Science Links Japan | An Autopsied Case of Subacute Progressive Dementia with Flactuated Disturbance of Consciousness, Myoclonus and Periodic Synchronous Discharges on EEGs.

An Autopsied Case of Subacute Progressive Dementia with Flactuated Disturbance of Consciousness, Myoclonus and Periodic Synchronous Discharges on EEGs.

Accession number;00A0018507

Title;An Autopsied Case of Subacute Progressive Dementia with Flactuated Disturbance of Consciousness, Myoclonus and Periodic Synchronous Discharges on EEGs.

Author;MIYAKAWA TOMOHIRO(Yokohama City Univ., Sch. of Med.) ISEKI EIZO(Yokohama City Univ., Sch. of Med.) SHIRAISHI MASAO(Kokuho Asahi Cent. Hosp.) FUJIWARA SHUICHIRO(Fed. of Natl. Public Serv. and Affil. Personnel Mutual Aid Assoc., Yokohama Minami Kyosai Hosp.) KOSAKA KENJI(Yokohama City Univ., Sch. of Med.)

Journal Title;Brain Nerve

Journal Code:Z0685A

ISSN:0006-8969

VOL.51;NO.11;PAGE.969-974(1999)

Figure&Table&Reference;FIG.6, REF.13

Pub. Country;Japan

Language;Japanese

Abstract;We present an autopsied case which developed progressive dementia at the age of 45. EEGs showed periodic synchronous discharges (PSD) when he showed fluctuated cloudy consciousness with myoclonus. Cerebrospinal fluid examination showed mild to moderate increase in protein and monocytes for duration of illness, but antivirotics and antibiotics were not effective He was not apallic even at the terminal stage when computed tomography (CT) revealed marked cerebral atrophy, and died of pneumonia 27 months after onset. He was clinically diagnosed as having Creutzfeldt-Jakob disease (CJD) because of subacute progressive dementia, myoclonus and PSD. Neuropathological examination revealed marked brain atrophy with severe neuronal loss and gliosis in the cerebral cortex and subcortical nuclei, although no spongiform degeneration was found. Senile plaques, neurofibrillaly tangles, argyrophilic glial inclusions and glial nodules were not detected. Prion protein immunoreactivity was negative. Therefore, this case can not be neuropathologically diagnosed as having CJD, Alzheimer's disease, non-Alzheimer-type degenerative dementias or any encephalitis. This case might have suffered from an unknown new disease. (author abst.)