Pure motor neurological disease Flashcards
Pure motor syndrome with fluctuating ptosis, diplopia, bulbar symptoms and limb weakness should make you think
NMJ
Fluctuating and fatiguable weakness
Myesthenia
tensilon test
edrophonium - boosts Ach improvement in response
Repetitive nerve stimulation
stimulate the nerve at low frequency
decremental response with recurrent stimulation
Managing myethenia graivs
1) symptomatic therapy
1- AntiAchesterase inhibitor
2- Plasma exchange
3- IVIG
Managing myethenia graivs
1) Disease modifying therapy
- steroids - prednisone
- steroid-sparing agents
- thymectomy
MG disease prognosis - today’s therapy
mortality < 5%
A pure motor syndrome with proximal weakness should make you think
myopathy
- hyperthyroid - graves
What investigations should be carried out for a myopathy?
- CK
- TSH
- ESR/CRP
- ANA
Progressive!
PURE MOTOR
Mixed upper and lower motor neuron
bulbar and limb muscles
Diffuse motor neuron disorder