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• Vol.44 No.9
• CLINICA NEUROL, 44: 618−622, 2004
• Vol.44 No.9 contents

Case Report

A case of steroid-responsive encephalopathy with positive 14-3-3 protein of the cerebrospinal fluid clinically resembling Creutzfelt-Jakob disease

Kanae Utsumiya, M.D.¹⁾, Ryuki Arakawa, M.D.¹⁾, Shin Fujimoto, M.D.¹⁾²⁾, Hidetsugu Ueyama, M.D.¹⁾ and Toshihide Kumamoto, M.D.¹⁾

¹⁾Department of Brain and Nerve Science (Third Department of Internal Medicine), Oita University Faculty of Medicine
²⁾Ueo Memorial Hospital

A 69-year old man developed subacutely progressive dementia, inactivity, and gait disturbance. On admission, he showed flutter-like oscillation of the bilateral eyes and myoclonus with upper extremities. Cerebrospinal fluid (CSF) analysis revealed elevation of protein (73.2 mg/dl) and the positive 14-3-3 protein. An electroencephalogram (EEG) revealed diffuse slowing (2-3 Hz, 80 μV). Brain MRI showed high intensity lesions in the white matter and left thalamus on FLAIR and diffusion imaging. We first suspected Creutzfelt-Jakob disease (CJD), but his symptoms didn't progress and showed no PSD on EEG. Oral corticosteroid therapy (prednisolone 60 mg/day) brought him remarkable recovery corresponding with improvement of CSF and EEG findings. Despite of etiology unknown, we made a diagnosis of steroid-responsive encephalopathy.

(CLINICA NEUROL, 44: 618−622, 2004)
key words: steroid-responsive encephalopathy, 14-3-3 protein, Creutzfelt-Jakob disease, flutter-like oscillation

(Received: 14-Feb-04)