Dement Neurocogn Disord.  2015 Dec;14(4):172-175. 10.12779/dnd.2015.14.4.172.

Hashimoto's Encephalopathy with Positive 14-3-3 Protein in the Cerebrospinal Fluid and Atypical Course Mimicking Creutzfeldt-Jakob Disease

• Yoon JE
• Kim JS
• Lee KB
• Roh H
• Ahn MY

• KMID: 2442936
• DOI: http://doi.org/10.12779/dnd.2015.14.4.172

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.

Keyword

Creutzfeldt-Jakob disease; Hashimoto's encephalopathy; 14-3-3 protein

MeSH Terms

14-3-3 Proteins*
Antibodies
Cerebrospinal Fluid*
Creutzfeldt-Jakob Syndrome*
Diagnosis
Humans
Male
Middle Aged
Myoclonus
Neurons
Phenotype
14-3-3 Proteins
Antibodies

Figure

• 

  Fig. 1 T2-weighted MRI on the first admission day showed high-signal-intense lesions in the basal ganglia, the thalamus and bilateral cerebral white matter (A). Follow-up MR images 8 months after the initial study revealed an increased extent of high-signal-intense lesions in the basal ganglia, the thalamus and multifocal cerebral hemisphere, and progression of diffuse cerebral atrophy (B).

• 

  Fig. 2 Initial EEG showed generalized continuous slow activity (A). Follow-up EEG recorded 8 months later shows periodic lateralized epileptiform discharges over the right hemisphere (B). EEG: electroencephalogram.

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