I-17 Functional evaluation of muscle impairment in neuromuscular disorders

The early pathophysiologic study showed increasing evidence that autoimmunity is implicated in the pathogenesis of neuromyotonia. Antibodies to voltage gated potassium channel were detected in the serum of patients who had peripherical nerves hyperexcitability and also Morvan’s disease or limbic encephalitis. These discoveries offered new approaches to treatments. Recently, antibodies previously attributed to VGKC recognise 2 surface antigens LGI1 and CASPR2 into the VGKC complex. Finally, VGKC antibodies are directed to 2 proteins the first one is a key hippocampic protein containing pre and post synaptic proteins. The second one CASPR2 is an hippocampic and paranodal protein. There clinical significance is different: hyperexcitability limbic encephalitis without thymoma for LGI1, hyperexcitability Morvan limbic encephalitis and frequent thymoma for CASPR2. In conclusion, the term NMT LE VGKC should be changed to NMTLE with LGI1 and CASPR2 antibodies and classified as auto immune synaptic disorders.