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KMID : 1100220150140040172
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Dementia and Neurocognitive Disorders
2015 Volume.14 No. 4 p.172 ~ p.175
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Hashimoto’s in the Cerebrospinal Fluid and Atypical Course Mimicking Creutzfeldt-Jakob Disease
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Yoon Jee-Eun
Kim Ji-Sun
Lee Kyung-Bok
Roh Hak-Jae
Ahn Moo-Young
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Abstract
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Background:Creutzfeldt-Jakob disease (CJD) shares common clinical features with Hashimoto’s encephalopathy (HE). The 14-3-3 protein is a relatively sensitive and specific marker of CJD but is not commonly detected in HE. We report the case of a patient with HE with unusual features including positive 14-3-3 protein in the cerebrospinal fluid (CSF) and an atypical course mimicking that of CJD.
Case Report: A 64-year-old male was admitted due to acute-onset cognitive dysfunction. HE was suspected based on increased titers of anti-thyroid microsomal antibody and an excellent response to steroid. However, 14-3-3 protein was detected in the CSF and a recurrent attack with progressive cognitive decline, pyramidal symptoms and myoclonus mimicking CJD occurred. Cognitive dysfunction showed progressive worsening and the response to steroid treatment was decreased.
Conclusions:4-3-3 protein could be considered a general marker of neuronal destruction and not specific to CJD. The clinical manifestations of HE are variable and its diagnosis is difficult due to the lack of a specific phenotype and reliable diagnostic criteria. We recommend that patients with clinical features of CJD and antithyroid antibodies should be considered for empirical steroid treatment for HE, despite a positive result for 14-3-3 protein.
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KEYWORD
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Creutzfeldt-Jakob disease, Hashimoto’s encephalopathy, 14-3-3 protein
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