NEURO: Febrile and Pediatric Seizures

Constitutional Symptoms (ie. unusual facial features) and Seizure

1' Metabolic Disorder, Storage Disease, or Structural Lesion

What is the definition of a febrile seizure?

A seizure associated with a fever in a child usually between six months and five years of age.

Vomiting and seizure

AM vomitting→ ↑ICP

Interruption of activity, staring, unresponsiveness Non convulsive. Can be accompanied by eyelid fluttering or automatisms (lip smacking, grimacing, swallowing) Lasts 5-15 seconds. Can occur more than 100 times a day! No aura or post-ictal period. Child unaware of event. 3Hz spike and wave discharges.

Absence Epilepsy Onset 3-8 years old Girls>Boys Genetic factors Tx: Ethosuximide

Precipitated by hyperventilation

Absence epilepsy

What is the AAP Practice Guideline for treating simple, febrile seizures with anti-convulsants? Are drugs recommended? Or are emotional, educational support preferred?

Based on the risks and benefits of the effective therapies, neither continuous nor intermittent anticonvulsant therapy is recommended for children with one or more simple febrile seizures. The AAP recognizes that recurrent episodes of febrile seizures can create anxiety in some parents and their children, and, as such, appropriate education and emotional support should be provided.

6 y/o boy, neurologically normal. Twitching of one side of face while sleeping or upon awakening. Anterior temporal lobe spike discharge.

Benign Partial Epilepsy of Childhood Tx: Carbamazepine

Onset 5-8 years old Boys>Girls Genetic component Occur during sleep, but sometimes upon awakening One-sided face twitching Unintelligible gurgling sounds Last <2 minutes Consciousness retained Infrequent episodes

Benign Partial Epilepsy of Childhood and Rolandic epilepsy/sylvian seizures

Eye rolling and a prolonged seizure indicate what kind of seizure?

COMPLEX

Impaired consciousness. Brief and infequent. Automatisms in 50-70% (brief unconcious behaviors). EEG: Temporal lobe focal spikes or sharp waves (20% are normal) CT/MRI: Common abnormality in TEMPORAL region

Complex Partial Seizure May generalize into tonic-clonic motor activity → during this, head turning, dystoning posturing or eye blinking may be noted.

Impaired consciousness Can begin as simple partial seizure Aura Occurs before consciousness is lost Auditory, olfactory, or visual hallucinations Can manifest as fear/unpleasant feeling Feelings of Déjà vu Automatisms Post-ictal confusion

Complex Partial Seizures

Intellectual Deterioration/Personality Changes and Seizure

Degenerative CNS Disease (ie. Leukodystrophy)

Management of Seizures

Diagnose potentially treatable underlying condition Patients with a single seizure, normal EEG and evaluation should be observed for recurrence 50% recur within 6 months 69% within 1 year 88% within 2 years If second seizure occurs, then initiate medication Goal is Monotherapy with antiepileptic medications Consider weaning/discontinuing medication after controlled for 2 years

Benign Partial Epilepsy of Childhood EEG? Remit age? Any neurologic impairment?

Drug of choice is carbamazepine EEG Findings: Midtemporal or centrotemporal spike discharges, frequent in light sleep Remit at age 9-12 years old No developmental or neurological impairment

Microcephaly or Macrocephaly

Familial craniosynostosis Hydrocephalus

What is the DOC for Lenox Gaustaut syndrome? Prognosis?

Goal: seizure control with few meds as possible Valproate and Ketogenic diet Better prognosis in healthy preschool children with normal background and fast polyspike-and-wave changes on EEG

Fall to ground posturing. CYANOSIS from diaphragm contraction. Biting of the tongue. Incontinence. Post ictal period (30-120min) vomitting, confusion, lethargy. LOSS OF CONSCIOUSNESS.

Grand Mal/Tonic-Clonic seizures Idiopathic or genetic. Partial seizure. Aura, head/eye deviation, focal clonic movmt at onset of seizure.

Evaluation of first AFEBRILE seizure

History and Physical Laboratory Evaluation Serum electrolytes, calcium, magnesium, glucose Lumbar puncture if meningitis or encephalitis suspected EEG Helps define seizure type Normal interictal tracings in 40% of patients Normal tracing does not exclude diagnosis X-rays of skull rarely helpful Can detect calcifications (ie. Tuberous sclerosis) CT or MRI Not warranted in every child Evaluate structural abnormalities CT in urgent situations MRI more sensitive (should be performed in kids w/ focal neurologic abnormalities or intractable epilepsy)

Ketogenic diet

In one study, 10% of treated patients were seizure free in 1 year 50% decrease in seizure frequency Unknown mechanism High-fat, low-protein, low-carb diet

hypsarrhythmia-High-voltage slow waves with irregularly interspersed multifocal spike-and-sharp waves

Infantile spasms EEG

West syndrome. What is it Onset? Gender?

Infantile spasms. Onset during the first year of life (peak 3-7 months) Boy>Girls. Sudden contraction of neck, trunk and extremities Dropping of head with abduction of shoulders and flexion of lower extremities. Rare during sleep. Occurs in clusters of up to 100 spasms!

Generalized, symmetrical polyspike and waves at 4-6 Hz Can be precipitated by photic stimulation in some patients

Juvenile Myoclonic Epilepsy

Myoclonic jerks of the upper extremities Commonly occur upon awakening from sleep 80% have generalized tonic-clonic and 25% have absence seizures in addition to myoclonic seizures Precipitated by lack of sleep, stress, alcohol, hormonal changes Retained consciousness Neurologically normal

Juvenile myoclonic epilepsy

4 y/o boy prior neurological deficits. Severe, variety of generalized seizures. Irregular, high-voltage, slow spike-wave. Tx?

Lennox-Gastaut Tx: Valproate

"Atypical spike and wave" pattern Irregular, high-voltage, slow spike-wave pattern

Lenox Gastaut Syndrome

Variety of generalized seizures (tonic, tonic-clonic, absence, atonic, myoclonic jerks). Patient will have neurologic deficits before onset. Will see in 3-5 yo. More often in boys.

Lenox Gastaut Syndrome-Ill-defined severe epileptic encephalopathy in childhood

Hepatosplenomegaly and seizure

Metabolic Disorders or Storage Disease (Niemann-Pick[sphingomyelinase deficiency], Gaucher's[GLucocerebroside deficiency], Tay Sach's Disease, Hurler's Syndrome

Juvenile Myoclonic Epilepsy. Onset? Genetic inheritance? History of what type of seizure?

Most common general Onset 12-18 years old Represents approximately 7% of all epilepsy Genetic (Autosomal Recessive) Often history of absence seizures earlier in childhood

Seizure Precautions

Position patient on their side Do not put anything inside mouth Should not swim alone Take showers instead of baths Should not use bike without helmet Sports ok if seizures are controlled

What is the most important and initial treatment for a simple, febrile seizure?

Prompt fever control: No evidence that fever control decreases the incidence of febrile seizures. In 10-30% of patients, the seizure is the first manifestation of the illness.

Retinal Hemorrhages and seizure

Shaken baby syndrome, papilledema, coloboma, or chorioretinitis

Maybe confused with TICS. Motor is most common. Sensory olfactory, autonomic(abdominal) or only an aura (headache, funny feeling) Lasts 10-20 seconds. Asynchronous tonic or clonic movements. EEG shows spikes and sharp waves. Consciousness maintained.

Simple partial seizure Differentiate from Tics because tics can be stopped. Tics: Shrugging of shoulders, blinking, grimacing, not tonic/clonic movements.

Preserved consciousness Focal. Restricted to part of body May spread to entire side (Jacksonian) May be followed by Todd paralysis Parasthesias, vertigo, gustatory, olfactory, auditory, visual phenomena Aura, deja-vu, fear

Simple partial seizures

Constrast simple febrile seizures vs. complex seizures. Focused or generalized? Are there neurological deficits? They last less than ___ minutes. How many within a 24hr period? Which one is more common?

Simple: -Generalized -No associated neurological deficit -Less than 15 minutes duration -Only one within a 24 hour period -Far more common that complex febrile seizures Complex manifest by one or more of the following parameters: -Focality -Greater than 15 minutes duration -Neurological Deficit -Multiple in a 24 hour period

Vagal nerve stimulation

Surgically implanted subcutaneously in anterior chest Stimulates left vagus nerve Unknown mechanism

Causes of infantile spasms. What is the prognosis?

Symptomatic Group-Abnormal neurological development prior to onset (75% of cases) Structural abnormalities of brain (ie. Tuberous Sclerosis) CNS infections or bleeds Metabolic disorders Hypoxic-ischemic events Cryptogenic Group- No etiology found Older at onset Prognosis Grave, 20% mortality Regression of development and milestones Intellectual disability Spasms remit by a few years of age, but approx 50% develop other forms of seizures

Tonic Clonic Seizure with LONG post-tictal phase. What red flag disease do you think of?

TUMOR

Describe the management for a seizing child. What do you first assess? If there are secretions what do you do next? Do you give an IV before or after fever control? When do you provide oxygen? What is a dextrostix?

The child is usually not seen until the seizure has ended. If the seizure is ongoing: -Assess respiratory and cardiovascular status -Gentle suction, or turn the head from side to side to clear secretions -Provide oxygen -Check Dextrostix (hypoglycemia test) -Fever control -Begin IV

What is the most common childhood seizure?

Tonic-clonic Grand Mal Note: May only have a tonic phase

Unilateral small hand or thumb

chronic disorder such as postencephalic cyst, unilateral cortical atrophy or AV malformation, or hemihypertrophy

Describe continuous therapy with anti-convulsants for simple, febrile seizures (3). Are carbamazepine and phenytoin effective? What do you give to prevent recurrences? What are the adverse effects?

-Carbamazepine and phenytoin are ineffective -Phenobarbital is effective in preventing recurrences; the adverse effects include behavioral and learning problems -Valproic acid is effective in preventing recurrences; the adverse effects include fatal hepatotoxicity, pancreatitis (both more common in children less than three years old)

Are EEG, blood studies, CTs or MRIs necessary to evaluate a child with a fever and their first simple, febrile seizure?

-EEG: No predictive value -Blood studies: No routine serum electrolytes, calcium, phosphorus, magnesium, CBC, or blood glucose -Neuroimaging: No need for skull films, CT or MRI -The EEG is best reserved for children with complex febrile seizures, particularly those with focality -Certainly if the child looks sick, or has fever 104 and is at risk for bacteremia, a CBC and blood culture is indicated

What are the risk factors for the development of a chronic seizure disorder in the child with febrile seizures (7)?

-Family history of chronic seizure disorder -Initial febrile seizure at less than nine months old -Complex febrile seizure -Developmental delay -Abnormal neurological exam -Risk of afebrile seizure disorder is 1% in the general population and patients with simple febrile seizures -Risk increases to 2% with one risk factor, and 10% with two to three risk factors

Seizures and meningitis. In what % is seizure a presenting sign of meningitis? In what % are the meningeal signs lacking? Is a lumbar puncture considered for a child less than 18 months?

-In 13 to 16% of children with meningitis, seizures are the presenting sign of disease -In 30 to 35% of these children (primarily less than 18 months old), meningeal signs are lacking -AAP Practice Guideline for First Simple Febrile Seizure recommends: Less than 12 months old lumbar puncture should be strongly considered; 12 to 18 months lumbar puncture should be considered

Describe the management for a seizure at home (3).

-Keep calm -Lay child on back, turning head from side to side, to clear secretions -Seek medical evaluation of fever

What are the possibilities when a child presents with a fever and their first seizure (3)?

-May be due to a febrile seizure -May be due to a chronic seizure disorder triggered by fever -May be due to an intracranial infection, meningitis, encephalitis, brain abscess

What oral drugs is used for simple, febrile seizures with anti-convulsants? What rectal drugs are used in acute management? What are the adverse effects?

-Oral diazepam: Given at the time of a fever, reduces the incidence of seizures by 44%; adverse effects include lethargy, drowsiness, ataxia; the adverse effects could mask evolving signs of a CNS infection, and make medical evaluation less accurate -Rectal diazapam or lorazepam: Used in acute management of the seizure; parents can be taught to administer the drug; effective in aborting prolonged seizures; still has the potential for adverse effects

Describe the presentation of febrile seizures. After how many hours of fever will the seizure occur? What causes the seizure? What are the most common infectious triggers?

-Seizure usually occurs within the first 24 hours of fever -Seizure is due to rapid change in body temperature, usually more than 39 C (102.2 F) -Usually occurs in the presence of a clinically recognizable infection -Most common infectious triggers are viral upper respiratory infection, acute otitis media and Roseola (HSSV 6)

What are the two types of febrile seizures?

-Simple -Complex

Are simple febrile seizures benign or malignant? Are there any treatments that can prevent it from becoming a chronic seizure disorder? If given phenobarbital what is a common side effect?

-Simple febrile seizures are a benign event -No treatment has been shown to prevent the rare development of a chronic seizure disorder after a simple febrile seizure -40% of children treated with phenobarbital have behavioral side effects (irritability, hyperactivity, disturbed sleep, learning problems)

Factors or findings that clue you in to an abnormal CSF.

-Suspicious findings on physical exam -Complex febrile seizures -Doctor visit within 48 hours before the seizure -Seizing upon arrival to emergency department -Prolonged postictal state -Initial seizure after three years old

What is the risk of death? Will the seizure affect the child's intelligence? Will the child develop epilepsy?

-What is the risk of death: Several large studies have not reported any deaths -Will the seizure affect the child's intelligence: No long term intellectual or neurological dysfunction has been reported -Will the child develop epilepsy: There is a slight (less than 1% difference) increase in comparison to the general population

Epidemiology of febrile seizures. What percentage of kids? Before what age? Average onset is what age? What is the recurrence rate if first seizure is at less than one year old?

-2 to 5 % of all children will have a febrile seizure before five years of age -The majority of febrile seizures occur prior to three years of age -The average age of onset is between 18 and 22 months -The recurrence rate is 50% if the first seizure occurs at less than one year old; 28% if onset is greater than 1 year old

What are the clinical features of febrile seizures (3)? What percentage are simple? complex? What kind of seizure is involved? What would you typically see in a focal motor activity seizure?

-80% are simple febrile seizures, 20% are complex febrile seizures -Most often have generalized motor seizure. Less commonly have focal motor activity or may be only staring episode or eye rolling -Usually brief; only 8% last longer than 15 minutes