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We report the results of an ultrastructural study of Pick bodies (PB). A histogram constructed with the maximal width of each filamentous component in PB revealed a wide range of sizes among the filaments, in contrast to the unique composition of the paired helical filaments (PHF) seen in the neurofibrillary tangle of Alzheimer type (NFT-AT).(More)
We describe three sibling patients with autosomal dominantly inherited sensory neuropathy, sensorineural hearing loss and dementia. The features of cognitive-behavioral deficits in the patients, including executive dysfunction, apathy, indifference and inattention, were consistent with a frontal lobe dysfunction. Magnetic resonance imaging showed a diffuse(More)
Unusual paired helical filaments (PHF) coexisting with single filaments were observed in neurofibrillary degeneration (NFD) in the globus pallidus, subthalamic nucleus, substantia nigra, and pontine tegmentum of a typical case of progressive supranuclear palsy (PSP). Each filament had a diameter of 10–12 nm and showed central low density and a smooth(More)
Severe degeneration of neuronal processes, including axons and dendrites, as well as accumulation of lipofuscin-like dense bodies have been induced in rats by continuous intraventricular administration by infusion of a protase inhibitor, leupeptin. The aggregation of degenerated processes in neuropils mingled with glial cells and their processes resembled(More)
Abnormal accumulation of alpha-synuclein is regarded as a key pathological step in a wide range of neurodegenerative processes, not only in Parkinson's disease (PD) and dementia with Lewy bodies (DLB) but also in multiple-system atrophy (MSA). Nevertheless, the mechanism of alpha-synuclein accumulation remains unclear. Leupeptin, a protease inhibitor, has(More)
(1) Accumulation of neuronal lipofuscin was clearly demonstrated in the chronic tellurium intoxication in young rats with histological, fluorescent and electron-microscopical methods; (2) The needle-shaped crystal in the neuronal lysosomes were demostrated to contain the tellurium with electron dispersive spectrophotometry analysis. (3) The vacuolation of(More)
The clinical and pathological features of a sporadic case of juvenile neuroaxonal dystrophy beginning at the age of 10 and leading to death at the age of 26 are described. Clinical manifestation began with cerebellar symptoms. The subject subsequently developed dementia, pes cavus (Friedreich's feet), epilepsy, myoclonus, and Parkinsonian syndrome, but(More)
We report a rare familial case of dementia with Lewy bodies (DLB). The patient was a man who died at the age of 51. His parents were first cousins. Among three siblings, two were diagnosed as probable cases of DLB, and one was a possible case, according to the clinical diagnostic criteria of the consortium on DLB. Following the patient's autopsy, he was(More)
Neurofibrillary tangles (NFTs), which are composed of paired helical filament (PHF)-like filaments, were induced by the long-term intraventricular infusion of leupeptin, a potent protease inhibitor. The fibrils composing the NFTs were 20 nm in maximal width and had periodic constrictions at 40-nm intervals. They were identical to the PHF that had been found(More)