Pediatric Neuromuscular Dysfunction Flashcards

1
Q

What is cerebral palsy?

A

Disorder of posture and movement from static brain injury perinatally or postnatally

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2
Q

What is the most common permanent physical disability in childhood?

A

cerebral palsy

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3
Q

What are associated impairments that are seen with cerebral palsy?

A

Seizures, hearing/vision, attentional, behavioral, communicative, cognitive, oral motor, speech

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4
Q

Why is it difficult to diagnose cerebral palsy at a young age?

A

lack of reliable neonatal neurological signs

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5
Q

What is a strong predictor when diagnosing cerebral palsy?

A

MRI

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6
Q

What are examples of therapeutic management of cerebral palsy?

A

Mobilizing devices
Surgery
Medication
Technical aids

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7
Q

When do neural tube defects develop?

A

early embryo development, GA 3-4 weeks

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8
Q

What are 3 of the main types of neural tube defects?

A

anencephaly, spinal bifida, myelomeningocele

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9
Q

What is a large reason that NTDs may occur?

A

Low folate levels

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10
Q

What is anencephaly?

A

Both cerebral hemispheres absent

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11
Q

What is myelodysplasia?

A

any malformation of spinal canal or cord

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12
Q

What is spina bifida?

A

midline defect involving failure of the osseous spine to close

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13
Q

Where is spina bifida occulta usually found?

A

Between L4 and L5

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14
Q

What does spina bifida cystica look like?

A

external sac-like protrusion

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15
Q

Does meningocele cause a neurological deficit?

A

no

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16
Q

What accounts for 90% of spinal cord lesions?

A

Myelomeningocele

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17
Q

Where is myelomeningocele found?

A

May be anywhere along the spinal column
Lumbar and lumbosacral areas are the most common, about 75%

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18
Q

What kind of allergy is a patient with a myelomeningocele at high risk for?

A

Latex

19
Q

What is important to remember when treating a patient with myelomeningocele?

A

Many patients with this defect have a neurogenic bladder. Upwards of 90% of children will have some from of voiding dysfunction

20
Q

What are the clinical manifestations of Guillain-Barré syndrome?

A

Paralysis rapidly ascends from the lower extremities; may involve the trunk, arms, and face
Hallmark sign is acute peripheral motor weakness

21
Q

What is the pathophysiology behind Guillain-Barré syndrome?

A

Immune mediated disease
Associated with viral or bacterial infections (within about 10 days)
Administration of certain vaccines
Acute, plateau and recovery phases

22
Q

What medications are used to treat Guillain-Barré syndrome?

A

Steroids, IVIG, plasmapheresis, immune suppressants, heparin (prevent DVT), stool softeners, analgesics, H2 blocker (prevent ulcer), gabapentin (chronic neuropathic pain)

23
Q

Why are H2 receptors given for patients with Guillain-Barré syndrome?

A

to prevent ulcers

24
Q

Why is gabapentin administered for patients with Guillain-Barré syndrome?

A

to treat chronic neuropathic pain

25
Q

What is tetanus?

A

Exotoxin produced by the anaerobic, spore-forming, gram-positive bacillus Clostridium tetani

26
Q

What is tetanus characterized by?

A

muscle rigidity

27
Q

Where are tetanus spores found?

A

in soil, dust, intestinal tracts of humans and animals

28
Q

How can tetanus be prevented?

A

Immunizations and boosters, wound care, prophylactic therapy

29
Q

What can ingestion of honey cause in infants?

A

clostridium botulinum

30
Q

What is the most common source of foodborne botulism?

A

improperly sterilized home-canned foods

31
Q

When do CNS symptoms of botulism typically occur?

A

12-36 hours after ingestion

32
Q

What is the most common reason for spinal cord injuries?

A

motor vehicle collision

33
Q

What are 4 reasons that spinal cord injuries may occur?

A

Motor vehicle collision is most common
Sports injuries
Birth trauma
Nonaccidental trauma

34
Q

What is the largest group of muscular disease in children?

A

Muscular dystrophies

35
Q

What are the manifestations of muscular dystrophies?

A

Gradual degeneration of the muscle fibers
Progressive weakness
Wasting of the skeletal muscles

36
Q

What is the most severe and most common form of muscular dystrophy?

A

Duchenne Muscular Dystrophy

37
Q

What is another name for Duchenne Muscular Dystrophy?

A

pseudohypertrophic

38
Q

When does Duchenne Muscular Dystrophy typically appear?

A

between 3 and 7 years

39
Q

What is the Gower sign? When is it seen?

A

A child will kneel than gradually pull his torso upright (with knees straight) by “walking” his hands up his legs

40
Q

What kind of genetic disorder is DMD?

A

X-linked recessive (2/3 of cases), 1/3 mother is not a carrier

41
Q

What is the therapeutic management of DMD?

A

No curative treatment
Maintain optimum function in all muscles is the primary goal
Corticosteroids

42
Q

What are complications of DMD?

A

Contractures, scoliosis, disuse atrophy, infections, obesity, cardiopulmonary complications

43
Q

What are the reasons that death normally occurs for patients with DMD?

A

cardiac failure or respiratory tract infections