Autoimmune limbic encephalitis and Morvan’s syndrome
Update:
Additional information on VGKC-complex antibodies.
Autoimmune limbic encephalitis—typical presentation is with acute or subacute onset of short term memory loss, seizures and disorientation. MRI characteristically shows striking abnormalities in the hippocampus. Antibodies against cell-surface proteins that are components of voltage-gated potassium channel (VGKC) complexes are found in a high proportion and are probably pathogenic. Aside from supportive care, treatment is with immunosuppression, often comprising corticosteroids with intravenous immunoglobulin and/or plasma exchange.
Morvan’s syndrome—a very rare condition presenting with varying degrees of neuromyotonia, memory loss, confusion, sleep disturbance, and autonomic features, also with antibodies to VGKC-complex proteins. About 50% have VGKC antibodies.
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