Autoantibodies in the CNS
Encephalopathy and potassium channelopathy
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Much has been made of gene mutations and the emerging field of channelopathies. The analysis of ion channel mutations not only provides insight into the underlying molecular mechanisms of disease but also may identify target molecules for therapeutic intervention. Moreover, the phenotypic consequences of an ion channel mutation often provide insight into its role in normal cellular function. It is important to remember that channelopathies may also be autoimmune in origin. In these cases, perhaps, dissecting the underlying molecular mechanisms is far more challenging, but nevertheless the phenotypic consequences of autoimmune channelopathies may open up intriguing scientific questions.
Two current reports, Thieben et al.1 in this issue of Neurology and Vincent et al.2 recently in Brain, describe the characteristic clinical features of a “limbic encephalitis”-like syndrome associated with autoantibodies to voltage-gated potassium channels which is potentially reversible. The major features at presentation are …
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