[An autopsied case of subacute progressive dementia with flactuated disturbance of consciousness, myoclonus and periodic synchronous discharges on EEGs].
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.
Miyakawa
T
T
Department of Psychiatry, Yokohama City University School of Medicine, Japan.
Iseki
E
E
Shiraishi
M
M
Fujiwara
S
S
Kosaka
K
K
D001921:Brain / Q000473:pathology; D003244:Consciousness Disorders / Q000209:etiology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis; D003704:Dementia / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D004569:Electroencephalography; D006801:Humans; D008297:Male; D008875:Middle Aged; D009207:Myoclonus / Q000209:etiology; D014057:Tomography, X-Ray Computed