ALS, MG, and GBS Flashcards
ALS: which neuros are effected (U or L, motor or sensory)
UMN and LMN
ALS- epidemiology
mostly sporadic
some association with chromosome 21 gene for superoxide dismutase - leading to free radical injury
ALS- age of onset
40-60
ALS- signs and symptoms
focal asymetric weakness
UMN- babinski and spasticity
LMN- weakness, fasiculations, and atrophy
atrophy is very noticeable in the back of the hand.
Normal bladder and bowel function
ALS- EMG
fibrilations and fasiculations
enlarged amplitude b/c of haphazard reinervation
ALS- nerve conduction study
SENSORY NERVES ARE NORMAL
motor nerves are slow, amplitude drops as the dz progresses
ALS- tx
Riluzole- glutamate antagonist
ALS- prognosis
3-5 yr survival
die due to respiratory failure
Mysthinia Gravis- MG pathogenesis
Autoimmune rxn against AchR, type II HSR
MG epidemiology (sex and age)
men in 50s and 60s
women in teens and 20s
MG- signs and symptoms
hallmark is muscle fatigueability first finding is ptosis (cant hold eyelid up) followed by diplopia) neck muscles are affected dysphagia for solids and liquids Normal reflexes and coodination
MG- Dx
Edrophonium (tensilon test)- acute and short-lived reversal of symptoms
EMG- diminished response with repeated stimulation
Serum AcHR- only 85-90% of pts have this
MG- treatment
Physostigmine
prednisone
plasmophoresis- removes Ab
MG- role of the thymus
65-80% of pts with MG have thymic hyperplasia and 15% have thymoma.
Pts with MG should always get CXR to see if this is the case.
This makes Tx easy- thymectomy
GBS- epidemiology
most common acute peripheral neuropathy
