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BACKGROUND: Limbic encephalitis is a potentially treatable immunological condition. The presence of voltage-gated potassium channel antibodies (VGKC-Ab) in the cerebrospinal fluid (CSF) and serum of patients with the condition is a marker of the disease associated with a non-paraneoplastic form and good response to treatment. Recent work has highlighted absent serum VGKC-Ab and distinct immunology in patients with the paraneoplastic form of limbic encephalitis. METHODS: The cases of four patients with the typical clinical presentation, neuropsychological features and brain imaging of acute limbic encephalitis, in the absence of any evidence for associated cancer during a follow-up of at least 18 months are described here. RESULTS: All patients had negative testing for VGKC-Ab measured during their acute presentation. All patients made some recovery, although they were left with marked cognitive deficits and persistent seizures. CONCLUSION: These cases demonstrate that the absence of VGKC-Ab in limbic encephalitis does not necessarily imply a paraneoplastic form. Further work is required to establish the immunological basis for the disorder in these patients, and the optimal treatment regimen.

Original publication

DOI

10.1136/jnnp.2006.093096

Type

Journal article

Journal

J Neurol Neurosurg Psychiatry

Publication Date

04/2007

Volume

78

Pages

391 - 394

Keywords

Acute Disease, Adult, Antibodies, Cognition Disorders, Female, Humans, Limbic Encephalitis, Magnetic Resonance Imaging, Male, Paraneoplastic Syndromes, Potassium Channels, Voltage-Gated, Predictive Value of Tests, Seizures, Treatment Outcome