07 Neuromuscular Dysfunction

Question 1

CP is a term that describes what?

Answer 1

A group of permanent disorders of the development of movement nad posture, causing activity limitation, that are attributed to non progressive disturbances that occurred in the developing fetal or infant brain.

Most common permanent physical disability in childhood

Question 2

What are some associated factors with CP?

Answer 2

Maternal Infections

Drug/alcohol use

Anemia

Rubella

ABO

Post natal causes like bacterial meningitis and traumatic brain injury

Question 3

KNOW: What is a primary goal of neuromuscular dysfunction?

Answer 3

A primary goal is to promote normal neuromuscular postures.

Promote nutrition, socialization, educational opportunities and integration of motor function

Question 4

What is Spina Bifida Occulta?

Answer 4

A type of NT defect. It’s a non visible vertebra defect with spinal cord and meninges intact.

Skin indicators: Sacral dimple, absence of that spine

Question 5

What is Spina Bifida Cystica?

Answer 5

Visible defect with external sac like protrusion

Question 6

What are two types of spina bifida cystica?

Answer 6

1. Meningocele
2. Meningomyelocele/Myelomeningocele

Question 7

What is a meningocele?

Answer 7

1. The sac has meninges and spinal fluid but no neural elements
2. Most kids have no neurologic deficiits because there are no nerves and no cord involvement

Question 8

What is a myelomeningocele?

Answer 8

1. Sac contains meninges, spinal fluid and nerves. Sac may be a fine membrane and prone to leakage of CSF, easily ruptured.

Question 9

What neurologic deficit occurs in myelomeningocele?

Answer 9

• Deficit and weakness is present below the defect, with bowel and bladder dysfunction.
• Clubfeet and disloacted hip and hydrocephalus may accompany. early treatment increases the chances for normal cognitive ability
• Bladder sphincter and muscle tone affected as result of defective nerve supply
• Fetal surgery may be an option

Question 10

What are some pre operative nursing care for myelomeningocele?

Answer 10

1. Protect sac from infection, sterile, moist and nonadherent dressings.
2. Proper position: prone, flat with hips slightly flexed, to reduce spinal fluid pressure
3. Monitor HC and fontanels for developing hydrocephalus

Question 11

What are some post operative treatment of myelomeningocele?

Answer 11

1. Observe for leakage of CSF

Question 12

What is the most common and severe muscular dystrophy?

Answer 12

Duchenne Muscular Dystrophy (DMD)

Question 13

how do you diagnose DMD?

Answer 13

Confirmed by EMG, muscle biopsy, and serum enzyme measurement

Question 14

What are some characteristics of DMD?

Answer 14

• —Early onset (3 to 5 years of age)
• —Progressive muscular weakness, wasting & contractures
• —Calf muscle hypertrophy (pseudohypertrophy)
• —Characterized by presence of Gower sign, waddling gait, and lordosis
• —Loss independent ambulation 9 to 12 years
• —Progression until death from respiratory & cardiac failure (adolescent/young adult)

Question 15

What is the primary goal when treating DMD?

Answer 15

Primary goal is to maintain function in unaffected muscles for as long as possible and promote ROM.

Question 16

What are other nursing considersations of DMD besides use of unaffected muscles as much as possible?

Answer 16

1. —Helping child and family cope with chronic, progressive debilitating disease
2. —Help design a program to foster independence and activity as long as possible
3. —Teach child self-help skills
4. —Appropriate healthcare assistance as child’s needs intensify (home health, skilled nursing facility, respite care for family, etc.)