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Case Reports
. 2007 Oct;64(10):1528-30.
doi: 10.1001/archneur.64.10.1528.

Potassium channel antibody associated encephalopathy presenting with a frontotemporal dementia like syndrome

Andrew McKeon  1 , Michael Marnane, Martin O'connell, John P Stack, Peter J Kelly, Timothy Lynch
Affiliations
Case Reports

Potassium channel antibody associated encephalopathy presenting with a frontotemporal dementia like syndrome

Andrew McKeon et al. Arch Neurol. 2007 Oct.

Abstract

Objective: To describe a patient who presented with features suggestive of frontotemporal dementia (FTD) but with some atypical findings and antibodies to neuronal voltage-gated potassium channels (VGKC-Abs).

Design: Case report.

Setting: Mater Misericordiae University Hospital, Dublin, Ireland.

Results: An 82-year-old man presented with progressive changes in personality, social conduct, and executive function with preservation of memory, deteriorating from baseline to requiring acute hospitalization within 6 months. Transient deterioration (episodic speech arrest) with spontaneous recovery, atypical for frontotemporal dementia, was observed. The patient had an elevated VGKC-Ab titer (2624 pM [normal range, < 100 pM]), elevated protein levels in cerebrospinal fluid, and a negative evaluation for malignancy. Magnetic resonance imaging of brain was normal but [(18)F]-fluorodeoxyglucose positron emission tomographic imaging revealed bifrontal hypometabolism. A marked and sustained improvement with steroid therapy was observed.

Conclusion: Workup for a potentially reversible autoimmune-mediated encephalopathy, including a VGKC-Ab titer, should be considered in patients presenting with rapidly progressive behavioral and cognitive decline.

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