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A case of voltage-gated potassium channel antibody-related limbic encephalitis

Abstract

Background A 56-year-old man presented to hospital with a 6-month history of recurrent episodes of altered behavior and 'odd' episodes. He had become apathetic and uninterested in his family. He had no relevant past medical or family history. General and physical neurological examinations were unremarkable, as was bedside cognitive testing.

Investigations Brain MRI scan, 24-h electroencephalogram, serum and cerebrospinal fluid testing for voltage-gated potassium channel antibodies, blood screening for tumors, CT scans of the chest, abdomen and pelvis, whole-body PET scan, neuropsychological examination, brain 18F-fluorodeoxyglucose-PET scan.

Diagnosis Voltage-gated potassium channel antibody-related limbic encephalitis.

Management Antiepileptic drugs, immunomodulatory therapy, oral steroids, plasma exchange.

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Figure 1: A comparison of serum sodium concentration (blue line and vertical axis on the left) and serum voltage-gated potassium channel antibody titer (red line and vertical axis on the right) over time (in weeks) and treatment courses.
Figure 2: A comparison of voltage-gated potassium channel antibody titer (red line with axis on the right) and memory (blue line; delayed verbal memory recall expressed in percentage of expected recall; vertical axis on the left) over time in weeks for second patient seen.
Figure 3: MRI images of brain of second patient.

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Acknowledgements

The patients and their families are thanked for their full co-operation at all stages of the assessment and treatment. The primary patient described is also thanked for allowing publication of the supplementary online material, for which his written consent was obtained. The radiological expertise of Drs Chawda, Antoun and Balaam is greatly appreciated. Professor Angela Vincent (Department of Clinical Neurology, University of Oxford, UK) is acknowledged for determining the VGKC-Ab titers in the serum and cerebrospinal fluid.

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Correspondence to Tim Harrower.

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Supplementary Video

Video clip of patient experiencing focal seizure (WMV 14412 kb)

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Harrower, T., Foltynie, T., Kartsounis, L. et al. A case of voltage-gated potassium channel antibody-related limbic encephalitis. Nat Rev Neurol 2, 339–343 (2006). https://doi.org/10.1038/ncpneuro0194

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