Course and outcome of acute limbic encephalitis with negative voltage-gated potassium channel antibodies.

Journal article

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.

Authors: Samarasekera, SR; Vincent, A; Welch, J; Jackson, M; Nichols, P

• Publication status: Published
• Journal: Journal of neurology, neurosurgery, and psychiatry
• Volume: 78
• Issue: 4
• Pages: 391-394
• Publication date: 2007-04-01
• DOI: 10.1136/jnnp.2006.093096
• EISSN: 1468-330X
• ISSN: 0022-3050
• Language: English
• Keywords: Acute Disease; Humans; Cognition Disorders; Treatment Outcome; Predictive Value of Tests; Potassium Channels, Voltage-Gated; Magnetic Resonance Imaging; Antibodies; Adult; Limbic Encephalitis; Paraneoplastic Syndromes; Female; Male; Seizures