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| Case Report | |||||
| Dementia and Neurocognitive Disorders 2015: 14: 4: 172-175 | |||||
| Hashimoto’s in the Cerebrospinal Fluid and Atypical Course Mimicking Creutzfeldt-Jakob Disease | |||||
|---|---|---|---|---|---|
| Jee-Eun Yoon, Ji Sun Kim, Kyung-Bok Lee, Hakjae Roh, Moo-young Ahn | |||||
| Department of Neurology, Soonchunhyang University Seoul Hospital, Soonchunhyang University School of Medicine, Seoul, Korea | |||||
| Hashimoto’s in the Cerebrospinal Fluid and Atypical Course Mimicking Creutzfeldt-Jakob Disease | |||||
| Jee-Eun Yoon, Ji Sun Kim, Kyung-Bok Lee, Hakjae Roh, Moo-young Ahn | |||||
| Department of Neurology, Soonchunhyang University Seoul Hospital, Soonchunhyang University School of Medicine, Seoul, Korea | |||||
| 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 14-3-3 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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| Key Words: Creutzfeldt-Jakob disease, Hashimoto’s encephalopathy, 14-3-3 protein. | |||||
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