- autoimmune disorder caused by antibodies against the voltage-sensitive calcium channels of the motor nerve terminals
- males more than female, with underlying malignancy (majority small lung lung CA)
- typically presents with weakness improved with exercise
- reduced/absent reflex (cardinal sign), because less transmitter is released, but reflexes may increase after maximum voluntary contraction (post-tetanic potentiation)
- also presents with autonomic symptoms (dry mouth)
- nerve conduction studies: compound motor action potential have small amplitude which after exercise or with repetitive stimulation will immediately increase by 100%
- voltage gated calcium channel antibodies are seen in almost 100% of LEMS patient with cancer and 90% of LEMS patient without cancer
- treatment: 3-4 diaminopyridine 20mg 5 times/day (potassium channel blocker, which helps maintain depolarisation of the nerve terminal by preventing repolarisation, leading to more calcium enter nerve terminal) or IVIG
Very well written and informative article, thank you :-)
ReplyDeleteTOSHIBA PLT-805AT