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Updated: Jul 11, 2026

Hippocampal Neuronal Cultures to Detect and Study New Pathogenic Antibodies Involved in Autoimmune Encephalitis
Published on: June 2, 2022
Potassium channel antibody associated encephalopathy presenting with a frontotemporal dementia like syndrome
Andrew McKeon1, Michael Marnane, Martin O'connell
1Department of Neurology, Mater Misericordiae University Hospital, Dublin, Ireland. mckeon.andrew@mayo.edu
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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