Pediatric Neuromuscular Dysfunction Flashcards
What is cerebral palsy?
Disorder of posture and movement from static brain injury perinatally or postnatally
What is the most common permanent physical disability in childhood?
cerebral palsy
What are associated impairments that are seen with cerebral palsy?
Seizures, hearing/vision, attentional, behavioral, communicative, cognitive, oral motor, speech
Why is it difficult to diagnose cerebral palsy at a young age?
lack of reliable neonatal neurological signs
What is a strong predictor when diagnosing cerebral palsy?
MRI
What are examples of therapeutic management of cerebral palsy?
Mobilizing devices
Surgery
Medication
Technical aids
When do neural tube defects develop?
early embryo development, GA 3-4 weeks
What are 3 of the main types of neural tube defects?
anencephaly, spinal bifida, myelomeningocele
What is a large reason that NTDs may occur?
Low folate levels
What is anencephaly?
Both cerebral hemispheres absent
What is myelodysplasia?
any malformation of spinal canal or cord
What is spina bifida?
midline defect involving failure of the osseous spine to close
Where is spina bifida occulta usually found?
Between L4 and L5
What does spina bifida cystica look like?
external sac-like protrusion
Does meningocele cause a neurological deficit?
no
What accounts for 90% of spinal cord lesions?
Myelomeningocele
Where is myelomeningocele found?
May be anywhere along the spinal column
Lumbar and lumbosacral areas are the most common, about 75%
What kind of allergy is a patient with a myelomeningocele at high risk for?
Latex
What is important to remember when treating a patient with myelomeningocele?
Many patients with this defect have a neurogenic bladder. Upwards of 90% of children will have some from of voiding dysfunction
What are the clinical manifestations of Guillain-Barré syndrome?
Paralysis rapidly ascends from the lower extremities; may involve the trunk, arms, and face
Hallmark sign is acute peripheral motor weakness
What is the pathophysiology behind Guillain-Barré syndrome?
Immune mediated disease
Associated with viral or bacterial infections (within about 10 days)
Administration of certain vaccines
Acute, plateau and recovery phases
What medications are used to treat Guillain-Barré syndrome?
Steroids, IVIG, plasmapheresis, immune suppressants, heparin (prevent DVT), stool softeners, analgesics, H2 blocker (prevent ulcer), gabapentin (chronic neuropathic pain)
Why are H2 receptors given for patients with Guillain-Barré syndrome?
to prevent ulcers
Why is gabapentin administered for patients with Guillain-Barré syndrome?
to treat chronic neuropathic pain
What is tetanus?
Exotoxin produced by the anaerobic, spore-forming, gram-positive bacillus Clostridium tetani
What is tetanus characterized by?
muscle rigidity
Where are tetanus spores found?
in soil, dust, intestinal tracts of humans and animals
How can tetanus be prevented?
Immunizations and boosters, wound care, prophylactic therapy
What can ingestion of honey cause in infants?
clostridium botulinum
What is the most common source of foodborne botulism?
improperly sterilized home-canned foods
When do CNS symptoms of botulism typically occur?
12-36 hours after ingestion
What is the most common reason for spinal cord injuries?
motor vehicle collision
What are 4 reasons that spinal cord injuries may occur?
Motor vehicle collision is most common
Sports injuries
Birth trauma
Nonaccidental trauma
What is the largest group of muscular disease in children?
Muscular dystrophies
What are the manifestations of muscular dystrophies?
Gradual degeneration of the muscle fibers
Progressive weakness
Wasting of the skeletal muscles
What is the most severe and most common form of muscular dystrophy?
Duchenne Muscular Dystrophy
What is another name for Duchenne Muscular Dystrophy?
pseudohypertrophic
When does Duchenne Muscular Dystrophy typically appear?
between 3 and 7 years
What is the Gower sign? When is it seen?
A child will kneel than gradually pull his torso upright (with knees straight) by “walking” his hands up his legs
What kind of genetic disorder is DMD?
X-linked recessive (2/3 of cases), 1/3 mother is not a carrier
What is the therapeutic management of DMD?
No curative treatment
Maintain optimum function in all muscles is the primary goal
Corticosteroids
What are complications of DMD?
Contractures, scoliosis, disuse atrophy, infections, obesity, cardiopulmonary complications
What are the reasons that death normally occurs for patients with DMD?
cardiac failure or respiratory tract infections
