Case Report/Case Series |

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

Ji Yeoun Yoo, MD1,2; Lawrence J. Hirsch, MD1
[+] 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.
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Published online

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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Figure 1.
Initial Magnetic Resonance Imaging (MRI) Findings Showing Abnormal Basal Ganglia

The MRI scans show T2-weighted and fluid-attenuated inversion recovery hyperintense signals in the right caudate and in the right putamen (A [arrowheads]) and in the left greater than right medial temporal lobe (B [arrowheads]). There is borderline diffusion-weighted imaging hyperintensity in the right caudate and putamen (C [arrowheads]), without a corresponding attenuation diffusion coefficient hypointensity (D).

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Figure 2.
Additional Magnetic Resonance Imaging (MRI) Findings Showing Improvement in the Basal Ganglia

The MRI fluid-attenuated inversion recovery axial scans reveal a marked improvement in the hyperintensity in the basal ganglia (A) and in the remaining hyperintensity in the bilateral medial temporal lobes (B [arrowheads]).

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Figure 3.
Positron Emission Tomographic (PET) Findings Showing Hypermetabolism in the Basal Ganglia

The PET scans of the patient’s brain show hypermetabolism in the left caudate and putamen (A [arrowheads]) and less prominent hypermetabolism in the right putamen (B [arrowheads]).

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