Neuromuscular

Question 1

Which nursing diagnosis is most relevant in the first 12 hours of life for a neonate born with a myelomeningocele?

a) Delayed growth and development
b) Risk for infection
c) Impaired physical mobility
d) Constipation

Answer 1

b) Risk for infection

All of these diagnoses are important for a child with a myelomeningocele. However, during the first 12 hours of life, the most life-threatening event would be an infection. The other diagnoses will be addressed as the child develops.

Question 2

The nurse is caring for a 2-year-old boy with cerebral palsy (CP). The medical record indicates “hypertonicity and permanent contractures affecting both extremities on one side.” Based on these findings, the nurse identifies this type of CP as which of the following?

a) Spastic hemiplegia
b) Ataxic
c) Spastic diplegia
d) Athetoid or dyskinetic

Answer 2

a) Spastic hemiplegia

Spastic hemiplegia involves hypertonicity and permanent contractures on both extremities on one side. Athetoid (dyskinetic) involves abnormal involuntary movements affecting all four extremities and sometimes the face, neck, and tongue. Ataxic affects balance and depth perception. Spastic diplegia affects the lower extremities.

Question 3

The nurse caring for an infant with myelomeningocele before surgical intervention will prioritize care in what way?

a) Prevent cold stress using an Isolette and blankets
b) Keep the mass uncovered and dry
c) Cover the sac with a saline-moistened dressing
d) Change position from side to side hourly

Answer 3

c) Cover the sac with a saline-moistened dressing

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.

Question 4

Why will it be necessary for the nurse to be very supportive of parents’ attempts to feed the infant with recently repaired myelomeningocele?

a) Nausea and vomiting often follow repair of the cystic mass.
b) The infant will have a poor sucking reflex.
c) Pain will interfere with the feeding process.
d) Assuming the usual feeding position will be difficult.

Answer 4

d) Assuming the usual feeding position will be difficult.

Because the repaired area will need to be protected, having to use an alternate feeding position is likely. The infant may need to be fed prone with the head turned to the side and may not be able to be held. Being able to provide food for the infant is central to parenting the child. Difficulty nurturing a child can be very stressful. Little pain will be experienced and should easily be controlled owing to loss of sensation in the area. The sucking reflex should not be affected by the myelomeningocele or its repair. Nausea and vomiting are unlikely after recovery from the anesthetic.

Question 5

A child with Duchenne muscular dystrophy is placed on prednisone & calcium. Parents view these meds as rather “common” & question their importance for the child. What explanation will be most helpful to the parents?

a) “Prednisone will help protect his vulnerable respiratory tract from developing reactive airway disease. Calcium is needed to guard against muscle cramping.”
b) “Prednisone will stimulate weight gain and appetite. Calcium is needed to ensure adequate supplies for the development of permanent teeth.”
c) “Prednisone will protect against nerve inflammation in his hips and legs. Calcium is necessary should dietary intake be insufficient to meet growth needs.”
d) “Prednisone helps to keep muscle fibers strong and delays progression of the disease. Calcium protects against osteoporosis caused by both prednisone and lack of weight bearing.”

Answer 5

d) “Prednisone helps to keep muscle fibers strong and delays progression of the disease. Calcium protects against osteoporosis caused by both prednisone and lack of weight bearing.”

Studies have shown that boys treated with prednisone have improved muscle strength and function. This is thought to be due to the protection that prednisone provides to muscle fibers. Calcium is needed to prevent osteoporosis, which is a side effect of prednisone that also occurs when weight bearing is limited. Respiratory infection is a risk in that those muscles weaken with progression of the disease, but reactive airway disease is not a particular risk. No peripheral nerve involvement is observed in Duchenne muscular dystrophy. Side effects of prednisone include weight gain and appetite stimulation, but these are not the reasons for the prednisone therapy. Calcium does augment dietary intake of the mineral and is important for tooth development, and it may play a role in prevention of muscle cramps, but these are not the main reasons for taking the calcium supplement.

Question 6

Which of the following strategies would be the first choice in attempting to maximize function in a child with muscular dystrophy?

a) Manual wheelchair
b) Motorized wheelchair
c) Walker
d) Long leg braces

Answer 6

d) Long leg braces

Long leg braces are functional assistive devices that provide increased independence and increased use of upper and lower body strength. Wheelchairs, both motorized and manual, provide less independence and less use of upper and lower body strength. Walkers are functional assistive devices that provide less independence than braces.

Question 7

A child is to undergo testing for suspected muscular dystrophy and is scheduled for the following tests. Which test would the nurse identify as most important to be completed first?

a) Electromyogram
b) Nerve conduction velocity
c) Muscle biopsy
d) Creatine kinase

Answer 7

d) Creatine kinase

The sample for creatine kinase must be obtained before the electromyogram or muscle biopsy because those tests may lead to a release of creatine kinase and provide false results. Nerve conduction velocity tests could be done at any time.

Question 8

A nurse who is discussing Duchenne muscular dystrophy characterizes it correctly using which descriptors?

a) Duchenne muscular dystrophy is diagnosed in boys who develop gait changes during the late school-age years.
b) Duchenne muscular dystrophy causes progressive muscular weakness that ends in death.
c) Duchenne muscular dystrophy is a nonprogressive disorder that severely affects muscle function through spinal cord atrophy.
d) Duchenne muscular dystrophy is a progressive disease of muscles and nerves that affects males and females equally.

Answer 8

b) Duchenne muscular dystrophy causes progressive muscular weakness that ends in death.

Duchenne muscular dystrophy is the most common of several muscular dystrophies and is a progressive, fatal disorder. It involves mainly skeletal muscles, but other muscles are affected over time. Onset occurs in early childhood. The disorder is X-linked recessive. An enzyme is lacking that is necessary for the maintenance of muscle cells. No structural abnormalities of the spinal cord or peripheral nerves are noted.

Question 9

Given knowledge of muscular dystrophy, the nurse would expect to see which form of this condition most commonly in children?

a) Myotonic
b) Becker’s
c) Limb-girdle
d) Duchenne’s

Answer 9

d) Duchenne’s

Duchenne’s accounts for 50% of all cases of muscular dystrophy.

Question 10

Based on knowledge of the progression of muscular dystrophy, which activity would a nurse anticipate the client having difficulty with first?

a) Breathing
b) Standing
c) Swallowing
d) Sitting

Answer 10

b) Standing

Muscular dystrophy usually affects postural muscles of the hip and shoulder first. Swallowing and breathing are usually affected last. Sitting may be affected, but a client would have difficulty standing before having difficulty sitting.

Question 11

The nurse is teaching a health and wellness course to young women of childbearing age. Which vitamin will the nurse encourage all to take daily?

a) Calcium
b) Ascorbic acid
c) Folic acid
d) Niacin

Answer 11

c) Folic acid

It is recommended that all women of childbearing age ingest 0.4 mg of folic acid daily. Ascorbic acid is vitamin C, niacin is a B vitamin, and calcium is a mineral, not a vitamin.

Question 12

Women attending a health and wellness event ask the presenting nurse what is meant by “neural tube defect.” Which is the accurate response?

a) “Neural tube defect refers to abnormal development of the brain and spinal cord in the fetus.”
b) “Neural tube defect refers to abnormal development of the brain and spinal cord in the embryo.”
c) “Neural tube defect refers to improper development of the digestive system in the fetus.”
d) “Neural tube defect refers to abnormal development of the genitourinary system in the embryo.”

Answer 12

b) “Neural tube defect refers to abnormal development of the brain and spinal cord in the embryo.”

The neural tube from which the brain and spinal cord develop closes between the third and fourth weeks of gestation in the embryonic period of development. Failure of the neural tube to close properly results in most congenital anomalies of the central nervous system.

Question 13

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 examination?

a) Move on to other assessments without calling attention to the difference
b) Inspect for precocious hair growth in the genital and underarm areas
c) Record and refer the finding for follow-up to the pediatrician
d) Snip the tuft of hair off close to the skin for hygienic reasons

Answer 13

c) Record and refer the finding for follow-up to the pediatrician

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.

Question 14

The nurse is assessing a child with spina bifida occulta. During the assessment, the parents say, “It’s going to be so difficult taking care of our child. He’ll never be able to walk.” The nurse identifies which nursing diagnosis as the priority?

a) Impaired physical mobility related to spinal cord defect
b) Deficient knowledge related to diagnosis and condition
c) Risk for injury related to lack of muscle control
d) Ineffective coping related to diagnosis of chronic condition

Answer 14

b) Deficient knowledge related to diagnosis and condition

The parents’ statement indicates a lack of understanding about the condition. Spina bifida is a term that is often used to refer to all neural tube disorders that affect the spinal cord. This can be confusing and a cause of concern for parents. There are well-defined degrees of spinal cord involvement, and it is important for healthcare professionals to use the correct terminology. Spina bifida occulta is a defect of the vertebral bodies without protrusion of the spinal cord or meninges. This defect is not visible externally and in most cases has no adverse affects. In most cases, spina bifida occulta is benign and asymptomatic and produces no neurologic signs; it may be considered a normal variant. Mobility typically is not impaired with spina bifida occulta. The child is at no greater risk for injury as any other child. The parents demonstrate a lack of knowledge, not problems with coping.

Question 15

The nurse is assessing a child with spastic cerebral palsy. Which of the following would the nurse expect to assess? Select all that apply.

a) Hypertonicity
b) Poor control of balance
c) Hemiplegia
d) Dysarthria
e) Drooling
f) Exaggerated deep tendon reflexes

Answer 15

a) Hypertonicity
b) Poor control of balance
c) Hemiplegia
f) Exaggerated deep tendon reflexes

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.

Question 16

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) Presence of high maternal levels of albumin after 12th week of gestation
c) Significant level of alpha-fetoprotein present in amniotic fluid
d) Amniocentesis for lecithin-sphingomyelin (L/S) ratio

Answer 16

c) Significant level of alpha-fetoprotein present in amniotic flui

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.

Question 17

Why does the nurse suspect cerebral palsy in the 8-month-old just assessed?

a) The baby drools almost constantly.
b) The child sits independently with a straight back.
c) When startled, a strong Moro reflex is noted.
d) The child does not crawl.

Answer 17

c) When startled, a strong Moro reflex is noted.

The Moro reflex is a primitive one that should have disappeared around 4 months of age. Persistence of this reflex may occur in children with cerebral palsy. Older children with cerebral palsy often drool owing to the inability to control oral muscles. Drooling in an 8-month-old is developmentally normal, as is sitting independently with the back straight. It fits developmentally that the 8-month-old does not yet crawl.

Question 18

Which statement about cerebral palsy would be accurate?

a) “Cerebral palsy is a condition that runs in families.”
b) “Cerebral palsy occurs because of too much oxygen to the brain.”
c) “Cerebral palsy is a condition that doesn’t get worse.”
d) “Cerebral palsy means there will be many disabilities.”

Answer 18

c) “Cerebral palsy is a condition that doesn’t get worse.”

By definition, cerebral palsy is a nonprogressive neuromuscular disorder. It can be mild or quite severe and is believed to be the result of a hypoxic event during pregnancy or the birth process and doesn’t run in families.

Question 19

Which condition would alert the nurse that a child may be suffering from muscular dystrophy?

a) Hyperactive lower extremity reflexes
b) Hypertonia of extremities
c) Upper extremity spasticity
d) Increased lumbar lordosis

Answer 19

d) Increased lumbar lordosis

An increased lumbar lordosis would be seen in a child suffering from muscular dystrophy secondary to paralysis of lower lumbar postural muscles. Increased lower extremity support may also be seen. Hypertonia isn’t seen in this disease. Upper extremity spasticity isn’t seen because this disease isn’t caused by upper motor neuron lesions. Hyperactive reflexes aren’t indications of muscular dystrophy.

Question 20

Muscular dystrophy is a result of which cause?

a) Genetic and environmental factors
b) Unknown nongenetic origin
c) Chromosomal aberration
d) Gene mutation

Answer 20

d) Gene mutation

Muscular dystrophy is a result of a gene mutation. It isn’t from a chromosome aberration or environmental factors. It’s genetic and there’s a known origin of the disease.

Question 21

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) Central venous catheter
b) Botulinum toxin
c) Baclofen pump
d) Vagal nerve stimulator

Answer 21

c) Baclofen pump

A baclofen pump can be placed surgically to deliver continuous medication intrathecally. Baclofen can also be taken orally. Botulinum toxin is injected by a practioner 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.

Question 22

The nurse receives a report on a child admitted with severe muscular dystrophy. The nurse suspects the child has been diagnosed with the most severe form of the disease, known as:

a) limb-girdle
b) facioscapulohumeral
c) Duchenne’s
d) myotonia

Answer 22

c) Duchenne’s

Studies have shown that Duchenne’s is the most severe form of muscular dystrophy. Myotonia isn’t a form of the disease; it’s a symptom.

Question 23

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) Diminished breath sounds
b) Pulse oximetry
c) Serial measurement of tidal volume
d) Ineffective cough

Answer 23

c) Serial measurement of tidal volume

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.

Question 24

The pediatric nurse practioner (PNP) records “positive Gowers’ sign” after finishing the assessment of a young boy. How will the student nurse reading the PNP’s note interpret this?

a) The boy rises from the floor by walking his hands up his legs.
b) The head is held tilted with limited side-to-side motion.
c) The boy has a large tan skin lesion on his torso.
d) Severe lordosis is evident in the lumbar spine.

Answer 24

a) The boy rises from the floor by walking his hands up his legs.

Gowers’ sign is a hallmark finding of Duchenne muscular dystrophy as muscles weaken. The boy cannot rise from the floor in the usual way and needs to turn to hands and knees, move feet under the body, and “walk” hands up his legs to stand. The other options do not describe Gowers’ sign, although lordosis is often a manifestation of Duchenne muscular dystrophy.

Question 25

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) Stress management techniques b) Establishment of plans for rest periods c) Ways to increase the temperature of the child's environment d) Signs and symptoms of infection e) How to administer anticholinergic drugs

Answer 25

a) Stress management techniques b) Establishment of plans for rest periods d) Signs and symptoms of infection e) How to administer anticholinergic drugs 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.

Question 26

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) Careful supine positioning d) Listening for a shrill cry

Answer 26

a) Inspection of the cystic sac on the child’s back for leakage 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.

Question 27

Which characteristic is true of cerebral palsy? a) It’s progressive. b) It results in mental retardation. c) It appears at birth or during the first 2 years of life. d) It’s reversible.

Answer 27

c) It appears at birth or during the first 2 years of life. Cerebral palsy is an irreversible, nonprogressive disorder that results from damage to the developing brain during the prenatal, perinatal, or postnatal period. Although some children with cerebral palsy are mentally retarded, many have normal intelligence.

Question 28

The child with a surgically repaired myelomeningocele has a neurogenic bladder. How will the nurse best explain this problem to the parents? ## Footnote a) “Old urine remains in the bladder because of poor emptying or overfilling, putting your child at risk for urinary tract infection.” b) “Your child dribbles urine because the bladder either is overactive, pushing urine out, or is not active enough, becoming overfilled and causing urine to leak.” c) “While your child is young, urine leaking from the bladder will not be a problem because diapering is expected.” d) “Your child cannot properly control holding urine or emptying the bladder. “

Answer 28

a) “Old urine remains in the bladder because of poor emptying or overfilling, putting your child at risk for urinary tract infection.” Parents need to understand that lack of urinary control is not the greatest problem. The larger threat is of urinary tract infection, which can result in kidney damage. Only one of the responses by the nurse carries the infection message. Continence is important. This along with the infection risk can be managed by clean intermittent catheterization (CIC) or other procedures.

Question 29

A teenager has been admitted to the hospital with respiratory complications related to Duchenne muscular dystrophy. How can the nurse best provide support for the parents, who are the caretakers of this adolescent? a) Provide accommodations for both parents to room-in with their teen b) Encourage the parents to assist their child with his activities of daily living while hospitalized c) Teach the parents how to add chest physical therapy to the care they provide d) Assume responsibility for the teen’s daily care while accepting input from parents and the teen

Answer 29

d) Assume responsibility for the teen’s daily care while accepting input from parents and the teen The parents have probably been caring daily for their son for longer than 10 years while his condition has deteriorated and his care has become more complex. They may use this hospitalization as an opportunity to be relieved briefly of some of this responsibility (respite). Chest PT and rooming-in are important but do not address the need for respite.

Question 30

Which type of spinal neural tube defect does the nurse recognize as common and usually benign? a) Spina bifida occulta b) Meningocele c) Spina bifida d) Myelomeningocele

Answer 30

a) Spina bifida occulta Spina bifida occulta usually is benign and is estimated to affect 20% of the population. It is a defect in the vertebral body without protrusion of the spinal cord or its coverings. Spina bifida is a general term that is often used to refer to all neural tube disorders of the spinal cord. Meningocele and myelomenigocele do involve protrusion of elements of the spinal portion of the central nervous system and require treatment.

Question 31

What reflex response will the nurse consider abnormal when assessing the 8-month-old infant? a) Brisk deep tendon b) Plantar grasp c) Parachute d) Babinski

Answer 31

a) Brisk deep tendon By 8 months, the infant should have +2 or average deep tendon reflexes. The protective parachute reflex would have developed between 6 and 7 months. The plantar grasp will remain until about 9 months and the Babinski until 12 months.

Question 32

In caring for the child with Guillain-Barré syndrome, the nurse will provide much supportive care while watching carefully for signs of deterioration in which body system? a) Cardiovascular b) Urinary c) Integumentary d) Respiratory

Answer 32

d) Respiratory Guillain-Barré is a life-threatening disease; the greatest risk occurs during the acute stage, when respiratory failure may occur. The child with this syndrome will be ill and will have limited mobility for an extended time. All body systems will be stressed, requiring supportive care.

Question 33

A child with cerebral palsy is referred for physical therapy. When describing the rationale for this therapy, the nurse would emphasize which of the following as the primary goal? a) Development of fine motor skills b) Development of gross motor movement c) Promote optimal self-care ability d) Enhance feeding capabilities

Answer 33

b) Development of gross motor movement Physical therapy focuses on assisting in the development of gross motor movements such as walking and positioning and helps the child develop independent movement. Occupational therapy assists in the development of fine motor skills and fashioning orthotics and splints. Occupational therapy assists the child in performing optimal self-care ability by working on skills such as activities of daily living. Speech therapy assists with feeding techniques for the child who has swallowing problems.

Question 34

How would the nurse best describe Gowers’ sign to the parents of a child with muscular dystrophy? a) A waddling-type gait b) The pelvis position during gait c) A transfer technique d) Muscle twitching present during a quick stretch

Answer 34

c) A transfer technique 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.