Pediatric Neuromuscular Dysfunction

Question 1

What is cerebral palsy?

Answer 1

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

Question 2

What is the most common permanent physical disability in childhood?

Answer 2

cerebral palsy

Question 3

What are associated impairments that are seen with cerebral palsy?

Answer 3

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

Question 4

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

Answer 4

lack of reliable neonatal neurological signs

Question 5

What is a strong predictor when diagnosing cerebral palsy?

Answer 5

MRI

Question 6

What are examples of therapeutic management of cerebral palsy?

Answer 6

Mobilizing devices
Surgery
Medication
Technical aids

Question 7

When do neural tube defects develop?

Answer 7

early embryo development, GA 3-4 weeks

Question 8

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

Answer 8

anencephaly, spinal bifida, myelomeningocele

Question 9

What is a large reason that NTDs may occur?

Answer 9

Low folate levels

Question 10

What is anencephaly?

Answer 10

Both cerebral hemispheres absent

Question 11

What is myelodysplasia?

Answer 11

any malformation of spinal canal or cord

Question 12

What is spina bifida?

Answer 12

midline defect involving failure of the osseous spine to close

Question 13

Where is spina bifida occulta usually found?

Answer 13

Between L4 and L5

Question 14

What does spina bifida cystica look like?

Answer 14

external sac-like protrusion

Question 15

Does meningocele cause a neurological deficit?

Answer 15

no

Question 16

What accounts for 90% of spinal cord lesions?

Answer 16

Myelomeningocele

Question 17

Where is myelomeningocele found?

Answer 17

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

Question 18

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

Answer 18

Latex

Question 19

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

Answer 19

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

Question 20

What are the clinical manifestations of Guillain-Barré syndrome?

Answer 20

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

Question 21

What is the pathophysiology behind Guillain-Barré syndrome?

Answer 21

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

Question 22

What medications are used to treat Guillain-Barré syndrome?

Answer 22

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

Question 23

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

Answer 23

to prevent ulcers

Question 24

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

Answer 24

to treat chronic neuropathic pain

Question 25

What is tetanus?

Answer 25

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

Question 26

What is tetanus characterized by?

Answer 26

muscle rigidity

Question 27

Where are tetanus spores found?

Answer 27

in soil, dust, intestinal tracts of humans and animals

Question 28

How can tetanus be prevented?

Answer 28

Immunizations and boosters, wound care, prophylactic therapy

Question 29

What can ingestion of honey cause in infants?

Answer 29

clostridium botulinum

Question 30

What is the most common source of foodborne botulism?

Answer 30

improperly sterilized home-canned foods

Question 31

When do CNS symptoms of botulism typically occur?

Answer 31

12-36 hours after ingestion

Question 32

What is the most common reason for spinal cord injuries?

Answer 32

motor vehicle collision

Question 33

What are 4 reasons that spinal cord injuries may occur?

Answer 33

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

Question 34

What is the largest group of muscular disease in children?

Answer 34

Muscular dystrophies

Question 35

What are the manifestations of muscular dystrophies?

Answer 35

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

Question 36

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

Answer 36

Duchenne Muscular Dystrophy

Question 37

What is another name for Duchenne Muscular Dystrophy?

Answer 37

pseudohypertrophic

Question 38

When does Duchenne Muscular Dystrophy typically appear?

Answer 38

between 3 and 7 years

Question 39

What is the Gower sign? When is it seen?

Answer 39

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

Question 40

What kind of genetic disorder is DMD?

Answer 40

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

Question 41

What is the therapeutic management of DMD?

Answer 41

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

Question 42

What are complications of DMD?

Answer 42

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

Question 43

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

Answer 43

cardiac failure or respiratory tract infections