neruo-myopathies

neuogenic

spontanous actions, can have re-inervation

sensory/motor can be affected

myopathic

no activity,
proximal limb weak
reduces # of muscle fibers

fibrillations
fasculatiosn

invisible twiches
visib twiches

ALS

UMN n LMN
adult-onset

if LMN signs - then earlier onset, and spinal atrophy

UMN - babskin, hoff (hand)

ALS (what does spinal cord look like)

harden SPinal cord
bc astrocytes and scarring

familial ALS

painless weakness, arms/legs- hyper-reflex
wallerian deng, Riluloze drug, blocks GLUTAMATergic transmission

Peripherial N disease

Neuopahtie
parathesias, abn sensory.
remove offending factor

myopathic disorder

progessiove muscle weakness & wasteing, muscle inntervation loss

DMD

XR rapid,
dystrophin gene mutated
3-6 yr/o
cardiac , CK MM up,
pseudohypertrop

Becker

onset later, less cardiac
remain ACTIVE (less active more wasting)

glucocorticoids

Myotonic

slowww, AD
20-40 yrs
cataracs, test atrophy, hair loss

myotonic congenita

muscle stiffness, exchaerbaceted by cold

limb girgle

men and w around shuold and hips

pseudo-calves
no pain, no mental change
hard to get up, climb stairs
20-30 yrs full progeression