Chapter 49: Neuromuscular or Muscular Dysfunction

4. Which problem is most often associated with myelomeningocele? a.Hydrocephalus c.Biliary atresia b.Craniosynostosis d.Esophageal atresia

ANS: A Hydrocephalus is an associated anomaly in 80% to 90% of children. Craniosynostosis is the premature closing of the cranial sutures and is not associated with myelomeningocele. Biliary and esophageal atresias are not associated with myelomeningocele.

1. Spastic cerebral palsy is characterized by: a.Hypertonicity and poor control of posture, balance, and coordinated motion. b.Athetosis and dystonic movements. c.Wide-based gait and poor performance of rapid, repetitive movements. d.Tremors and lack of active movement.

ANS: A Hypertonicity and poor control of posture, balance, and coordinated motion are part of the classification of spastic cerebral palsy. Athetosis and dystonic movements are part of the classification of dyskinetic/athetoid cerebral palsy. Wide-based gait and poor performance of rapid, repetitive movements are part of the classification of ataxic cerebral palsy. Tremors and lack of active movement may indicate other neurologic disorders.

3. The most common problem of children born with a myelomeningocele is: a.Neurogenic bladder. c.Respiratory compromise. b.Intellectual impairment. d.Cranioschisis.

ANS: A Myelomeningocele is one of the most common causes of neuropathic (neurogenic) bladder dysfunction among children. Risk of intellectual impairment is minimized through early intervention and management of hydrocephalus. Respiratory compromise is not a common problem in myelomeningocele. Cranioschisis is a skull defect through which various tissues protrude. It is not associated with myelomeningocele.

11. A young boy has just been diagnosed with pseudohypertrophic (Duchenne's) muscular dystrophy. The management plan should include: a.Recommending genetic counseling. b.Explaining that the disease is easily treated. c.Suggesting ways to limit the use of muscles. d.Assisting the family in finding a nursing facility to provide his care.

ANS: A Pseudohypertrophic (Duchenne's) muscular dystrophy is inherited as an X-linked recessive gene. Genetic counseling is recommended for parents, female siblings, maternal aunts, and their female offspring. No effective treatment exists at this time for childhood muscular dystrophy. Maintaining optimal function of all muscles for as long as possible is the primary goal. It has been found that children who remain as active as possible are able to avoid wheelchair confinement for a longer time. Assisting the family in finding a nursing facility is inappropriate at the time of diagnosis. When the child becomes increasingly incapacitated, the family may consider home-based care, a skilled nursing facility, or respite care to provide the necessary care.

23. The nurse in the neonatal intensive care unit is caring for an infant with myelomeningocele scheduled for surgical repair in the morning. Which early signs of infection should the nurse monitor on this infant (Select all that apply)? a.Temperature instability b.Irritability c.Lethargy d.Bradycardia e.Hypertension

ANS: A, B, C The nurse should observe an infant with unrepaired myelomeningocele for early signs of infection, such as temperature instability (axillary), irritability, and lethargy. Bradycardia and hypertension are not early signs of infection in infants.

22. The nurse is conducting discharge teaching with parents of a preschool child with myelomeningocele, repaired at birth, who is being discharged from the hospital after a urinary tract infection (UTI). Which should the nurse include in the discharge instructions related to management of the child's genitourinary function (Select all that apply)? a.Continue to perform the clean intermittent catheterizations (CIC) at home. b.Administer the oxybutynin chloride (Ditropan) as prescribed. c.Reduce fluid intake in the afternoon and evening hours. d.Monitor for signs of a recurrent UTI. e.Administer furosemide (Lasix) as prescribed.

ANS: A, B, D Discharge teaching to prevent renal complications in a child with myelomeningocele include: (1) regular urologic care with prompt and vigorous treatment of infections; (2) a method of regular emptying of the bladder, such as clean intermittent catheterization (CIC) taught to and performed by parents and self-catheterization taught to children; and (3) medications to improve bladder storage and continence, such as oxybutynin chloride (Ditropan) and tolterodine (Detrol). Fluids should not be limited, and Lasix is not used to improve renal function for children with myelomeningocele.

21. Which assessment findings should the nurse note in a school-age child with Duchenne's muscular dystrophy (DMD) (Select all that apply)? a.Lordosis b.Gower's sign c.Kyphosis d.Scoliosis e.Waddling gait

ANS: A, B, E Difficulties in running, riding a bicycle, and climbing stairs are usually the first symptoms noted in DMD. Typically, affected boys have a waddling gait and lordosis, fall frequently, and develop a characteristic manner of rising from a squatting or sitting position on the floor (Gower's sign). Lordosis occurs as a result of weakened pelvic muscles, and the waddling gait is a result of weakness in the gluteus medius and maximus muscles. Kyphosis and scoliosis are not assessment findings with DMD.

20. A 14-year-old girl is in the intensive care unit after a spinal cord injury 2 days ago. Which nursing care interventions are needed for this child (Select all that apply)? a.Monitoring and maintaining systemic blood pressure. b.Administering corticosteroids. c.Minimizing environmental stimuli. d.Discussing long-term care issues with the family. e.Monitoring for respiratory complications.

ANS: A, B, E Spinal cord injury patients are physiologically labile, and close monitoring of blood pressure and respirations is required. They may be unstable for the first few weeks after the injury. Corticosteroids are administered to minimize the inflammation present with the injury. Minimizing environmental stimuli and discussing long-term care issues with the family do not apply to providing care for this patient.

16. The nurse is caring for an infant with myelomeningocele scheduled for surgical closure in the morning. Which interventions should the nurse plan for the care of the myelomeningocele sac? a.Open to air b.Covered with a sterile, moist, nonadherent dressing c.Reinforcement of the original dressing if drainage noted d.A diaper secured over the dressing

ANS: B Before surgical closure, the myelomeningocele is prevented from drying by the application of a sterile, moist, nonadherent dressing over the defect. The moistening solution is usually sterile normal saline. Dressings are changed frequently (every 2 to 4 hours), and the sac is closely inspected for leaks, abrasions, irritation, and any signs of infection. The sac must be carefully cleansed if it becomes soiled or contaminated. The original dressing would not be reinforced but changed as needed. A diaper is not placed over the dressing because stool contamination can occur.

7. The nurse is caring for a neonate born with a myelomeningocele. Surgery to repair the defect is scheduled the next day. The most appropriate way to position and feed this neonate is to place him: a.Prone and tube feed. b.Prone, turn head to side, and nipple feed. c.Supine in infant carrier and nipple feed. d.Supine, with defect supported with rolled blankets, and nipple feed.

ANS: B In the prone position, feeding is a problem. The infant's head is turned to one side for feeding. If the child is able to nipple feed, no indication is present for tube feeding. Before surgery, the infant is kept in the prone position to minimize tension on the sac and risk of trauma.

10. Which finding should cause the nurse to suspect a diagnosis of spastic cerebral palsy? a.Tremulous movements at rest and with activity b.Sudden jerking movement caused by stimuli c.Writhing, uncontrolled, involuntary movements d.Clumsy, uncoordinated movements

ANS: B Spastic cerebral palsy, the most common type of cerebral palsy, will manifest with hypertonicity and increased deep tendon reflexes. The child's muscles are very tight and any stimuli may cause a sudden jerking movement. Tremulous movements are characteristic of rigid/tremor/atonic cerebral palsy. Slow, writhing, uncontrolled, involuntary movements occur with athetoid or dyskinetic cerebral palsy. Clumsy movements, loss of coordination, equilibrium, and kinesthetic sense occur in ataxic cerebral palsy.

19. A home care nurse is caring for an adolescent with a T1 spinal cord injury. The adolescent suddenly becomes flushed, hypertensive, and diaphoretic. Which intervention should the nurse perform first? a.Place the adolescent in a flat right side-lying position. b.Place a cool washcloth on the adolescent's forehead and continue to monitor the blood pressure. c.Implement a standing prescription to empty the bladder with a sterile in-and-out Foley catheter. d.Take a full set of vital signs and notify the health care provider.

ANS: C The adolescent is experiencing an autonomic dysreflexia episode. The paralytic nature of autonomic function is replaced by autonomic dysreflexia, especially when the lesions are above the mid-thoracic level. This autonomic phenomenon is caused by visceral distention or irritation, particularly of the bowel or bladder. Sensory impulses are triggered and travel to the cord lesion, where they are blocked, which causes activation of sympathetic reflex action with disturbed central inhibitory control. Excessive sympathetic activity is manifested by a flushing face, sweating forehead, pupillary constriction, marked hypertension, headache, and bradycardia. The precipitating stimulus may be merely a full bladder or rectum or other internal or external sensory input. It can be a catastrophic event unless the irritation is relieved. Positioning the adolescent, placing a cool washcloth on the adolescent's forehead, continuing to monitor blood pressure and vital signs, and notifying the health care provider would not reverse the sympathetic reflex situation.

15. The nurse is admitting a child with Werdnig-Hoffmann disease (spinal muscular atrophy type 1). Which signs and symptoms are associated with this disease? a.Spinal muscular atrophy b.Neural atrophy of muscles c.Progressive weakness and wasting of skeletal muscle d.Pseudohypertrophy of certain muscle groups

ANS: C Werdnig-Hoffmann disease (spinal muscular atrophy type 1) is the most common paralytic form of floppy infant syndrome (congenital hypotonia). It is characterized by progressive weakness and wasting of skeletal muscle caused by degeneration of anterior horn cells. Kugelberg-Welander syndrome is a juvenile spinal muscular atrophy with a later onset. Charcot-Marie-Tooth disease is a form of progressive neural atrophy of muscles supplied by the peroneal nerves. Progressive weakness of the distal muscles of the arms and feet is found. Duchenne's muscular dystrophy is characterized by muscles, especially in the calves, thighs, and upper arms, that become enlarged from fatty infiltration and feel unusually firm or woody on palpation. The term pseudohypertrophy is derived from this muscular enlargement.

14. The nurse is preparing to admit a newborn with myelomeningocele to the neonatal intensive care nursery. Which describes this newborn's defect? a.Fissure in the spinal column that leaves the meninges and the spinal cord exposed b.Herniation of the brain and meninges through a defect in the skull c.Hernial protrusion of a saclike cyst of meninges with spinal fluid but no neural elements d.Visible defect with an external saclike protrusion containing meninges, spinal fluid, and nerves

ANS: D A myelomeningocele is a visible defect with an external saclike protrusion, containing meninges, spinal fluid, and nerves. Rachischisis is a fissure in the spinal column that leaves the meninges and the spinal cord exposed. Encephalocele is a herniation of brain and meninges through a defect in the skull, producing a fluid-filled sac. Meningocele is a hernial protrusion of a saclike cyst of meninges with spinal fluid, but no neural elements.

2. The parents of a child with cerebral palsy ask the nurse if any drugs can decrease their child's spasticity. The nurse's response should be based on knowing that: a.Anticonvulsant medications are sometimes useful for controlling spasticity. b.Medications that would be useful in reducing spasticity are too toxic for use with children. c.Many different medications can be highly effective in controlling spasticity. d.Implantation of a pump to deliver medication into the intrathecal space to decrease spasticity has recently become available.

ANS: D Baclofen given intrathecally is best suited for children with severe spasticity that interferes with activities of daily living and ambulation. Anticonvulsant medications are used when seizures occur in children with cerebral palsy. The intrathecal route decreases the side effects of the drugs that reduce spasticity. Few medications are presently available for the control of spasticity.

13. What is the most appropriate nursing response to the father of a newborn infant with myelomeningocele who asks about the cause of this condition? a."One of the parents carries a defective gene that causes myelomeningocele." b."A deficiency in folic acid in the father is the most likely cause." c."Offspring of parents who have a spinal abnormality are at greater risk for myelomeningocele." d."There may be no definitive cause identified."

ANS: D The etiology of most neural tube defects is unknown in most cases. There may be a genetic predisposition or a viral origin, and the disorder has been linked to maternal folic acid deficiency; however, the actual cause has not been determined. There is no evidence that children who have parents with spinal problems are at greater risk for neural tube defects.