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Michael D. Geschwind, MD, PhD; K. Meng Tan, MD; Vanda A. Lennon, MD, PhD; Ramon F. Barajas Jr, BS; Aissa Haman, MD; Christopher J. Klein, MD; S. Andrew Josephson, MD; Sean J. Pittock, MD

Arch Neurol. 2008;65(10):1341-1346.

Background  Rapidly progressive dementia has a variety of causes, including Creutzfeldt-Jakob disease (CJD) and neuronal voltage-gated potassium channel (VGKC) autoantibody–associated encephalopathy.

Objective  To describe patients thought initially to have CJD but found subsequently to have immunotherapy-responsive VGKC autoimmunity.

Design  Observational, prospective case series.

Setting  Department of Neurology, Mayo Clinic, and the Memory and Aging Center, University of California, San Francisco.

Patients  A clinical serologic cohort of 15 patients referred for paraneoplastic autoantibody evaluation. Seven patients were evaluated clinically by at least one of us. Clinical information for the remaining patients was obtained by physician interview or medical record review.

Main Outcome Measures  Clinical features, magnetic resonance imaging abnormalities, electroencephalographic patterns, cerebrospinal fluid analyses, and responses to immunomodulatory therapy.

Results  All the patients presented subacutely with neurologic manifestations, including rapidly progressive dementia, myoclonus, extrapyramidal dysfunction, visual hallucinations, psychiatric disturbance, and seizures; most (60%) satisfied World Health Organization diagnostic criteria for CJD. Magnetic resonance imaging abnormalities included cerebral cortical diffusion-weighted imaging hyperintensities. Electroencephalographic abnormalities included diffuse slowing, frontal intermittent rhythmic delta activity, and focal epileptogenic activity but not periodic sharp wave complexes. Cerebrospinal fluid 14-3-3 protein or neuron-specific enolase levels were elevated in 5 of 8 patients. Hyponatremia was common (60%). Neoplasia was confirmed histologically in 5 patients (33%) and was suspected in another 5. Most patients' conditions (92%) improved after immunomodulatory therapy.

Conclusions  Clinical, radiologic, electrophysiologic, and laboratory findings in VGKC autoantibody–associated encephalopathy may be confused with those of CJD. Serologic evaluation for markers of neurologic autoimmunity, including VGKC autoantibodies, may be warranted in suspected CJD cases.

Author Affiliations: Memory and Aging Center, Department of Neurology, University of California, San Francisco (Drs Geschwind, Haman, and Josephson and Mr Barajas); and Departments of Laboratory Medicine and Pathology (Drs Tan, Lennon, and Pittock), Neurology (Drs Lennon, Klein, and Pittock), and Immunology (Dr Lennon), Mayo Clinic College of Medicine, Rochester, Minnesota.

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