Hiroshi Ishino

Joseph disease in a non‐Portuguese family

We studied four patients with Joseph disease in a Japanese family. There were two clinical types in the family. One was characterized by pyramidal and cerebellar signs with or without extrapyramidal signs; the other, by cerebellar signs, loss of tendon reflexes, and peripheral sensory loss. The family tree indicated autosomal-dominant inheritance. Neuropathologic examination revealed marked degeneration of the substantia nigra, dentate nuclei, Clarke column, and anterior horn cells of the spinal cord. This is the first report of pathologically proven Joseph disease in a non-Portuguese family.

Frequency of Alzheimer's neurofibrillary tangles in the cerebral cortex in progressive supranuclear palsy (subcortical argyrophilic dystrophy)

The frequency of Alzheimers neurofibrillary tangles was studied, employing large sections of the cerebral hemispheres, in the cerebral cortex in 2 cases of progressive supranuclear palsy. The majority of the neurofibrillary tangles were found in the smaller nerve cells of the third layer. The typical triangular form was rare, and most of them showed argyrophilic neurofibrillary filaments which coiled around the well-preserved nucleus. We concluded that their occurrence in the cerebral cortex is one of the morphological manifestations of the disease process.

Motor nuclear involvement in progressive supranuclear palsy

Abstract A case of progressive supranuclear palsy is described in which there was also spinal anterior horn and oculomotor nucleus involvement. Previous reports describing similar lesions are reviewed and this combination of pathological findings is shown to be very rare. It is concluded that they represent variable manifestations of one pathological process, viz., heterogenous system degeneration (Verhaart).

Contribution to clinical pathology of progressive supranuclear palsy (subcortical argyrophilic dystrophy)

Abstract A new case of progressive supranuclear palsy is reported, and the pathological findings in 2 cases of this disease are compared. We consider the marked accentuation of argyrophilic dystrophy (neurofibrillary tangles) in the basal ganglia and the brain-stem to be the basic lesion of this disease and in our view atrophy is a secondary phenomenon. The presence of numerous neurofibrillary tangles in the brain-stem reticular formation is considered to be responsible for dementia of a mild or moderate degree, often accompanied by night delirium and confusion.

A case of Wilson's disease with enormous cavity formation of cerebral white matter

Since Wilson’s original description of progressive lenticular degeneration,’ the idea that the cerebral degeneration is limited to the lenticular nuclei has been accepted widely. However, a number of cases have been reported in the literature in which gross destructive lesions in the convolutional white matter of the cerebral hemispheres equaled or exceeded those in the lenticular nuclei. In 1930, Braunmuhl’ and, later, in 1948, Richter3 called attention to the foci of necrosis involving both cortex and convolutional white matter. Pathologic reports, however, describing enormous cavity formation of the cerebral white matter, except for changes in the occipital lobes, are rare.4 The authors report in this paper the clinicopathologic findings in a case of Wilson’s disease with cavity formation in the white matter as well as changes in the pallium and cerebellum. The pathogenesis of the cavity formation is briefly discussed.

Frequency of Alzheimer's Neurofibrillary Tangles in the Basal Ganglia and Brain-stem in Alzheimer's Disease, Senile Dementia and the Aged

The authors studied the frequency of neurofibrillary tangles in the basal ganglia and brain-stem of two cases with Alzheimers disease (average 55 years of age), six cases with senile dementia (average 82 years) and ten cases with cerebral softening and arteriosclerosis in old age (average 79 years). The distribution pattern of ANC was almost the same in Alzheimers disease and senile dementia, i.e. plenty of ANC were found in the nucl. dorsalis raphe, nucl. centralis superior, nucl. mamilloinfundibularis and nucl. basilaris, but in senile dementia ANC were much fewer in the thalamus and lenticular nuclei. In the aged with cerebral softening and arteriosclerosis, a negligible number of ANC in the brain-stem made a marked contrast to a large quantity of ANC in the cases with senile dementia. Hence, as far as ANC in the brain-stem is concerned, no continuum seems to exist between normal senility and senile dementia.

Distribution of Alzheimer's Neurofibrillary Tangles in the Basal Ganglia and Brain Stem of Progressive Supranuclear Palsy and Alzheimer's Disease

The authors studied the distribution of neurofibrillary tangles in the basal ganglia and brain stem of progressive supranuclear palsy and Alzheimers disease, with the result that almost no similarity in the distribution and frequency of neurofibrillary tangles exists between both diseases. In two cases with progressive supranuclear palsy, neurofibrillary tangles were found most numerously in the subthalamic nucleus. Next in order came the globus pallidus, reticular formation of midbrain, pons and medulla oblongata, pontine nuclei, locus coeruleus, red nucleus, substantia nigra, periaqueductal grey matter and olivary nuclei. Neurofibrillary tangles were rare in the thalamus. In two cases with Alzheimers disease, neurofibrillary tangles were found most numerously in the nucleus mamilloinfundibularis, nucleus basilaris, nucleus dorsalis raphe, nucleus centralis superior, and next in order came the thalamus. They were found scarcely in the lenticular nuclei and reticular formation of the pons. In both diseases, almost no neurofibrillary tangles were found in the nucleus supraopticus, nucleus paraventricularis, nuclei tuberales and nuclei corporis mamillare.

“Neuro‐Behçet's syndrome”–Case report with pathological findings–

A case of Neuro‐Behcets syndrome was reported clinicopathologically.

Alcoholic hallucinosis and its marginal mental disorders.

We studied four cases of intermediate or marginal types of alcoholic hallucinosis. According to the clinical features, they are classified as follows:

A Case of Subcortical Arteriosclerotic Encephalopathy (Binswanger's disease)

Clinico‐pathological descriptions were made on a case of Binswangers disease, in whom the illness began with Parkinsonism and followed by progressive dementia. The authors compared the clinico‐pathological findings with those of the cases reported in the literature.