Chapter 28: Alterations in Neuromuscular Function

After teaching a group of students about medications commonly used for neuromuscular disorders, the nursing instructor determines that the teaching was successful when the students identify which agent is a centrally acting skeletal muscle relaxant? A. Baclofen B. Prednisone C. Lorazepam D. Botulin toxin

ANS: A Rationale: Baclofen is a centrally acting skeletal muscle relaxant used to treat painful spasms and decrease spasticity in children with motor neuron lesions. Prednisone is a corticosteroid that is used to help slow the progression of Duchenne muscular dystrophy. Lorazepam is a benzodiazepine used for adjunctive relief of skeletal muscle spasm associated with cerebral palsy. Botulin toxin is a neurotoxin used to relieve spasticity in cerebral palsy.

The nurse is assessing the neuromusculoskeletal system of a newborn. What is an abnormal assessment finding? A. Sluggish deep tendon reflexes B. Full range of motion in extremities C. Absence of hypotonia D. Lack of purposeful muscular control

ANS: A Rationale: Deep tendon reflexes are present at birth and are initially brisk in the newborn and progress to average over the first few months. Sluggish deep tendon reflexes indicate an abnormality. The newborn is capable of spontaneous movement but lacks purposeful control. Full range of motion is present at birth. Healthy infants and children demonstrate normal muscle tone; hypertonia or hypotonia is an abnormal finding.

How would the nurse best describe Gowers sign to the parents of a child with muscular dystrophy? A. A transfer technique B. A waddling-type gait C. The pelvis position during gait D. Muscle twitching present during a quick stretch

ANS: A Rationale: Gowers' sign is a description of a transfer technique present during some phases of muscular dystrophy. The child turns on the side or abdomen, extends the knees, and pushes on the torso to an upright position by walking his hands up the legs. The child's gait is unrelated to the presence of Gowers sign. Muscle twitching present after a quick stretch is described as clonus.

An infant has been born and diagnosed with a meningocele. Which action will the nurse incorporate into each contact with this infant? A. Inspection of the cystic sac on the child's back for leakage B. Auscultation for bowel sounds C. Listening for a shrill cry D. Careful supine positioning

ANS: A Rationale: Leakage from the cystic area indicates loss of cerebrospinal fluid (CSF) and risk of infection of the central nervous system. Prompt intervention is needed, probably surgical. Listening for bowel sounds confirms intestinal peristalsis but is not necessary with each infant contact. A shrill cry may indicate increased intracranial pressure (ICP). This is important to note yet is not as pressing as being aware of leakage. The baby would be positioned prone, not supine, to protect the sac.

The nurse is caring for a 7-year-old with Guillain-Barré syndrome (GBS). Which of the following would be the most effective intervention to monitor for respiratory deterioration? A. Serial measurement of tidal volume B. Pulse oximetry C. Ineffective cough D. Diminished breath sounds

ANS: A Rationale: Serial measurement of tidal volumes may reveal respiratory deterioration in a child with GBS. Pulse oximetry gives no information regarding ventilation, only oxygen saturation. A decrease in oxygen saturation noted on pulse oximetry would be helpful for determining a change in respiratory function. However, it would not be the most effective method. Ineffective cough may indicate a change in respiratory function, but this change is nonspecific. Diminished breath sounds reveal a change in respiratory function; however, they are nonspecific.

The nurse is assessing a child with spastic cerebral palsy. What findings would the nurse expect to assess? Select all that apply. A. Exaggerated deep tendon reflexes B. Hemiplegia C. Poor control of balance D. Hypertonicity E. Drooling F. Dysarthria

ANS: A Rationale: Spastic cerebral palsy is associated with exaggerated deep tendon reflexes; poor control of posture, balance, and movement; hypertonicity of the affected extremities; and hemiplegia, quadriplegia, or diplegia, based on the limbs affected. Drooling and dysarthria are associated with athetoid cerebral palsy.

Parents of a preschooler with cerebral palsy ask the nurse what the surgeon plans to implant in their child's body to control spasticity. What is the nurse's answer? A. Baclofen pump B. Vagal nerve stimulator C. Central venous catheter D. Botulinum toxin

ANS: A Rationale: A baclofen pump can be placed surgically to deliver continuous medication intrathecally. Baclofen can also be taken orally. Botulinum toxin is injected by a practitioner into specified muscle groups to reduce spasticity. A central venous catheter places medication directly into rapidly moving blood and would not be used. A vagal nerve stimulator is used to control seizures.

A nurse is developing a teaching plan for the parents of a child with myasthenia gravis. Which of the following would the nurse include? A. How to administer anticholinergic drugs B. Establishment of plans for rest periods C. Signs and symptoms of infection D. Stress management techniques E. Ways to increase the temperature of the child's environment

ANS: A, B, C, D Rationale: The teaching plan for a child with myasthenia gravis should include instructions about administering anticholinergic agents, usually 30 to 45 minutes before meals, on time and exactly as ordered; measures to allow for rest periods for energy conservation; signs and symptoms of infection and the need to notify the physician because infection can precipitate a myasthenic crisis; stress management techniques because stress can precipitate a myasthenic crisis; and ways to maintain the child's environmental temperature because exposure to extreme temperatures can precipitate a myasthenic crisis.

The child has a meningocele and a neurogenic bladder. Which of the following topics should the nurse include in the teaching plan when educating the child and the child's caregivers? Select all that apply. A. How and when to administer oxybutynin chloride B. The importance of antibiotic use to prevent urinary tract infections from occurring C. How and when to perform clean intermittent urinary catheterization D. Signs and symptoms of a urinary tract infection E. Different types of surgeries used to treat this condition

ANS: A, C, D, E Rationale: Ditropan is used to increase the child's bladder capacity when they have a spastic bladder. The caregivers and the child should be taught about urinary catheterization techniques to allow the bladder to empty. The child and caregivers should be educated about the clinical manifestations associated with a urinary tract infection so that it can be treated promptly. Sometimes surgical interventions such as vesicostomy and the creation of a continent urinary reservoir are used to treat neurogenic bladders.

The nurse is conducting a wellness examination of a 6-month-old child. The mother points out some dimpling and skin discoloration in the child's lumbosacral area. How should the nurse respond? A. "This could be an indicator of spina bifida; we need to evaluate this further." B. "This can be considered a normal variant with no indication of a problem; however, the doctor will want to take a closer look." C. "Dimpling, skin discoloration, and abnormal patches of hair are often indicators of spina bifida occulta." D. "This is often an indicator of spina bifida occulta as opposed to spina bifida cystica."

ANS: B Rationale: Dimpling and skin discoloration in the child's lumbosacral area can be an indication of spina bifida occulta. It would be best to respond that the dimpling and discoloration is possibly a normal variation with no problems and indicate that the doctor will want to take a closer look; this response will not alarm the parent, but it also does not ignore the findings. Spina bifida is a term that is often used to generalize all neural tube disorders that affect the spinal cord. This can be confusing and a cause of concern for parents. It is probably best to avoid the use of the term initially until a diagnosis is confirmed. Nursing care would then focus on educating the family.

A nurse is caring for an infant with spinal muscle atrophy (SMA) type 1. What will the nurse note when assessing the child? A. Spastic upper and lower extremities B. Narrow chest and protuberant abdomen C. Enlarged head with low-set ears D. Lusty cry with voracious appetite

ANS: B Rationale: SMA type 1 is also known as Werdnig-Hoffman disease and infantile SMA. It is the most severe of the three types. This disease is autosomal recessive and affects the ability of spinal nerves to communicate with muscle, eventually leading to atrophy. The infantile form progresses rapidly to early childhood death, usually from respiratory complications. The narrow chest and large abdomen are characteristic. Over time, the chest develops pectus excavatum, which restricts respiration further when combined with muscle weakness. Extremities would not be spastic but hypotonic. Head size and ear placement are normal in the infant with SMA type 1. Difficulties in sucking and swallowing are common, and a lusty cry is not found.

Which diagnostic measure is most accurate in detecting neural tube defects? A. Flat plate of the lower abdomen after the 23rd week of gestation B. Significant level of alpha-fetoprotein present in amniotic fluid C. Amniocentesis for lecithin-sphingomyelin (L/S) ratio D. Presence of high maternal levels of albumin after 12th week of gestation

ANS: B Rationale: Screening for significant levels of alpha-fetoprotein is 90% effective in detecting neural tube defects. Prenatal screening includes a combination of maternal serum and amniotic fluid levels, amniocentesis, amniography, and ultrasonography and has been relatively successful in diagnosing the defect. Flat plate X-rays of the abdomen, L/S ratio, and maternal serum albumin levels aren't diagnostic for the defect.

The mother of a 3-year-old with a myelomeningocele is thinking about having another baby. The nurse should inform the woman that she should increase her intake of which acid? A. Folic acid to 0.4 mg/day B. Folic acid above 0.4 mg/day C. Ascorbic acid to 0.4 mg/day D. Ascorbic acid to 4 mg/day

ANS: B Rationale: The American Academy of Pediatrics recommends that a woman who has had a child with a neural tube defect increase her intake of folic acid to above 0.4 mg per day 1 month before becoming pregnant and continue this regimen through the first trimester. A woman who has no family history of neural tube defects should take 0.4 mg/day. All women of childbearing age should be encouraged to take a folic acid supplement because the majority of pregnancies in the United States are unplanned. Ascorbic acid hasn't been shown to have any effect on preventing neural tube defects.

The nurse caring for a toddler immediately after a fall from a grocery cart will avoid moving which body area as the child is examined? A. Lower extremities B. Head and neck C. Torso D. Clavicle

ANS: B Rationale: The head and neck should remain immobilized until cervical spine injury is ruled out. Motion in this area could damage the spinal cord. The rest of the child's body should be examined carefully so as not to aggravate an unsuspected injury. The clavicle is the bone most frequently fractured during childhood.

A child with spastic cerebral palsy is to receive botulin toxin. The nurse prepares the child for administration of this drug by which route? A. Oral B. Subcutaneous injection C. Intramuscular injection D. Intravenous infusion

ANS: C Rationale: Botulin toxin is administered by injection into the muscle. It may cause dry mouth. It is not administered orally, by subcutaneous injection, or by intravenous infusion.

What will be the nurse's next action after noting dimpling and a tuft of hair located in the lumbosacral area of the preschool child during the examination? A. Snip the tuft of hair off close to the skin for hygienic reasons B. Move on to other assessments without calling attention to the difference C. Record and refer the finding for follow-up to the pediatrician D. Inspect for precocious hair growth in the genital and underarm areas

ANS: C Rationale: Dimpling and hair growth may signal spina bifida occulta, which usually is benign. However, some complications can be associated, and further investigation is warranted to prevent possible damage to the spinal cord. Magnetic resonance imaging (MRI) is often the diagnostic tool used. No hygienic concerns need prevail. These findings do not suggest development of precocious puberty or any other hormonal problem. The dimpling and hair tuft must be clearly explained to the parents.

The nurse caring for an infant with myelomeningocele before surgical intervention will prioritize care in what way? A. Keep the mass uncovered and dry B. Prevent cold stress using an Isolette and blankets C. Cover the sac with a saline-moistened dressing D. Change position from side to side hourly

ANS: C Rationale: Protection of exposed neural tissue is of high priority. Keeping the cystic mass moist prevents damage to neural elements from drying. Blankets may cause trauma to the sac. An Isolette can be used for warmth, but much attention will have to be directed toward keeping the sac moist. Side-to-side hourly position changes increase the risk of damage to protruding nervous tissue. Unnecessary handling should be avoided.

A nurse is teaching the parents of a child who has been diagnosed with spina bifida. Which statement by the nurse would be the most accurate description of spina bifida? A. "It has little influence on the intellectual and perceptual abilities of the child." B. "It's a simple neurologic defect that's completely corrected surgically within 1 to 2 days after birth." C. "Its presence indicates that many areas of the central nervous system (CNS) may not develop or function adequately." D. "It's a complex neurologic disability that involves a collaborative health team effort for the entire first year of life."

ANS: C Rationale: When a spinal cord lesion exists at birth, it commonly leads to altered development or function of other areas of the CNS. Spina bifida is a complex neurologic defect that heavily impacts the physical, cognitive, and psychosocial development of the child and involves collaborative, lifelong management due to the chronicity and multiplicity of the problems involved.

Other than providing direct care to children, what is the major role of nurses in the care of nearly all children with neuromuscular disorders? A. Consoling parents B. Teaching children self-care C. Helping with specialized equipment D. Coordinating care by specialists

ANS: D Rationale: Being part of a multidisciplinary team and coordinating the care the child usually needs from a variety of specialists is an essential and major role. The other nursing activities are important as well, but many children/families require individual interventions.

The young child has been diagnosed with Guillain-Barré syndrome and it is progressing in a classic manner. Rank the following sequence of events in the order that they typically occur. A. The child is having difficulty producing facial expressions. B. The child states that it is difficult to move his legs. C. The child reports numbness and tingling in his toes. D. The child states that it is difficult to move his arms.

ANS:B, C, D, A Rationale: Guillain-Barré syndrome paresthesias and muscle weakness. Classically it initially affects the lower extremities and progresses in an ascending manner to upper extremities and then the facial muscles. Progression is usually complete in 2 to 4 weeks, followed by a stable period leading to the recovery phase.