Assessment, Diagnosis and Management of Common Neurological Problems

12. What are the different types of meningitis? Describe the pathophysiology and clinical manifestations for each type.

Many organisms can cause (group B streptococcus, Escherichia coli, Listeria monocytogenes, enterococci, S. pneumoniae, N. meningitidis, H. influenzae). The causative organism varies with age. N. meningitidis is a gram-negative diplococcus. common organism in the human nasopharynx. Groups A, B, C, W-135, and Y are largely the causes of invasive disease. Groups B, C, and Y account for 90% of invasive meningococcal disease in the U.S. and share equal incidence. Group B is a greater threat to younger children The organism is spread from person to person via respiratory tract secretions and in most cases causes asymptomatic colonization. This can persist for weeks to months. The incubation period is 1 to 14 days. Patients are contagious until 24 hours after initiation of treatment. Colonization can lead to invasive disease. Bacteremia and sepsis result and, depending on hematogenous spread, multiple patterns of illness can occur. These include bacteremia without sepsis, meningococcemic sepsis without meningitis, meningitis with or without meningococcemia, meningoencephalitis, and specific organ infection. Presenting symptoms of meningitis can include: • Occult bacteremia: a febrile child with URI or gastrointestinal-like symptoms. maculopapular rash. treated as having a viral illness. Some have recovered without antimicrobial intervention, whereas others have developed meningococcal meningitis (58% of cases) • Meningococcemia: fever (characteristic), chills, cold hands and feet, pharyngitis, headache, anorexia, purulent conjunctivitis, photophobia, myalgias/limb pain/refusal to walk (7%), myocarditis, malaise, stiff neck (less in infants), seizures, prostration, irritability, emesis, and a maculopapular or petechial rash (characteristic, follows other symptoms within 5 to 20 hours and occurs in about 7% of cases) that may quickly progress to purpura and septic shock manifested by hypertension, DIC, acidosis, adrenal hemorrhage, renal failure, myocardial failure, and coma. Fever and irritability may be the only initial symptoms in young children, whereas fever and headache are more typical in older children and adolescents • Aseptic meningitis: fever, stiff neck, and headache. Altered sensorium and seizures are common. Most cases appear in epidemics or as unique cases; most patients recover completely. Tuberculous Meningitis: the most serious complication of TB. generally follows primary pulmonary disease in 0.3% of untreated infants and young children 6 months to 4 years of age. Meningeal infection is also common in miliary TB. Bacilli migrate to the subarachnoid space. Caseous lesions can enlarge, encapsulate, and form a tuberculoma that can act just like any other CNS mass lesion. Symptoms can evolve slowly or rapidly; infants and children generally experience rapid onset. Tuberculin skin testing is negative in 50% of cases, with 20% to 50% of cases also having negative chest x-rays. Diagnosis is via CSF culture. Symptoms include fever, malaise, irritability, drowsiness, decreased developmental milestones, nuchal rigidity, positive Kernig or Brudzinski signs, hypertonia, vomiting, seizures, and other neurologic symptoms. The provider should consider TB in the differential diagnosis for any child who presents with basilar meningitis and hydrocephaly, cranial nerve palsy, or stroke without other apparent cause. Tuberculoma (brain tumor presenting as headache, fever, seizure) is also possible in children. Meningitis is usually caused by bacterial, viral, or fungal infection, but may also result from injury, cancer, or drugs. Classic symptoms of meningitis include headache, nausea or vomiting, neck stiffness, and fever. Each type has characteristic risk factors, signs, and symptoms. from review book: Etiology/Incidence of bacterial meningitis—related to age 1. Most frequently a result of hematogenous dissemination 2. Bacterial pathogens—related to age a. Newborn (1) Escherichia coli (2) Group B streptococci (3) Listeria monocytogenes (less frequent) (4) Enterobacteriaceae b. Etiologic agents responsible for 95% of cases that occur in children over 2 months of age have been: (1) Haemophilus influenzae type b (dramatic decline in incidence with H. influenzae immunization) (2) Streptococcus pneumoniae (3) Neisseria meningitidis 3. Other etiologic agents a. Mycobacteria b. Fungal infections c. Viral (aseptic)—most common cause of meningitis d. Protozoa 4. Highest risk—infants between 6 and 12 months of age; 90% of all cases occur in children between 1 month and 5 years • Signs and Symptoms 1. Newborn a. Often nonspecific and indistinguishable from those of septicemia b. Most frequent signs (1) Temperature instability (2) Respiratory distress (3) Irritability, lethargy (4) Poor feeding (5) Vomiting c. Seizures present in 40% 2. Older infants and children a. Nausea and vomiting b. Irritability, confusion c. Anorexia d. Headache, back pain, nuchal rigidity e. Hyperesthesia, cranial nerve palsy, ataxia f. Photophobia Physical Findings 1. Newborn a. Bulging fontanel (with or without suture diastasis) b. Increased ICP c. Cardinal signs of meningitis in older children are usually absent in infants (1) No stiff neck (2) No evidence of Kernig's and Brudzinski's signs 2. Older infants and children a. Common clinical signs associated with meningeal irritation are: (1) Kernig's sign (a) Flexion of the leg 90° at hip (b) Pain on extension of leg (2) Brudzinski's sign—involuntary flexion of legs when neck is flexed b. Headache is frequent sign of increased intracranial pressure from epocrates: Viral meningitis: the most common cause of aseptic meningitis. Causative agents include human enteroviruses (most commonly), HSV, mumps, arboviruses such as West Nile, HIV, and (rarely) influenza. However, it is typically self-limiting without serious sequelae. patho: Enteroviruses are spread by the fecal-oral route. The nonpolio enteroviruses and arboviruses initially replicate outside the CNS in tissues such as muscle, liver, and the respiratory or GI tracts, and then reach the CNS by hematogenous spread. Viral penetration of the blood-brain barrier occurs by either infection of endothelial cells or of migrating leukocytes. It is unclear whether HSV infects the CNS as a result of hematogenous spread or by retrograde spread along peripheral nerves. viruses spread through the subarachnoid space leading to meningitis, and may go on to infect neurons and glial cells leading to encephalitis or myelitis. The cellular immune response to viral infection of the CNS leads to the accumulation of lymphocytes within the CSF and the release of inflammatory cytokines such as interleukin (IL)-6 and TNF. The inflammatory response increases the permeability of the blood-brain barrier and this allows diffusion of circulating immunoglobulins into the CSF. The importance of the cell-mediated response in controlling infection is illustrated by the increased incidence and severity of infection with varicella zoster virus and CMV in patients with impaired T-cell responses. clinical presentation: headache, nausea and vomiting, photophobia, neck stiffness, fever, rash Bacterial meningitis: rare but serious. Streptococcus pneumoniae, Hemophilus influenzae type b (Hib), and Neisseria meningitidis are the predominant causative pathogens in both adults and children. Rapid assessment and prompt antimicrobial therapy are essential. patho: Bacteria reach the CNS either by hematogenous spread (the most common route) or by direct extension from a contiguous site. Neonates can acquire pathogens from nonsterile maternal genital secretions, through the placenta, or from their surroundings. The bacteria multiply quickly once they have entered the subarachnoidal space. Bacterial components in the CSF induce the production of various inflammatory mediators, which in turn enhance the influx of leukocytes into the CSF. The inflammatory cascade leads to cerebral edema and increased intracranial pressure, which contribute to neurologic damage and even death. clinical presentation: headache, neck stiffness, fever, altered mental status, confusion, photophobia, vomiting, seizures, hypothermia (infants), irritability (infants), lethargy (infants) poor feeding (infants), apnea (infants), high pitched cry (infants) Meningococcal disease: may progress rapidly to septic shock with hypotension, acidosis and disseminated intravascular coagulation. Prompt evaluation and treatment are essential, as the case fatality rate is high. patho: Sepsis caused by meningococci is multifactorial. Bacterial factors, chiefly lipooligosaccharide, stimulate a proinflammatory cytokine response. The signs and symptoms of meningitis are a result of local inflammatory responses leading to cerebral edema, elevated intracranial pressure, and vascular thrombosis. Hypotension results from increased vascular permeability and dysregulation of vascular tone. Both myocarditis and myocardial depression may contribute to poor tissue perfusion. Bacteria release endotoxin, which triggers the inflammatory response; this in turn leads to activation of the coagulation cascade and downregulation of anticoagulant and fibrinolytic pathways. DIC is caused by these acquired deficiencies of protein C, protein S, and antithrombin III, increases in plasminogen activator inhibitor and thrombin-activatable fibrinolysis inhibitor, and reduced activation of protein C on endothelial cells. Resulting small-vessel thrombosis and skin necrosis cause purpura fulminans. More rarely, thrombosis of larger blood vessels results in ischemia or infarction of digits or extremities. Waterhouse-Friderichsen syndrome is caused by bilateral adrenal hemorrhage and necrosis with acute adrenal insufficiency. clinical manifestations: rapid onset of illness, fever, irritability, leg pain, seizures, neck pain, headache, confusion, altered consciousness, hypotension, pallor or mottled skin, rash, cold hands and feet, neck stiffness, photophobia, hypotonia, lethargy poor appetite or feeding, nausea or vomiting, thirst coryza, sore throat, or cough, respiratory distress, tachycardia Fungal meningitis A progressive, life-threatening, chronic or subacute meningitis that is most commonly caused by Cryptococcus species. It is often accompanied by systemic involvement in immunosuppressed patients. Infants and neonates are also at increased risk. Other causative agents include Coccidioides species, Candida species, or Histoplasma capsulatum. patho: many fungal pathogens are thought to be acquired through inhalation. Meningeal involvement results from hematogenous dissemination from the lungs. This may occur either following primary infection, or after a period of controlled, latent infection, when host immunity is compromised. Protection from Cryptococcus neoformans and the endemic mycoses is associated with an active granulomatous inflammatory response, and depends on intact cell-mediated immunity involving both CD4 and CD8 T cells (Th1 pattern of cytokine release). Cryptococcal infection is probably acquired through the inhalation of small yeasts or basidiospores. The primary pulmonary infection is often asymptomatic. Reactivation of latent infection may be important in HIV-associated cryptococcal meningitis. The organism has a particular predilection for dissemination to the brain. This may relate to the production of melanin, which may interfere with oxidative killing by phagocytes, from L-dopa in the brain by a cryptococcal laccase enzyme. [48] In histoplasmosis, hematogenous dissemination may occur throughout the reticuloendothelial system via parasitized macrophages. There are numerous reports of histoplasmosis in patients who have not returned to endemic areas for many years. This suggests the importance of reactivation of latent infection. In endemic areas, the relative importance of reactivation versus progressive primary infection is less clear. Coccidioidal meningitis occurs in approximately half of individuals with disseminated disease. Following the initial primary infection, dissemination to the brain, when it occurs, typically takes weeks to months. In high-risk, symptomatic populations, the dissemination rate may be >15%. Candidal meningitis may occur as a complication of candidemia, especially in neonates and premature infants. It is also associated with head trauma and neurosurgical procedures. In contrast to cryptococcosis and the endemic mycoses, the major risk factor for invasive candidiasis in adults is neutropenia clinical presentation: progressive headache, severe headache, meningismus, symptoms of hydrocephalus (impaired cognitive function, confusion, coordination and gait disturbances, and urinary incontinence), behavioral or personality change, reduced visual acuity and papilledema, nausea or vomiting, fever, reduced conscious level cranial nerve palsies Extrapulmonary tuberculosis Tuberculous meningitis results from hematogenous spread of Mycobacterium tuberculosis with the development of submeningeal or intrameningeal foci called Rich foci. With rupture of a Rich focus into the subarachnoid space, meningitis develops. It may result from reactivation (more common in adults) or primary infection (more common in children). Diagnosis is dependent upon cerebrospinal fluid examination, and its rapid diagnosis is essential for improved outcomes. clinical presentation: enlarged lymph node pleuritic chest pain, skeletal pain, urinary symptoms, abdominal swelling, abdominal pain

Cerebral Palsy a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. a mixed group of motor disorders that affect motor function and are caused by static injury to developing brains b. a chronic, nonprogressive disorder that impairs control of movement by damaging motor areas in the brain. Symptoms appear within the first few years of life. mental impairment (up to 66%); seizures (up to 50%); a lag in growth and development; neurosensory disorders affecting touch, pain, and continence; perceptual disorders; impaired vision and hearing; speech, swallowing, or chewing difficulties; and other learning or emotional difficulties. The etiology is unknown in a large percentage of cases. Risk factors: Small-for-gestational-age, low birth-weight (less than 1000 g), preterm babies (less than 37 weeks of gestation), multiple births Complicated labor and delivery, breech presentation, Apgar score of less than 3 at 10 minutes or more; traumatic delivery; microcephaly; exposure to maternal infection (evidenced by chorioamnionitis, inflamed placental membranes, umbilical cord inflammation, foul-smelling amniotic fluid, maternal temperature greater than 100.4° F [38° C] during labor, or UTI; maternal vaginal bleeding (between the sixth and ninth month of pregnancy); severe proteinuria late in pregnancy; maternal hyperthyroidism, mental retardation, and seizures; intracranial hemorrhage; toxemia; preeclampsia; antepartal hemorrhage; postmaturity; fetal distress; maternal stroke; coagulation in the fetus or newborn; and neonatal seizures intrauterine drug exposure (e.g., alcohol, cocaine, tobacco, crack cocaine), intrauterine infections (e.g., cytomegalovirus, toxoplasmosis, rubella), congenital brain malformations. meningitis, encephalitis, head trauma (e.g., secondary to shaken baby syndrome or other abuse, car accidents, falls), and kernicterus c. 3 major types of CP: spastic, athetoid (or dyskinetic), and ataxic. Spastic: muscles stiffen, causing muscle tightness. Inability of a muscle to relax Often evident after 4-6 months; retarded speech; convergent strabismus; *toe-walking*; flexed elbows; delayed walking until 18-24 months; one third have seizures Athetoid: muscles that enable smooth, coordinated movement and maintain body posture; without this control movement becomes involuntary and purposeless. Inability to control muscle movement (continuous, writhing movements) Infant has difficult feeding as a result of tongue thrust, is initially hypotonic with head lag; increasing tone with rigidity over time; speech delay ataxic: affects balance and coordination. tremors Children may exhibit varying degrees of involvement and severity; capabilities may improve over time depending on the degree of involvement and treatment. Diplegic: Affects both legs more than both arms Most have limited use of legs; can walk often with aids; walk typically "scissor- like" with knees bent in and crisscross over each other Hemiplegic: Affects one side of the body (upper extremity is usually affected more than the lower extremity) Often not detected at birth; right side often more affected than left; 50% develop seizures; growth arrest of affected limb(s); individuals usually able to walk Tetraplegic/Quadriplegic: Affects all four extremities, trunk and head Affects upper extremities more than lower; 50% with grand mal seizures; IQ impairment can be severe; most unable to walk or stand Specific Problems With Movement or Function Dystonia: Involuntary, slow, sustained muscle contraction, Abnormal posture, writhing motion of arms, legs, trunk Choreic: Disorganized tone, Uncontrollable jerky movements of toes and fingers Tremor: Involuntary, rhythmic movements of opposing muscles; can affect extremities, head, face, vocal cords, trunk Ballismus: Violent, jerky movements; may affect only one side of body Rigidity: Stiffness Pathology • Prenatal/natal history of risk factors • Seizures • Hearing and vision or ocular problems, such as strabismus, nystagmus, optic atrophy • Change in growth parameters, especially decreased head circumference • Early head injury or meningitis • Muscle tone (can be hypotonic before 6 months old then become hypertonic, as evidenced by unusual posture or favoring one side). Preterm infants with generalized, prolonged, and cramped synchronized movements are more often diagnosed with CP at a later time Development Milestones delayed but should still be attained; persistent primitive reflexes are common (e.g., Moro and tonic neck). *Hand preference before 1 year old is highly suspect.* Functional Health Patterns • Feeding history of regurgitating through the nose, inability to coordinate suck and swallow, inability to advance the diet to textured foods—oral-motor coordination problems • Irritability or depressed affect (including unusual sleepiness) • Difficulty with movement, cuddliness, grasp and release, self-feeding, and head control to look around; inability to change position per developmental level • Persistent primitive reflexes • Communication problems, either in language or speech proficiency Physical Examination • Skin: Dermatologic signs of syndromes, such as neurofibromatosis • Orthopedic examination: Scoliosis, contractures, and dislocated hip • Neurologic examination: Deep tendon reflexes increased Tone increased, although occasionally decreased; hypotonia before 6 months old is common. Tone may also be mixed. Minimal muscle atrophy No fasciculations Persistent primitive reflexes (e.g., tonic neck and Moro after 6 months old) Delayed reflexes (e.g., parachute reflex remains absent after 9 to 10 months old; side-protective reflexes remain absent after 5 months old) Asymmetric movements Preferred handedness before 1 to 2 years old Structural defects, such as hydrocephaly or microcephaly • Vision and hearing: Visual refractive errors occur in 50% of children; strabismus is found in 33%. Hearing problems may have resulted from the initial brain insult. • Development: Assess gross motor, fine motor, language, and personal social skills. The Denver Developmental Screening Test II (Denver II) can be used for initial screening. Motor milestones are commonly delayed. Note quality of movements (e.g., smoothness of gait, grasping, clarity of speech). • Feeding: Note a reversed swallow wave; uncoordinated suck and swallow; decreased tone of the lips, tongue, and cheeks; increased gag reflex; involuntary tongue and lip movements; increased sensitivity to food stimuli; poor occlusion; and delayed inhibition of the suck reflex. • Evaluate the diet, height, weight, and BMI for adequate nutrition. d. Diagnostic Studies: •CT scan - identify brain malformations. MRI - aid the visualization of structures and abnormalities that are nearer to bony structures. • Chromosomal and metabolic studies - identify genetic disorders, especially single-gene defects. • Lumbar puncture if sepsis is suspected. management : • *Referral* of suspected cases. evaluated and cared for at centers that provide interdisciplinary caregivers, including a developmental pediatrician, gastroenterologist, orthopedist, neurologist, nurse, speech pathologist, physical and occupational therapists, education consultant and psychologist, and social worker. Care may also involve an ophthalmologist, feeding clinic and nutritionist services, and genetics counseling. • *Family education*: nonprogressive but incurable characteristics. extent of brain damage is not always related to the extent of disability; no one can predict what the future for a given child will be. special services —physical therapy, speech therapy—have better outcomes than children who are left to develop on their own. United Cerebral Palsy has educational materials, services available. • *Family support*. Support groups or opportunities to meet other families with affected children. The emotional needs of siblings. The social worker helpful to families • *Financial resources.* long term and expensive. Supplemental Security Income or state program benefits for the severely handicapped. Respite care may be available. The Individuals with Disabilities Education Act of 1997 (IDEA) requires children with disabilities to be assessed for and instructed in the use of assistive devices along with appropriate referrals to regional centers. Some insurance companies try to avoid the costs of long-term care and therapy. Medical social workers and public health nurses can be very helpful in connecting families to appropriate services. • *Nutrition*. inadequate nutrition because of their problems with biting, sucking, chewing, swallowing, and self- feeding. children with athetosis may need as much as 50% to 100% more calories to support their increased caloric needs because of their constant writhing movements. Children with spasticity may need fewer calories because of their decreased movements. Occasionally gastrostomy is needed, sometimes with fundoplication to prevent reflux and aspiration. Special positioning, feeding therapy, and special feeding devices can help. High nutrient density is a key to providing a nutritious diet (i.e., getting more nutrients into the same volume of food) Feeding clinics are often helpful. • *Elimination*. Constipation is common because of lack of exercise, inadequate fluid and fiber intake, medications, poor positioning, low abdominal muscle tone. *Stool softeners, such as docusate sodium. Laxatives, such as senna concentrate or Milk of Magnesia*, not be used long term. Osmotic agents may also be used (e.g., *polyethylene glycol*). Bladder control and urinary retention are also problems; these children are 3x more likely to suffer UTIs • Most children achieve bladder control between 3 and 10 years old. For some, toilet training may be difficult, especially for those with mental retardation. • *Dentistry*. Orofacial muscle tone can contribute to malocclusion. Problems with oral mobility make daily dental hygiene difficult, leading to more gum disease. The side effects of some seizure medications can include swollen gums and tooth decay. A careful dental care program is necessary *Drooling*. Inability to manage oral secretions results in drooling. Social isolation, wet clothing, skin excoriation, malodorous breath, discomfort, choking, gagging, and aspiration can make these oral secretions a serious problem. The *anticholinergic, glycopyrrolate, is approved for use in those 3 to 16 years old* with chronic excessive drooling from neurologic conditions. This cherry-flavored oral solution (1 mg/5 mL) is dosed at 0.05 to 1 mg by mouth two or three times daily. Side effects: dry mouth, vomiting, constipation, flushing, urinary retention, and nasal congestion. Clinical improvement in drooling has been demonstrated in up to 78% of children and adolescents who used this drug. *Surgical intervention is a last resort* and commonly involves removing the submandibular gland or nerves, or cutting or rerouting the salivary duct. • *Respiratory*. Positioning problems, an increase in GERD and difficulty in clearing secretions -higher risk for respiratory problems, pneumonias (especially from aspiration). The duration of respiratory symptoms with URIs is increased in these children because they may have congenital paralysis or sleep-related obstruction. A tracheotomy may be necessary in severe cases of upper airway obstruction or difficulty. • *Skin*. The skin in sedentary children is more likely to break down and cause decubitus. There is an increased incidence of skin latex allergies • *Movement and mobility*. Positioning and seating, standing, transportation, bathing, dressing, mobility for play and getting to school are important to assess and manage. *Occupational and physical therapists* are essential, families need their help incorporating various strategies into their homes and lifestyles. The goals of therapy are to improve physical conditioning and gain maximal independence in mobility, fine motor activities, self-care, and communication by promoting efficient movement patterns, inhibiting primitive reflexes, and achieving isolated extremity movements. Bracing, postural support and seating systems, adaptive devices, and early intervention programs beginning in infancy are important. Open-front walkers, quadrupedal canes, gait poles, wheelchairs, and motorized wheelchairs are beneficial in helping children explore their environment more efficiently. contractures, scoliosis, dislocated hips, and other deformities can develop if the child is allowed to maintain abnormal positions for long periods; range-of- motion exercises are a long-term need. *Orthopedic care* may be necessary. Constraint-induced therapy (the use of the more functional side is restricted) can both improve mobility function and sustain function longer than conventional physical therapy • *Medications*. *Antispasmodic medications (baclofen, tizanidine, diazepam, and dantrolene)* may be used to minimize contractures and spasticity. They are appropriate for children needing only a mild decrease in their muscle tone or in those with widespread spasticity. dosages often need to be high side effects: drowsiness, upset stomach, high blood pressure, and possible liver damage with chronic use *Botulinum toxin A injections*: eliminate pain, minimize contractures, delay or prevent surgery, and maximize function used off-label use is dependent on the recommendation of a pediatric physiatrist, pediatric neurologist, or pediatric orthopedic surgeon, and after input of therapists and family. injected directly into muscles (sometimes guided by an electromyogram or electrical stimulation). The child may experience mild flulike symptoms and transient worsening of spasticity; injections are best followed by physical and occupational therapies that help strengthen the antagonist and agonist muscles The dosage administered depends on which muscles are being selected and muscle size. Results are generally seen within 5 to 7 days and last 3 to 4 months. The toxin has been safely used in infants older than 1 month. Resistance can occur because neutralizing antibodies can develop. only the smallest possible effective dose must be used, and at least 3 months must lapse between injections. Contraindications: diffuse hypertonia, myasthenia gravis (MG), motor neuron disease, injection into an infected muscle, caution in pregnancy (fetal complications have been seen in animal studies), and caution when it is coadministered with an aminoglycoside or another agent that interferes with neuromuscular transmission (toxin effect can be increased) numerous side effects • *Communication*. combined problems of lack of oral-motor control and the high incidence of mental retardation, communication can be a problem. *Speech therapy* may be of assistance; augmentative devices, such as computers with voices, can allow for language development and communication of needs even without oral speech. *Hearing deficits need to be identified and managed by an audiologist.* • *Vision*. Visual acuity, eye tracking, and binocularity are key factors to be assessed by a pediatric ophthalmologist • *Osteopenia.* at risk of bone density loss secondary to their inability to ambulate and place weight on their bones. *bisphosphonates off-label* • *Pain*. Spastic muscles, strain on compensatory muscles, and frequent or irregularly occurring muscle spasms can cause chronic and acute pain. *Diazepam, gabapentin, and complementary therapies (distraction, biofeedback, relaxation, therapeutic massage)* can help • *Special Education*. Early intervention programs and specialized educational programs through school systems • Other Treatments. *Surgery is used to release contractures or to sever overactivated nerves* (called a selective dorsal root rhizotomy). *Selective dorsal root rhizotomy (of spinal nerves) plus intrathecal baclofen decrease spasticity and increase range of motion of affected limbs.* Intrathecal baclofen uses an implantable pump to deliver the drug, a muscle relaxant. The pump is programmable with an electronic telemetry wand. Pumps have been successfully implanted in children as young as 3 years of age; this treatment has small but significant risks of complications. It is most efficacious in children who have some motor movement control and who have few muscles to treat that are not fixed or rigid Intense physical therapy is an instrumental adjunct treatment. • Strength training can help with balance and weakness. Functional electrical stimulation (involves insertion of a microscopic wireless device into specific muscles or nerves) has been used to activate and strengthen muscles in the hand, shoulder, and ankle. experimental, used only as an alternative treatment if other treatments fail to relax muscles or relieve pain • Alcohol "washes" (injections of alcohol into targeted nerves) are sometimes used. Benefits can last from months to 2 years or more; side effects : significant risk of pain or numbness. Research - chemodenervation techniques that deliver injected antispasmodic medications more precisely to target and relax muscles • "Patterning" is a controversial physical therapy (child is taught elementary movements, such as crawling, before advancing to walking skills). AAP do not endorse this technique because of the lack of evidence-based studies. the Bobath technique (involves inhibiting abnormal movement patterns in favor of more normal ones) has provoked strong reservations about efficacy. Conduction education (use of rhythmic activities combined with physical maneuvers on special equipment) has failed to consistently produce improvement

5. List possible causes for each of the following injuries: a. extradural hematomas b. intracerebral hemorrhage c. compound fractures d. basilar skull fracture

a. post-traumatic injuries, delivery by forceps or vacuum extraction, falls, child abuse, motor vehicle collisions b. hypertension, High alcohol intake, Black ethnicity, Lower cholesterol and lower LDL cholesterol, Lower triglycerides, chronic kidney disease, SSRIs c. CNS trauma, head molding, cephalhematoma, TBI, falls, recreational activities, MVA, assault, child abuse d. TBI, eyelid contusion

Concussions (Book)

occur in 1.6 to 3.6 million young athletes annually Head and neck injuries cause 70% of sports-related traumatic deaths and 20% of permanent disabilities sports w the highest risk: football, bicycling, basketball, soccer, and trauma on playground equipment. a child was six times more likely to acquire a severe concussion during an organized sport than from other recreational activities repeated concussions are often progressively more serious or can result in second impact syndrome. The symptoms from a concussion may affect four domains (physical, cognitive, emotional, and sleep). symptoms may appear even several hours after the injury. children and adolescents take longer to recover from concussions If severe enough, the injury may precipitate a seizure lasting 1 to 2 minutes. The incidence of posttraumatic epilepsy is less than 10% after a concussion or contusion factors have been identified that may modify or intensify concussion severity and lengthen recovery: • Increased number, severity and duration of symptoms (especially if lasting >10 days) • Prolonged loss of consciousness (LOC) >1 minute, amnesia • Concussive convulsions • Repeated concussions; recent past concussion; second-impact syndrome (this second blow may have seemed minor but can cause massive brain swelling and herniation with risk of mortality or morbidity within minutes; occurs in an athlete who has not fully recovered from a previous concussion) • Age less than 18 years • Comorbidities: migraines, depression, ADHD, learning disabilities, sleep disorders • Use of some psychoactive medications or anticoagulants An evolving body of research indicates that young children are particularly vulnerable to mild traumatic brain injury (TBI), also known as concussion. Most TBIs occur secondary to acceleration-deceleration or rotational forces, and long-term sequelae are much more likely in children with developing brains The CDC defines mild traumatic brain injury (MTBI) as a complex pathologic brain process that results from primary or secondary forces on the head that disrupt brain processes and functioning MTBI results in physical, cognitive, emotional and sleep symptoms Epidemiology TBI is a common cause of trauma in pediatrics, resulting in almost 2200 deaths, 35,000 hospitalizations, and 474,000 ED visits annually in the U.S. for children 0 to 14 years old Approximately 2 to 5 million children sustain head traumas of varying intensities each year in the U.S. when all ages during childhood and adolescence are considered. Common causes of ED-treated TBI include falls, sports-related injuries, motor vehicle accidents, violence and assaults, and being struck by or against objects Boys experience head injury twice as frequently as girls. Many children die each year from head trauma, and children who survive their injuries can have significant long-term disability. Children can also experience subtle symptoms of TBI that may not appear until days or weeks after the injury. Various types of head injuries can result in pathologic conditions: skull fracture, concussion, posttraumatic seizure, cerebral contusion, epidural hematoma, subdural hematoma, cerebral edema, and penetrating injury. Clinical Presentation Glasgow Coma Scale (GCS), which has been traditionally used to measure the severity of head injury. Modification to the GCS for pediatrics has resulted in the implementation of the Pediatric GCS scoring system Symptoms of TBI can mimic those of other medical conditions, thus making the diagnosis challenging. It is recommended that providers use an evidence-based assessment tool like the CDC's Acute Concussion Evaluation (ACE) tool • History of how injury occurred; if injury involved a fall, the height from which the child fell needs to be determined. Specifically, providers should ascertain injury cause, body part affected, forces, and circumstances. • Loss of or alteration in consciousness or memory, confusion, irritability, inappropriate behavior, repetitive questioning • Presence of vomiting and frequency • Presence of headache, description of the headache pain • Presence of blurred vision, diplopia, or other vision problem • Numbness or loss of sensation, loss of balance, or difficulty walking • Specify symptoms occurring at the time of injury and interval changes Child abuse should be strongly suspected when a head injury is present in a child without a history of a fall or with a history of a fall from a relatively low height of less than 4 feet. It is also recommended that a skeletal survey be obtained in children younger than 3 years when inflicted head injuries are suspected because younger children are at higher risk for skeletal trauma as well Physical Examination A sideline evaluation of any player with a head injury should be performed, using a standardized tool such as the Standardized Concussion Assessment Tool 2 (SCAT2) • Pupils: Symmetry and reaction • Coordination: Finger-nose-finger; tandem walk • Sensation: Finger-nose with eyes closed; Romberg test • Physical: having the youth do a 40-yard sprint, five push-ups, five sit-ups, and five knee bends Check vital signs (temperature, blood pressure, pulse, and respiration) and compare findings with normal parameters expected for children of varying ages. Changes in vital signs can indicate shock or intracranial hypertension. careful oral examination and a careful neurologic examination including level of consciousness, mental status, motor function (both gross and fine motor), sensory function, cranial nerve functioning, and reflexes. neck injury, internal abdominal injuries, or bone fractures. Periorbital hemorrhage ("raccoon-eyes"), ecchymosis behind the ear (Battle sign), blood behind the eardrum, and bleeding from the ears or nose indicate a basilar skull fracture. TABLE 399 Glasgow Coma Scale Eye opening (E) Spontaneous 4 To speech (command) 3 To pain 2 None 1 Motor (M) Obeys (command) 6 Localizes 5 Withdraws 4 Abnormal flexion 3 Extensor response 2 None 1 Verbal (V) Oriented 5 Confused conversation 4 Inappropriate words 3 Incomprehensible sounds 2 None 1 Recognizing Signs and Symptoms of a Concussion Physical: headache (persistant, mild), nausea, vomiting, balance problems, dizziness, visual problems, fatigue, sensitivity to light and/or noise, numbness/tingling, dazed or stunned (befuddled facial expression), seizure (transport to hospital in order to differentiate between seizures due to concussion, post traumatic brain injury, convulsive syncope, drug related epilepsy (cocaine), or idiopathic primary epilepsy) Cognitive: feeling mentally foggy, feeling slowed down (slurred, incoherent speech; disjointed or incomprehensible statements), difficulty concentrating (cannot recall words, numbers; unable to follow through with normal activities), difficulty remembering time, place, date, forgetful of recent information or conversations, confused about recent events, delayed verbal and motor responses (slower to answer questions or follow instructions; walking in wrong direction), repeats questions, gross incoordination (stumbling, unable to walk tandem or in a straight line.), confusion, altered concentration, mental torpor, altered memory, forgetfulness (esp conversations or recent events), needs to repeat or slowly answer questions emotional: irritability (low frustration tolerance), sadness, more emotional (appears distraught, crying for no reason) nervousness sleep: drowsiness, sleep disturbance (sleeping less or more than usual) trouble falling asleep mild/low risk: length of time patient was unconscious or had PTA <1 hr, no or brief LOC (<30 min), GCS 13-15, symptom: usually alert in the ED with headache, dizziness, lethargy, irritability; withdrawn, may or may not be labile no neuro focal deficity sequalae: repeated minor damage (head trauma with sports) can result in change in neuropsychology (attention, arousal, and information processing). ADHD, decreased attention span, emotional changes, sleep disturbances, memory problems, headache, language deficits can result may have linear skull fractures moderate risk: unconscious or PTA: 1-24hr, variable LOC, GCS 9-12, sx: focal signs, occasional brain swelling and hematoma, brief LOC, seizure, vomiting, headache, concentration, problem-solving and memory problems; symptoms can last for several months. sequelae: same as for mild with concentration, problem solving and memory problems, symptoms can last for several months may have depressed skull fracture or intracranial hematoma major/high risk: unconscious or PTA: >24 hours, prolonged LOC, GCS 3-8, sx: focal signs, impaired LOC, focal neurologic findings, skull injuries, approximately 50% mortality rate. sequel: seizures, hemiparesis, aphasia, cognitive problems, behavior changes, anxiety, attention problems (concentration, problem solving and memory problems) symptom can last for several months (ADHD) often have depressed skull fractures and intracranial hematoma Diagnostic Studies CT imaging is negative in a concussion; imaging is needed if there is suspicion of an intracranial hemorrhage. MRI with magnetic resonance angiogram is indicated if carotid dissection or stroke is suspected; PET is used in cases in which the individual has persistent symptoms All children with moderate (GCS 9 to 12) and severe (GCS 3 to 8) acute trauma should have a cranial CT scan. recommends including plain radiographs (cervical spine and a series of skull views) with significant head injury, loss of consciousness, focal neurologic signs (GCS 3 to 8), and further neurologic study. Indications for obtaining a CT scan include any of the following: • Penetrating trauma • Altered level of consciousness (excessive irritability or lethargy) • History of loss of consciousness (exceeding 1 minute) • Amnesia about the injury • Focal neurologic signs or deficit • Depressed skull fracture or signs of basilar injury • Seizures • Persistent vomiting • History of coagulopathy CT is the preferred imaging technique because it can be obtained rapidly, and the child can be monitored easily during the study. Skull fractures are better visualized on skull radiographs. Acute hemorrhage is detected more easily by CT (order without contrast) than by MRI. If CT is ordered after several days (3 or more days past injury), it should be done both with contrast (to pick up extravasated blood) and without. CT can demonstrate brain edema, midline displacements, hydrocephalus, loss of brain tissue, and most skull fractures. Although CT itself is a safe procedure, some healthy children require sedation or anesthesia (with some risk), so the benefits gained from CT should be carefully weighed against the possible harm of sedating or anesthetizing a child. In addition, CT scans obtained for asymptomatic children may show incidental findings that lead to subsequent unnecessary medical or surgical interventions. CT scans, MRI, or skull radiographs are generally not indicated for mild or minor closed head trauma without focal neurologic signs or loss of consciousness Differential Diagnosis Head trauma may cause injuries of the scalp, skull, dentition, and intracranial contents. Children with intracranial lesions after minor closed head injury are not easily distinguishable clinically from the large majority with no intracranial injury. Children with mild nonspecific signs such as headache, vomiting, or lethargy after minor closed head injury may be more likely to have intracranial lesions than children without such signs. most children with headache, lethargy, or vomiting after minor closed head injury do not have demonstrable intracranial injury. In addition, some children with intracranial injury do not have any such signs or symptoms, showing a normal neurologic assessment. some experts recommend a liberal policy on the ordering of cranial CT scans following any head trauma Management requires physical and cognitive rest until symptoms resolve. Cognitive recovery may lag behind physical symptom resolution, and this domain plays an important part in return-to-play decisions. Once symptoms resolve, the athlete can progress through steps to gradually return to play. first concussion: grade 1 (no LOC; transient confusion; post traumatic amnesia (PTA), postconcussion signs/symptoms other than amnesia (PCCS) <30 minutes): athlete may return to play that day in select situations if clinical exam results are normal at least and with exertion; otherwise return to play in 1 week. Athlete should be taken to the ED if mental status abnormalities last more than 1 hour. Grade 2 (LOC <1 minute; duration of PTA >= 30 minutes but <24 hours; PCCS >30 min but <7 days): athlete may return to play in 2 weeks if asymptomatic at rest and with exertion for 7 days Grade 3 (LOC >1 minute; PTA >24 hours; PCCS > 7 days): athlete may return to play in 1 month if asymptomatic at rest and with exertion for 7 days. second concussion: grade 1: return to play in 2 weeks if asymptomatic for 1 week. grade 2: minimum of 1 month without playing; after that may return to play if asymptomatic at rest or with exertion for 1 week; consider terminating season. grade 3: terminate season. may return to play next season if asymptomatic third concussion: grade 1: terminate season; may return to play next season if asymptomatic grade 2: terminate season; may return to play next season if asymptomatic grade 3: terminate contact sports for 1 year; RTP in non contact sports after that Graduated Return To Play Protocol Rehabilitation Stage∗ Functional Exercise at Each Stage of Rehabilitation Complete physical and cognitive rest Objective of Each Stage 1. No activity - Recovery 2. Light aerobic exercise - Walking, swimming, or stationary cycling keeping intensity <70% MPHR, No resistance training - Increase HR 3. Sport- specific exercise - Skating drills in ice hockey, running drills in soccer. No head impact activities. - Add movement 4. Noncontact training drills - Progression to more complex training drills (e,g., passing drills in football and ice hockey), May start progressive resistance training - Exercise, coordination, and cognitive load 5. Full-contact practice - Following medical clearance, participate in normal training activities Restore confidence and assess functional skills by coaching staff 6. Return to play - Normal game play LOC is key determinant of the child's prognosis. Prompt identification of a deteriorating LOC and quick medical and/or surgical intervention are essential components of the management plan. Management of the Child With Minor Closed Head Injury and No Loss of Consciousness Observation in the clinic, office, ED, or home, under the care of a competent caregiver, who understands what signs and symptoms to watch for, is able to closely and reliably monitor, and can quickly bring the child back for treatment or access emergency medical services if necessary, Observation implies regular monitoring by a competent adult who would be able to recognize abnormalities and seek appropriate assistance. Management of the Child With Minor Closed Head Injury and Brief Loss of Consciousness no other neurologic or physical deficits reported or detected on examination, observation in the office, clinic, ED, hospital, or home, when under the care of a competent caregiver, may be used to evaluate such a child. The use of CT scan, skull radiographs, or MRI in the initial management of children with minor closed head injury and loss of consciousness is not routinely recommended. However, CT scanning along with observation is also accepted. If the provider is not assured that the child will be closely and reliably monitored at home, hospitalization is indicated. Management of the Child With Moderate Head Injury or Worrisome Symptoms Children with moderate head injuries (GCS 9 to 12) may require admission or prolonged observation in the ED until their mental status stabilizes; children with severe head injuries (GCS less than 8 or coma and physical findings) need immediate hospital admission and consultation with a neurologist. Children with any of the following should be hospitalized: • Changing vital signs • Seizures • Altered mental status • Slurred speech • Prolonged unconsciousness (greater than 30 seconds) • Persisting memory deficit or focal neurologic signs • Depressed or basilar skull fractures • Persistent headache (particularly with stiff neck) • Recurrent vomiting or unexplained fever • Unexplained injury (suspected child abuse) • CT scan or MRI findings that are worrisome A child with a skull fracture or transient neurologic findings whose level of consciousness is normal may be admitted for overnight observation. Posttrauma Sequelae The duration of coma is predictive of subsequent neurologic function, and prognosis is good if mortality does not occur in the first 48 hours Minor head injury without neurologic changes generally has no resulting deficit. In major head injuries, correlation between the extent of the injury to the extent of the dysfunction is predictive of outcome in 70% of cases. After severe injury, cognitive function changes generally will not improve after 12 months, but speech and motor difficulties may continue to see improvement for up to several years. A neuropsychological evaluation may be helpful to plan appropriate educational and behavioral management. Children (2 to 6 years old) are usually more impaired than adolescents, secondary to immature brain development and general vulnerability. children and adolescents are more likely to show improvement in cognitive and social skills Approximately 5% of hospitalized children with a head injury suffer a seizure within the first week; another 5% experience a seizure after this time Prevention • Wear helmets when using bicycles, skateboards, scooters, motorcycles, inline skates, snowboarding, ski racing, and when appropriate for sports participation. • Protect children from falls in the home or from playground equipment. Discourage the purchase of residential trampolines • Use appropriate seat restraints when riding in motor vehicles. Complications concussions, posttraumtic seizures, cerebral contusion, epidural hematoma, subdural hematoma, intracerebral hematoma, subarachnoid hemorrhage, acute brain swelling, and penetrating injuries. Intracranial lesions, particularly epidural hematomas, are life-threatening and have significant complications. Features indicative of serious injury include loss of consciousness (longer than 1 minute), persistent vomiting, depressed level of consciousness, seizures, unequal pupil size, severe headache, and GCS less than 15. Patient and Parent Education Give caregivers a "head injury sheet," and make every effort to ensure that they understand the instructions and will comply with them. instructions about when to contact the health care provider or take the child to an ED: • Increased drowsiness, sleepiness, inability to wake up, unconsciousness • Vomiting more than twice • Neck pain • Watery or bloody drainage from ear or nose • Seizures, "fit," or fainting • Unusual irritability, personality change, confusion, or any unusual behavior • Headache that gets worse or lasts more than a day • Unequal pupils • Trouble with vision (blurred), hearing, or speech • Trouble with walking (e.g., clumsiness or stumbling) or weakness of any muscle of arms, legs, or face In addition, parents or caregivers should be given the following specific instructions: • Wake child every 2 to 4 hours for the first 24 hours after injury; child should wake easily and be able to stay awake for a few minutes. • Make sure child is moving his or her arms and legs normally. • Give only acetaminophen, if needed for headache or relief of soft tissue pain. Parents should also be informed that sometimes symptoms from head trauma occur days, weeks, or months after the initial trauma. Neurologic sequelae following mild head injury in children often improve or resolve within 9 to 12 months. These sequelae include the following: • Headache • Vertigo or dizziness • Difficulty concentrating or loss of memory • Depression, fatigue • Poor school performance and neurobehavioral problems Postconcussive Syndrome Typical postconcussive syndrome in adolescents is manifested by headache, dizziness, irritability, and impaired ability to concentrate. In younger children it is manifested as aggression, disobedience, behavioral regression, inattention and anxiety.

Describe each of the following: a. focal brain injury b. epidural hematoma c. subdural hematoma d. intracerebral hematoma e. diffuse brain injury f. concussion

a. Brain contusion - arise from blunt trauma between brain and skull after acceleration and deceleration of the head. •Intraparenchymal brain hemorrhage - tears in the brain tissue and/or vasculature, complicate blunt or penetrating trauma. •Subdural hematoma - hemorrhage into the space between the dura and the arachnoid membranes. two major sources of bleeding: bridging blood vessels that cross the subdural space and cerebral cortical hemorrhage caused by direct brain trauma. commonly complicates severe brain injury, including abusive head trauma in children. •Epidural hematoma - extradural hematoma, hemorrhage into the space between the dura and the overlying calvarium. After blunt trauma, may result from disruption of the middle meningeal artery, the middle meningeal vein, diploic veins, or venous sinuses. •Subarachnoid hemorrhage - develops from tearing of small vessels in the pia mater. may complicate other brain injuries or be found in isolation b. blood clot formation between skull and dura of the brain, result of trauma. morbidity and mortality result from mass effect on the brain as the hematoma grows and strips the dura away from the skull. managed similarly to adults, but it requires special knowledge of location and clinical presentation. Prompt diagnosis is critical to the successful management. c. blood clot formation on brain surface beneath the dura mater differs significantly from SDH in adults because inflicted head injury is a common etiology, especially under two years of age. operative management of SDH are less clear, and surgery is less likely to prevent morbidity and mortality d. less common than in adults, hemorrhagic stroke can affect children, resulting in significant morbidity and mortality. encompasses spontaneous intracerebral hemorrhage, isolated intraventricular hemorrhage, and nontraumatic subarachnoid hemorrhage. defined by intraparenchymal hemorrhage or a combination of intraparenchymal and intraventricular hemorrhage. e. the most common type of severe TBI resulting in death in children and is usually produced by impact, acceleration and deceleration forces. Diffuse traumatic axonal injury (DAI) is a severe form of DBI. DAI develops as the result of tissue shearing at the interface of grey and white matter; focal injuries may also be present in patients with DAI. Although not associated with structural changes on imaging, concussion is a milder form of DBI. f. mild TBI following a biomechanical force. may be caused by a direct blow to the head, face, neck, or elsewhere on the body with an 'impulsive' force transmitted to the head. typically results in the rapid onset of short-lived impairment of neurologic function that resolves spontaneously. signs and symptoms may evolve over a number of minutes to hours. may result in neuropathological changes, but the acute clinical signs and symptoms largely reflect a functional disturbance rather than a structural injury and no abnormality is seen on standard structural neuroimaging studies. results in a range of clinical signs and symptoms that may or may not involve LOC. Resolution of the clinical and cognitive features typically follows a sequential course. symptoms may be prolonged.

Dysautonomia (notes from class)

Dysautonomia - constellation of symptoms, most people don't know about it Treat the symptoms Effects autonomic nervous system - weakness, so tired, can't get out of bed, when get up so weak have to go back to bed, can't make it school Dizziness, constipation, anxiety, diarrhea, chronic fatigue, fibromyalgia Not getting better - start connecting the dots Diff dx: Autoimmune disease, lyme disease, alcoholism, lead, cancers, chemotherapy Dysregulation of parasympathetic and sympatheic nervous system: impotence in men, orthostatic hypotension, gi motility disorders - instead of stomach starting to digest, it doesn't do anything, feels bloated all the time, constipation Hypertension, autoimmune, autonomic failure, reflex failure Sympathetic - if doing opposite that what is should be doing Taking hot shower - get tunnel vision and feel like pass out, but don't pass out Short or long term problem - chronic Typically see girls 12 -13 year olds, some males too, more common in girls Always keep it on the chart - can happen in 40-50s Destruction in neurotransmitters which cause symptoms Risk factors: females > males, affects 70 million people S&S: drop in blood pressure, dizziness, lightheadness, bradycardia, orthostatic hypotension, orthostatic intolerance, syncope, near syncope, heat intolerance, sweating, super sweaty, anhidrosis, always cold, constipation, gastroparesis, urinary retention, frequent UTI sinus or yeast infection, dry eyes, dry mouth, fatigue, weakness, internal tremor. Anxious all the time, insides are quivering. Shortness of breath, insomnia, headaches, migraines, hypersensitivity Systolic blood pressure changes > 20 mg, diastolic changes >5 mg Pulse greater than 30 increase when standing Neurocardriogenic syncope - can die, most dangerous, may need pace maker Taking BP lying down, sitting and standing - take yourself Evaluate medication - iron or b12 deficiency? Internal tremor - inderol Anxiety - SSRI/SNRI Gastroparesis - GI doctor, erythromycin to help stimulate, small frequent meals, fibrous foods and vegetables, oranges and broccoli, low fat foods, walk after meals, drink water Blood pressure low - salt and water, Gatorade, electrolyte, mineral water, smart water Severe, nothing is helping - IVIG Patients are so disabled they can't work, may need home school Compression stocking - helps for orthostatic intolerance, push blood flow back up Elevate had of bed Heat and cold intolerance - no significant temps change, short cool shower, avoid alcohol and caffeine

Dysautonomia (UpToDate)

Hereditary sensory and autonomic neuropathy type 3 (HSAN3) is more commonly known as familial dysautonomia or Riley-Day syndrome. This disorder is a progressive sensorimotor neuropathy, but sympathetic autonomic dysfunction is responsible for most clinical manifestations ●Episodes of dysautonomic crises occur in many patients, characterized by nausea and vomiting, and by symptoms of sympathetic storm with irritability, tachycardia, hypertension, facial flushing, bronchorrhea, and diminished oral coordination resulting in swallowing and speech dysfunction. These crises can be triggered by physical or emotional stress. ●Additional autonomic symptoms include orthostatic hypotension, excessive salivation, gastrointestinal motility dysfunction, bladder dysfunction, decreased or absent tearing, pupil dilation, hypohidrosis and episodic hyperhidrosis (leading to defective temperature dysregulation), and blotchy skin. ●Glomerulosclerosis and chronic kidney disease may develop, unrelated to autonomic dysfunction. ●Neuropathic symptoms include loss of reflexes, hypotonia, and decreased perception of pain and temperature. ●Additional clinical features include small stature, kyphoscoliosis, a smooth tongue that lacks fungiform papillae, recurrent aspiration or vomiting, multiple sites of skin trauma, dysarthria, mental deficiencies, and emotional lability. The respiratory, cardiovascular, and cerebrovascular responses to hypoxia are markedly diminished, but responses to hypercarbia remain intact. The disease affects central as well as peripheral myelin. Genetics — Familial dysautonomia (HSAN3) is transmitted as an autosomal recessive trait and is essentially limited to children of Ashkenazi Jewish decent. The carrier frequency is 1 in 30, and the incidence is approximately 1 in 3700 live births. Only a few non-Jewish children have been described. Diagnosis — Genetic evaluation is sensitive and specific for the diagnosis of HSAN3. No other specific diagnostic test exists, although the following tests are suggestive: ●Intradermal histamine may produce a wheal but no flare or pain at the injection site. Histamine should be used at a 1:1000 dilution in adolescents, while a 1:10,000 dilution should be used in infants. ●Urine excretion of vanillylmandelic acid excretion may be diminished, and excretion of homovanillic acid increased. ●Methacholine (2.5 percent) administration into the conjunctival sac produces no response in normal children, but induces pupillary constriction in children with familial dysautonomia. ●Blood pressure responses are exaggerated following intravenous norepinephrine, and methacholine infusion generates an increased hypotensive response. Management — No specific treatment exists for familial dysautonomia (HSAN3). Supportive care and symptomatic therapies are the mainstay of management: ●Vomiting and dysautonomic crisis may require intravenous fluids to prevent dehydration. Diazepam, clonidine, pregabalin, or a peripheral decarboxylase inhibitor such as carbidopa may be helpful to control symptoms. ●Nutrition, airway protection, and avoidance of aspiration are important, particularly during infancy. ●Gastroesophageal reflux management includes upright positioning during feeding, prokinetic agents (eg, bethanechol), H2 antagonists, and gastrostomy with or with fundoplication. ●For patients with chronic lung disease from recurrent aspiration pneumonia, daily chest physiotherapy (ie, nebulizers, bronchodilators, postural drainage) is suggested. High frequency chest wall oscillation was beneficial in one report. ●Orthostatic hypotension can be treated with elastic stockings, leg exercises, and physical maneuvers such as squatting and bending forward. Fludrocortisone and midodrine may help orthostatic symptoms. Observational data suggest these agents prolong survival in patients with familial dysautonomia ●For patients with bradyarrhythmia and/or syncope, a pacemaker may be protective ●Corneal scarring is a risk due to decreased corneal sensation and diminished tearing. Artificial tear solutions that contain methylcellulose can be given three to six times daily. Other measures include maintenance of normal hydration, moisture chamber spectacle attachments, and soft contact lenses. Tarsorrhaphy is an option if conservative measures fail. ●A yearly spine examination is suggested to evaluate for kyphoscoliosis; some patients may benefit from spinal fusion. ●Patients are at risk for decubitus ulcers due to decreased pain sensitivity. Awareness of pressure points is needed when fitting orthopedic devices. ●Exercise may be helpful to correct or prevent contractures. Dysautonomic crisis — To control vomiting and dysautonomic crisis, some experts treat with intravenous or rectal diazepam (0.2 mg/kg every three hours); oral clonidine (0.005 to 0.01 mg/kg daily in three or four divided doses) is another option. Clonidine may be increased gradually up to 0.025 mg/kg per day in four divided doses, with a maximum total dose of 0.9 mg/day. carbidopa is effective as an antiemetic for adolescents and adults with HSAN3 and severe nausea and retching that is refractory to treatment with benzodiazepines and clonidine. carbidopa was started at 12 mg daily and the dose was increased every other day in 25 mg increments up to a total of 600 mg daily given in three divided doses. significantly less nausea and retching while on carbidopa treatment with pregabalin was associated with improvement in nausea and symptoms of dysautonomic crisis in 13 of 15 patients, most of whom were maintained on their pre-study regimen of a benzodiazepine and/or clonidine Pregabalin was started at 25 to 50 mg twice daily and gradually titrated over one month to benefit or a maximum total daily dose of 6 mg/kg. Prenatal screening — Intrauterine diagnosis of familial dysautonomia (HSAN3) can be made with up to 98 percent accuracy using linked genetic markers in affected families although higher accuracy would be anticipated by testing for the known familial mutations. Since the advent of prenatal screening for the gene linked to familial dysautonomia in 2001, the incidence of newborns with the disease has markedly declined in the United States.

Concussions (notes from class)

Injury to brain - blow to head or face or neck T bone car - side to side, front to back, fall on the floor and throw head back, doesn't have to be a big hit They don't feel right, have brain fog Sports injuries Symptom resolution - variable, 1 week or 2, more than 2 years Side to side impact - most likely have symptoms like LOC Depression, headaches - monitor for a couple of weeks Pituitary with brain hypothalamus region - can become damaged Long term moods, memory, anxiety, personality changes, diff with daily function Someone who can do everything themselves and never had an issue, now they have this problem, they can become very emotional Hemorrhages - microscopic bleeding, iron breaks down, toxic to neurons, can cause movement disorders, neurons can become stretched out Falls - high risk Previous concussion Assess and make sure if it is a safe activity for this child Motor vehicle Physical abuse History of depression Clinical symptoms: nausea, dizziness, tiredness, depression, headaches, difficulty sleeping, and sensitivity to light More severe symptoms: amnesia, vomiting, blurred vision, seizures, trouble sleeping, confusion, weakness, diff coordination -> ER Children may present differently, not able to verbalize how they feel, what do you see, irritable cranky when they weren't before, loss of balance, walking funny, changing in eating or sleeping patterns, lack of interest in things they used to like 4 major categories: memory: difficulty processing information, forming words, foggy, brain feels slow, difficulty concentration physical: ha, n/v, sensitivity to light, tiredness, balance, mood, irritable, sleep patterns complete neuro eval: cranial nerves, sensation, hearing, balance, tandem walk, memory, computer tests, if you were testing before you can see how they are now. But might not know how they were before orientation to person place time, recall stepping test - balance, close eyes, standing right in front of them, you can catch them, hands by side, stepping, if problem with one side or the other - you can see which lesion is one what side of the brain, depending on what side they face, take about 50 steps without opening their eyes really good history: LOC, location, mental status, n/v, speech, ha, difficulties, did they have ha before, is this something new? CT of head or MRI brain to r/o hemorrhage, ER skull fracture - seizure, augmented MS, LOC - referred further to neurology Seen in the office - not to much disability, ha, sensitivity to light, vomiting, I don't know if I lost consciousness, could be loss of memeory Moderate risk, or significant issues - ER Cervical spine - neck pain, ha, whiplash, where is the pain coming from? Advanced cognitive testing - computer tests - language skills, memory, concentration, if impaired or normal. If normal you don't know if they were really high before. EEG Vestibular testing - sitting there and keep falling backward First line treatment - rest until symptoms improve, no driving, video games, texting, or exercise. Step wise approach for the first few weeks Concussion changes the level of brain chemicals - takes a week for levels to stabilize again HA - eating regular meals, drinking enough water, stress, ice packs, Tylenol, no evidence of bleeding can use ibuprofen, if persist for several weeks - walking might help, stationary bike, swimming, shouldn't get worse Magnesium, riboflavin, fatty acids can help headaches Topamax helps well too - watch is hx of kidney stones and glaucoma, can cause drowsiness and fatigue, take in evening to help sleep better Amitriptyline - can help with mood issues and headache If already on something like that can always increase Propranolol - twice a day, helps with headaches and sleep Cymbalta - helps with ha, neck pain Abortive meds - triptans for migraines, be careful with cardiac ischemia and strokes NSAIDs School - home, have a hard time sleeping might not make it through the day Physical therapy is a great choice - strengthen muscles in neck Occupational therapy - concussion, and a few falls in a short time period Psychologist - if mood is low, feels like world has ended Massage therapist Biofeedback Neurocognitive rehab - helps with dementia, memory back, exercises Discuss risk of future concussions, continuation of sports More likely to have in future if already had one Don't return to play that day - evaluate by health care professional School has specific concussion center that they send kids to Complications: vertigo, post concussion syndrome, ha, dizziness, can last for years Important to rest to allow concussion to heal If anything gets worse get help immediately, should never get worse, always better, if worsens ER right away

9. Discuss the therapeutic management for the following types of chronic headaches: a. migraine b. tension c. cluster

Parents seek medical attention for pain relief for their child, in addition to reassurance that there are no intracranial processes occurring (brain tumors). For nonorganic headaches (e.g., no tumor, aneurysm, or metabolic or structural cause), the patient should be taught *pain and stress management techniques; NSAIDs are the first-line* pharmaceutical for acute treatment. There can be significant loss of school attendance as a result of headaches, but attendance should be mandatory. A quiet rest period may be allowed at school if needed, and school nurses can be helpful in developing a plan for this. If the child remains home, activities should be restricted to bed and all homework completed. The child should be returned to school if the pain improves during the school day. Minimize attention to the headache. Relaxation exercises or biofeedback training can be helpful. Trigger factors should be avoided. a. goals: abortive therapy; reducing frequency, severity, and length of treatment; reducing loss of impairment; improving overall quality of life; avoiding escalation of medications; optimizing self-care abilities of the patient and family; using beneficial and cost-effective treatment; and minimizing medication side effects. *Many of the newer medications for migraines (e.g., triptans) have not been adequately tested for safety and efficacy in children and adolescents, with the exception of sumatriptan and zolmitriptan.* An 8- to 12-week course of preventive treatment is necessary to determine efficacy, and daily regimens should be used only for a set period of time. treating throughout the school year and then gradually curtailing daily agents during the summer months. An alternative for younger children is to use shorter courses of preventive medications (6-8 weeks) followed by gradual weaning. All individuals with migraines benefit from regular sleep, exercise, moderate caffeine intake, and adequate hydration. Medications should be taken as soon as possible after the onset of the headache; should be taken in the prescribed dosage; should be available at home, school, or work; and the overuse of analgesics is to be avoided (more than three doses per week) Prophylactic therapy is considered when migraines cause a child to miss school regularly and when the child suffers severe headaches two to four times a month with a clear sense of functional disability. Anticonvulsants are also used, especially if the child has a seizure disorder. Other medications to consider might include beta-blockers, antidepressants, NSAIDs, or calcium channel blockers. analgesics, triptans, biofeedback and relaxation chronic daily low grade: acute only: ibuprofen, naproxen, acetaminophen, other NSAID (with clear limits to their use); antidepressants; amitriptyline (>12 yrs); antihistamine cyproheptadine (3-12 yrs) b. tension: acute: analgesics, NSAIDS, amitriptyline, tizanidine chronic: antidepressants (treatment of choice; start with low dose and increase every 3-7 days; try for at least 1-2 months), beta-blockers, anticonvulsants other: massage; relaxation techniques; cold; alternating warm compresses to occipital area c. cluster: prevention: lithium, verapamil, valproic acid, topiramate, melatonin suppression: prednisone 1mg/kg/day for 5 days, then taper for 2 weeks acute attack: sumatriptan (injection or nasal), oxygen, lithium. Oxygen is treatment of choice (inhale 100% oxygen for 20 min at 8-15L/min following onset; zolmitriptan. Acute Treatment of Nonspecific Acute Migraine Medications (these should be tried first in acute management) • Acetaminophen (gel capsule)* 10-15 mg/kg PO every 4 hr up to 500 mg every 4 hr. Acetaminophen has faster onset of action than ibuprofen • Ibuprofen* (gel capsule) 7.5-10 mg/kg PO every 4 hr up to 800 mg every 8 hr Ibuprofen showed greater headache resolution than acetaminophen (rebound headache can occur) • Naproxen sodium Children: 5-7 mg/kg PO every 8-12 hr, Adolescents: 250-325 mg PO Safe and effective Dimenhydrinate 2-5 yr: 12.5-25 mg PO every 6-8 hr (max 75 mg/24 hr), 6-12 yr: 25-50 mg PO every 6-8 hr (max 150 mg/24 hr), >12 yrs: 50-100 mg PO every 4-6 hr (max 400 mg/24 hr) *Use when vomiting is a major symptom* Migraine-Specific Acute Medications* 5-HT1-receptor agonists (triptans) • Sumatriptan*: Consider for children >12 yr old when there is no response to analgesics Nasal spray*: 5 mg/spray; 5-20 mg each nostril once (may repeat every 2 hr if headache unresolved; max dosage 40 mg/24 hrs) Subcutaneous (self- administered): 3 or 6 mg once (may repeat in 1 hr; max dosage 12 mg/24 hrs) Oral: 25-100 mg once (may be repeated every 2 hr; max 200 mg/24 hr); available in tablets: 25, 50, or 100 mg •Triptans are all FDA approved for those ≥18 yr old; they are regarded safe, and well tolerated in children ≥12 yr old; efficacy rates for the triptans (except for sumatriptan nasal spray and oral zolmitriptan) are essentially the same as for placebos; they may prolong an aura. •If the first dose is given in the outpatient setting, the patient should be monitored for 1 hr. Side effects: Hot flushes; nausea and vomiting, chest/neck/head pressure, tingling, bad taste (nasal agents) DBCT: When compared with placebo, nasal sumatriptan significantly reduces headache; side effects present with sumatriptan vs placebo. Inadequate data to support use of subcutaneous sumatriptan use in children; do not use in basilar-type and hemiplegic migraine or in those with cardiovascular disease, uncontrolled hypertension, or who have used MAO inhibitor in prior 2 weeks. Has been used off-label in children <12 yrs who have not responded to typical analgesic regimens. • Zolmitriptan: ≥18 yr: 2.5-5 mg PO repeated every 2 hr prn (max 10 mg/24/hr) Limited studies in children <18 yrs: no significant benefit seen in one study in children 12-17 yrs. • Rizatriptan: ≥18 yr: 5-10 mg PO repeated every 2 hr prn (max 30 mg/24/hr) (ODT available) Studies limited in children; one study found no difference in symptom relief between drug and placebo; side effects well tolerated. • Naratriptan, Almotriptan, Frovatriptan, Eletriptan: ≥18 yr: 2.5 mg PO once, repeated once in 4 hr, prn ≥12 yr: 6.25 mg PO once, repeat once in 2 hr prn (max 2 doses/24 hr) ≥18 yr: 2.5 mg PO at onset; repeat every 2 hr, prn (max 7.5 mg/24 hr) ≥18 yr: 20-40 mg PO, repeated every 2 hr prn (max 80 mg/24 hr) Do not use concurrently with an SSRI or SNRI because serotonin syndrome can occur. Prophylaxis Treatment (Maintain Use for at Least 1 Year) Antidepressants •Amitriptyline (nortriptyline and desipramine have not been studied): Starting dosage 5-10 mg PO at bedtime, increasing every 2 wk towards maximum of 1 mg/kg/day, Not assessed in controlled studies but is one of the most widely used agents, Efficacy in 50%-80% of children, Adverse effects: somnolence, dry mouth, dysrhythmia (order an ECG if dose exceeds 25 mg/day), Use with caution in children ages <12 yr • Trazodone: 7-18 yr: 1 mg/kg/day PO divided tid • Mixed results; no appreciable side effects Anticonvulsants • Divalproex sodium: 10-30 mg/kg/day PO bid in divided doses up to 40 mg/kg/day • Open-label trials only done: Showed 50% reduction in headache frequency in children ages 7-16 yr • Adverse effects: Weight gain, heartburn, hair loss, dizziness • Not for use in children ages <2 yr • Topiramate: 5-10 mg/kg/day PO divided bid up to maximum dose 100 mg/day •Adolescents: Start with 15-25 mg PO dose at bedtime; gradually increase to 50 mg bid on a weekly or every- other-week basis; benefit may be seen at 25 mg at bedtime • Gaining wide acceptance for efficacy; well-designed study showed benefit at 50 mg bid dosing with more than 80% of patients showing >50% improvement after 8 weeks of treatment • Adverse effects: Weight loss, episodes of paresthesia, cognitive slowing, loss of appetite, dizziness, irritability; monitor any change in school/cognitive performance • Indicated in epilepsy for children as young as 2 yr • Levetiracetam: Open label studies done in children (no well-controlled studies) showed promising results of >50% reduction in migraine frequency. • Zonisamide: Small open label study of children 10-17 yrs showed 2⁄3 of children had >50% reduction in mixed refractory headaches. Antiserotonergic agents •Cyproheptadine: Age <2 yr: Not recommended 2-6 yr: 0.25 mg/kg/day PO (tablet or syrup) Age ≥7 yr: 2 mg PO daily; then titrate to 3-4 mg PO daily OR < 10 yrs without overweight problems: 2-4 mg PO at bedtime; may be gradually increased to bid- tid use if side effects not problematic • Used more in toddlers because weight gain (due to appetite stimulation) and somnolence are primary adverse effects in older children; sedation more problematic at doses higher than 4-8 mg/24 hr •In children ages 3-12 yr, drug was effective in up to 83% of patients, per retrospective study. • Do not use methysergide in children <10 yr and do not use for more than 3 mo (prolonged use can cause retroperitoneal or pulmonary fibrosis). Antihypertensives Beta-blocker: propranolol (timolol, atenolol, metoprolol, nadolol have not proven effective •≥7-8 yr: 10-20 mg PO tid; start with 10 mg/24 hr and increase by 10 mg/week • May take up to several weeks to a month to be effective • May lower blood pressure or cause depressive adverse effects or exercise-induced asthma • 71% of children 7-16 yr had complete remission using 60- 120 mg/day in a DBCT; other trials failed to show any improvement in headache frequency. • Do not use in children with history of asthma; use with caution in children with depression • Alpha-agonist (clonidine): No significant difference in headaches between clonidine and placebo groups • Calcium channel blocker (Flunarizine): 5-13 yr: 5 mg PO at bedtime (may be increased to 10 mg at bedtime), Probably effective for reducing frequency

10. At what point should the nurse practitioner refer and/or consult with a specialist ?

Refer all patients with organic (structural) headaches. Brain tumors, abscesses, hematomas, and arteriovenous malformations in children are generally associated with ataxia, papilledema, intellectual changes, or behavioral changes. These processes crowd out other intracranial structures, precipitating edema and interfering with the normal actions of CSF and vessels. Infants may initially accommodate well to the increase in intracranial pressure because of the ability of their cranial sutures to expand.

Phenylketonuria a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. Classic most common form, results from deficiency of the enzyme phenylalanine hydroxylase, which converts phenylalanine to tyrosine. b. Untreated leads to elevated phenylalanine concentrations in the blood and brain and results in CNS damage with profound mental retardation. an autosomal recessive trait c. No clinical manifestations are noted at birth, and the effects of high phenylalanine levels may not be apparent in the first few months, if untreated, irreversible brain damage has occurred. Children with more advanced, untreated disease tend to have *lighter skin and hair* than typical for their race and develop an *eczematous rash and a musty or mousy odor* related to buildup of phenylacetic acid. d. detected on newborn screening. ingesting a normal diet will have serum phenylalanine levels greater than 1200 mmol/L on confirmatory testing, whereas others with milder hyperphenylalaninemia will have intermediate levels. mgmt: limiting the dietary intake of phenylalanine, with a *goal of serum phenylalanine levels between 120 and 360* mmol/L in infants and young children. Phenylalanine is an essential amino acid and cannot be eliminated entirely, thus patients need to receive enough to meet growth needs. To obtain the essential amino acids and to meet energy and other nutritional needs, the *diet is supplemented with a medically modified formula, free of phenylalanine*. Over the child's first year or two of life, parents are *educated on the phenylalanine content of foods*; the child's *phenylalanine level is frequently monitored*; and a *phenylalanine "allowance" is established* based on the child's dietary tolerance. *The current recommendation is "diet for life"* to prevent long- term cognitive and neurologic sequelae. Medically modified low- phenylalanine food products are available online and directly, with insurance reimbursement in some states. Pregnant teenagers with PKU must maintain very strict dietary restrictions to protect the fetus. *Consultation with or referral to a dietitian is essential.* • Intervene promptly. Infants who begin treatment before 3 weeks old do not suffer mental retardation secondary • All children require phenylalanine in their diet. • The goal of PKU dietary therapy is to prevent excess phenylalanine accumulation in the body. • Recommended daily intake of phenylalanine decreases with age. Dietary restrictions continue for life. • Most foods contain phenylalanine (approximately 5% of all protein is phenylalanine). • Involve older children in preparation of nutritional supplements. • Supplements may be more palatable if served as frozen drinks or flavored with juices or fruits.

Myelomeningocele a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. Failure during embryogenesis of the vertebrae, skull, meninges, brain, or spinal cord to be encapsulated by the lamina of the vertebrae along the dorsal midline of the body is referred to as a dysraphic defect. refers to the protrusion of both the spinal cord nerve roots (myelo) and the three layers of membranes (meninges) that cover the spinal cord and brain through this spinal defect. The protruding dural sac may contain only the meninges (10% to 20% of cases) or both meninges and nerve roots (the remaining cases). The term spina bifida cystica is often used interchangeably When the vertebral arches fail to close, but there is no subsequent herniation of cord or meninges, the term spina bifida occulta is used. Most cases of spina bifida cystica occur in the thoracolumbar area (90%). result of failure to close the posterior neural tube and the vertebral column. This is the most severe form of neural tube defect occurring in 1 per 4000 live births. Genetic and environmental factors are believed to play a causative role. b. Closure of the neural tube usually occurs during the third and fourth weeks of gestation. Genetic and environmental factors are believed to play a causative role in the failure of the closure to occur. A woman who has had a previous child born with dysraphia has about a 3% to 4% recurrence rate with future pregnancies and Hispanic women are at higher risk. A lack of sufficient levels of folic acid and vitamin A increases the incidence of neural tube defects Intake of certain drugs and toxins is associated with neural tube defects; they include folic acid antagonists (trimethoprim, carbamazepine, phenytoin, phenobarbital, primidone), retinoic acid derivatives (e.g., vitamin A, a paradox given that insufficient levels also cause the defect), valproic acid, and alcohol. Diabetes mellitus (including gestational diabetes), maternal hyperthermia during the first month of pregnancy, trisomy 13 and 18, and Meckel syndrome are also risk factors Myelomeningocele is due to failure of closure of the spinal neural tube and leads to malformation of the vertebral column and spinal cord, and ultimately the skull and brain. The diagnosis of myelomeningocele is usually obvious at birth because of the grossly visible lesion. In approximately 80 percent of cases, the vertebral defect involves the lumbar and sacral region (including the thoracolumbar or lumbosacral area), which is the last portion of the neural tube to close. However, any segment of the vertebral column may be involved, and the defect typically includes the entire spine distal to the most proximal malformed vertebra. At birth, the neural plate appears as a raw, red, fleshy plaque seen through a defect in the vertebral column (spina bifida) and the skin. A protruding membranous sac containing meninges, cerebrospinal fluid (CSF), and nerve roots is under the dysplastic spinal cord, which protrudes through the defect. Anatomy of the lesion — The exposed neural tissue may be flat or elevated by a CSF sac below. Rostral to the open lesion, the spinal cord is closed. There may be an associated split cord malformation, in which case the dorsal portion of the spinal cord is only a hemicord, and the other portion of the split cord is ventral to the lesion. At the superior margin of the lesion in the midline is a small opening that is the opening into the central canal of the closed spinal cord. If the sac has not ruptured, CSF from the central canal of the spinal cord can be seen coming out of this opening. Running caudally in the midline is the embryonic ventral sulcus. On either side of the sulcus are the motor plates with the motor nerves exiting from the ventral surface of these plates. The outer lateral tissues are the sensory (alar) plates and the sensory nerves enter at the lateral edge. At the lateral edge, the lesion is attached to dysplastic meninges and skin. c. Poor intake of folic acid and exposure to hyperthermia or valproic acid • Saclike cyst containing meninges and spinal fluid covered by thin layer of partially epithelialized skin; 75% found in the lumbosacral area • Flaccid paralysis of lower extremities • Absence of deep tendon reflexes • Lack of response to touch and pain • Constant urinary dribbling signs at birth include lesion at some point along thoraco-lumbar-sacral spine, often with a cystlike structure protruding; neural elements may be apparently absent or may be easily visualized within the sac Other physical anomalies: cleft lip and palate, omphalocele, diaphragmatic hernia, tracheoesophageal fistula, congenital heart disease, bladder exstrophy, and imperforate anus. d. prevention: *A maternal serum test showing an increase in the concentration of alpha-fetoprotein is diagnostic*; if elevated, an *ultrasound* and alpha-fetoprotein is diagnostic; if elevated, an ultrasound and *amniocentesis* are performed (alpha-fetoprotein is the primary plasma protein within the fetus, and amniotic fluid and is elevated if there is a defect in the skin of the fetus). *Cranial ultrasounds should be done to look for hydrocephaly and cephaloceles* (and in turn the Arnold-Chiari type II malformation). It is preferable that these infants be *delivered by cesarean section.* mgmt: In the neonatal period, serial cranial ultrasounds are conducted to watch for the development of hydrocephaly, if this condition has not shown up prenatally. *Surgical resection* and closure of the involved neural tube structures are done within a week after birth; *often shunting for hydrocephaly is also required*. If surgery is not done during that time, death may result in the first year from meningitis or sepsis. *Intrauterine surgery* has also been successful in closing the defect and preventing exposure of the neural tube to amniotic fluid and possible postnatal infection. If the defect occurs in a high spinal region or there is clinical hydrocephalus at birth, survival is also compromised. The PCP's role includes delivering well-child care, assessing and treating acute illnesses (especially UTIs and constipation), monitoring shunt function, checking for skin breakdown, and communicating with and often coordinating services between a myriad of specialists that will be involved (e.g., orthopedists, ophthalmologists [strabismus is common], neurologists, nephrologists, physical therapists, social workers, geneticists). Genitourinary management entails teaching parents (and eventually the child) how to regularly catheterize a neurogenic bladder. Periodic urine cultures, assessing renal function (with serum electrolytes, creatinine), ordering appropriate imaging studies (renal scans, intravenous pyelograms [IVPs], ultrasounds) the PCP needs to be alert to the onset of symptoms indicative of Arnold-Chiari type II malformation and tethered cord, and watch for seizures (15% incidence), learning difficulties, and attention-deficit/hyperactivity disorder (ADHD). Bowel training can help control stool incontinence Surgical repair and multidisciplinary supportive management are indicated. The mortality rate is 10% to 15% in aggressively treated children with most deaths occurring before 4 years old. At least 70% have normal intelligence, but seizure disorders, hydrocephalus, learning disabilities, and neurogenic bowel and bladder are more common than in the general population Folic acid supplementation (400 mcg/day) with a daily multivitamin is helpful in preventing neural tube defects and should be taken by all females of childbearing age. Prenatal vitamins have at least 400 mcg/vitamin; however, additional folic acid supplementation (4000 mcg) is recommended for those women who have had a child with a neural tube defect

4. Define coup and contrecoup injuries.

from uptodate: Mild TBI -> cortical contusions due to coup and contrecoup injuries. If head is struck while *immobilized*, the focus of the injury will be at the *impact site - a coup injury*. If head is *not immobilized* when struck, the majority of the injury may be to the *brain on the opposite side* of the head from *impact* - a contrecoup injury. Contrecoup injuries d/t *acceleration or deceleration* (in the case of falls) imparted to the brain by the impact and are particularly notable in the *occipital region*.

Describe the pathophysiology of headaches.

the exact physiologic mechanism and etiology for have not been conclusively determined. Headache pain occurs when pain-sensitive intracranial structures are activated. Such structures include the arteries of the circle of Willis and some of their branches, meningeal arteries, large veins and dural venous sinuses, and part of the dura near blood vessels. Muscles around the head, neck, scalp, eyes, jaw teeth, sinuses and the external carotid artery and its branches, are pain sensitive structures external to the skull. Stimulation of these structures results in more localized pain that is carried by cranial nerves V, VII, IX, and X. intracranial stimulation refers pain imprecisely (e.g., occipital lobe tumor). The pain-sensitive blood vessels can be stimulated by inflammation, trauma, traction, compression, malignant infiltration, and other disturbances coenzyme Q10 (CoQ10) deficiency may be present in children with frequent migraines

7. What should the nurse practitioner do in order to perform a thorough evaluation of a child presenting with complaints of a headache?

A complete physical and neurological examination • Blood pressure, supine and standing with 2-minute interval between them • Height and weight • Head circumference (all children) • Eyes: Palpate for tenderness; check disks, movements • Ears: Patency of canals, normal tympanic membranes, absence of tumors • Neck: Palpate muscles; check range of motion for nuchal rigidity • Sinuses (frontal and maxillary) • Teeth (percuss, inspect) • Temporomandibular joints (mouth and jaw): Palpate and check range of motion • Thyroid gland • Bones and muscles of skull: Palpate for tenderness; listen for cranial bruits; check range of motion of cervical spine • Extremities: Tandem gait • Nerves: Palpate supraorbital, trochlear, occipital nerves; assess CN IX-XII • Reflexes: Pronator drift test

8. What type of diagnostic exams can be requested in order to help in the diagnosis?

Imaging studies are rarely indicated unless the history suggests intracranial pressure; there is a sudden onset, increased severity, or change in headache pattern; the neurological examination is abnormal; or complaint of "dizziness" A CT scan without contrast is usually adequate to determine the presence of a brain tumor. If abnormal, an MRI should be done. An EEG should be obtained if the history and physical examination suggest a seizure process. If there is a history of external trauma, such as from a motor vehicle accident, cervical and spinal x-rays should be ordered. How to Proceed When the Complaint Is Dizziness Complaint of Dizziness Studies Indicated Lightheaded - None Double vision (posterior fossa location) - MRI Sensation of whirling motion of oneself or of room or objects (vertigo) - MRI Confusion - MRI, EEG, comprehensive metabolic screen

8. Describe the clinical manifestation for each of the following: a. focal brain injury b. diffuse brain injury

a. Focal motor signs related to increased intracranial pressure (ICP) EDH: Classic clinical sign is delayed onset of symptoms, Initial neurological symptoms may be minimal or absent Secondary injury from increased ICP as hematoma enlarges, may result in neurological symptoms (a) Headache (b) Confusion (c) Vomiting (d) One-sided weakness (e) Agitation (5) Symptoms may progress to lethargy, coma, and even death if left untreated Head trauma, especially to the temporal or occipital region History of loss of consciousness History of altered mental status per caregiver Severe headache Vomiting Lethargy (GCS 9-14) or coma (GCS ≤8) Irritability, pallor, cephalohematoma (infants ≤12 months) Lucid interval for minutes up to several hours followed by clinical deterioration (classic temporal EDH) Ataxia, dizziness with potential for abrupt catastrophic deterioration (posterior fossa EDH) Lateralizing signs (anisocoria, hemiparesis, hemiplegia) and Cushing triad (hypertension, bradycardia, respiratory depression) are late findings that indicate cerebral herniation SDH: acute presentation: sx appear within 48 hours, signs of intracranial hypertension including irritability or lethargy, vomiting, headache subacute: sx appear bw 2-21 days, seizures, motor abnormalities (hypertonicity and agitation), systemic (irritability, vomiting, fever, anemia, poor weight gain) impaired consciousness irritability, vomiting, bulging anterior fontanelle, increased head circumference, pallor, lethargy, coma, or seizures retinal hemorrhages, skeletal or skull fractures lateralizing signs such as a fixed, dilated pupil and contralateral hemiparesis hemorrhagic stroke: headache, nausea, emesis, seizures (generalized or focal), hemiparesis, aphasia, neck pain, altered LOC, altered mentation, general deterioration, increased crying, sleepiness, irritability, feeding difficulty subarachnoid: sudden, severe headache, worst headache of my life, LOC, nausea, vomiting, seizures b. physical: ha (persistent, mild), nausea, vomiting, balance problems, dizziness, visual problems, fatigue, sensitivity to light and/or noise, numbness/tingling, dazed or stunned (befuddled facial expression), seizure (transport to hospital in order to differentiate between seizures due to concussion, post TBI, convulsive syncope, drug related epilepsy, or idiopathic primary epilepsy.) cognitive: feeling mentally foggy, feeling slowed down (slurred, incoherent speech, disjointed, incomprehensible statements), difficulty concentrating (cannot recall words, numbers; unable to follow through with normal activities), difficulty remembering time, place, date, forgetful of recent information or conversations, confused about recent events, delayed verbal and motor responses (slower to answer questions or follow instructions; walking in wrong direction), repeats questions, gross incoordination (stumbling, unable to walk tandem or in a straight line) emotional: irritability (low frustration tolerance), sadness, more emotional (appears distraught, crying for no apparent reason), nervousness sleep: drowsiness, sleep disturbances (sleep less or more than usual) trouble falling asleep

Epilepsy a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. Seizures are due to the misfiring of the cortical neurons of the brain. Convulsive seizures occur when misfiring causes episodes of involuntary contraction of voluntary muscles. When seizures are recurrent and unrelated to fever, the disorder is called epilepsy. Seizures represent either brain dysfunction or significant underlying disorders. A patient may demonstrate characteristics of more than one type of seizure. Epilepsy most often has two characteristics: the seizures recur, and the seizure events are unprovoked. b. Different kinds of seizures arise from disorders in diverse parts of the brain. can result from a variety of genetic, symptomatic, or idiopathic conditions. 20% of individuals with epilepsy have a genetic etiology. Several familial epilepsies have been identified. These include benign neonatal convulsions, juvenile myoclonic epilepsy, and progressive myoclonic epilepsy c. Description of the seizure: Focal or generalized, loss of consciousness, aura, length of postictal sleep or confusion, duration of the episode, and associated illness • Any underlying medical diagnosis (e.g., diabetes, renal disease, cardiovascular disorder) • Previous CNS infection or birth trauma • Intrauterine infection, trauma, bleeding • Toxic exposure or drug use • Anticonvulsant medication stopped abruptly • Recent head injury • Family history of seizures • Any noted missed milestones Physical Examination • Focal abnormalities, weakness • Presence of seizure activity during the examination • Hypertension (for renal disease) • Systemic disease • Cardiovascular disorder • Neurocutaneous disease, café au lait spots of neurofibromatosis, ash leaf spots or adenoma sebaceum of tuberous sclerosis, facial hemangioma of Sturge-Weber syndrome • Signs of head trauma • Transillumination of the skull in infants partial or focal focal simple: consciousness not impaired; lasts 20-30 seconds with sensory or somatosensory symptom: pins and needles, numb, auras include lights, tastes, sounds with autonomic symptom: recurrent abdominal pain, headache, sweat, laugh, cry, tachycardia, dilated pupil, may have migraine quality Complex focal: consciousness impaired, clonic activity, forced head/eye deviation, focal tonic posturing, automatisms - purposeless motor activities (lip smacking, repetitious swallowing, chewing, finger/hand fidgeting, tics) lasts 1-2 minutes impaired consciousness: staring spell with cognitive sx: may have confusion with affective sx: aura of fear with psychosensory sx: may have odd smell/taste; visual or auditory hallucination with psychomotor sx: automatism focal seizure, secondary generalized: begins in one part of body but then generalized, aura can let person seek safe position idiopathic localization related seizures benign focal (or rolandic) diurnal: alert, unilateral twitching, drooling, paresthesia of face, gums, tongue, buccal mucosa, may progress into hemiclonic or hemitonic movements, with or without postictal weakness of affected side nocturnal: advances to generalization Generalized seizure: usually no aura absence (Petit mal): lasts 5-30 seconds; lapse of consciousness (short staring spell) can have associated movements, no falling, no aura, hyperventilation for 3-4 minutes can trigger seizure, blinking lights can also trigger myoclonic (infantile spasms): head drops or sudden flexing, may suddenly cry out, older children exhibit trunk or extremity flexion, hypsarrhythmia on EEG, with no normal background activity clonic: rhythmic jerking tonic: intense muscle contraction tonic-clonic: at any age; *most common type of seizure*. Grand mal; begins with LOC, stiffening, violent jerking, postictal phase of sleep and confusion, aura in some; may have abdominal pain or headache, life threatening producing hypercarbia, respiratory acidosis, lactic acidosis, some occur in sleep atonic: similar to myoclonic, aka drop attacks, duration <15 minutes, child often falls, injury prevention important d. Diagnostic Studies • *Complete blood count* (including platelets, liver function tests)—useful for diagnostic purposes or as a baseline before anticonvulsant therapy is started • *Metabolic screen* • *Blood glucose*—standard in all patients • *Urine and serum toxicology*—only if illicit drug exposure is suspected • *Lumbar puncture*—<6 months; any aged patient with persistent changes in mental status or failure to return to baseline functioning; patients with meningeal signs • *EEG*—standard in all children after first nonfebrile seizure. An abnormal EEG supports the seizure diagnosis. a normal EEG when the child is not seizing does not rule out a seizure disorder. *Video EEG* (VEEG) over 1 to 6 days is another option to identify seizure activity. • *MRI*—not routinely indicated if the initial seizure is followed by a normal neurologic examination and return to baseline mental status. Imaging is recommended: (1) if the patient demonstrates cognitive changes after several hours and postictal focal dysfunction (signs of increased intracranial pressure, such as found with tumors, abscesses, strokes, or vascular malformations); (2) if the seizure lasted more than 15 minutes; (3) in infants younger than 6 months old; and (4) if any new onset of focal neurologic deficit has occurred. An *MRI is preferred imaging study over CT scans* because of its increased sensitivity. • *CT scan*—used only in cases of marked cognitive, motor, or neurologic dysfunction of unknown etiology (e.g., head injury, brain infection or tumor, abscesses); abnormal EEGs; or focal seizure symptoms that may or may not evolve into a generalized seizure. •*Polysomnography* (simultaneous EEG, electromyogram, electrocardiogram [ECG], and electrooculogram) can be useful to assess nocturnal seizures. Management *Referral* - refer to a neurologist *Antiepileptic drugs (AEDs)* are usually prescribed, especially after a second seizure. Delaying treatment does not affect ultimate control, but early diagnosis and intervention can often reduce the disabilities and risks associated with the condition. Management of Stable Patients With Diagnosed Seizure Disorders The PCP can monitor stable children with seizures, including continuing the prescription for their anticonvulsant(s), monitoring drug levels, and performing case management. 1/3 of children who have a hx of cognitive or motor impairments will have a recurrent unprovoked seizure within 1 year. Drug Monitoring All providers working with patients receiving anticonvulsants should be familiar with the common drugs and their major side effects. Helping with compliance issues is also a component of the monitoring role If possible, *start with one drug with the fewest side effects and describe how the drug works with the child and parent, with an explanation of side effects.* Key points for drug monitoring include: • Patients can be controlled with *subtherapeutic blood levels.* • Patients can be free of side effects at levels beyond the therapeutic range. • *Phenytoin saturates the enzyme system; therefore, even a small increase in dosage can cause a marked increase in blood levels.* • Half-lives and steady state concentrations vary by patient and when new AEDs are introduced or when other non-AEDs being taken (e.g., antibiotics, antipyretics). Half-lives are longer with the introduction to a new drug; steady concentrations (and elimination) of the drug are achieved at five half-lives. • If gastrointestinal side effects occur, decreasing the dosage and increasing the frequency of administration may help; try changing to an enteric-coated pill or taking the drug after eating. • Administer drug twice daily or daily for better compliance. • The first signs of toxicity usually include sedation, changes in behavior, and changes in cognition; other drug toxicities may cause decreases in memory and attention span or interpersonal relationship difficulties. It is important to note that some patients may exhibit these changes and have drug levels within the normal range. • Metabolites of the drugs can cause hypersensitivity side effects. • Monitor routine drug levels based on the clinical picture with trough levels (random drug levels are rarely helpful). • Some herbal products interfere with seizure control (e.g., ginkgo and kava) Carbamazepine: partial generalized tonic clonic in children >2 year old; contraindicated for absence and myoclonic. baseline CBC at 6-12 weeks, then annually, drug blood levels Phenytoin: partial, generalized, status epilepticus. drug blood levels phenobarbital: simple partial, tonic-clonic ethosuximide: absence valproic acid: first-line generalized seizures if <10 year old, myoclonic, absence, baseline CBC with diff, LFTs; CBC, SGOT, and drug levels in first 6 mo primidone: partial, tonic-clonic, myoclonic vigabatrin: partial, myoclonic, infantile spasm, visual fields every 3-6 mo felbamate: partial, generalized, Lennox-Gastault syndrome, LFTs, CBC with diff, platelets, reticulocyte count monthly Gabapentin (adjunct drug with other AEDS used for partial seizures) - refractory partial onset, rolandic lamotrigine: partial, lennox-gastaut syndrome, absence, atonic, juvenile myoclonic topiramate: partial, generalized and lennox gastaut (adjunct drug), partial, tonic-clonic oxcarbazepine: partial lacosamide: partial zonisamide: partial, generalized levetiracetam: partial tiagabine (adjunct drug only): partial, generalized clonazepam: partial, absence, myoclonic, infantile spasms, Lennox-Gastaut syndrome, akinestic - LFTs, CBC with diff Anti epileptic Drug Withdrawal After 2 years or longer without seizures, pediatric neurologist will consider gradually withdrawing anticonvulsant therapy after obtaining an EEG. Patients with hx of benign epilepsy with rolandic spikes or with idiopathic generalized seizures are more likely to be successfully withdrawn. Those with complex partial seizures and juvenile myoclonic seizures are more likely to have a recurrence once medication has been withdrawn. Drug withdrawal should occur over a span of several months because abrupt weaning might cause status epilepticus (SE) Children with mental retardation, CP, focal motor deficits, age of onset younger than 2 years, symptomatic seizures, and abnormal EEGs are not appropriate candidates for weaning. Weaning is supervised closely, with one drug removed at a time. Of children who have been seizure-free for 2 years and have low risk factors, 70% to 75% remain seizure-free without drugs. If seizures do recur, 50% do so within the first 6 months of weaning and 60% to 75% within 2 years. 3/4s of recurrences occur during weaning or within the first year If the onset of seizures occurred during a time of anoxia, head injury, meningitis, or encephalitis, the AED treatment can be stopped after recovery from the condition is complete. The child can always be restarted should there be a recurrence. Ketogenic Diet useful in young children with all types of seizures, myoclonic forms, infantile spasms, atonic- kinetic types, and with the mixed seizures of Lennox-Gastaut syndrome considered when the side effects of AEDs are intolerable or when allergies to AEDs preclude administration. between 2 and 5 years old because the desired steady state of ketosis is easier to maintain. The diet is stringent and requires utmost vigilance to the ratios of calories, protein, fat, carbohydrates, vitamins, and minerals. It is best managed under very tight control with medical and dietetic leadership. Side effects usually involve abdominal pain and diarrhea. A prescreening process, including psychological testing to determine the child's and family's emotional functioning, coping, and problem-solving abilities, is recommended. A dietitian should screen the child for nutrition and growth status. A nurse should interview the family for understanding of and education about the protocol. The diet is started while the child is admitted to the hospital, where metabolic and neurologic states can be monitored. ketogenic diet for 3 to 6 years demonstrated that 34% had a greater than 90% reduction in their seizures Surgery: complex partial seizures; Focal resection surgery is used only in children whose epileptic focus is localized, who have failed to respond to AEDs, and whose development has been assessed over time. Seizure-free rates after resection - 60% to 80%, with minimal loss of neurologic function depending on the type of surgery Hemispherectomy or interhemispherectomy can be curative. Side effects: hemiparesis, incontinence, stuttering, and poor hand coordination. Temporal lobotomies are an option for treating intractable partial complex seizures localized to the temporal area; side effects are aphasia and superior quadrant visual loss. Slightly more than 50% of candidates achieve freedom from seizures. Callosotomy and vagus nerve stimulation, as palliative surgeries, are options for children with multiple regions of hemispheric involvement that result in intractable seizures. vagus nerve stimulation (VNS), a programmed device—"pacemaker of the brain"—is implanted in the anterior chest wall. A wire wraps around the left vagus nerve and sends regular, mild pulses of electrical energy to the brain via the nerve. A patient with an aura can stimulate the device to prevent a seizure. For those without an aura, the device is set on specific parameters given the patient's seizure pattern. With VNS, seizures and side effects can be better controlled. Counseling diagnosis, treatment (including specifics of the prescribed AED), necessary follow-up, and long-term prognoses. Myths and negative attitudes may need to be addressed. Laws vary from state to state regarding driving, but generally a teenager who has been seizure-free for 2 years and has demonstrated good drug compliance should be allowed to drive. Many antiseizure medications are teratogenic; therefore, contraception is essential for sexually active females. social stigmas and problems with self-esteem. Other mental health problems treat children as normally as possible and seek appropriate support groups. Safety Uncontrolled seizures can present safety hazards for an unsupervised child. including school personnel. Safety helmets worn at all times are sometimes warranted if falls and head injury occur frequently. Swimming alone is never recommended, but swimming, contact sports, and climbing are to be allowed if the child is well controlled and there is constant supervision during these activities. Immunizations *The decision to give pertussis vaccine to children with neurologic seizures or other neurologic conditions needs to be made on an individual basis.* Children who will be in childcare centers, special clinics, or residential care centers should be immunized if possible. Progressive neurologic conditions with developmental delays or nonfebrile seizures are reasons for deferral of pertussis vaccine until the underlying cause has been determined. Infants and children with a personal history of seizures have been noted to have an increased risk of post-DPT immunization seizures, less so with the acellular pertussis form of DPT (DTaP). These seizures generally have been found to be febrile, self-limited, brief, and generalized. There has been no evidence that such febrile seizures lead to brain damage, predispose one to epilepsy, or aggravate neurologic disorders. Other neurologic conditions that predispose one to seizures or neurologic deterioration or a seizure history in an infant younger than 1 year old should result in consideration of deferral of pertussis immunization until the child is near or at 1 year of age. DTaP and acetaminophen at the time of administration and every 4 hours for the first 24 hours may be given to allay concerns of fever or other adverse effects.

6. Microcephaly and Congenital Hydrocephalus a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. a head circumference 2 SD below the mean for age and sex, gestation or a head in which the growth is increasingly slower than normal. b. can result from conditions in which the brain never formed correctly because of genetic or chromosomal abnormalities. Disease processes that interfere with normal brain growth can also be causative (these infants have normal head circumferences at birth). reflects an abnormally small brain; common causes are intrauterine infections (e.g., herpes, rubella, syphilis); genetic defects; drug usage during pregnancy (especially alcohol) Primary: Caused by a variety of genetic/ chromosomal disorders Secondary: Acquired from multiple causes including: a. Intrauterine infections, e.g., TORCH— toxoplasmosis, other agents [HIV, listeria, syphilis, gonococcus, group streptococcus, varicella-zoster, malaria], rubella, cytomegalovirus inclusion disease, herpes simplex b. Fetal exposure to toxic substances including alcohol, medications (e.g., phenytoin), phenylketones (maternal PKU) c. Fetal exposure to radiation in first and/or second trimesters d. Placental insufficiency, prenatal hypoxia, trauma, maternal hypoglycemia, degenerative diseases (e.g., Tay Sachs) e. Extremely poor nutrition during the first six months of life f. Significant risk for isolated microcephaly— alcohol use, inadequate weight gain during pregnancy, inadequate prenatal care, African-American, and low educational level c. On examination the skull appears to be normally shaped; palpation may reveal some overlapping bones along the suture lines. Brain damage that occurs prenatally may or may not always be evident initially in the newborn; a decreasing head circumference curve may start to occur after the infant reaches 3 to 6 months of age. delayed developmental milestones and neurologic problems. mental retardation Small head—in full term birth and up to six months of age, chest circumference surpasses head circumference (unless child is very obese) Family history of microcephaly Early closure of fontanel; prominent cranial sutures Marked downward sloped forehead with narrowed temporal diameter and occipital flattening (familial microcephaly) Skull asymmetries, high arched palate, and dysplastic teeth (dysmorphic and chromosomal disorders) d. supportive, may involve an interdisciplinary team, and is directed toward management of the resulting deficits. Protein-calorie malnutrition, craniosynostosis, and hypopituitarism are treatable causes of microcephaly. Referral to a neurologist should be made for diagnostic purposes. Diagnostic Tests/Findings 1. Karyotyping for chromosomal disorders, e.g., fragile X syndrome 2. Antibody titers for TORCH infections of the mother and child 3. Test infant serum and urine for amino and organic acids 4. CT or MRI to detect calcification, malformations, or atrophy • Management/Treatment 1. Complete history and physical findings crucial to finding treatable disorders, e.g., hypopituitarism, severe protein-calorie malnutrition 2. Premature closure of sutures may be surgically corrected 3. Most disorders are untreatable; accurate diagnosis is essential for appropriate genetic and family counseling 4. Management is supportive; especially placement in a program that will maximize development since many are mentally retarded congenital hydrocephalus: definition, clinical manifestations - see other card b. depends upon the underlying cause, how quickly the condition develops, and the presence or absence of compensatory mechanisms: ●Hydrocephalus that begins in infancy before fusion of the cranial sutures, if untreated, typically results in marked enlargement of the head and in less compromise of brain tissue, compared with hydrocephalus that develops acutely. This is because the skull expands, partially relieving the intracranial pressure (ICP). In addition, the force of the ICP is distributed over the greater surface area of an enlarged ventricular system, so there is less pressure on the brain parenchyma compared with hydrocephalus that develops in a ventricular system that is not previously enlarged. ●If hydrocephalus occurs acutely or occurs after fusion of the cranial sutures, the head does not enlarge. This results in significantly increased ICP and in more rapid destruction of brain tissue. The progression of ventricular dilatation is usually uneven. The frontal and occipital horns typically enlarge first and to the greatest extent. Their progressive enlargement disrupts the ependymal lining of the ventricles, allowing the cerebrospinal fluid (CSF) to move directly into the brain tissue. This reduces CSF pressure but also leads to edema of the subependymal areas and to progressive involvement of the white matter. As the hydrocephalus progresses, edema and ischemia develop in the periventricular brain tissue, leading to atrophy of the white matter. The gyri become flattened, and the sulci become compressed against the cranium, obliterating the subarachnoid space over the hemispheres. The width of the cerebral mantle may be substantially reduced; gray matter is better preserved than white matter, even in advanced stages. The vascular system is compressed, and the venous pressure in the dural sinuses increases. d. cranial ultrasonography shows dilated ventricles. often an MRI is obtained to further define anatomy. mgmt: medications that decrease CSF production (acetazolamide), a ventriculoperitoneal shunt, or both are used. referral should be prompt. Diagnostic Tests/Findings 1. Cranial radiograph—separated sutures 2. CT scan/ultrasound—impaired CSF circulation causing ventricular enlargement 3. Ventriculography—obstruction detection • Management/Treatment 1. Most cases require extracranial shunts, particularly ventriculoperitoneal types 2. Treatment of underlying cause, e.g., mass or lesion, inflammation, infection, vasogenic edema 3. Anticipatory guidance for families and caregivers throughout child's life a. Referral to support groups b. Teaching signs and symptoms of ICP c. Daily head circumference measurements d. Management of psychomotor challenges

1) Define head injury 2) Define blunt (closed) trauma and open (penetrating) trauma. 3) What is the most common cause of most traumatic brain injuries.

Traumatic brain injury (TBI) - tissue damage to brain and surrounding structures, mild to severe. open or closed. Open - more focal injuries Closed - more multifocal or diffuse damage. primary effects form tear connections within the brain and cause contusions where the brain hits the skull surfaces. Axons to distant areas, fibers in the corpus callosum connecting the two hemispheres, or both can be torn. Contusions and hemorrhage can occur. Secondary effects - hypoxia, ischemia, hypotension, brain swelling, hemorrhage, contusion, and status epilepticus, can affect recovery. most common causes: Infants and toddlers - falls and physical abuse. 0 to 4 years and 15 to 24 years old - highest risk of TBI Young children - falls, pedestrian, and bicycle accidents adolescents - motor vehicle accidents Concussions d/t to sports injuries.

13. Define hydrocephalus. What are the two subtypes? Describe them. Discuss the course of the disease, pathogenesis, and clinical manifestations.

congenital: an accumulation of CSF in the brain's ventricles at birth, occurring in 1 of 1000 live births. Malformations, infections, intraventricular hemorrhage, and disorders in brain development. clinical presentation: Head circumference enlarging or rapidly increasing in size Cranial sutures separated by large, tense fontanelles Diagnostic Studies Cranial ultrasonography shows dilated ventricles. MRI to further define anatomy. From review book: Definition: Increased production, impaired absorption, or obstruction in flow of CSF, leading to excessive CSF in the cerebral ventricles and ventricular enlargement • Etiology/Incidence 1. Obstruction of CSF flow anywhere along its pathway; CSF is produced in the ventricles, exits into subarachnoid space (area of reabsorption) Causes a. Congenital, due to primary cerebral malformation b. Acquired postnatally; secondary to tumor, hemorrhage, or CNS infection (e.g., meningitis) c. Choroid plexus papilloma— overproduction of CSF; rare cause 3. Types a. Obstructive or noncommunicating hydrocephalus (1) Major cause of hydrocephalus (2) Obstruction of CSF within ventricular system before CSF reaches subarachnoid space (3) Sites include aqueduct of Sylvius and obstruction of fourth ventricle (4) Congenital defects—most common cause; can be isolated (aqueductal stenosis) or part of a syndrome (Arnold-Chiari and Dandy-Walker) (5) Acquired conditions—common complication of tumors b. Nonobstructive or communicating hydrocephalus (1) Impairment of CSF absorption within subarachnoid space (2) Usually caused by scarring due to subarachnoid hemorrhage (result of intraventricular hemorrhage in the premature infant) or meningitis • Signs and Symptoms: Presentation is variable depending on age and etiology 1. Birth to 12 months—apparent large head, sluggish feeding, vomiting, piercing cry, and irritability 2. 12 months through adolescence—signs of increased ICP, headache following sleep, lethargy or irritability, confusion, personality changes, possible decline in academic performance, signs and symptoms related to specific focal lesion or tumor, possible sixth nerve palsies, chronic papilledema Physical Findings 1. Infancy—bulging anterior fontanel, scalp vein distention, bossing, "setting sun sign," separated skull sutures, slow PERRL, hypertonia, hyperreflexia, spasticity 2. Childhood—strabismus, extrapyramidal tract signs (ataxia), papilledema, optic atrophy, growth failure from endocrine dysfunction from up to date a disorder in which an excessive amount of CSF accumulates within the cerebral ventricles and/or subarachnoid spaces, resulting in ventricular dilation and increased ICP Normal pressure hydrocephalus, a condition seen predominantly in adults in which the cerebral ventricles are pathologically enlarged, but the ICP is not elevated ●Obstructive hydrocephalus - (also called noncommunicating hydrocephalus) refers to excess accumulation of CSF due to structural blockage of CSF flow within the ventricular system. most common form of hydrocephalus in children and is almost always associated with increased ICP. ●Communicating hydrocephalus - CSF accumulation due to impaired absorption that occurs in the subarachnoid spaces. Rarely, CSF accumulates because of excessive production. typically associated with increased ICP. Many causes of hydrocephalus have both obstructive and absorptive components and the absorptive component may change over time. ●Normal pressure hydrocephalus - In normal pressure hydrocephalus (NPH), the cerebral ventricles are pathologically enlarged, but the ICP is not elevated. ●Ventriculomegaly - Ventriculomegaly is a general term used to describe enlargement of the ventricles as seen on neuroimaging. Ventriculomegaly is a common finding in all forms of hydrocephalus. epidemiology: The reported prevalence of congenital and infantile hydrocephalus ranges from 0.5 to 0.8 per 1000 live and still births. *Myelomeningocele is the most common cause of congenital hydrocephalus* and accounts for approximately 15 to 25% of these cases. The most common cause of *acquired* hydrocephalus in infants is *hemorrhage*, usually as a consequence of prematurity. Other common causes of *acquired hydrocephalus include tumors and infections*. Factors associated with an increased risk of infantile hydrocephalus include ●Birth weight <1500 g ●Prematurity (gestational age ≤30 weeks) ●Maternal diabetes ●Low socioeconomic status ●Male sex ●Race/ethnicity (the risk is decreased in Asians) There is substantial familial aggregation for congenital hydrocephalus. pathogenesis: results from an imbalance between the intracranial CSF inflow and outflow. It is caused by obstruction of CSF circulation, by inadequate absorption of CSF, or (rarely) by overproduction of the CSF. the excessive volume of CSF causes increased ventricular pressure and leads to ventricular dilatation. Obstruction — most common mechanism, anatomic or functional obstruction to CSF flow. The obstruction occurs at the foramen of Monro, at the aqueduct of Sylvius, or at the fourth ventricle and its outlets. Dilatation of the ventricular system occurs proximal to the obstruction. The ventricle just proximal to the obstruction usually dilates most prominently. ●Obstruction of the aqueduct of Sylvius (aqueductal stenosis) causes dilation of the lateral and third ventricles, while the size of the fourth ventricle remains relatively normal. This is a very common cause of hydrocephalus in infants and children. ●Obstruction at the body of the lateral ventricle causes dilation of the distal temporal horn and atrium ●Obstruction of one foramen of Monro causes dilatation of the lateral ventricle on that side ●Obstruction of outflow from the fourth ventricle causes dilation of all four ventricles Impaired absorption — Less commonly, communicating hydrocephalus. d/t inflammation of the subarachnoid villi but also may be caused by impaired CSF absorption or increased pressure in the venous sinuses. The *radiographic hallmark* of communicating hydrocephalus is *dilation of the entire ventricular system, including the fourth ventricle*. Impaired CSF absorption also can occur when cranial venous sinus pressure is elevated. Excessive production — Excessive production of CSF is a rare cause of hydrocephalus. This condition may occur with a functional choroid plexus papilloma. It leads to enlargement of the entire ventricular system and of the subarachnoid spaces, with a radiographic appearance that is similar to communicating hydrocephalus from other causes Clinical Presentation: signs and symptoms result from increased ICP and dilatation of the ventricles. ●Timing of onset (relative to closure of cranial sutures) - infancy (prior to fusion of the cranial sutures): absent or mild. most common presenting complaint is *enlarging head circumference*. older children: neurologic complaints and symptoms of increased ICP are more common. ●The duration and rate of rise in ICP - If accumulation of excessive CSF is slow, allowing adjustments to occur, the patient may have a long period without symptoms. Rapid progression of ventricular dilatation typically results in early development of symptoms. ●The presence of associated structural abnormalities - Infants and children with hydrocephalus caused by space-occupying lesions may present with focal neurologic signs rather than or in addition to signs of increased ICP. Common signs and symptoms — nonspecific and independent of the etiology. Infants and children with mild hydrocephalus may be asymptomatic. ●Headache, Behavior changes, Developmental delays, Nausea and vomiting, Lethargy head circumference is enlarged at birth or in whom serial measurements cross percentiles in standard growth curves, indicating excessive head growth severe headache and other features suggesting increased ICP (eg, persistent nausea/vomiting; altered mental status; ataxia, weakness, diplopia, or abnormal eye movements; severe and persistent headache; headache worsened in recumbent position or by cough, micturition, or defecation). ●Vital signs - bradycardia, systemic hypertension, and altered respiratory rate. ●Head - Macrocephaly, anterior fontanelle may become full or distended. The sutures feel more widely split due to an enlarging head circumference. There is an abnormal percussion note to the head when the sutures are spread (the "cracked pot" sound or Macewen's sign) •Frontal bossing - an abnormal skull contour in which the forehead becomes prominent •Prominent scalp veins ●Neurologic examination •Spasticity - Stretching of the fibers from the motor cortex around the dilated ventricles may result in spasticity of the extremities, especially the legs. •Compression of the third or sixth cranial nerve may result in extraocular muscle paresis leading to diplopia. •Pressure on the midbrain may result in impairment of upward gaze. This is referred to as the "setting-sun" sign because of the appearance of the sclera visible above the iris. It may be part of a larger constellation of neuro-ophthalmologic signs known as Parinaud syndrome ●Fundus - Infants with open sutures are less likely to have papilledema compared with older children. ●Spine - acquired Chiari II malformation associated with spinal dysraphism, such as a pit located above the gluteal crease, a palpable lumbar mass, or skin stigmata of spinal dysraphism . if the pit is located between the upper buttocks in the intergluteal cleft and if the coccyx is palpable, the lesion usually is benign and does not require imaging unless neurological or urinary symptoms develop. ●Growth and pubertal development - Accelerated pubertal development and disturbed growth may result from pressure of the dilated third ventricle on the hypothalamus ●Syndromic features - bilateral adducted thumbs (suggestive of X-linked hydrocephalus), ocular anomalies (suggestive of Walker-Warburg syndrome)

Meningitis a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

for a, b, and c - see question 12 d. Diagnostic Studies dx confirmed with positive culture or Gram stain from blood, CSF, synovial fluid, sputum, or petechial or purpura lesion scraping. PCR useful when antibiotics are given before testing, and organism growth has been suppressed. CBC shows leukopenia or leukocytosis with increased bands and neutrophil percentages, hypoalbuminemia, hypocalcemia, metabolic acidosis with increased lactate levels, decreased platelets, and elevated ESR and CRP. IV antibiotics are started pending culture results; cefotaximine or ceftriaxone are drugs of choice. If susceptibility is confirmed, aqueous penicillin G (250,000 to 300,000 units/kg/day IV divided every 4 hours for 5 to 7 days) has been the drug of choice for infants and children. Alternative drugs (and for those with penicillin allergies) include cefotaxime, ceftriaxone, chloramphenicol (hematologic concerns with this drug), or ciprofloxacin. The patient is kept in respiratory isolation until 24 hours after the induction of treatment. Early dexamethasone given within minutes before antimicrobials may reduce the incidence of residual neurologic and audiologic complications, but its use in children remains controversial. Activated protein C for meningococcal sepsis in children and adults has been studied but also remains controversial Control Measures Exposed contacts must be carefully monitored. Household, school, or child contacts who develop a febrile illness must be evaluated for invasive disease promptly. • Chemoprophylaxis should be given within 24 hours of identification of the index case. At-risk individuals include close contacts (household, daycare, nursery school, those who shared oral secretions [kissing, shared utensils or toothbrushes]) of the index case 7 days before the onset of symptoms are at increased risk of invasive disease. Airline travel of greater than 8 hours while sitting next to an infected individual qualifies an individual for prophylaxis Casual contact with the index case, casual contact with a high-risk contact, or medical personnel (unless they performed mouth-to-mouth resuscitation, intubation, or suctioning before antibiotic therapy was instituted) are usually not considered high risk. Oral rifampin, 10 mg/kg/dose PO (maximum dose 600 mg) twice daily for a total of four doses is the prophylactic treatment of choice for those older than 1 month. Infants younger than 1 month old should be given 5 mg/kg/dose PO twice daily for a total of four doses. Ceftriaxone (125 mg IM for those less than 15 years old; 250 mg IM for those older than 15 years) in a single dose is as effective as oral rifampin; it can be given to pregnant women. Ciprofloxacin (500 mg PO in a single dose) can be given to nonpregnant adults 18 years and older (it can be given for those 1 month old or older but this is not routinely recommended; dose 20 mg/kg PO [maximum 500 mg]). Azithromycin (500 mg single dose) is effective but not recommended for routine use • Prophylaxis during outbreak: Vaccine with serogroups A, C, Y, and W-135, in conjunction with chemoprophylaxis, is advisable to prevent extended outbreaks only if the identified strain is contained in the vaccine. No vaccine covers serogroup B infection.

6. What is the differential diagnoses for chronic headaches? Give a brief description of each.

vascular (migraine): benign occipital epilepsy of childhood: same visual changes as migraine but are either followed by either unilateral or tonic-clonic, complex partial seizure pattern, then headache and nausea - occurs usually as child falls asleep) hemiplegic or basilar type migraine benign paxoysmal torticolois or vertigo: a peripheral vestibular disorder that manifests as sudden, short-lived episodes of vertigo elicited by specific head movements. one of the most common causes of vertigo. self-limited, but can become chronic and relapsing may be incorrectly diagnosed as epilepsy. It typically develops in toddlers (median age 18 months) but rarely occurs after 5 years old. BPV is also a known precursor to the development of migraines later in life. The history may include rapid onset of an attack that lasts seconds to minutes; daily attacks that occur in clusters over several days and then may not recur for weeks or months; and a possible history of motion sickness. Symptoms are likely to have resolved by the time the child is examined. The physical examination findings consist of: • Acute unsteadiness; the child may fall or refuse to walk or sit; may grab on to a parent or object for steadiness. • Nystagmus may be present; no loss of consciousness with events. • Vomiting and nausea may be present and be quite prominent. • Appearance of child is frightened and/or pale. • Child may be lethargic or drowsy; some children may sleep and return to normal activities on awakening • Neurologic examination is essentially negative except for abnormal vestibular function. One possible diagnostic study involves ice water caloric testing to detect abnormal vestibular function. The clusters of attacks may be managed with diphenhydramine, 5 mg/kg/24 hr (maximum 300 mg/24 hr) PO, IM, IV, or per rectum. Once diagnosis is made, parental reassurance is key. Children may be inappropriately diagnosed as having epilepsy and started on anticonvulsants; attacks will not respond to such drugs. abdominal migraine: part of a continuum with migraine and cyclic vomiting syndrome. It typically occurs in children. The diagnosis becomes evident with cyclic episodes. The diagnostic criteria Rome III diagnostic criteria: • Paroxysmal episodes of intense, acute periumbilical (midabdominal) pain that lasts from 1 to 72 hours • Intervening periods of usual health lasting weeks to months • Pain that interferes with normal activities • Pain associated with two or more of the following: Nausea, vomiting, anorexia, headache, photophobia, or pallor • No evidence of an inflammatory, anatomic, metabolic, or neoplastic process that explains the symptoms • These criteria need to be present two or more times in the preceding 12 months. cyclic (or cyclical) vomiting syndrome: recurrent, discrete, self-limited episodes of vomiting between which are completely symptom-free periods. often associated with abdominal migraines intense nausea and unremitting vomiting (a median of six times per hour at peak) often with bilious emesis (83%) and severe abdominal pain (80%) Accompanying symptoms include pallor, listlessness, anorexia, nausea, retching, abdominal pain, headache, and photophobia. The periods of vomiting may last hours or even days; the symptom-free periods may last for weeks or even years. cluster: chronic paroxysmal one sided head pain (hemicranial) migraines temporal arteritis: Giant cell arteritis (GCA) is a granulomatous vasculitis of large and medium-sized arteries. It primarily affects branches of the external carotid artery. typically occurs in people 50 years of age or older and is more common in women. Symptoms of polymyalgia rheumatica are frequently present. The most common serious consequence of GCA is irreversible loss of vision due to optic nerve ischemia. GCA is sometimes also referred to as temporal arteritis, cranial arteritis, or granulomatous arteritis. sinusitis: a symptomatic inflammation of the mucosal lining of the nasal cavity and paranasal sinuses, where clinical symptoms have been present for 4 weeks or less. It can be caused by either a viral or a bacterial infection. glaucoma: Angle-closure glaucoma (ACG) is a group of diseases in which there is reversible (appositional) or adhesional (synechial) closure of the anterior-chamber angle resulting in elevation of the intraocular pressure (IOP). The angle closure may occur in an acute or chronic form. In the acute form, the IOP rises rapidly as a result of relatively sudden blockage of the trabecular meshwork by the iris, via the pupillary block mechanism (mechanism that pushes iris from behind leading to angle closure). The chronic form may develop after acute angle closure where synechial closure of the angle persists. It may also develop over time, as the angle closes from prolonged or repeated contact between the peripheral iris and the trabecular meshwork, which often leads to peripheral anterior synechiae (PAS) and functional damage to the angle. chronic daily low grade: tension headaches migraine with or without aura tension: migraine brain tumor cervical, ocular disorders temporomandibular disorders: describing several painful disorders involving the mandibular joint. The 3 most common subtypes are myofascial pain and dysfunction, internal derangement, and osteoarthritis. Myofascial pain and dysfunction is the most common subtype and affects the muscles of mastication. Internal derangement involves the dislocation of the articular disc in the glenoid fossa. Osteoarthritis of the TMJ involves degeneration of the articular cartilage. chronic sphenoid sinusitis intracranial mass pseudotumor cerebri: Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of increased intracranial pressure that occurs mainly in overweight women of childbearing years, often in the setting of weight gain. Its cause is not known (hence the preferred name IIH). It is a syndrome characterized by increased intracranial pressure and its associated signs and symptoms in an alert and oriented patient, but without localizing neurologic findings. There is no evidence of deformity or obstruction of the ventricular system, and neurodiagnostic studies are normal except for increased CSF pressure and the related neuroimaging signs. Furthermore, no secondary cause of intracranial hypertension is apparent. IIH can either be self-limited or have a life-long chronic course. sleep disorder hyperthyroidism hypertension Drugs Cocaine: Migraine-like pain in patient with no history of migraine headaches Marijuana: Frontal, mild Analgesics, methylphenidate, oral contraceptives, steroids, and cardiovascular agents: Pain follows administration (of drug) or withdrawal (typical of analgesics) Food additives (nitrites, monosodium glutamate common): Pain occurs only in individual genetically sensitive; pain is diffuse, throbbing after ingestion Physiologic Vasculitis: Uncommon in children; can occur as part of a collagen-vascular disease, such as systemic lupus erythematosus. inflammation of blood vessels. It may result in vessel wall thickening, stenosis, and occlusion with subsequent ischemia. Necrotizing inflammation can completely destroy segments of the wall. Vasculitis can involve vessels of any size and can affect any organ system. The clinical presentation varies according to the histologic type of inflammation, the size of the involved blood vessel segment, and the distribution of the involved vessels. Chronic hypertension: Low-grade occipital pain on awakening or frontal during day Eyestrain: Dull, aching pain behind eyes relieved when eyes are closed; caused by muscular fatigue during prolonged ocular convergence; not a refractive error Temporomandibular joint (TMJ) syndrome: >8 years old; pain on one side of face and vertex of TMJ; may be a history of jaw injury Whiplash and neck injury: Pain dull, aching in neck, shoulders, upper arms with poor neck rotation; no nausea or vomiting; caused by muscles contracted to "splint" area of dysfunction in cervical joint areas or soft tissue Following partial or generalized seizure: Diffuse pain Infectious illness (viral or bacterial): meningitis, sinusitis, pharyngitis, upper respiratory infection; fever: Pain may be nonspecific Dental disease: Uncommon Malfunctioning shunt or hydrocephalus: History of ventriculoperitoneal, ventriculopleural, or ventriculoatrial shunt Toxins—Carbon monoxide, lead: Dull, aching pain Tumor, brain abscess, subarachnoid or intracranial hemorrhage: Progressive worsening; can be severe; worst in early morning and with lying down (brain tumors) Exertional: Sharp and occurs after exercise Posttraumatic head injury: Pain can be severe when associated with epidural hematoma; if not associated with epidural hematoma, can start within hours up to weeks following injury

Reye Syndrome a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. an encephalopathy process often associated with influenza A and B. b.systemic disorder of mitochondrial function occurring during or after a viral infection, more often with use of salicylates. Infrequent cases seen with varicella or nonspecific respiratory infections, notably H. influenzae type B. c. clinical course proceeds in predictable stages after the initial prodromal symptoms of the illness: severe vomiting progresses to irrational behavior; to stupor and coma; to apnea, fixed pupils, and decorticate posturing with increasing brain edema; and then to death. d. immediate referral with admission to a hospital for supportive care. About 70% of patients survive, some with severe neurologic sequelae. Infants are more severely affected than older children.

Static Encephalopathies a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. chronic nonprogressive brain disorders in children, primarily cerebral palsy and mental retardation an altered mental state or brain damage that is permanent, unchanging Hypoxic-ischemic insult in the newborn is divided into three stages of injury (stages I, II, and III, or mild, moderate, and severe) b. Brain damage results from fetal hypoxia or ischemia over an extended period. The initial hypoxic or ischemic insult is followed by metabolic and respiratory acidosis. Compensatory mechanisms, such as shunting blood through the ductus to maintain perfusion of the brain, heart, adrenals, kidneys, liver, and intestines, ultimately fail if the insult is severe enough. 15% to 20% of infants with hypoxic-ischemic encephalopathy die in the neonatal period, and up to 30% develop permanent neurodevelopmental disabilities. Causes of the initial hypoxic or ischemic insult include abruptio placentae, hemorrhage, cord compression, mechanical injury, severe maternal hypertension or diabetes, and inadequate resuscitation of the infant c. Stage 1: hyper alert, normal muscle tone, normal posture, hyperactive tendon reflexes/clonus, myoclonus, stong moro reflex, mydriasis, <24 hours if progresses, otherwise may remain normal, good outcome Stage 2: lethargic, hypotonic muscle tone, flexion posture, hyperactive tendon reflexes/clonus, myoclonus, weak moro reflex, miosis, seizures common, EEG low voltage changing to seizure activity, duration is 24h - 24 days, outcome variable Stage 3: stuporous, coma, flaccid muscle tone, decerebrate posture, tendon reflexes/clonus absent, myoclonus absent, moro reflex absent, unequal pupils, poor light reflex, decerebration seizures, EEG burst supression to isoelectric, duration days to weeks, outcome death or severe deficits Physical Examination • Seizure activity • Pallor • Cyanosis, apnea • Bradycardia and unresponsiveness to stimulation d. difficult or impossible to treat prevent further damage and implement rehabilitation to allow the individual to perform at his or her highest possible functional level. The prognosis depends on the effectiveness of managing the underlying symptoms. Severe complications (hypoxia, hypoglycemia, shock) and encephalopathy characterized by flaccid coma, apnea, and seizures are associated with a poor prognosis. An infant who remains neurologically abnormal after the initial recovery phase (2 weeks) likely has suffered permanent neurologic impairment. A low Apgar score at 20 minutes, absence of spontaneous respirations, and persistence of abnormal neurologic signs at 2 weeks old predict death or severe cognitive and motor deficits; Apgar scores done at 1 and 5 minutes are far less predictive of outcome.

Meningocele a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. may protrude through the skull and may or may not be covered with skin. cranial - consists only of a CSF filled meningeal sac; no nerve roots are involved and therefore no neurologic deficits exist. however, there may be brain malformation under the mass that does have neurologic consequences. herniation of the meninges b. Sacral cysts are thought to arise when the accessory neurenteric canal (ANC) partially resorbs both ventrally and dorsally, leaving only a cyst-like structure, with no connection to the intestines or skin. This cyst-like structure is incorporated in the mesenchyma destined to form the sacrum, forming a sacral cyst. The spinal meninges with or without the terminal spinal cord can then herniate into this defect, forming a sacral meningocele. Sacral meningeal cysts usually communicate freely with the subarachnoid space and are known as perineural or Tarlov cysts. Most of these cysts are asymptomatic; cysts that do not communicate with the subarachnoid space are more likely to cause symptoms. c. Tethered cord syndrome: •Neurologic abnormalities in the legs (ie, motor weakness, sensory loss, reflex changes, abnormal plantar responses) •Urologic symptoms (ie, urinary incontinence/retention, urinary tract infections) •Orthopedic problems (ie, foot deformities, scoliosis, leg length discrepancy, kyphosis) ●dimples, hypertrichosis, nevi, hyper/hypopigmentation, and hemangiomas ●subcutaneous back mass Cutaneous — dermal sinus tracts, dimples or pits , hypertrichosis, hyperkeratosis, areas of hyperpigmentation or hypopigmentation, hemangiomas, capillary malformations (port wine stains), subcutaneous lipomas, caudal appendages (true tail or pseudotail), and isolated deviation of the intergluteal cleft. Neurologic — autonomic and sphincteric dysfunction being more common and occurring earlier than sensorimotor deficits in the legs Less commonly, patients can present with meningitis due to a ruptured dermal sinus or cyst Autonomic symptoms: urinary retention or incontinence, dysuria or recurrent urinary tract infections, or bowel obstruction in infancy or intractable constipation in childhood. Sensorimotor symptoms: leg weakness, decreased or increased muscle tone, or sensory deficits of the legs and perineal area. In some cases, sensory loss can lead to atrophic ulceration of the skin. Tethered cord syndrome — stretch-induced dysfunction of the caudal spinal cord and conus. back pain, bladder dysfunction, leg weakness, calf muscle atrophy, diminished or absent deep tendon reflexes, and dermatomal sensory loss. Orthopedic signs include progressive scoliosis and various foot deformities. progressive motor and sensory dysfunction, gait abnormalities and loss of bladder control pain in the lumbosacral region, perineum, and legs. Urologic — neurogenic bladder dysfunction or urogenital malformations. Urinary tract problems, Incontinence, Recurrent urinary tract infections, Abnormal renal tract investigations Other malformations — anorectal malformations (eg, imperforate anus) or urogenital malformations Pain — lower back, sacrococcygeal or gluteal areas, with or without a radicular pattern Pain may be exacerbated with Valsalva maneuver in the cases of lesions that are in communication with the subarachnoid space, such as Tarlov cysts EVALUATION AND DIAGNOSIS — * confirmed by radiologic demonstration* of a spinal dysraphic lesion. *evaluation with MRI of the entire spine for infants and children* who have two or more cutaneous lumbosacral spine lesions, a subcutaneous back mass, or neurologic symptoms suggestive of tethered cord syndrome. MRI of the spine for neurologically asymptomatic infants and children who have an isolated midline cutaneous lumbosacral spine lesion that is potentially high-risk for the development of CSD. These cutaneous lesions include atypical dimples (those >5 mm in size or located >2.5 cm from the anus), hemangiomas, cutis aplasia, and upraised lesions (ie, masses, tails, and hairy patches). *Plain radiographs are useful for detecting vertebral defects. CT is also useful when the evaluation for bony abnormalities is important*, especially thin-section multi-planar CT with reformatted images. *prenatal ultrasonography of the spine* can identify spinal anomalies if they are large enough, but this modality is not always accurate. Urodynamics — *Urodynamic testing* can detect preclinical urologic dysfunction in children with CSD. often used for preoperative evaluation of children who might benefit from neurosurgery for tethered cord release. *Ultrasound of abdomen and pelvis* — can assess the urinary system for pathologic changes such as a thickened bladder wall (suggesting detrusor hypertrophy), incomplete bladder emptying, upper tract dilatation, small kidney size, and renal scars TREATMENT — *Surgery is considered the mainstay* of treatment Patients with impairments due to neurologic deficits, bladder dysfunction, or chronic pain may benefit from *physical and occupational therapy* . *Surveillance and monitoring is needed to detect new or progressive deficits.* neurosurgical intervention include cases where the spinal cord is internally exposed, such as with intrasacral meningocele, to decrease the risk of infection and meningitis and patients who need vertebral stabilization or pain relief. In contrast, severely disabled patients with static deficits related to CSD are unlikely to benefit from surgery Conservative management with watchful monitoring is also an acceptable approach in patients who are asymptomatic or mildly symptomatic, given the highly variable natural history of CSD. ●Spinal neurosurgery aimed at altering the natural course of the disease, preventing further neurologic deterioration, and relieving, as much as possible, presurgical neurologic deficits. ●Surgeries to correct various comorbid conditions associated with CSD, such as urinary retention, incontinence, impotence, constipation, anorectal malformations, tumors, and dorsal cutaneous lesions. These interventions involve the fields of urologic, gastroenterologic, colorectal, oncologic, plastic, and general surgery. Monitoring — Asymptomatic patients with CSD who do not have surgery still require close monitoring to watch for the onset of neurologic, genitourinary or gastrointestinal symptoms, especially with respect to incontinence or constipation. Patients who have surgery for CSD should remain under close monitoring because of the risk of future worsening, which can occur with spinal cord retethering or progression of a preexisting syrinx. Urodynamics are generally considered to be a good monitoring tool for both nonoperative patients and postoperative patients, and particularly for early detection of cord retethering.

Anencephaly and Encephalocele a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. most common neural tube defects include anencephaly, which affects brain and skull development and is incompatible with life Hypothalamic or pituitary disorder Anencephaly: total or partial absence of brain and calvarium. Many fetuses are spontaneously aborted; few survive infancy. open defect in the calvaria and skin, such that the cranial neural tube is exposed. It is a severe defect and is not compatible with survival. Infants that are alive at birth generally die within hours, but occasionally survive for a few days or weeks. Encephalocele: defect in calvarium with protrusion of brain, most often in occipital region. Occipital encephaloceles are associated with intellectual disability, hydrocephalus, spasticity, and seizures. Patients with frontal encephaloceles have better developmental outcomes. cranial lesions that contain a meningocele sac plus cerebral cortex, cerebellum, or portions of brainstem that protrude from fissures in the occipital (most common), frontal, or nasal cavity areas of the skull b. The malformation that causes neural tube defects occurs during embryogenesis, typically no later than 26 days after fertilization. Two different processes lead to formation of the CNS. The first is primary neurulation, which refers to the invagination of the neural plate into the neural tube, and subsequently the embryonic brain and spinal cord. Secondary neurulation refers to the formation of the lower spinal cord, which gives rise to the lumbar and sacral elements. Any disruption that occurs when the neural plate begins its first fold and fuses to form the neural tube (days 17-23) can cause craniorachischisis, the most severe form of neural tube defect. *Closure of the rostral neuropore occurs between days 23 and 26. Disruption during this phase of embryogenesis results in anencephaly.* Myelomeningocele results when the closure of the caudal neuropore is disrupted during days 26 to 30. The CNS appears as a plate of thickened ectoderm called the neural plate at the beginning of the third week of embryonic life. The lateral edges of the neural plate become elevated to form the neural folds and fuse to form the neural tube; the fusion begins in the cervical region and proceeds in both the rostral and caudal directions. The rostral neuropore closes on the 25th day after conception, and the caudal neuropore closes two days later. NTDs result from failure of the neural tube closure between 25 and 27 days after conception. Anencephaly is a severe defect of development of the neuraxis, in which the developing forebrain and variable amounts of the brainstem are exposed in utero and fail to develop or are destroyed. results from failure of the rostral neuropore to close around postovulatory day 25 The craniofacial abnormalities in anencephaly are caused by abnormal neural induction from prosencephalic and mesencephalic neural crest tissue. This mechanism is also seen in other anomalies of craniofacial development such as holoprosencephaly. Craniorachischisis totalis is a related NTD, in which anencephaly is accompanied by a contiguous open defect of the spine (spina bifida totalis). It is an extreme example of defective closure of the neural tube during early embryogenesis, around 20 to 22 days gestation An encephalocele is a type of malformation in which there are calvarial and dural defects with extracranial herniation of leptomeninges and sometimes of brain and CSF. The mechanism causing primary (congenital) encephalocele is uncertain, although it involves defective closure of the anterior neural tube. Onset of the most severe lesions likely occurs prior to 26 days after conception; less severe lesions that primarily involve skull or meninges may occur later. Nasofrontal lesions are thought to result from defective separation of neural and surface ectoderm at the site of final closure of the rostral neuropore. Anterior encephaloceles (sincipital and basal) probably result from defective development of the prosencephalic neural crest tissue. This mechanism is suggested by the patency of the midline foramina which are open only transiently during normal craniofacial development. By contrast, the embryology of occipital encephaloceles suggests defective segmentation of the bones of the posterior cranium (supraoccipital, exoccipital, and basioccipital, and the posterior part of the parietal bones). These bones are not of neural crest origin c. maternal risk factors for child with spina bifida, hx of elevated triple or quadruple screening test during prenatal assessment, hx of abnormality on prenatal ultrasound, open spina bifida lesion: myelomeningocele, myeloschisis, meningocelem closed spina bifida lesion: asymmetric gluteal fold or dimple, hemangioma, hairy patch, or other cutaneous markings, bulging fontanelle, rapid head growth, abnormal urinary voiding, leakage of meconium or stool, absence of anal wink/rectal tone, downward deviation of the eyes (sundowning), upward and lateral deviation of eyes abnormal cry, breathing abnormalities: apnea, inspiratory stridor, snoring, facial asymmetry, asymmetry of spontaneous arm and leg movement difficulty with diapering or dressing, abnormal muscle tone and bulk in arms and legs, decreased sensation, hip subluxation or dislocation, clubfoot (equinovarus deformity), vertical talus deformity, hip and knee flexion contractures anencephaly: forebrain and variable amounts of upper brainstem are involved. Exposure in utero results in destruction of neural tissue, which appears as a hemorrhagic, fibrotic, nonfunctioning mass. Major portions of the CNS are absent or malformed. The hypothalamus is typically missing. The cerebellum, brainstem, optic nerves, and spinal cord may be malformed. Underdevelopment or absence of the pituitary leading to adrenal hypoplasia is always present. Large portions of the cranium are absent in this disorder. The frontal, parietal, and portions of the occipital bones are most often affected. The absent cranial vault results in the *characteristic appearance of bulging eyes and absent neck*. The defect in the skull sometimes extends through the level of the foramen magnum and involves the cervical spine. This abnormality is known as holoacrania. Associated malformations — cleft lip and/or palate or omphalocele. Craniofacial and ocular anomalies often occur. Cardiac, pulmonary, renal, and skeletal malformations may be associated. Aganglionosis of the intestine is a frequent finding Neurologic function — typically have brainstem function, with spontaneous breathing and often with suck, root, and gag responses. they are permanently unconscious. Without intensive care, the majority of them die within two days of birth, and none survive longer than two weeks Sincipital (fronto-ethmoidal) encephaloceles may be occult lesions that are not noticeable or may present with marked craniofacial deformities (hypertelorism, telecanthus, orbital dystopia, or unilateral micro/anophthalmos). ●Basal encephaloceles may or may not be apparent on external inspection, but there may be a broadened nasal bridge, hypertelorism, or other midfacial anomalies. Affected patients may present as a nasal or epipharyngeal mass, difficulty breathing, recurrent upper tract infections, nasal discharges, recurrent meningitis, or CSF leaks. ●Occipital encephaloceles usually are obvious at the time of birth and many are diagnosed prenatally by ultrasonography. The neural tissue usually is covered by skin. Those of relatively large size may be associated with cranial nerve deficits, poor sucking and feeding, spasticity, blindness, seizures, or developmental delay. Neurologic deficits may progress after birth if hydrocephalus develops. Occipital encephalocele also may be associated with hind-brain anomaly (Chiari III malformation) in which herniating occipital/cerebellar tissues distort the posterior fossa structures The protruding tissue of an encephalocele may consist of normal brain or fibrous atrophic gliotic tissue which has little or no function. In a nasofrontal encephalocele, most of the herniating mass consists of nonfunctional gliotic neural tissue occipital encephaloceles may include a variety of tissues. d. evaluation: prenatal triple/quadruple test elevated, prenatal ultrasound may show flattened or inwardly scalloped frontal bones (lemon sign); obliteration of cisterna magna and loss of normal roundness of cerebellar hemispheres may be seen (banana sign), fetal MRI identifies topography, contents of sacs, CNS and non-CNS findings cranial ultrasound: cortical mantle thickness, ventricle size spinal ultrasound: may show tethering, diastomyelia, hydromyelia, or syringomyelia CT head: evaluates ventricular size and tonsillar ectopia; confirms ventriculoperitoneal catheter placement urine culture: positive or negative serum BUN and creatinine: normal or elevated renal ultrasound: evaluates kidney size and configuration; estimates bladder capacity; determines presence of hydronephrosis urodynamic study: determines detrusor leak-point pressure, presence of detrusor sphincter dyssynergia, as well as bladder capacity and compliance voiding cystourethrogram (VCUG): may show vesicoureteral reflux other tests: prenatal amniocentesis or postnatal chromosomal analysis: may show trisomy 13 or trisomy 18 fluorescence in situ hybridization (FISH) testing may show 22q deletion syndrome MRI brain and spine: agenesis of corpus callosum, cerebellar tonsil ectopia, brainstem compression, ventriculomegaly, syrinx hip ultrasound: subluxation at hip in infants ≤3 months of age hip x-ray: abnormal relationship between femoral head and acetabulum polysomnography: may be abnormal The diagnosis is apparent at birth in most of the occipital encephaloceles. Basal encephaloceles may present as a midline mass in the nose or may not be visible. An encephalocele may be mistaken for a nasal polyp if it is located within the nose or for a soft tissue tumor if it is covered with skin and anterior to the nose. These basal encephaloceles, either ethmoidal or sphenoidal, tend to present with meningitis. Neuroimaging should be performed to evaluate the intracranial components of the malformation and identify any associated brain or vascular anomalies. *Computerized tomography (CT)* scans are effective for detection of the extent of cranial defects. *Magnetic resonance imaging (MRI)* is effective for detection of the extent of neural herniation. It is especially useful for basal encephaloceles. Neuroimaging will also detect hydrocephalus, if present. Prenatal diagnosis — *maternal screening of serum AFP levels and ultrasonography* - encephaloceles appear as a defect in the calvarium containing a cystic or solid mass with a gyral pattern that is contiguous with the brain. detects approximately 80% of encephaloceles. Fetal MRI has higher sensitivity for NTDs, and can be used in cases where ultrasound results are not optimal or the diagnosis is uncertain, or to evaluate high-risk cases. Management: prenatal diagnosis: parental genetic counseling + multidisciplinary approach specialized obstetric care + planned birth Acute neonate or infant: surgical repair of cele + intravenous antibiotics monitoring of head circumference and supportive care Chiari decompression surgery and/or tracheostomy and/or Nissen/gastrostomy tube placement with severe hydrocephalus: shunt placement with high-risk neurogenic bladder: intermittent catheterization and ongoing urologic surveillance prophylactic antibiotic therapy, anticholinergic with bacteriuria: antibiotic therapy with orthopedic deformities: physical therapy ± orthopedic surgery, ankle-foot orthoses (AFOs) 75% of anencephalic infants are stillborn. Liveborn infants generally die within hours but occasionally survive for a few days or weeks. There are no neurosurgical management options. In most developed countries where abortion is legal these pregnancies are interrupted earlier. PREVENTION — Several lines of evidence suggest a link between folic acid deficiency and the development of NTDs Risk factors for NTDs include dietary deficiency of folic acid, administration of valproate or folic acid antagonists such as trimethoprim, carbamazepine, phenytoin, and phenobarbital, and genetic polymorphisms in genes encoding folate-dependent enzymes. randomized trials have consistently shown that folic acid supplementation reduces the incidence of NTDs. Other contributors to NTD risk include maternal diabetes mellitus with poor glycemic control during the first trimester, hyperthermia, and some genetic syndromes. *Periconceptional folic acid supplementation is recommended for all women who are pregnant or who may become pregnant.* Higher doses of folic acid supplements are usually recommended for women who are taking anticonvulsant drugs or who have had a previous pregnancy affected by a NTD. mgmt of encephaloceles: When diagnosed prenatally, vaginal delivery may be safe if the lesion is relatively small. Large encephaloceles require cesarean section. *Surgical treatment* is appropriate in most cases unless the encephalocele is massive and there is severe microcephaly or other lethal anomalies. The procedure basically consists of removing the overlying sac and closing the defect including the dural defect. In patients with basal encephaloceles or CSF leakage, prompt closure is important to reduce the risk of infection. Patients with hydrocephalus usually undergo ventriculo-peritoneal shunt placement prior to encephalocele repair to prevent postoperative CSF leaks. Sincipital encephaloceles — Unless there is CSF leakage, sincipital encephaloceles can be treated electively, ideally early in infancy to avoid deleterious effect of an enlarging encephalocele on craniofacial structures. Complete surgical repair includes: ●Resection of the herniated mass to be flush with the floor of the anterior cranial base ●Repair of the dural and cranium defect ●Correction of hypertelorism, when appropriate, with: •Reconstruction of the nasal elements •Alignment of the horizontal ocular axis •Cannulation of obstructed nasolacrimal ducts Basal encephaloceles — Basal encephaloceles require prompt surgical repair because of high prevalence of meningitis. Ideally, a craniofacial team usually consisting of neurosurgeons and plastic surgeons perform the repair. Reconstruction of the skull and dural defect is important to prevent meningitis. In the case of sphenoidal encephalocele, particular caution must be used during the repair because the third ventricle, hypothalamus, pituitary gland, and optic pathway may be present in the encephalocele sac. Occipital encephaloceles — Surgical management of patients with occipital encephalocele depends on the type of neural tissue that is protruding beyond the skull, as assessed by inspection during surgery. If the herniating mass consists of gliotic tissue, the mass is transected flush with the skull. If the mass contains normal brain, attempts should be made to preserve it. Several techniques have been described: ●Expansion cranioplasties have been used to accommodate large amounts of herniated neural tissues. One technique of expansion cranioplasty uses tantalum mesh to create an extracranial space for herniating tissues. ●Ventricular volume reduction is a two-stage technique in which the ventricles are artificially expanded, followed by intracranial transposition of the neural tissue. In the first stage, the dural sac is closed; this increases ventricular pressure to produce ventriculomegaly. After hydrocephalus develops, a ventriculoperitoneal shunt is placed, and then herniated brain is transpositioned into the new intracranial space as the ventricle contracts ●To preserve herniated occipital and cerebellar parenchyma, the tentorium is incised, creating infratentorial space, and the cerebral cortex retracted into this space

Retinoblastoma a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. one type of intraocular tumor. a rare malignant tumor of the retina, most common tumor in childhood. highest in infants and young children and extremely low in children 6 years and older A single or multiple tumors may be found in one or both eyes. Bilateral disease and multifocal tumors are usually found in hereditary forms, nonhereditary forms are unilateral and unifocal (30% to 40% of cases). Unilateral disease occurs 60% to 70% of the time, is due to genetic mutation, and is commonly recognized by 25 months of age. The median age of diagnosis in children with bilateral disease is 12 months and 24 months in children with unilateral disease b. Abnormal fetal neural crest cell maturation Hereditary—germinal mutation (40%); siblings and subsequent offspring at risk Acquired—somatic mutations from epocrates: inherited in the germline or occurring spontaneously, a critical mutation occurs in the RB1 gene of horizontal interneurons in the inner nuclear layer of the retina. This loss of heterozygosity results in a cell that is homozygous null for this gene. The presence of other RB family proteins and unrelated proteins (such as senescence proteins) can inhibit the progression of retinoblastoma. For reasons that are not clear, but likely related to the local retinal microenvironment, a high percentage of these lesions develop aneuploidy and high copy number changes, resulting in genomic instability and a predictable series of losses of proteins critical for cell cycle control. Oncogenesis results and clinical retinoblastoma can be detected. c.• Positive family history Strabismus - the most common finding. • Unilateral or bilateral white pupil (leukokoria), an intermittent "glow, glint, gleam, or glare", usually in low-light settings • Decreased visual acuity • Possible orbital cellulitis and photophobia (causes pain), hyphema, abnormal red reflex, nystagmus, glaucoma, hypopyon (pus in anterior chamber of eye), or signs of global rupture d. CT scan with contrast and/or echography and/or MRI. Other tests: fundus photography, fluorescein angiography, ocular ultrasonography, or fine-needle aspiration. Refer patient to ophthalmologist criteria of tumors based on their size, location, number, and degree of invasiveness or seeding. treatment may involve external beam radiation, cryotherapy, laser photocoagulation, episcleral plaque brachytherapy, or systemic chemotherapy. Early detection and advances in treatment -> less enucleation (removal of eye) and less use of external beam radiation, preserving sight. advanced tumors requiring enucleation, the hydroxyapatite implant provides excellent cosmetic appearance and acceptable motility of the implant Siblings and parents should receive a referral for fundi examinations. Frequent follow-up (every 3 months until 6 or 7 yo) to assess treatment and monitor for recurrence Up to 45% of children treated with eye-preserving therapy will develop new or recurrent ocular tumors that require further treatment

Spina Bifida Occulta a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

a. when the vertebral arches fail to close but there is no subsequent herniation of the cord or meninges common radiographic finding characterized by simple lack of fusion of vertebral spinous processes. The spinal cord itself is normal. Closed spinal dysraphism (also known as occult spinal dysraphism or spina bifida occulta) is characterized by failure of fusion of the vertebral bodies due to abnormal fusion of the posterior vertebral arches, with unexposed neural tissue; the skin overlying the defect is intact. The more common and least severe forms consist of isolated vertebral bony defects. However, the vertebral defects may occur in association with other more severe anomalies of the spinal cord and sacral structures, such as split spinal cord malformation or various cavitary defects of the spinal cord. b. not well established. anomaly to disruption in a specific stage of nervous system development may have a common genetic or environmental trigger. the normal neural arch ossification is not complete until the age of two to three years. Therefore, an incompletely ossified neural arch in a child younger than three years of age should not be mistaken for spina bifida occulta c. dimples, hemangiomas, tufts of hair, or other lesions along the spine may be associated with spinal abnormalities skin covered with no visible abnormalities of the back) Vertebral fusion defect only, without spine involvement common and almost always without consequence, some developmental abnormalities may occur—such as a spinal cord lipoma or a fibrous cord—that can cause subtle or rare neurologic signs. A fibrous cord may extend from an interdural component of one of these developmental abnormalities to the skin, producing a dimple, an area of pigmentation, or a hairy patch at the base of the spine Patients with a fibrous cord may have problems with micturition, or they may have subtle neurologic signs, such as a foot deformity (most commonly, a cavus foot). When a lipoma is present, there may be a lipomeningocele, a lipomyelomeningocele, or a lipomyelocele. These may be associated with areas of fluid in the cord, which may be a syringomyelia. cutaneous lesions are often associated, with the bifid vertebra lying at the level of entry of the tract into the dural space see meningocele card for more info about closed spinal defects (CSD)

5. What signs and symptoms would you expect to find with an acute headache and a chronic headache?

acute: transitory neuro changes: nausea, vomiting, malaise, personality changes, photophobia, phonophobia appears sick, incapacitates period "ice pick" pain on top of head resolves after sleeping lasts 1-3 hours but up to 72 hours in some cases chronic: throbbing pain, anxiety, malaise if does take analgesia occurs at least 15 days/month for 3 months low intensity, dull ache, may interfere with activities and school attendance occurs at variable times of day tension acute: no n/v, photophobia, phonophobia or neuro changes, negative neuro exam chronic: may have nausea, lasts 30 minutes, all day or for up to 7 days migraine with aura: individual experiences >2 attacks Individual has at least three of the following symptoms: • ≥ one reversible aura symptom* • At least one aura develops slowly over >5 minutes, or two or more aura symptoms occur in succession • Aura does not last >1 hour • Headache begins during or follows the aura in <1 hour • Headache cannot be attributed to another disorder migraine without aura: Individual experiences ≥ five attacks Headache episode lasts 1 to 72 hours Headache has two or more of the following characteristics: • Located bilaterally or unilaterally frontotemporal region (not occipital) • Pulsation present • Intensity is moderate to severe • Is aggravated by routine physical activity Headache has one or both of the following and is not attributable to another disorder: • Nausea and/or vomiting • Photophobia and/or phonophobia Signs and Symptoms Suggestive of Intracranial Structural Pathology Infants: Full anterior fontanelle Open metopic and coronal sutures Poor growth Impaired upward gaze Abnormal head growth Shrill cry Lethargy Vomiting Children Persistent unilateral headache Papilledema Abnormal eye movements (or one or both eyes suddenly turn in) Ataxia Hemiparesis Abnormal deep tendon reflexes Severe, excruciating headache of recent onset, unlike any previously experienced; no normal period of functioning between episodes of headache Cranial bruits Personality changes

10. Describe subarachnoid hemorrhage. Explain the pathophysiology. Define Kernig and Brudzinski sign. List the clinical manifestations.

from epocrates definition: bleeding into the subarachnoid space *emergency*. most common cause of nontraumatic SAH is intracranial aneurysm. When a cerebral aneurysm ruptures, blood flows into the subarachnoid space, sometimes seeping into brain parenchyma and/or ventricles. The sudden increase in intracranial pressure, as well as the destructive and toxic effects of blood on brain parenchyma and cerebral vessels, accounts for most complications. patho: Cerebral aneurysms arise at the bifurcation of major arteries that form the circle of Willis. The majority are located at the anterior communicating/anterior cerebral artery junction, distal internal carotid artery/posterior communicating artery junction, and middle cerebral artery bifurcation. Less than 10% arise from the vertebral or basilar arteries. Up to 19% of patients are found to have multiple aneurysms. Greater pressures at the apexes of arterial bifurcation, pulsatile flow patterns, and turbulence The risk of rupture depends on its size, location, the presence of symptoms, the presence of multiple aneurysms, and whether previous aneurysms have ruptured. Patient-related predictors of rupture are age and smoking. Small, asymptomatic aneurysms (<7 mm) are less prone to rupture than bigger ones that exert mass effect on surrounding structures. Aneurysms located at the basilar tip, in the vertebrobasilar, posterior cerebral distribution, or posterior part of the circle of Willis are more likely to rupture compared with aneurysms in other locations. An unruptured aneurysm discovered during workup for SAH (caused by a different aneurysm) has a higher annual incidence of rupture than a single unruptured aneurysm. kernig sign: positive if resistance and head or neck pain are elicited when the patient bends over from the waist and touches fingers to toes. In an infant, the Kernig sign can be tested by extending the leg at the knee with the infant lying supine. A positive sign can be as subtle as facial grimacing. Brudzinski sign: positive is evidenced by the patient spontaneously flexing the hip and knees after the examiner passively flexes the neck. clinical manifestations: *sudden, severe headache* is characteristic. It is the most important clue to diagnosis and is often described as *"the worst ever headache".* photophobia, LOC

9. Define arteriovenous malformation. Describe the pathophysiologic basis of this disorder. List the clinical manifestations.

from epocrates: definition: congenital vascular lesions consisting of direct connections between cerebral arteries and veins. two most common presentations of AVMs are intracerebral hemorrhage (50%-70% of cases) and seizures (approximately 20%). patho: shunting of arterial blood into veins is the development of "arterialized" veins with proliferation of smooth muscle and elastin in the vessel wall. a mass of abnormal vascular channels with widely varying caliber, from hypertrophied to thin-walled sinusoidal vessels with varying degrees of arterialization. Hemorrhage may be related to the feeding arterial pressure and venous pressure that presumably exceeds the tolerable transmural pressure of the AVM channels when they rupture. present a parallel, high-pressure vascular circuit that causes local arterial hypotension and venous hypertension, challenging the local cerebrovascular physiology and autoregulation. When local autoregulation is preserved, the lower limit of the autoregulation curve is displaced to the left in an attempt to maintain normal cerebral blood flow with arterial hypotension (adaptive autoregulatory displacement). The consequent ischemia/hypoxia in the environment surrounding the AVM may cause neurologic deficits, seizure activity, or cognitive impairment. clinical manifestations: *sudden onset focal neurologic deficit, seizures*, reduced conscious level, sudden onset headache, nausea, vomiting, confusion, gradual onset headaches, hypertension, coma

Brain Tumors a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

from review book a. A group (mass) of expanding intracranial abnormal cells; the most common tumors in childhood b. Etiology is unknown; may be genetic predisposition, congenital factors, environmental exposures The primary tumors seen in children Astrocytomas - Usually slow growing, Often develop between 5 and 8 years Medulloblastoma - The most common brain cancer in children, Usually develop around age 5 Occur more often in boys Brain stem gliomas - Occur almost exclusively in children, Often grow very large before symptoms develop, Average age of development is 5 years, Five-year survival rate is low Ependymomas: Are located in the ventricles, Block the flow of CSF In children between 4 and 11 years, infratentorial (posterior fossa; posterior third of the brain) tumors predominate (60%), including cerebellar and brain stem tumors; the others are supratentorial in the anterior two thirds of the brain c. In infants with open sutures: Increased head circumference, Irritability, Head tilt, Loss of developmental milestones, Bulging fontanelles, No red reflex in the eye Older children: Headache; symptoms usually increase in frequency, becoming more severe in the morning followed by vomiting 85% of children with malignant tumors have abnormal neurologic or ocular examinations within 2 to 4 months of onset of headaches Certain specific neurologic symptoms may occur later and suggest localization of the CNS tumor: Ataxia, hemiparesis, cranial nerve palsies Somnolence Seizures Head tilt Diencephalic syndrome—failure to thrive (FTT), emaciation Diabetes insipidus Physical Findings: Directly related to anatomic location, size, and to some extent the age of the child. May include: Tense bulging fontanel at rest (in infant) Cranial enlargement (present in infants and young children) Papilledema once sutures are fused (edema of optic nerve) Nuchal rigidity Poor coordination or clumsiness Poor fine motor control Hypoflexia or hyperflexia Positive Babinski sign Spasticity/paralysis Behavioral changes—may be earliest symptom in child/adolescent d. Diagnostic Tests/Findings MRI—superior, sensitive neuroimaging technology CT scan: Without contrast, can detect: Whether lesion is cystic or solid Presence of calcifications, hemorrhage, edema, and hydrocephalus With contrast can detect: Small tumors Differentiation of areas of edema Angiography—determines blood supply to affected structures CT—may include guided biopsy Lumbar puncture contraindicated in presence of increased ICP Management/Treatment: Therapy selected depends on type and site of tumor 1. Surgery (usually treatment of choice, although some tumors cannot be removed entirely and can only be debulked) 2. Radiation therapy 3. Chemotherapy 4. Follow-up EEG for seizures VER (visual evoked response) for visual problems BAER (brainstem auditory evoked response) for hearing problems Multidisciplinary approach for comprehensive health and developmental needs of child/family

Describe each of the following brain and spinal cord tumors: a. primary intracerebral tumor (glioma) b. astrocytoma c. oligodendroglioma d. ependymoma e. meningioma f. neurilemmoma g. intramedullary tumor h. extramedullary tumor

from up to date a. great majority of primary tumors that arise within the brain parenchyma. tumors that have histologic features similar to normal glial cells (ie, astrocytes, oligodendrocytes, and ependymal cells). span a broad spectrum of biologic aggressiveness. the slower-growing lesions, corresponding to WHO grades I and II, referred to as low-grade gliomas, more rapidly progressive tumors are referred to as high-grade gliomas. many of which have significantly different biologic properties, prognoses, and treatment approaches. Brain stem gliomas (1) Occur almost exclusively in children (2) Often grow very large before symptoms develop (3) Average age of development is 5 years (4) Five-year survival rate is low tumors of neuroepithelial tissue. astrocytomas and ependymomas are two major tumor types in this category. account for almost 50 percent of all CNS tumors in children and adolescents. b. (1) Usually slow growing (2) Often develop between 5 and 8 years primary tumor can occur throughout the CNS and range from low-grade indolent lesions to highly malignant aggressive tumors. Low-grade are the largest group of CNS tumors in children. Pilocytic is the most common brain tumor in children. High-grade malignant are divided into anaplastic gliomas, diffuse intrinsic pontine glioma and glioblastoma. These highly malignant and aggressive tumors carry an extremely poor prognosis c. type of astrocytoma, anaplastic glioma all harbor IDH mutations rounded nuclei, often with perinuclear halos, calcification, and delicate, branching blood vessels exist on a spectrum ranging from well-differentiated, slow-growing tumors to malignant tumors with rapid growth patients may survive for many years after symptom onset. good prognosis less aggressive biological behavior and a favorable response to chemotherapy d. (1) Are located in the ventricles (2) Block the flow of CSF an uncommon group of glial tumors that typically arise within or adjacent to the ependymal lining of the ventricular system. occasionally occur within the brain parenchyma or outside the CNS. account for <10% of tumors arising in the CNS and 25% of primary tumors originating in the spinal cord. e. Tumors of the meninges arise from the arachnoidal cap cell in the arachnoid membrane and have varying degrees of malignancy. the most frequent primary brain tumors most are benign, their location in the CNS can cause serious morbidity or mortality. can occur in spine f. also called Schwannomas are encapsulated tumors made entirely of benign neoplastic Schwann cells. most common tumor of peripheral nerves. do not transform to malignancy grow from peripheral nerves or nerve roots in an eccentric fashion with the nerve itself usually incorporated into the capsule. In large schwannomas, nerve fibers can be difficult to find show a biphasic architecture of Antoni A (dense) and B (loose) patterns, as well as nuclear palisading (Verocay bodies) and a fibrous capsule containing the parent nerve. Neoplastic Schwann cells typically show spindle shaped nuclei g. subgroup of intradural spinal tumors that arise from cells within the spinal cord. much less common than brain tumors and are thought to account for only 2-4% of all intrinsic tumors of the CNS. most common initial symptom is generalized back pain, which is very difficult to distinguish clinically from back pain from musculoskeletal conditions. involve the spinal cord h. arise outside of the spinal cord. Nerve sheath tumors (NSTs) constitute about 25% of tumors arising in the intradural extramedullary space majority are confined to the intradural extramedullary space, some of these tumors extend into either the extradural compartment or the spinal cord. occasional spinal NSTs are confined to either the extradural or intramedullary spaces derived from the Schwann cells and perineurial cells of the peripheral nervous system. 65% of intradural nerve sheath tumors are schwannomas, and most of the remainder are neurofibromas. Malignant NSTs are rare, constituting about 5% of such tumors. Intradural, extramedullary NSTs may be either sporadic or associated with one of three inherited disorders: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis.

Embryonal Tumors a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

from up to date and medscape a. typically occur in infants and young children. most common tumors in this group are medulloblastomas; other more rare tumor types include embryonal tumor with multilayered rosettes (ETMR), atypical teratoid/rhabdoid tumors, and CNS embryonal tumor not otherwise specified (NOS), previously called CNS primitive neuroectodermal tumor (PNET). All CNS embryonal tumors are highly malignant and correspond to World Health Organization (WHO) grade IV. include a heterogeneous group of immature-appearing neoplasms that are highly cellular and mitotically active, thus having a superficial resemblance to the developing nervous system. [1] These highly malignant tumors most often arise in children and not only invade nervous tissue but also can disseminate in the cerebrospinal fluid (CSF) and subarachnoid space. b. commonly arise in the supratentorial compartment, often in the frontal or parietotemporal lobes. Approximately 30 percent of tumors arise in the cerebellum or brain stem. poorly differentiated (WHO grade IV), rapidly growing, neuroepithelial tumors that originate from the germinal matrix of the primitive neural tube. They have the potential to differentiate along multiple different cell lines. Tumors with pure neuronal differentiation are classified as CNS neuroblastomas, while those with mixed neuronal and ganglion cell differentiation are classified as CNS ganglioneuroblastomas. Some PNETS can be classified according to specialized tissue of origin (eg, retinoblastoma or pineoblastoma), but most of the remainder originate in the cerebral hemispheres or suprasellar region and were previously referred to as supratentorial PNETs. The cerebral hemispheres are the preferred site above the tentorium, with occasional cases arising in the pineal region or, rarely, as suprasellar lesions. The cerebellum is the most common location in the infratentorial compartment where the hemispheres are most commonly involved. Such tumors may also be found in the cerebellopontine angle, brainstem, and in the spinal cord. may involve multiple nervous system sites at presentation; dissemination along cerebrospinal fluid (CSF) pathways is identified in more than 20% of patients at the time of presentation. c. increased intracranial pressure or focal neurologic signs older: headache, nausea, vomiting, hemiplegia infants: lethargy, irritability, anorexia, and/or an enlarging head circumference. seizures or focal deficits referable to the tumor location. leptomeningeal dissemination, manifested by signs of cranial nerve palsies, encephalopathy, or spinal cord symptoms d. CT, MRI maximal surgical resection plus chemotherapy and/or radiation therapy

6. Describe the damage that occurs from each of the following mechanisms: a. primary injury b. secondary injury c. tertiary injury

from up to date, wikipedia a. direct trauma to the brain wiki: initial insult, results from displacement of the physical structures of the brain includes contusion, damage to blood vessels, and axonal shearing, in which the axons of neurons are stretched and torn. The blood brain barrier and meninges may be damaged in the primary injury, and neurons may die. Cells are killed in a nonspecific manner. Tissues have a deformation threshold: if they are deformed past this threshold they are injured. myelinated tissues may have different properties than other tissues. some tissues may experience more force and be more injured. The primary injury leads to the secondary injury. Intracerebral hemorrhage Subdural hemorrhage Subarachnoid hemorrhage Epidural hemorrhage Cerebral contusion Cerebral laceration Axonal stretch injury b. the result of a cascade of biochemical, cellular, and metabolic responses to direct injury which worsens in patients who develop hypoxia, hypotension, or both. occurs gradually and may involve an array of cellular processes not caused by mechanical damage initially following brain injury, cerebral blood flow is decreased in children. Hypoperfusion in conjunction with increased metabolic demand makes the brain more susceptible to hypoxemia and hypotension. Cerebral perfusion may be particularly dependent upon maintaining adequate blood pressure because cerebral autoregulation is often impaired following severe pediatric TBI. In addition, release of excitatory neurotransmitters, such as acetylcholine, glutamate, and aspartate causes neuronal damage. Following this initial phase, cerebral swelling develops that generally peaks 24 to 72 hours after the injury. The resulting intracranial hypertension can further compromise cerebral perfusion leading to more ischemia, swelling, herniation, and death. Impaired cerebral autoregulation after initial TBI may further reduce cerebral blood flow and result in cerebral ischemia. For example, impaired cerebral autoregulation during the first week after severe TBI is associated with a poor, six-month Glasgow Outcome Score. Diffuse cerebral swelling following severe TBI is more common among infants and children compared with adults. A diffuse pattern of brain injury may develop because the infant skull is more compliant and can tolerate considerable deformation before fracture occurs. brain atrophy that probably begins in young adulthood, permits more room in the adult skull for the brain to expand. enhanced diffusion of excitotoxic neurotransmitters, the inflammatory response of the developing brain, and changes in blood-brain-barrier permeability may also be involved. wiki: an indirect result of the injury. occurs in the hours and days following the primary injury and plays a large role in the brain damage and death that results from TBI. people killed by brain trauma do not die right away but rather days to weeks after the event. 40% of people with TBI deteriorate after hospitalization. can damage neurons that were unharmed in the primary injury. occurs after subarachnoid hemorrhage, stroke, and TBI and involves metabolic cascades. ischemia (insufficient blood flow); cerebral hypoxia (insufficient oxygen in the brain); hypotension (low blood pressure); cerebral edema (swelling of the brain); changes in the blood flow to the brain; and raised intracranial pressure (the pressure within the skull). If intracranial pressure gets too high, it can lead to deadly brain herniation, in which parts of the brain are squeezed past structures in the skull. hypercapnia (excessive carbon dioxide levels in the blood), acidosis (excessively acidic blood), meningitis, and brain abscess. alterations in the release of neurotransmitters (the chemicals used by brain cells to communicate) can cause. Imbalances lead to excitotoxicity, damage to brain cells that results from overactivation of biochemical receptors for excitatory neurotransmitters (those that increase the likelihood that a neuron will fire). damage to cells by free radicals, potentially leading to neurodegeneration. loss of cerebral autoregulation, the ability of the brain's blood vessels to regulate cerebral blood flow. breakdown of the blood-brain barrier, edema, ischemia and hypoxia. Cerebral edema Impaired metabolism Altered cerebral blood flow Free radical formation Excitotoxicity c. i don't know what a tertiary brain injury and couldn't find any info anywhere

Acrania and Craniosynostosis a. Define each term b. Describe the pathophysiology c. List the clinical manifestations d. Discuss, briefly, the evaluation and management.

from wikipedia a. Acrania is a rare congenital disorder, the flat bones in the cranial vault are either completely or partially absent. The cerebral hemispheres develop completely but abnormally. The condition is frequently associated with anencephaly. the fetus should have a perfectly normal facial bone, a normal cervical column but without the fetal skull and a volume of brain tissue equivalent to at least one third of the normal brain size. b. During the fourth week of human development the neuropore in a normally developing fetus closes. When this process is either interrupted or never initiated, acrania occurs. The desmocranium becomes a membranous coverage instead of forming the epidermis of the scalp. Whether from being blocked by amniotic bands or by just not initiating, the migration of mesenchyme under the ectoderm does not occur. Because this migration does not occur, the skull, and all involved muscles, are never formed. Without the presence of the neurocranium, the brain fails to separate into two separate lobes. The hindbrain proceeds to develop normally, allowing for the child to be carried to term, but the diencephalon and ocular lobe remain small and underdeveloped. d. diagnosed early in pregnancy through an ultrasound. appears during the beginning or end of the fourth week of the fetus's development. An absence of the skull is needed in order to make a diagnosis. A presence of brain tissue will confirm the diagnosis of acrania and differentiate it from other developmental problems such as anencephaly Prognosis is poor. 100% mortality rate for those with acrania. early detection is of extreme importance so that actions may be taken to help the mother and child. Families may choose either to terminate the pregnancy, or to carry the child to term. Acrania may cause a fetus to spontaneously abort before reaching term. craniosynostosis a. Skull malformations may be due to primary or secondary causes. Congenital (or "true" or "primary") involves early closure or absence of one or more cranial sutures. b. When more than one suture is involved, there is more likely to be an associated genetic disorder; can be found in more than 60 genetic syndromes (e.g., Crouzon, Apert, Carpenter, Chotzen, Pfeiffer), but all of these syndromes also have extracranial features Growth along the remaining open suture lines produces progressive skull deformity in one or more directions. The skull is flat over the closed suture(s). Increased intracranial pressure may result as the brain tries to grow within the confined space, but this does not always occur. The sagittal suture is most commonly fused (referred to as scaphocephaly or dolichocephaly) and accounts for 60% of all cases. Secondary synostosis results when outside forces put pressure on the growing cranium, causing the skull to become misshapen (referred to as deformational plagiocephaly). most commonly seen with premature infants (termed deformational scaphocephaly), after shunting an infant with hydrocephaly, in children who have microcephaly and aberrant positioning in utero, during birth, or perinatally because of torticollis or positioning traditions. The success of the Back to Sleep campaign has resulted in an increase of infants with secondary (or pressure-related) occipital flattening. Such occipital deformity is not accompanied by compensatory suture line growth that would be seen with a primary lambdoidal synostosis. This type tends to correct when the child attains vertical posture Primary—due to abnormalities of skull development; cause unknown in majority of cases but in approximately 10% to 20% of cases due to a genetic syndrome or familial pattern 2. Secondary—due to failure of brain growth (microcephaly); uncommon cause c. Monitor cranial symmetry for the first year. This is best done by looking down at the top of the head, noting the position of the ears and cheekbones. a deformational plagiocephaly will form a parallelogram characterized by unilateral occipital flattening and contralateral occipital bossing, ipsilateral ear displacement anteriorly, and associated parietal bossing and cheekbone prominence on the side of the occipital flattening. the deformity of lambdoidal craniosynostosis does not assume a parallelogram shape, may be present at birth, has less frontal asymmetry than positional plagiocephaly, the ear ipsilateral to the occipital flattening is posterior and displaced inferiorly to the contralateral ear; the deformity may become more severe over time. Symmetry of neck rotation should also be included in the examination to rule out torticollis. Infants with torticollis typically have some limitation of neck rotation away from the side of their occipital flattening. 1. Abnormal head shape 2. Head size often small but depends on which suture is closed 3. Typically no symptoms unless part of a genetic syndrome or multiple suture closures; rarely may develop signs/symptoms of increased intracranial pressure 4. FH of abnormal head shape and/or genetic syndrome • Physical Exam 1. Abnormal head shape 2. Palpable bony ridge along affected suture line 3. Anterior fontanel may be large or small depending on suture closing d. evaluation: A *CT scan* is standard for a skull shape deformity. Deformational plagiocephaly does not require imaging studies in most situations when the history and physical examination are diagnostic. *If the child has neurologic findings, an MRI is also indicated* 1. Plain skull x-rays can identify 2. Chromosomal analysis if indicated Management 1. Surgery to open fused suture 2. Performed primarily for cosmetic reasons, unless neurologic complications *refer* the child to an experienced pediatric neurosurgeon or craniofacial plastic surgeon. Treatment is often *surgical*, but in some cases *reassurance, repositioning, exercises* for any associated torticollis, and *clinical follow-up* are sufficient. If the condition is genetic, management needs to be planned according to the problems associated with the syndrome. Genetic counseling is important. PCPs can anticipate concern about deformational plagiocephaly by counseling parents at the newborn visit to: (1) lay infant down in the Back to Sleep position for sleep, alternating positions (i.e., left and right occiputs); (2) when awake and observed, place infants prone; (3) during feedings have parents avoid holding an infant in a manner that avoids putting pressure on the flattened part of the skull; and (4) have infants spend minimal time in car seats or other upright devices that maintain supine positioning. Improvement should occur over a 2- to 3-month period if interventions are instituted early. Throughout the first year, emphasize tummy time. Monitor head shape during all well-child visits. The majority of positional plagiocephalies are self-limited; sometimes physical therapy is indicated in recalcitrant cases (e.g., with torticollis). For positional plagiocephaly, *orthotic cranial molding helmet therapy* may be prescribed when repositioning and exercises are not successful. The helmet is individually engineered to allow growth where needed and restrict it where the head is prominent; it needs to be worn for 23 hours a day for 4 to 6 months, can lead to odor and skin breakdown, is costly, and may not be covered by health insurance.

2. What is the incidence of headaches in the pediatric population?

one of most common reasons parent seek medical care for their children common during childhood, increasing in frequency and incidence during adolescence vascular: 1.2-3.2% occurence in children 3-7 years old (greater incidence in males), 4-11% occurrence prepuberty, 8-23% post puberty (female:male = 3:2) cluster: uncommon in children before 20 years; male predominance chronic daily low grade: all ages, increased incidence in midteen females tension: all ages; commonly starts during adolescence; both sexes affected 40% of children experience a headache by the age of 7 years and 75% by 15 years of age migraine headaches are common in children and may go unrecognized by a health care provider Prevalence rates for migraine headaches are reported to be: age 3 (3% to 8%); age 5 (19.5%); age 7 (37% to 51%); and 7 to 15 years of age (57% to 82%). Before 10: incidence is higher in males than females. After 11: higher in females The mean age at onset of migraine is at age 7.2 years for males and 10.9 years for females Chronic and recurrent headaches are most common neurologic complaints; occur in 3% to 20% of children and adolescents Migraines—account for 50% of chronic or recurrent headaches; 60% of affected children are male Cluster headaches—least common in children

4. Identify the anatomic sites from which headaches can originate.

vascular: bilateral in young children changing to unilateral in adolescence cluster: unilateral chronic, daily, low grade: pain generally bilateral (bifrontal or bitemporal) tension: bilateral, diffuse (site may shift), aching pain (tight band around head), located in neck and back of head, bifrontal or occipital occipital or consistently localized: underlying pathology, neck pain - brain tumors originate in posterior fossa in children facial pain: sinusitis periorbital discomfort: ocular motor imbalance TMJ: localized around the periauricular or temporal areas

3. When taking a history for headache, what specific questions should the nurse practitioner ask?

vascular: positive family history (90%, particularly on maternal side of family); history of motion sickness cluster: family hx rare. rarely begins in childhood; headache generally recur on the same side. History of cigarette smoking or second hand cig smoke exposure during childhood chronic daily low grade: episodic symptoms suggestive of migraine with or without aura that evolved into daily chronic headache (70%), family hx of migraine, chronic use of nonprescription analgesics or caffeine (including caffeinated drinks); social, psychological; emotional factors, posttrauma tension: positive family history with childhood onset; fatigue, stress, depression, exertion Children less than 12 years of age often have a poor sense of time and may not serve as the best historians Important questions to ask the child and parent(s) include: • Duration. Recent severe onset is worrisome. • Frequency and triggers. recurrent, low-intensity headaches, with no neurologic changes, and who recover completely between episodes unlikely to have serious intracranial etiology. Triggers can include ovulation or menstruation, exercise, food or odors, and stress. chocolate, processed meats, aged cheeses, nuts, altered amounts of caffeine intake, dairy products, shellfish, some dried fruits. perimenstrual exacerbation, food triggers, and a stable pattern with intervals of wellness over a long time period are reassuring symptoms that suggest primary headache. • Location. Occipital or consistently localized headaches can indicate underlying pathology. Facial pain might be sinusitis. Ocular motor imbalance can produce a dull periorbital discomfort, whereas temporomandibular joint pain tends to localize around the periauricular or temporal areas. • Quality and severity of pain. Sharp, throbbing, or pounding pain is probably vascular (migraine). Dull and constant pain may be tension or organic. ask about limitations to activities and missed school days. How many "different kinds of headaches" are experienced? • Age of onset. Progression of the headaches over time, and longest period of time without symptoms. • Home management and medications including dosage and self- coping activities. • Associated symptoms can include nausea, vomiting, visual changes, dizziness, paresthesia, neck/shoulder pain, back pain, otalgia, abdominal pain, hypersomnia, food cravings, confusion, ataxia, pallor, photophobia, and phonophobia. Changes in gait, personality, mentation, or behavior that do not occur at the same time as the headache are worrisome and merit further evaluation with medical referral. precursor symptoms and conditions that can indicate a predisposition to migraines: cyclic vomiting , abdominal migraine, and benign paroxysmal vertigo. These conditions may evolve into migraine without aura in later childhood • Head trauma. If associated with headache, a subdural hematoma or postconcussive syndrome must be considered. • Psychologic symptoms. Evaluate for the presence of depression, school stressors, or concerns about family functioning. Family history. Most children, especially migraine, have a family history of headaches. Distinguishing Features of Headache Types • Migraine and migraine with aura can be differentiated by the presence or absence of aura symptoms. Characteristics of migraines include nausea; abdominal pain; vomiting; unilateral pain; pulsating pain; relief with sleep; an aura; visual changes such as dark or blind spots; and a history of a family member (usually on the maternal side) with migraine without aura in 90% of cases Dizziness and motion sickness may be described. Infants and toddlers may present with irritability, sleepiness, and pallor. In preadolescents, common migraine symptoms are more likely. Nausea and vomiting might not occur, and the pain can be more frontal. Lethargy and sleep can follow. Visual changes are rare, and the pain quality is variable. Times between headaches are pain free. • Abdominal migraine is rare; symptoms include midline pain, nausea, and vomiting with minimal or no headache. Such symptoms can also be suggestive of complex partial seizures • Muscle contraction or tension headaches. The pain is dull and bifrontal or occipital, with nausea and vomiting occurring only rarely; there is no prodrome. can last for days or weeks but generally do not interfere with activities. In children it can be difficult to differentiate migraine and tension-type headaches. Psychosocial stress seems to be a major factor in tension and chronic daily headaches in both children and adolescents. • Secondary headaches (or those headaches that have a pathologic process) Key historical markers are sudden onset of hyperacute or increasing pain severity or accompanying neurologic signs. These require prompt referral. Presenting symptoms of these headaches include pain that is worse in the morning on awakening and standing up, then fades increases in frequency and severity over a period of only a few weeks Pain that wakens the child from sleep Vomiting but not nausea; vomiting may relieve the headache Visual disturbances, diplopia, edema of the optic disc Increased pain with straining, sneezing, coughing, defecation, or changes in position Occipital region and neck pain (brain tumors tend to originate in the posterior fossa area in children Educational, mental, personality or behavioral alterations, irritability Seizures Unsteadiness Fever Family history of neurological disorders (e.g., brain tumors, neurofibromatosis, vascular malformations) Child has a history of a ventriculoperitoneal shunt, meningitis, hydrocephaly, or tumor.