Hashimoto's Encephalopathy with Positive 14-3-3 Protein in the Cerebrospinal Fluid and Atypical Course Mimicking Creutzfeldt-Jakob Disease
Dementia and Neurocognitive Disorders
;
: 172-175, 2015.
Article
in English
| WPRIM
| ID: wpr-197191
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Phenotype
/
Cerebrospinal Fluid
/
Creutzfeldt-Jakob Syndrome
/
14-3-3 Proteins
/
Diagnosis
/
Antibodies
/
Myoclonus
/
Neurons
Type of study:
Diagnostic study
/
Prognostic study
Limits:
Humans
/
Male
Language:
English
Journal:
Dementia and Neurocognitive Disorders
Year:
2015
Type:
Article
Similar
MEDLINE
...
LILACS
LIS