Continuous muscle activity, Morvan's syndrome and limbic encephalitis: ionic or non ionic 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 peripheral nerves hyperexcitability and also Morvan's disease or limbic...

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Bibliographic Details
Published in:Acta myologica 2011-06, Vol.30 (1), p.32-33
Main Authors: Serratrice, G, Serratrice, J
Format: Article
Language:English
Subjects:
Autoantibodies - analysis
Humans
in Tribute of V. Askanas and K. Engel
Isaacs Syndrome - diagnosis
Isaacs Syndrome - immunology
Limbic Encephalitis - diagnosis
Limbic Encephalitis - immunology
Membrane Proteins - immunology
Nerve Tissue Proteins - immunology
Potassium Channels, Voltage-Gated - immunology
Proteins - immunology
Syringomyelia - diagnosis
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Summary: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 peripheral 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 NMT--LE with LGII and CASPR2 antibodies and classified as auto immune synaptic disorders. Mutations in genes encoding both these proteins are found in hereditary epilepsy and other syndromes. Various potassium channelopathies are closely linked to Morvan's syndromes. A new classification of antibodies will be necessary.
ISSN:1128-2460
1128-2460