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328 Pediatrics Exam 2 > Neuromuscular/Muscular Dysfunction > Flashcards

Neuromuscular/Muscular Dysfunction Flashcards

1
Q

What is Cerebral Palsy (CP)?

A

group of permanent movement, balance, and posture disorders that appear in infancy or early childhood

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2
Q

Cerebral palsy (CP) risk factors?

A
  • Preterm birth
  • Low birth weight
  • CNS malformation
  • Asphyxia during birth
  • Intracranial hemorrhage after birth
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3
Q

Cerebral palsy (CP) clinical manifestations?

A
  • Delay in achieving developmental milestones
  • Difficulty swallowing or uncontrolled drooling
  • Poor sucking
  • Clenched fists beyond 3 months of age
  • Stiff or rigid limbs
  • Arching of back
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4
Q

Types of Cerebral palsy (CP)?

A
  • Spastic
  • Dyskinetic
  • Ataxic
  • Mixed
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5
Q

What is Spastic CP?

A

stiffness and tightness in muscles (most common type)

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6
Q

What is Dyskinetic CP?

A

involuntary, uncontrolled, repetitive movements

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7
Q

What is Ataxic CP?

A

incoordination with decreased muscle tone

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8
Q

What is Mixed CP?

A

symptoms from spastic, dyskinetic, and ataxic

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9
Q

Cerebral palsy (CP) management?

A
  • Medications
    • Botulinum toxin type A (Botox) - reduce spasticity to improve mobility and pain control
    • Baclofen (Reduce Spasms-Implanted pump for intrathecal administration –(into spinal canal)
  • Management of Seizures
  • Mobilization and Assistive Devices
  • Physical Therapy
  • Surgical interventions for contractures
  • Nutrition interventions
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10
Q

What are neural tube defects?

A
  • congenital anomalies due to incomplete closure of the neural tube (forms the brain and spinal cord)
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11
Q

Describe Spina Bifida Occulta.

A
  • Dimple or hair noted at the base of the spine. No protrusion of meninges.
  • Ultrasound to determine if there is any underlying problem.
  • Treatment is usually not needed.
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12
Q

Describe Meningocele.

A

protrusion of meninges, CSF, no nerves within a sac of skin

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13
Q

Describe Myelomeningocele.

A

protrusion of meninges, CSF, and nerves within sac of skin

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14
Q

Neural tube defects nursing management?

A
  • Side lying or prone position to protect defect
  • Prevent infection (cover w/ moist sterile dressing)
  • LATEX precautions- all spina bifida patients
  • Antibiotics
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15
Q

Neural tube defects therapeutic management?

A
  • Management of genitourinary function
  • Bowel control
  • Prevention of pressure ulcers
  • Promote mobility, PT/OT
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16
Q

What is Guillain Barre Syndrome (GBS)?

A
  • Rare autoimmune disorder where the body’s immune system attacks peripheral nervous system
  • Usually preceded by viral illness that induces an abnormal autoimmune response targeting peripheral nerves
17
Q

Guillain Barre Syndrome (GBS) etiology?

A

unknown

18
Q

Guillain Barre Syndrome (GBS) s/s?

A
  • Acute symmetric ascending paralysis that occurs 2-4 weeks after viral or bacterial illness
  • Gait abnormalities
  • Diminished or lost reflexes
  • Respiratory failure due to paralysis
19
Q

Guillain Barre Syndrome (GBS) management?

A
  • Treatment is supportive
  • Respiratory support
  • Steroids, IV immunoglobulin, and plasmapheresis (plasma exchange).
  • Low–molecular-weight heparin, gabapentin (neuropathic pain), analgesics
  • Risk of recurrence low
  • 10-20% of patients will require mechanical ventilation
20
Q

What is Duchenne Muscular Dystrophy (DMD)?

A

genetic disorder characterized by progressive muscle degeneration and weakness

21
Q

Who does Duchenne Muscular Dystrophy (DMD) affect more?

A

primarily affects boys (x-linked), typically becoming evident around ages 3 to 5

22
Q

Duchenne Muscular Dystrophy (DMD) signs and symptoms?

A
  • Progressive muscle weakness*, wasting, and contractures
  • Affects proximal before distal limb muscles, & lower extremities before upper extremities
  • Waddling gait, frequent falls, Gower’s sign
  • Pts. usually wheelchair bound by ~12 years of age
  • Average expectancy 28 years
23
Q

How is DMD diagnosed?

A
  • child’s blood sample and test it for creatine kinase
    • an enzyme that muscles release when they are damaged

high CK level is a sign that child could have DMD

24
Q

How to treat DMD?

A
  • steroid medications to maintain muscle strength
  • “exon skipping” medicine which increases the amount of dystrophin protein
  • gene therapy, replaces mutated dystrophin gene with a dystrophin gene that works

328 Pediatrics Exam 2 (7 decks)

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