Hashimoto's Encephalopathy with Positive 14-3-3 Protein in the Cerebrospinal Fluid and Atypical Course Mimicking Creutzfeldt-Jakob Disease.
10.12779/dnd.2015.14.4.172
- Author:
Jee Eun YOON
1
;
Ji Sun KIM
;
Kyung Bok LEE
;
Hakjae ROH
;
Moo Young AHN
Author Information
1. Department of Neurology, Soonchunhyang University Seoul Hospital, Soonchunhyang University School of Medicine, Seoul, Korea. jisunkim@schmc.ac.kr
- Publication Type:Case Report
- Keywords:
Creutzfeldt-Jakob disease;
Hashimoto's encephalopathy;
14-3-3 protein
- MeSH:
14-3-3 Proteins*;
Antibodies;
Cerebrospinal Fluid*;
Creutzfeldt-Jakob Syndrome*;
Diagnosis;
Humans;
Male;
Middle Aged;
Myoclonus;
Neurons;
Phenotype
- From:Dementia and Neurocognitive Disorders
2015;14(4):172-175
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
