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Patient With Voltage-Gated Potassium-Channel (VGKC) Limbic Encephalitis Found to Have Creutzfeldt-Jakob Disease (CJD) at Autopsy
Christopher R. Newey, M.D.; Brian S. Appleby, M.D.; Steven Shook, M.D.; Aarti Sarwal, M.D.
The Journal of Neuropsychiatry and Clinical Neurosciences 2013;25:E05-E07. doi:10.1176/appi.neuropsych.11120355
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All authors contributed equally to the writing of the case and formatting the images. Authors have no financial disclosures to report.

Dept. of Adult Neurology Lou Ruvo Center for Brain Health (BSA) Cleveland Clinic Cleveland, OH
Dept. of Neurology and Critical Care Wake Forest Baptist University Medical Center Winston-Salem, NC

Correspondence: Christopher Newey, M.D.; e-mail: neweyc@ccf.org

Copyright © 2013 American Psychiatric Association


To the Editor: Key differential diagnoses of patients presenting with rapidly-progressive dementia include autoimmune encephalitis, such as antibodies to the voltage-gated potassium channel (VGKC), and Creutzfeldt-Jakob disease (CJD). Correct and timely diagnosis of the etiology is paramount, given the catastrophic progression of CJD and the potential for response to immunomodulatory therapy in autoimmune encephalitis.

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FIGURE 1. Brain MRI Showing Restricted Diffusion of Cortex and Subcortical Structures [A], With Correlation on Apparent Diffusion Coefficient Sequence (ADC) [B]

The hyperintensity can also be seen on fluid-attenuated inversion recovery sequence (FLAIR) [C]. There is no gadolinium enhancement [D]. These sequences are suggestive of Creutzfeldt-Jakob disease.

FIGURE 2. Immunohistopathology, Showing Hematoxylin-Eosin Staining of Spongiform Degeneration of the Frontal Cortex, Deposition of the Misfolded Prion Protein (PrP) Around the Rim of Vacuoles (circle) or as Isolated PrP Aggregates of the Frontal Cortex, and PrP Deposits Found in Granular and Molecular Layers of the Cerebellum, With a Kuru Plaque (inset) Detected in the Purkinje Layer


World Health Organization: Global Surveillance, Diagnosis, and Therapy of Human Transmissible Spongiform Encephalopathies: Report of a WHO Consultation. Geneva, Switzerland, 1998
Zerr  I;  Kallenberg  K;  Summers  DM  et al:  Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease.  Brain 2009; 132:2659–2668
[CrossRef] | [PubMed]
Tan  KM;  Lennon  VA;  Klein  CJ  et al:  Clinical spectrum of voltage-gated potassium-channel autoimmunity.  Neurology 2008; 70:1883–1890
[CrossRef] | [PubMed]
Geschwind  MD;  Tan  KM;  Lennon  VA  et al:  Voltage-gated potassium-channel autoimmunity mimicking Creutzfeldt-Jakob disease.  Arch Neurol 2008; 65:1341–1346
[CrossRef] | [PubMed]
Parchi  P;  Giese  A;  Capellari  S  et al:  Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects.  Ann Neurol 1999; 46:224–233
[CrossRef] | [PubMed]
Pozo-Rosich  P;  Clover  L;  Saiz  A  et al:  Voltage-gated potassium-channel antibodies in limbic encephalitis.  Ann Neurol 2003; 54:530–533
[CrossRef] | [PubMed]
Rueff  L;  Graber  JJ;  Bernbaum  M  et al:  Voltage-gated potassium-channel antibody-mediated syndromes: a spectrum of clinical manifestations.  Rev Neurol Dis 2008; 5:65–72
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