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Biomedical subjects

T Inose

Publications and source records attributed to T Inose.

At least 19 recordsLinked to original sources

Familial parkinsonism and dementia with ballooned neurons, argyrophilic neuronal inclusions, atypical neurofibrillary tangles, tau-negative astrocytic fibrillary tangles, and Lewy bodies.

We report four patients with a new type of familial parkinsonism and dementia consisting of an autosomal dominant inheritance, dopa-responsive parkinsonism, severe dementia, variable myoclonus and autonomic disturbances. Autopsy of two patients revealed symmetrical cerebral atrophy with fronto-temporal dominant distribution, and marked depigmentation in the substantia nigra and locus ceruleus. Neuronal loss and gliosis were observed in the deep cerebral cortex and amygdala as well as in the areas vulnerable to Parkinson's disease. In the cerebral cortex, swollen neurons with frequent granulovacuolar changes were observed, consisting of ballooned neurons and those with argyrophilic intracytoplasmic inclusions, in addition to neuropil threads. Atypical neurofibrillary tangles, which barely stained with tau antibodies, were numerous in the upper cortical layers, consisting of 15-nm straight tubules. In addition, tau-negative astrocytic fibrillary tangles were also frequent. Electron microscopically, the ballooned neurons and argyrophilic neuronal inclusions contained filamentous structures coated with fuzzy electron-dense deposits. The inclusions showed immunohistochemical features different from those of cortical Lewy bodies and Pick bodies. Occasional Lewy bodies were present in the brain stem lesions of both patients. In two of our patients, the pathology in the brain stem was similar to that of Parkinson's disease, whereas their cerebral pathology was unusual and has not been reported previously.

Adult

Purification and characterization of microbial gellan lyase.

Gellan lyase was purified from the culture fluid of soil samples incubated in a medium containing gellan as a sole carbon source. The enzyme was a monomer with a molecular mass of 140 kDa and was most active at pH 7.5 and 45 degrees C. The enzyme was highly specific to gellan and lowered the viscosity of the polymer.

Carbohydrate Sequence

Analysis of lysine-dependent yeast sporulation: a decrease in cyclic AMP is not required for initiation of meiosis and sporulation in Saccharomyces cerevisiae.

Cells of the yeast Saccharomyces cerevisiae sporulated in nutrient-rich medium containing L-lysine. Sporulation was specific to the presence of L-lysine and was initiated when the cellular content of this basic amino acid reached approximately 0.2-0.5 mmol (g cells)-1, at early stationary phase. The formation of asci was most efficient at pH 7.0 and 50-100 mM L-lysine; in these optimum conditions, the sporulation frequency reached about 60% after 5 d incubation. The L-lysine-dependent sporulation system in nutrient-rich conditions was distinct from the currently used potassium-acetate-dependent system in nutrient-deficient conditions. Analysis of the L-lysine-dependent system indicated that, prior to entrance into meiosis and/or sporulation processes, the yeast cells change in shape, their pool sizes for L-cysteine and glutathione alter, and they synthesize a protein with a molecular mass of 15 kDa. A low level of cAMP was not required for the entrance into meiosis and/or sporulation.

Acetates

Corticonigral degeneration with neuronal achromasia presenting with primary progressive aphasia: ultrastructural and immunocytochemical studies.

We describe a clinico-pathological variant of a degenerative disorder involving Broca's, Wernicke's, and supplementary motor areas, which presented as primary progressive aphasia, dysarthria, bucco-facial apraxia, and hearing loss as initial symptoms, followed by organic personality changes. Postmortem examination revealed severe focal atrophy of the cerebral convolutions in the frontal operculum, superior frontal gyrus, and superior and transverse temporal gyri in addition to diffuse atrophy of the frontal and temporal lobes in both hemispheres. Microscopical examination revealed argyrophilic neuronal inclusions (ANIs) in the neuronal perikarya and presynaptic terminal throughout the central nervous system, as well as neuronal loss and swollen chromatolytic neurons in the affected cortices. Neocortical ANIs showed a positive immunoreaction with an anti-tau antibody but only a weak reaction with an anti-ubiquitin antibody immunohistochemically. Ultrastructurally, neocortical ANIs consisted of 15-nm thick smooth-surfaced tubules and tubules with constrictions at 120-150-nm intervals; thus they were different from the typical paired helical filaments of the 80-nm interval constrictions observed in the subiculum. ANIs were also found in the basal ganglia, brain stem nuclei, and cervical cord. Accordingly, ANIs appear distinct from neurofibrillary tangles (NFTs) of progressive supranuclear palsy, NFTs of Alzheimer-type dementia, and Pick bodies. The authors consider that this case fits the histopathological criteria of corticonigral degeneration with neuronal achromasia except for the unusual extension to the temporal lobes.

Aphasia

Immunocytochemical and ultrastructural studies of neuronal and oligodendroglial cytoplasmic inclusions in multiple system atrophy. 1. Neuronal cytoplasmic inclusions.

Neuronal alterations in five cases of multiple system atrophy (MSA) were investigated histologically, immunocytochemically and ultrastructurally. Argentophilic neuronal cytoplasmic inclusions (NCIs) were observed in all cases. They were distributed, in order of decreasing frequency, in the pontine nucleus, striatum, subiculum, amygdala, hippocampus, dentate fascia, substantia nigra and inferior olivary nucleus. Anti-ubiquitin antibodies visualized many thickened neurites in the degenerating gray matter as well as NCIs. Some NCIs were also recognized by anti-phosphorylated neurofilament antibodies. Ultrastructurally, NCIs consisted of a meshwork of granule-associated filaments, the diameter ranging from 18 to 28 nm, that were mixed with neurofilaments. The granule-associated filaments were also present in the axoplasm of myelinated fibers. Our studies demonstrate widespread distribution of NCIs in the central nervous system of MSA. The same pathological process that forms the granule-associated filaments in axons may also be responsible for the formation of ubiquitin-positive thickened neurites. These axonal alterations, as well as neuronal perikaryal changes, may play an important role in the impaired neuronal function in MSA.

Adult

Presenile dementia with progressive supranuclear palsy tangles and Pick bodies: an unusual degenerative disorder involving the cerebral cortex, cerebral nuclei, and brain stem nuclei.

Degeneration of heterogeneous systems in the central nervous system, with widespread distribution of argyrophilic neuronal fibrillary inclusions, was found in a patient with presenile dementia. Atrophy was circumscribed in the frontal and temporal lobes. Neuronal loss was severe in the basal ganglia, subthalamic nucleus, and substantia nigra. Immunocytochemical study using anti-phosphorylated tau and anti-ubiquitin antibodies in conjunction with ultrastructural observations revealed two types of inclusions: neurofibrillary tangles (NFTs) of progressive supranuclear palsy (PSP) in the Edinger-Westphal nucleus, locus coeruleus, cerebellar dentate nucleus, inferior olivary nucleus, and posterior horn of the spinal cord; and Pick bodies (PBs) in the atrophied cerebral cortex and red nucleus. PSP-type NFTs and PBs have been demonstrated in a single case for the first time. Despite their pathognomonic significance in certain disorders, we suggest that these inclusions may reflect a form of cytoskeletal disorganization, which is not entirely restricted to a single disease entity.

Adult

Immunocytochemical and ultrastructural studies of neuronal and oligodendroglial cytoplasmic inclusions in multiple system atrophy. 2. Oligodendroglial cytoplasmic inclusions.

Oligodendroglial cytoplasmic inclusions (OCI) in multiple system atrophy were investigated immunocytochemically and ultrastructurally. Among the 17 cases examined, 16 had OCIs. Almost all OCIs were positive for both alpha B-crystallin and ubiquitin. The antibodies against tubulin, paired helical filament and tau stained OCIs to various extents. Ultrastructurally OCIs consisted of meshworks of granule-associated filaments about 25 nm in diameter that sometimes formed flame-shaped tangle-like structures. Immunoelectron microscopy showed that an epitope of alpha B-crystallin was located on the granule-associated filaments composing OCIs. Our studies further support a cooperative role of alpha B-crystallin, ubiquitin and cytoskeletal protein in the formation of some types of intracytoplasmic inclusions.

Adolescent

Clinical and neuropathological study of a familial case of juvenile parkinsonism.

This is a detailed autopsy case of rare juvenile parkinsonism with dominant heredity. The patient displayed parkinsonian symptoms which began at the age of 24, and expired in a state of quadriplegia-in-flexion at 35. In the later stage, myoclonic jerks, epileptiform convulsions and dementia appeared. L-dopa was effective only in the early stages. The autopsy revealed severe degeneration and the formation of atypical Lewy bodies in the cerebral cortex, as well as typical lesions of idiopathic parkinsonism with a Lewy body formation in the brain stem. This case was considered to belong essentially to idiopathic parkinsonism. The pathology of juvenile parkinsonism is reviewed briefly.

Adult

[A case of Pick's disease with long duration--an extraordinary cerebral change in the fore part of cerebrum].

An autopsied case of Pick's disease, having an extraordinary cerebral change in the anterior portion of Lobus frontalis and temporalis, was reported. Our case is a 71 year-old woman at death with a fourteen year history of chronic progressive dementia and mental deterioration, and it may be stressed that the existence lasted 8 years, over the latter half of clinical course, was depended on the tube feeding. The first symptoms suddenly appeared in 1964, 2 months after her husband's death of illness, when she was 57. She prepared the table for breakfast late at night, calculated wrongly in her domestic account book, and stole foods in the grocery. Two years later, her illness was diagnosed as presenile dementia by characteristic personality change and marked dilatation of anterior horn of lateral ventriculus. On admission to National Musashi Sanatorium, three years after the first symptoms' appearance, she presented restless walking, insomnia, memory loss, weakness of concentration, and high degree of disorientation. Particularly, it was noticeable that she behaved with bizzare contact. After 1970, tube feeding was introduced continuously, because of swallowing difficulty. Death occurred in July 1978 from a general weakness and a broncho-pneumonia, 14 years after the onset of the first symptoms. Autopsy revealed small and atrophied brain weighed 820 g. Cerebral cortical atrophy extended to frontal, temporal, insular, and parietal lobes, but right T-1 was relatively well preserved. On section, frontal and temporal ventriculus were remarkably enlarged and caudate nuclei were extremely atrophic.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged

Clinical and pathological studies in a case of reticulum cell sarcoma.

A case of reticulum cell sarcoma associated with involvement of the central nervous system was presented. This seems to be an atypical case of reticulum cell sarcoma for the following reasons. 1. Hepatosplenomegaly and enlarged lymph nodes were not observed through the entire clinical course. The main abnormalities were psycho-neurological. 2. These cells did not form a tumour mass, but infiltrated diffucely into the central nervous system and extraneural sites, and the primary focus could not be determined. Four cases of reticulum cell sarcoma in Japan, in which the initial symptoms were of neurological involvement, are reviewed.

Adult