Limbic Encephalitis Associated With Anti–Voltage-Gated Potassium Channel Complex Antibodies Mimicking Creutzfeldt-Jakob Disease | Clinical Decision Support | JAMA Neurology | JAMA Network
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Case Report/Case Series
January 2014

Limbic Encephalitis Associated With Anti–Voltage-Gated Potassium Channel Complex Antibodies Mimicking Creutzfeldt-Jakob Disease

Author Affiliations
  • 1Yale Comprehensive Epilepsy Center, New Haven, Connecticut
  • 2Department of Neurology, Mount Sinai Hospital, New York, New York
JAMA Neurol. 2014;71(1):79-82. doi:10.1001/jamaneurol.2013.5179
Abstract

Importance  Limbic encephalitis that is associated with anti–voltage-gated potassium channel complex (VGKCC) antibodies (VGKCC syndrome) is an autoimmune, usually nonparaneoplastic form of encephalitis that is responsive to immunotherapy. Differentiating this treatable disease from others that have a similar presentation is thus important.

Observations  We present the case of a 58-year-old man who had a rapid onset of progressive confusion, twitching of the face and hand, and abnormal basal ganglia detected by magnetic resonance imaging. His conditions were initially diagnosed as Creutzfeldt-Jakob disease (CJD). Faciobrachial dystonic seizures, possibly pathognomonic for the VGKCC syndrome, had been misdiagnosed as myoclonus. Treatment led to a complete resolution of his symptoms.

Conclusions and Relevance  Given the similarities of the clinical features and, at times, the neuroimaging findings of VGKCC syndrome to CJD, recognizing VGKCC syndrome and the highly associated and distinctive faciobrachial dystonic seizures is very important. Because this syndrome is the most common treatable condition that mimics CJD, we believe that it is crucial to screen all patients with presumed CJD for this reversible condition.

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