Chronic Neuromuscular Disorders

Question 1

Cerebral Palsy

Answer 1

• Non-progressive neuromuscular disorder
• Oxygenation issue
• Can happen in utero or right after birth

Question 2

Cerebral Palsy - Assessment

Answer 2

• Thorough hx is reviewed
  > is child meeting milestones?
  > did child have traumatic delivery?
  > did mom have healthy pregnancy?
  > has child had any head trauma?
• Types of CP
  > spastic, athetoid, ataxic, mixed
  > depends on where injury to plave in brain

Question 3

Cerebral Palsy - Manifestations

Answer 3

• Extreme irritability & crying
• Feeding difficulties
• Alterations of muscle tone; stiff & rigis arms or legs
• Delayed gross motor development
  > not meeting milestones
• Persistence of primitive infantile reflexes (moro, tonic neck) after 6 months
  > most primitive reflexes disappear by 3-4 months of age
• Abnormal posturing, such as opisthotonos
  > exaggerated arching of back
• Seizures may occur

Question 4

Cerebral Palsy - Therapeutic Management

Answer 4

• Goal is early recognition & intervention to maximize child’s abilities
• PT, OT, speech, education, recreation
• Prepare for using mobilizing devices to help prevent or reduce deformities
• 30-50% also have some form of cogniti e impairment

Question 5

Cerebral Palsy - Medications

Answer 5

• May be prescribed to relieve muscle spasms & antiseizure meds
  > Baclofen: antispasmodic
  > Diazepam: muscle relaxant
  > Botulinum toxin A injections

Question 6

Neural Tube Defects

Answer 6

• Largest group of congenital anomalies
• Normally: spinal cord & cauda are encased in protective sheath of bone & meninges
• Failure of neural tube closure: produces defects of varying degrees

Question 7

Neural Tube Defects - Types

Answer 7

• Anencephaly
• Encephalocele
• Spina bifida occulta
• Spina bifida cystica
  > meningocele
  > myelomeningocele

Question 8

Neural Tube Defects - Patho

Answer 8

• Failure of neural tube to close during embryo’s early development
  > suppose to close approx 3-4wks after conception
• Multifactorial causes
• Genetic mutation
• Highest risk for neural tube defects:
  > lack of folic acid
• Additional Factors:
  > maternal obesity
  > maternal DM
  > low vit B12 lvl

Question 9

Neural Tube Defects - Postop Care Management

Answer 9

• Pre/post surgical intervention
• Support fam & educate abt home care
• Orthopedic problems
  > hips, back, legs, feet
  > incrd risk: clubfoot, bowel issues
• Bladder/bowel control
• latex allergies
• ICP signs
  > high pitched cry
  > drowsy
  > bulging fontanels

Question 10

Juvenile Idiopathic Arthritis - Description

Answer 10

• Autoimmune inflammatoru disease
• Impacts joints & other tissues
• No cure, not usually life threatening
• Can subside overtime but may have residual impaired joint function or deformities
• no definitive tests for assessment

Question 11

Juvenile Idiopathic Arthritis - Interventions

Answer 11

• Encourage normal performance of faily activities
• Minimize damage & preserve joint function - treatment is supportive
  > ROM
  > heat/ice
• Pain control
• Meds
  > steroids
  > opioids/non-opioids
• Eye care
  > incrd risk for uveitis due to inflamm & steroid use
  > make sure child has regular vision care