Chapter 32

Chapter 32: The Child with Neuromuscular or Muscular Dysfunction
MULTIPLE CHOICE
1. The nurse is planning a staff in-service on childhood spastic cerebral palsy. Spastic cerebral
palsy is characterized by:

hypertonicity and poor control of posture, balance, and coordinated motion.
athetosis and dystonic movements.
wide-based gait and poor performance of rapid, repetitive movements.
tremors and lack of active movement.
a.
b.
c.
d.
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.
PTS: 1
DIF: Cognitive Level: Understand
REF: 1092
TOP: Integrated Process: Teaching/Learning
MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation
2. The parents of a child with cerebral palsy ask the nurse whether any drugs can decrease their
childs spasticity. The nurses response should be based on which statement?

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 currently available for the control of
spasticity.
PTS: 1
REF: 1094
MSC: Area of Client Needs: Physiologic Integrity: Pharmacologic and Parenteral Therapy
3. The nurse is preparing to admit a newborn with myelomeningocele to the neonatal intensive
care nursery. Which describes this newborns 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.
PTS: 1
REF: 1098
TOP: Integrated Process: Nursing Process: Assessment
4. The nurse is conducting a staff in-service on common problems associated with
myelomeningocele. Which common problem is associated with this defect?

Hydrocephalus
Craniosynostosis
Biliary atresia
Esophageal atresia
a.
b.
c.
d.
ANS: A
Hydrocephalus is a frequently 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 atresia is not associated with myelomeningocele.
PTS: 1
REF: 1100
5. The nurse is teaching a group of nursing students about newborns born with the congenital
defect of myelomeningocele. Which common problem is associated with this defect?

Neurogenic bladder
Mental retardation
Respiratory compromise
Cranioschisis
a.
b.
c.
d.
ANS: A
Myelomeningocele is one of the most common causes of neuropathic (neurogenic) bladder

dysfunction among children. Risk of mental retardation 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.
PTS: 1
REF: 1101
6. The nurse is reviewing prenatal vitamin supplements with an expectant client. Which
supplement should be included in the teaching?

Vitamin A throughout pregnancy
Multivitamin preparations as soon as pregnancy is suspected
Folic acid for all women of childbearing age
Folic acid during the first and second trimesters of pregnancy
a.
b.
c.
d.
ANS: C
The widespread use of folic acid among women of childbearing age has decreased the
incidence of spina bifida significantly. Vitamin A is not related to the prevention of spina
bifida. Folic acid supplementation is recommended for the preconception period and during
the pregnancy. Only 42% of women actually follow these guidelines.
PTS: 1
REF: 1102
TOP: Integrated Process: Nursing Process: Implementation
7. How much folic acid is recommended for women of childbearing age?
a. 1.0 mg
b. 0.4 mg
c. 1.5 mg
d. 2.0 mg
ANS: B
It has been estimated that a daily intake of 0.4 mg of folic acid in women of childbearing age
will prevent 50% to 70% of cases of neural tube defects; 1.0 mg is too low a dose; 1.5 to 2.0
mg are not the recommended dosages of folic acid.
PTS: 1
DIF: Cognitive Level: Remember
REF: 1102
TOP: Integrated Process: Nursing Process: Evaluation
8. 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 which
position?
a. Prone and tube-fed
b. Prone, head turned to side, and nipple-fed
c. Supine in an infant carrier and nipple-fed
d. Supine, with defect supported with rolled blankets, and nipple-fed
ANS: B
In the prone position, feeding is a problem. The infants head is turned to one side for
feeding. If the child is able to nipple-feed, tube feeding is not needed. Before surgery, the
infant is kept in the prone position to minimize tension on the sac and risk of trauma.
PTS: 1
DIF: Cognitive Level: Apply
REF: 1103
MSC: Area of Client Needs: Physiologic Integrity: Basic Care and Comfort
9. The nurse is talking to a parent with a child who has a latex allergy. Which statement by the
parent would indicate a correct understanding of the teaching?

a. My child will have an allergic reaction if he comes in contact with yeast
products.
b. My child may have an upset stomach if he eats a food made with wheat or
barley.
c. My child will probably develop an allergy to peanuts.
d. My child should not eat bananas or kiwis.
ANS: D
There are cross-reactions between latex allergies and a number of foods such as bananas,
avocados, kiwi, and chestnuts. Children with a latex allergy will not develop allergies to other
food products such as yeast, wheat, barley, or peanuts.
PTS: 1
DIF: Cognitive Level: Analyze
REF: 1104
MSC: Area of Client Needs: Physiologic Integrity: Reduction of Risk Potential
10. Latex allergy is suspected in a child with spina bifida. Appropriate nursing interventions
include which action?

Avoid using any latex product.
Use only nonallergenic latex products.
Administer medication for long-term desensitization.
Teach family about long-term management of asthma.
a.
b.
c.
d.
ANS: A
Care must be taken that individuals who are at high risk for latex allergies do not come in
direct or secondary contact with products or equipment containing latex at any time during
medical treatment. There are no nonallergenic latex products. At this time, desensitization is
not an option. The child does not have asthma. The parents must be taught about allergy and
the risk of anaphylaxis.
PTS: 1
REF: 1104
11. The nurse is admitting a child with Werdnig-Hoffmann disease (spinal muscular atrophy type
1). Which signs and symptoms are associated with this disease?
Spinal muscular atrophy
Neural atrophy of muscles
Progressive weakness and wasting of skeletal muscle
Pseudohypertrophy of certain muscle groups
a.
b.
c.
d.
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 disease is a juvenile spinal muscular atrophy with a later onset. CharcotMarie-Tooth disease is a form of progressive neural atrophy of muscles supplied by the
peroneal nerves. Progressive weakness is found of the distal muscles of the arms and feet.
Duchenne muscular dystrophy is characterized by muscles, especially in the calves, thighs,
and upper arms, which become enlarged from fatty infiltration and feel unusually firm or
woody on palpation. The term pseudohypertrophy is derived from this muscular enlargement.
PTS: 1
REF: 1105
12. Which clinical manifestations in an infant would be suggestive of spinal muscular atrophy
(Werdnig-Hoffmann disease)?
a.
b.
c.
d.
Hyperactive deep tendon reflexes

Hypertonicity
Lying in the frog position
Motor deficits on one side of body
ANS: C
The infant lies in the frog position with the legs externally rotated, abducted, and flexed at
knees. The deep tendon reflexes are absent. The child has hypotonia and inactivity as the most
prominent features. The motor deficits are bilateral.
PTS: 1
REF: 1105
13. A 4-year-old child has just been diagnosed with pseudohypertrophic (Duchenne) muscular
dystrophy. The management plan should include which action?

Recommend genetic counseling.
Explain that the disease is easily treated.
Suggest ways to limit use of muscles.
Assist family in finding a nursing facility to provide his care.
a.
b.
c.
d.
ANS: A
Pseudohypertrophic (Duchenne) 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 to
provide his care 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.
PTS: 1
REF: 1109
14. Therapeutic management of a child with tetanus includes the administration of:
a. nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation.
b. muscle stimulants to counteract muscle weakness.
c. bronchodilators to prevent respiratory complications.
d. tetanus immunoglobulin therapy.
ANS: D
Tetanus immunoglobulin therapy, to neutralize toxins, is the most specific therapy for tetanus.
Tetanus toxin acts at the myoneural junction to produce muscular stiffness and lowers the
threshold for reflex excitability. NSAIDs are not routinely used. Sedatives or muscle relaxants
are used to help reduce titanic spasm and prevent seizures. Respiratory status is carefully
evaluated for any signs of distress because muscle relaxants, opioids, and sedatives that may
be prescribed may cause respiratory depression. Bronchodilators would not be used unless
specifically indicated.
PTS: 1
REF: 1112

15. The nurse is conducting reflex testing on infants at a well-child clinic. Which reflex finding
should be reported as abnormal and considered as a possible sign of cerebral palsy?

Tonic neck reflex at 5 months of age
Absent Moro reflex at 8 months of age
Moro reflex at 3 months of age
Extensor reflex at 7 months of age
a.
b.
c.
d.
ANS: D
Establishing a diagnosis of cerebral palsy (CP) may be confirmed with the persistence of
primitive reflexes: (1) either the asymmetric tonic neck reflex or persistent Moro reflex
(beyond 4 months of age) and (2) the crossed extensor reflex. The tonic neck reflex normally
disappears between 4 and 6 months of age. The crossed extensor reflex, which normally
disappears by 4 months, is elicited by applying a noxious stimulus to the sole of one foot with
the knee extended. Normally, the contralateral foot responds with extensor, abduction, and
then adduction movements. The possibility of CP is suggested if these reflexes occur after 4
months.
PTS: 1
REF: 1091
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.
PTS: 1
REF: 1102
17. The nurse is admitting a school-age child with suspected Guillain-Barr syndrome (GBS).
Which is a priority in the care for this child?

Monitoring intake and output
Assessing respiratory efforts
Placing on a telemetry monitor
Obtaining laboratory studies
a.
b.
c.
d.
ANS: B
Treatment of GBS is primarily supportive. In the acute phase, patients are hospitalized
because respiratory and pharyngeal involvement may require assisted ventilation, sometimes
with a temporary tracheotomy. Treatment modalities include aggressive ventilatory support in
the event of respiratory compromise, intravenous (IV) administration of immunoglobulin
(IVIG), and sometimes steroids; plasmapheresis and immunosuppressive drugs may also be
used. Intake and output, telemetry monitoring and obtaining laboratory studies may be part of
the plan of care but are not the priority.
PTS: 1
REF: 1110
18. The nurse is caring for an intubated infant with botulism in the pediatric intensive care unit.
Which health care provider prescriptions should the nurse clarify with the health care provider
before implementing?
a. Administer 250 mg botulism immune globulin intravenously (BIG-IV) one time.
b. Provide total parenteral nutrition (TPN) at 25 ml/hr intravenously.
c. Titrate oxygen to keep pulse oximetry saturations greater than 92.
d. Administer gentamicin sulfate (Garamycin) 10 mg per intravenous piggyback every
12 hours.
ANS: D
The nurse should clarify the administration of an aminoglycoside antibiotic. Antibiotic

therapy is not part of the management of infant botulism because the botulinum toxin is an
intracellular molecule, and antibiotics would not be effective; aminoglycosides in particular
should not be administered because they may potentiate the blocking effects of the neurotoxin.
Treatment consists of immediate administration of botulism immune globulin intravenously
(BIG-IV) without delaying for laboratory diagnosis. Early administration of BIG-IV
neutralizes the toxin and stops the progression of the disease. The human-derived botulism
antitoxin (BIG-IV) has been evaluated and is now available nationwide for use only in infant
botulism. Approximately 50% of affected infants require intubation and mechanical
ventilation; therefore, respiratory support is crucial, as is nutritional support because these
infants are unable to feed.
PTS: 1
REF: 1113-1114
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 adolescents 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. Placing a cool washcloth on the adolescents forehead, continuing to monitor blood
pressure and vital signs, and notifying the healthcare provider would not reverse the
sympathetic reflex situation.
PTS: 1
REF: 1115
MULTIPLE RESPONSE
1. A 14-year-old girl is in the intensive care unit after a spinal cord injury 2 days ago. Nursing
care for this child includes which action(s)? (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 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. It is not necessary to minimize
environmental stimuli for this type of injury. Discussing long-term care issues with the family
is inappropriate. The family is focusing on the recovery of their child. It will not be known
until the rehabilitation period how much function the child may recover.
PTS: 1
REF: 1115-1116
2. Which assessment findings should the nurse note in a school-age child with Duchenne
muscular dystrophy (DMD)? (Select all that apply.)

Lordosis
Gower sign
Kyphosis
Scoliosis
Waddling gait
a.
b.
c.
d.
e.
ANS: A, B, E
Difficulties in running, riding a bicycle, and climbing stairs are usually the first symptoms
noted in Duchenne muscular dystrophy. 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 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.
PTS: 1
REF: 1107
3. The nurse is conducting discharge teaching to parents of a preschool child with
myelomeningocele, repaired at birth, 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 childs 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 urinary tract infection.
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; (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.
PTS: 1
REF: 1101
4. Which should the nurse expect to find in the cerebral spinal fluid (CSF) results of a child with
Guillain-Barr syndrome (GBS)? (Select all that apply.)

Decreased protein concentration
Normal glucose
Fewer than 10 white blood cells (WBCs/mm3)
Elevated red blood cell (RBC) count
a.
b.
c.
d.
ANS: B, C
Diagnosis of GBS is based on clinical manifestations, CSF analysis, and EMG findings. CSF
analysis reveals an abnormally elevated protein concentration, normal glucose, and fewer than
10 WBCs/mm3. CSF fluid should not contain RBCs.
PTS: 1
REF: 1110
5. 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.
PTS: 1
REF: 1102
SHORT ANSWER
1. A toddler is admitted to the hospital with a possible diagnosis of tetanus. The health care
provider has prescribed lorazepam (Ativan) intravenously 0.05 mg/kg/dose every 6 hours prn
as a muscle relaxant. The child weighs 22 lb. How many milligrams of Ativan should the
nurse administer per dose? (Record your answer using one decimal place.)
ANS:
0.5
Find the childs weight in kilograms by dividing 22 by 2.2 = 22/2.2 = 10 kg. Multiply the
0.05 mg dose by 10 = 0.05 mg 10 kg = 0.5 mg per dose.
PTS: 1
REF: 1112

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Chapter 32

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Chapter 32: The Child with Neuromuscular or Muscular Dysfunction

palsy is characterized by:

childs spasticity. The nurses response should be based on which statement?

care nursery. Which describes this newborns defect?

A myelomeningocele is a visible defect with an external saclike protrusion, containing

myelomeningocele. Which common problem is associated with this defect?

Hydrocephalus is a frequently associated anomaly in 80% to 90% of children.

defect of myelomeningocele. Which common problem is associated with this defect?

Myelomeningocele is one of the most common causes of neuropathic (neurogenic) bladder

supplement should be included in the teaching?

parent would indicate a correct understanding of the teaching?

include which action?

Hyperactive deep tendon reflexes

dystrophy. The management plan should include which action?

Pseudohypertrophic (Duchenne) muscular dystrophy is inherited as an X-linked recessive

DIF: Cognitive Level: Understand

TOP: Integrated Process: Nursing Process: Implementation

should be reported as abnormal and considered as a possible sign of cerebral palsy?

Which is a priority in the care for this child?

The nurse should clarify the administration of an aminoglycoside antibiotic. Antibiotic

The adolescent is experiencing an autonomic dysreflexia episode. The paralytic nature of

muscular dystrophy (DMD)? (Select all that apply.)

Discharge teaching to prevent renal complications in a child with myelomeningocele include:

Guillain-Barr syndrome (GBS)? (Select all that apply.)

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