Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of Creutzfeldt-Jakob disease presenting with nonconvulsive status epilepticus in the early stages

Masato Kinboshi, M.D., Ph.D.1), Yu Tamura, M.D.1), Hiroki Yoshida, M.D.1), Ryota Matsunari, M.D.1), Jumpei Togawa, M.D.1) and Morito Inouchi, M.D, Ph.D.1)

1) Department of Neurology, National Hospital Organization Kyoto Medical Center

A 64-year-old Japanese woman presented with 1 week of recurrent convulsive seizures. At the time of admission, she was in a coma and did not present with convulsions. Intravenous diazepam administration improved her consciousness, although severe psychomotor excitement developed. Brain MRI demonstrated diffusion restriction in the cerebral cortex of the right hemisphere. Electroencephalography (EEG) showed periodic discharges centered around the parietal regions with right-sided dominance. Nonconvulsive status epilepticus (NCSE) was suspected, and the patient was actively treated with anti-epileptic drugs. She developed akinetic mutism and generalized myoclonus 1 month after admission. Follow-up EEG studies disclosed periodic synchronous discharges. Abnormal prion protein in the cerebral fluid was detected using a real-time quaking-induced conversion assay. The clinical diagnosis in the present case was sporadic Creutzfeldt-Jakob disease (CJD). Seizures as an initial symptom in patients with CJD are relatively rare. Our case suggests that CJD should be considered as a differential diagnosis when a patient presents with refractory NCSE.
Full Text of this Article in Japanese PDF (4725K)

(CLINICA NEUROL, 62: 357−362, 2022)
key words: Creutzfeldt-Jakob disease, epileptic seizure, nonconvulsive status epilepticus, periodic synchronous discharge, SPECT

(Received: 9-Aug-21)