The Child with Neuromuscular or Muscular Dysfunction

Question 1

Cerebral Palsy

Answer 1

• CP is defined as a disorder of posture and movement from static brain injury perinatally or postnatally which limits activity
• 60% do not have any cognitive disability
• 40% have epilepsy • 50-60% can walk independently

Question 2

Causes of cerebral palsy: prenatal/first trimester

Answer 2

• Teratogens
• Genetic syndromes
• Chromosomal abnormalities
• Brain malformation

Question 3

Causes of cerebral palsy: 2nd-3rd trimester

Answer 3

• intrauterine infections
• Problems in fetal/ placental functioning
• Maternal thrombosis or hemorrhage

Question 4

Causes of cerebral palsy: labor and delivery

Answer 4

• preeclampsia
• Complications of labor and delivery

Question 5

Causes of cerebral palsy: perinatal

Answer 5

• Sepsis/CNS infection
• Kernicterus
• Hypoglycemia
• Asphyxia
• Prematurity** (most important determinant)

Question 6

Causes of cerebral palsy: childhood

Answer 6

• Meningitis
• Traumatic brain injury
• Toxins

Question 7

S/S: of cerebral palsy

Answer 7

• May involve one or both sides
• Hypertonicity with poor control of posture, balance, and coordination motion, persistent reflexes
• Impairment of fine and gross motor skills
• abnormal involuntary movement (Chorea)
• Athetosis, characterized by slow, wormlike, writhing movements that usually involve the extremities, trunk, neck, facial muscles, and tongue
• Involvement of the pharyngeal, laryngeal, and oral muscles causes drooling and dysarthria.
• Wide-based gait
• Rapid, repetitive movements performed poorly
• Disintegration of movements of the upper extremities when the child reaches for objects
• Delayed Gross Motor Development – is a universal manifestation of CP.
• *Abnormal Motor Performance – An early sign is preferential *unilateral hand use that may be apparent at approximately 6 months of age
• “Bunny hop”; spasticity may cause the child to stand or walk on the toes
• Uncoordinated or involuntary movements.
• *Alterations of Muscle Tone
• Abnormal Posture
• Reflex Abnormalities

Question 8

Cerebral palsy: Potential Disabilities and Problems

Answer 8

• Orthopedic complications
• Constipation
• Increased incidence of dental caries
• Malocclusion `
  -Gingivitis
• Nystagmus and amblyopia
• Hearing loss
• Intellectual impairment may be a concern (60% are within normal limits)
• Attention deficit-hyperactivity disorder (ADHD)
• Seizures
• Drooling
• Aspiration
• Inadequate gas exchange

Question 9

cerebral palsy: nursing Considerations

Answer 9

• ** Recognize early and promote optimum development**
• Address Health Maintenance Needs
  — Physical and Occupational therapy
  — Medication: baclofen (oral/ intrathecally - in CSF)
  — Drowsiness, weakness, constipation
  — Mobilizing devices
  — Speech Therapy
  — Surgery
  — Special Education
• Support Family
• Support Hospitalized Child

Question 10

Neural Tube Defects (NTD): umbrella term

Answer 10

• Failure of the neural tube to close during the third to fourth week gestation
• Anencephaly & Spina Bifida (Occulta (gap in spine but closed), Meningocele (gap with opening/ fluid) and Myelomengocele)
• Etiology: Low prenatal folic acid intake, maternal malnutrition, maternal drug use, exposure to chemicals or radiation
• Prevention: 50-70% can be prevented with 0.4mg daily folic acid for women of child bearing age

Question 11

Anencephaly

Answer 11

• The most serious NTD, both cerebral hemispheres are absent
• Have an intact brainstem, maintain vital functions, for a few hours die of respiratory failure.
• Provided comfort measures

Question 12

Spina Bifida Occulta

Answer 12

• 10% to 30% of the general population
• Superficial cutaneous indications, skin depression or dimple
• Portwine angiomatous nevi, dark tufts of hair, & soft, subcutaneous lipomas
• Nothing is protruding to the outside
• No issue

Question 13

Spina Bifida-Meningocele

Answer 13

• Sac which encases meninges and spinal fluid.
• No nerve involvement
• Protrusion through gap with CSF

Question 14

Myelomeningocele

Answer 14

• Detected prenatally or at birth
• Involves meninges, spinal fluid and nerves
• Lumbar or lumbosacral area 75%
• Hydrocephalus is common- 80-85%
• Chiari malformation type II: need shunt
• Protrusion with nerve involvement

Question 15

Initial Nursing Care: spina bifida

Answer 15

• • Thermoregulation
• • Moistening solution is usually sterile normal saline
• • Prone Positioning
• • Infection prevention
• Preparation for surgical closure: NPO, IV/fluids, consent
• Neurologic assessment: increased intercranial pressure

Question 16

Spina Bifida: Long Term Nursing Considerations

Answer 16

• Orthopaedic Considerations
• Musculoskeletal problems that will affect later locomotion should be evaluated early, scoliosis
• • Bowel and Bladder management issues
• Latex Allergy
• Hydrocephalus

Question 17

Duchenne Muscular Dystrophy

Answer 17

• Most severe and common MD
• X linked recessive trait defect
• 30% are new mutations
• Dystrophin (skeletal muscle protein is absent)

Question 18

Duchenne Muscular Dystrophy:
Clinical Manifestations

Answer 18

• Muscle weakness appears between 3- 7 years and is progressive
• Gross motor delays
• Calf muscle hypertrophy (enlarge)
• Contractures
• Mild to moderate mental impairment
• Immobilization complications
• Cardiopulmonary issues

Question 19

Gowers Sign**

Answer 19

The child stands by getting to knees, then walking hands up the legs then standing with lordosis

Question 20

Duchenne Muscular Dystrophy:
Nursing Management

Answer 20

• Corticosteroids to prolong ambulation, preserve respiratory function and decrease scoliosis and cardiomyopathy
• *Maintain muscle function is the primary goal and prevent contractures secondly- physical therapy
• Cardiopulmonary care: exercise
• Assist family with coping