Chapter 30: The Child with Neuromuscular or Muscular Dysfunction Hockenberry: Wong's Essentials of Pediatric Nursing, 10th Edition

The nurse is planning a staff in-service on childhood spastic cerebral palsy. What

characterizes spastic cerebral palsy?

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.

DIF: Cognitive Level: Understand REF: p. 978

TOP: Integrated Process: Teaching/Learning

MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation

The parents of a child with cerebral palsy ask the nurse whether any drugs can decrease

their child's spasticity. The nurse's 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.

DIF: Cognitive Level: Understand REF: p. 979

TOP: Integrated Process: Teaching/Learning

MSC: Area of Client Needs: Physiologic Integrity: Pharmacologic and Parenteral Therapies

The nurse is reviewing prenatal vitamin supplements with an expectant client. Which

supplement should be included in the teaching?

a. Vitamin A throughout pregnancy

b. Multivitamin preparations as soon as pregnancy is suspected

c. Folic acid for all women of childbearing age

d. Folic acid during the first and second trimesters of pregnancy

c. Folic acid for all women of childbearing age

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.

DIF: Cognitive Level: Understand REF: p. 988

TOP: Integrated Process: Nursing Process: Implementation

MSC: Area of Client Needs: Physiologic Integrity: Pharmacologic and Parenteral Therapies

Which clinical manifestations in an infant would be suggestive of spinal muscular

atrophy (Werdnig-Hoffmann disease)?

a. Hyperactive deep tendon reflexes

b. Hypertonicity

c. Lying in the frog position

d. Motor deficits on one side of body

c. Lying in the frog position

A 4-year-old child has just been diagnosed with pseudohypertrophic (Duchenne)

muscular dystrophy. The management plan should include which action?

a. Recommend genetic counseling.

b. Explain that the disease is easily treated.

c. Suggest ways to limit use of muscles.

d. Assist family in finding a nursing facility to provide child's care.

a. Recommend genetic counseling.

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 the child's 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.

DIF: Cognitive Level: Understand REF: p. 992

TOP: Integrated Process: Nursing Process: Implementation

MSC: Area of Client Needs: Physiologic Integrity: Reduction of Risk Potential

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?

a. Tonic neck reflex at 5 months of age

b. Absent Moro reflex at 8 months of age

c. Moro reflex at 3 months of age

d. Extensor reflex at 7 months of age

d. Extensor reflex at 7 months of age

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.

DIF: Cognitive Level: Apply REF: p. 979

TOP: Integrated Process: Nursing Process: Implementation

MSC: Area of Client Needs: Physiologic Integrity: Reduction of Risk Potential

The nurse is admitting a school-age child with suspected Guillain-Barré syndrome

(GBS). Which is a priority in the care for this child?

a. Monitoring intake and output

b. Assessing respiratory efforts

c. Placing on a telemetry monitor

d. Obtaining laboratory studies

b. Assessing respiratory efforts

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.

DIF: Cognitive Level: Analyze REF: p. 996

TOP: Integrated Process: Nursing Process: Evaluation

MSC: Area of Client Needs: Physiologic Integrity: Reduction of Risk Potential

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.

DIF: Cognitive Level: Apply REF: p. 1000

TOP: Integrated Process: Nursing Process: Implementation

MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation

Which assessment findings should the nurse note in a school-age child with Duchenne

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

a. Lordosis

b. Gower 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

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.

DIF: Cognitive Level: Apply REF: p. 992

TOP: Integrated Process: Nursing Process: Assessment

MSC: Area of Client Needs: Physiologic Integrity: Reduction of Risk Potential

Which should the nurse expect to find in the cerebral spinal fluid (CSF) results of a

child with Guillain-Barré syndrome (GBS)? (Select all that apply.)

a. Decreased protein concentration

b. Normal glucose

c. Fewer than 10 white blood cells (WBCs/mm3)

d. Elevated red blood cell (RBC) count

b. Normal glucose

c. Fewer than 10 white blood cells (WBCs/mm3)

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.

DIF: Cognitive Level: Understand REF: p. 996

TOP: Integrated Process: Nursing Process: Evaluation

MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation