A highly sensitive silver technique for glial cytoplasmic inclusions (GCI) in olivopontocerebellar atrophy (OPCA) was applied to tissues from 15 patients with neurodegenerative disorders including OPCA, Joseph disease, Alzheimer's disease (AD), Huntington's chorea, Pick disease and three control non-neurological subjects. Brain tissue from both OPCA and AD impregnated positively. Neurons, astroglia and oligodendroglia in the putamen, pontine nucleus and inferior olivary nucleus all impregnated in addition to white matter oligodendroglia. Neuronal inclusions in the pontine nucleus appeared as compact or fibrillary masses, and GCI-bearing oligodendroglia and astrocytes showed homogeneously impregnated somata. The myelinated pontocerebellar tract and the white matter surrounding the inferior olivary nucleus contained a small number of impregnated nerve fibres with a hollow structure, which resembled the myelin sheath. Immunocytochemical studies to clarify these argyrophilic structures in the OPCA subjects employed paired helical filament (PHF), microtubule associated proteins (MAPs), MAP1, MAP2, MAP5, tau, ubiquitin, neurofilament (200 or 70 kilodaltons) and myelin basic protein (MBP) antisera. GCI-bearing white matter oligodendroglia expressed PHF, tau, MAP5 and ubiquitin immunoreactives and non-argyrophilic astroglia were positive for MAP5 antiserum alone. In the putamen, pontine nuclei and inferior olivary nuclei, impregnated neurons as well as the GCI-bearing oligodendroglia immunostained with PHF, tau, MAP5 and ubiquitin antisera and impregnated astroglia were also immunoreactive to these antisera except for being tau negative in the putamen. Silver impregnated nerve fibres showed only MBP immunoreactivity. These findings indicate that the argyrophilia in the OPCA subjects closely correlates with PHF and tau immunoreactivities.
Three Japanese patients with Joseph disease from different families developed sleep disturbance, followed by delirium at the middle to end stage. Brain CT scans of the three patients showed brainstem tegmental atrophy. EEG revealed slowing of background activity. Two necropsy cases showed degeneration of the reticular formation, raphe nuclei and locus ceruleus in the brainstem tegmentum in addition to the common pathological findings of Joseph disease. The clinicopathological correlation between the delirium and the brainstem tegmental atrophy in Joseph disease is discussed.
We clinically studied 4 patients with Joseph disease in a Japanese family, who developed mild dementia, and neuropathologically examined one autopsied case among them. Neurological findings included cerebellar ataxia, progressive external ophthalmoplegia, peripheral amyotrophy, and pyramidal signs. Mental state examination revealed mild impairment of intelligence. The dementia was characterized by mild memory disturbance with preservation of orientation, slowing of thought processes, affective changes including apathy and depression, impaired ability to manipulate acquired knowledge, and the absence of aphasia, apraxia and agnosia. CT demonstrated severe atrophy of the brain stem and severe dilatation of the fourth ventricle with mild atrophy of the cerebral and cerebellar hemispheres. EEG showed slowing of background activity. The autopsied case showed atrophy of the tegmentum of the brain stem in addition to the common pathological findings of Joseph disease. The features of dementia in the 4 patients were similar to those seen in ‘subcortical dementia’, suggesting that its pathological basis may be involvement of the tegmentum of the brain stem.
Eight Japanese patients with Joseph disease were studied using computed tomography (CT). Morphometric analysis using the two-dimensional (area) measurement by CT was performed in the infratentorial region. The brain-stem index, an index of brain-stem atrophy or pontine atrophy, revealed a significant decrease (P less than 0.01), with a mean of 66.7% when compared with 16 control subjects. The patients showed a significant increase (P less than 0.01), with a mean of three times that of the controls in the fourth ventricular index, an index of fourth ventricular dilatation. There were no differences in the cerebellar index, an index of cerebellar atrophy, between these patients and the controls, although the patients had an increased number of visible cerebellar vermian (2.0, SD 0.7) and hemispheric sulci (2.6 SD 0.6) as compared with the controls (vermian sulci: 0.4, SD 0.7: hemispheric sulci: 0). These data indicate severe pontine atrophy, fourth ventricular dilatation with mild involvement of the cerebellum and correlate well with the common pathological features of Joseph disease. The present morphometric evaluation by CT may be useful in the clinical diagnosis of Joseph disease.
The experimental epileptogenic focus produced by cobalt-gelatine stick implantation in the cerebral cortex of rabbits has been studied by electron microscopy. Since our cobalt focus was latent, the bemegride maneuver was intravenously performed to identify the cobalt lesion of the cortex as an epileptogenic focus. The cobalt lesion comprised three different zones. Of the three zones, the reactive zone showed a glio-mesenchymal scar and prominent ultrastructural changes. The major change of this zone was found in the neurons, neuropils and astrocytes. Electron-lucent neurons deprived of the usual neuropils surrounding them, widening of extracellular spaces and swollen dendrites in neuropils, and proliferating astrocytes were observed constantly throughout the experimental period. These findings may be significant for the genesis of this type of epilepsy.
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