motor neuron and radiculopathies Flashcards
infantile spinal musc atrophy
Au Rec, hypotonicity, hyporeflex, fatal, tongue fascic, poor suck reflex, abdominal resp
juvenile prox chronic spinal musc atrophy
Au Rec, slowly prog, fascic, prox weakness, resembles myopathy
adult onset spinal spinal musc atrophy
sporadic, prox, distal weakness, hypoton, hyporeflex
may see frog leg posture
infantile spinal musc atrophy
EMG for infantile spinal musc atrophy
signs of denervation and fascic
muscle bx for infantile spinal musc atrophy
group fiber atrophy
gene for infantile spinal musc atrophy
chrom 5 survival motor neuron gene
EMG and bx for juvenile spinal musc atrophy
denervation changes, serum CK usually normal
size of SMN2 determines
if pt has neonatal (smaller) or juvenile (larger). SNM1 is absent in spinal musc atrophy
progressive bulbar palsy
sporadic, fascic, bulbar muscle weakness, rapidly prog, tongue atrophy
ALS
mostly sporadic, hyperreflex, spasticitiy, musc atrophy, fascic, tongue atrophy, rapidly prog
progressive lateral sclerosis
sporadic, involves only UMN, spasticity, hyperreflex, more benign course than ALS
progressive spinal musc atrophy
mostly sporadic, progressive weakness, muscle atrophy, fascic, areflexia
muscle bx for motor neuron dz shows
atrophic fibers and fiber grouping
pts should always have ____ before diagnosing ALS
MRI
cervical spondylosis can mimic
ALS
Kennedy dz
gynecomastia, testicular atrophy, LMN syndrome. mut on x chrom. test DNA serum CK may be elevated
Bunina body
eosino inclusion seen in ant horn cell neuron - ubiquitin accum. ALS
ALS genes
SOD in AD familial - chrom 21; expanded section on chrom 9 - C90RF72
juvenile ALS
chrom 2
ALS associated with frontotemp dementia
chrom 17
ALS tx possibilities
glu antags - riluzole. Phys therapy. experimental antiox, nerve growth factors, COX2 inh.
tx ALS symptoms
antidep, bracing, gastrostomy, resp assistance, stool softener, botox, antispastic drugs, antichol for excess saliva
GBS (aka acute inflam demy polyneurop)
ascending motor weakness, absent muscle stretch reflex
