Biomedical subjects

S Oyanagi

Publications and source records attributed to S Oyanagi.

At least 19 recordsLinked to original sources

PubMed2001

Distribution of cerebral cortical lesions in Pick's disease with Pick bodies: a clinicopathological study of six autopsy cases showing unusual clinical presentations.

We investigated six Japanese autopsy cases of Pick's disease with Pick bodies (PDPB) both clinically and pathologically, and examined the distribution of their cerebral cortical lesions using hemisphere and/or bisphere specimens. The lesions were classified into three categories (slight, moderate, and severe). Two patients with a clinical diagnosis of primary progressive apraxia and of slowly progressive aphasia had speech apraxia as their initial signs, and the other two patients were suspected as having Alzheimer's disease, with the clinical diagnosis of the remainder two patients being presenile dementia and depression, respectively. Extrapyramidal signs, believed to be rare in PDPB, were present in four patients. Severe lesions were multicentrically present in the cerebral cortices of all six cases. In two patients with speech apraxia, severe lesions were seen in the primary motor area, which generally has not been regarded as an "atrophic center" in Pick's disease. Furthermore, in a patient with depression, severe lesions were more widespread in the convexity than in the orbital region of the frontal lobe. The parietal lobes, including the postcentral gyrus usually believed to be spared in Pick's disease, were severely involved in three patients. We postulate that the clinical features of PDPB have a much wider spectrum than previously believed. In addition, we believe that the distribution of the cerebral cortical lesions in PDPB is more widespread than previously assumed, and that clinical manifestations of PDPB depend to some extent on the topographic distribution of the cerebral cortical lesions.

Aged↗

PubMed2000

Hereditary dentatorubral-pallidoluysian atrophy.

Pathology and associated clinical symptoms of hereditary dentatorubral-pallidoluysian atrophy (H-DRPLA) which was established as a new inherited neurodegenerative disease in 1982 are described. Obligatory lesions in the central nervous system combine with degeneration of the dentatorubral and pallidoluysian pathway, and occasional degenerative lesions are found in the cerebral white matter, putamen, Goll's nucleus of the medulla oblongata, and lateral corticospinal and Goll's tract of the spinal cord. The main clinical symptoms are myoclonus, epilepsy, dementia or mental retardation, cerebellar ataxia and choreoathetosis. Furthermore, newly developing aspects in the pathology of H-DRPLA following the discovery of the gene locus of H-DRPLA in 1994 are briefly described.

Cerebellar Nuclei↗

PubMed1998

Hereditary dentatorubral-pallidoluysian atrophy: ubiquitinated filamentous inclusions in the cerebellar dentate nucleus neurons.

We examined the cerebellar dentate nucleus (CDN) in 16 patients with hereditary dentatorubral-pallidoluysian atrophy (DRPLA), one of the neurodegenerative diseases caused by expansion of a CAG repeat encoding a polyglutamine tract in the disease protein. In all patients, some CDN neurons were found to contain ubiquitinated filamentous inclusions in their cytoplasm. On hematoxylin and eosin preparations, these filamentous inclusions were eosinophilic, basophilic or amphophilic, and were often found in areas of pale cytoplasm. Electron microscopy revealed that they consisted of bundles of filaments that were somewhat thicker than neurofilaments. These features of the present inclusions were indistinguishable from those of skein-like inclusions (SLI) previously described in the lower motor neurons in sporadic amyotrophic lateral sclerosis. We conclude that SLI can also occur in the CDN in DRPLA and believe that they reflect a characteristic pathological process in this disease.

Adolescent↗

PubMed1998

Dentatorubropallidoluysian atrophy: clinicopathological study of dementia and involvement of the nucleus basalis of Meynert in seven autopsy cases.

This report concerns a clinicopathological study including a quantitative pathological study on the nucleus basalis of Meynert (nbM) of seven Japanese autopsy cases (four male, three female) of dentatorubropallidoluysian atrophy (DRPLA) with special reference to the clinicopathological correlation of dementia in DRPLA. In each case the pattern of the inheritance was consistent with that of an autosomal dominant trait. The neurological examination revealed that all seven individuals had cerebellar signs. Six patients had epilepsy and choreoathetoid involuntary movement; myoclonus was evident in five patients. Dementia was noted in all seven patients. Degeneration of the globus pallidus (particularly the lateral segment) and of the dentate nucleus was the principal pathological feature. Brain weights at autopsy ranged from 1020 to 1400 g (average 1241 g: male 1320 g, female 1135 g). The quantitative evaluation revealed no significant loss of neurons in the nbM as compared with a control group. There was no clinicopathological correlation between dementia and involvement of the nbM. We suggest that the dementia of DRPLA is due not to the involvement of the nbM, but to - as yet - unidentified pathology elsewhere.

Adolescent↗

PubMed1996

[Effect of ulinastatin on delayed neuronal death in the gerbil hippocampus].

The effect of ulinastatin on delayed neuronal death was studied in the gerbil. The animals were divided into 2 groups according to the temperatures of the temporalis muscle, tympanic membrane, and the rectum. One group consisted of 36 animals in whom the temperature was maintained at 36.5 degrees C and the another was at 38 degrees C. They were anesthetized with isoflurane and injected intravenously with 5 ml.kg-1 of normal saline solution, 100 mg.kg-1 of alpha-tocopherol, 250,000 units.kg-1 of ulinastatin, or 500,000 units.kg-1 of ulinastatin, respectively. Transient ischemia was then induced by occluding bilateral common carotid arteries for 5 min. Six days later, they were sacrificed, and the brain tissues were fixed and stained for histopathological analysis of hippocampal CA1 regions. alpha-Tocopherol and ulinastatin prevented delayed neuronal death in the 36.5 degrees C group, but did not in the 38 degrees C group. alpha-tocopherol is recognized as an intrinsic radical scavenger, and known to prevent delayed neuronal death. Compared with 100 mg.kg-1 of alpha-tocopherol, 500,000 units.kg-1 of ulinastatin had the same protective effect on delayed neuronal death at 36.5 degrees C. We concluded that administration of ulinastatin prevented delayed neuronal death at 36.5 degrees C.

Animals↗

PubMed1995

Aluminium detected in senile plaques and neurofibrillary tangles is contained in lipofuscin granules with silicon, probably as aluminosilicate.

Aluminium and silicon are incidentally found in senile plaques (SP) and neurofibrillary tangles (NFT) of brains with Alzheimer's disease (AD), and have been considered as one of the risk factors for senile dementia. Since lipofuscin granules were concentrated concomitantly with SP and NFT in the high density fraction in subcellular fractionation of autopsied brains and are solid in nature, it was suspected that aluminum and silicon are accumulated in lipofuscin granules. Therefore, elemental analyses of partially purified lipofuscin granules from autopsied brains with AD and those without dementia, were attempted by energy dispersive X-ray spectrometry with a scanning electron microscope. It was demonstrated by a mapping method that aluminum and silicon were accumulated in some lipofuscin granules, probably as aluminosilicate. In addition, it was demonstrated by fluorescence microscopy of Bodian stained paraffin sections, that many SP and NFT contained lipofuscin granules, although lipofuscin granules were not always specific to those neuropathological changes. These results imply that aluminum detected in SP and NFT is due to lipofuscin granules and can explain the cause of discrepancies in the reports on the presence of aluminum in SP and NFT.

Aged↗

PubMed1995

X-ray microprobe analysis of corpora amylacea.

Since corpora amylacea is concentrated in the high density fraction in the subcellular fractionation of autopsy brain. It is suspected that inorganic materials accumulate in corpora amylacea. Therefore, elemental analyses of partially purified corpora amylacea from autopsy brain from a patient with Alzheimer's disease and those from brain of a non-demented patient were performed by the X-ray microprobe method. Prominent peaks of sodium, phosphorus, sulphur and chloride were observed, and mapping analyses confirmed that these elements were actually contained within the corpora amylacea. A similar result was obtained using cryostat sections. Corpora amylacea are characteristically distributed along the margin of blood vessels, beneath the pial border of the hippocampus and in the subependymal zones of ventricles of aged brains, namely in the vicinity of blood and cerebrospinal fluid. From this distribution and from the results of the present paper, we suggest that corpora amylacea play a role in the absorption and accumulation of inorganic materials which have been extravasated from blood and cerebrospinal fluid (CSF) and taken up by astrocytes. This may reflect alteration of the blood-brain and blood-CSF barriers in the ageing brain.

Alzheimer Disease↗

PubMed1995

Accumulation of aluminium and silicon in lipofuscin granules.

Since lipofuscin granules were concentrated in the high density fraction in subcellular fractionation of autopsy brains of normal aged subjects and of patients with Alzheimer's disease (AD), it was suspected that inorganic materials accumulated in lipofuscin granules. Therefore, elemental analyses of clusters of lipofuscin granules in the high density fraction were attempted by energy dispersive X-ray spectrometry with a scanning electron microscope. It was demonstrated that aluminium (Al) and silicon (Si) accumulated in some lipofuscin granules. Al has been considered as one of risk factors of AD since Al and Si were found in senile plaques (SP) and neurofibrillary tangles (NFT) of the brains with AD, although conflicting results also have been reported. It was also demonstrated in this experiment by fluorescence microscopy of Bodian stained paraffin sections of AD brain that many SP and NFT contained lipofuscin granules, although lipofuscin granules were not always specific to those neuropathological changes. The results of this experiment imply that Al detected in SP and NFT is contained in lipofuscin granules.

Aged↗

PubMed1995

A family with adult type ceroid lipofuscinosis (Kufs' disease) and heart muscle disease: report of two autopsy cases.

Two cases in a family with Kufs' disease had lethal arrhythmias and heart muscle disease. Autopsy findings showed an abundant accumulation of lipofuscin-like lipopigments in most neurons in the central nervous system (CNS). The heart showed a slight increase in the accumulation of the lipofuscin-like lipopigments in the myocardial fibers, slight to severe fibrosis and infiltration of fat cells in the myocardium. The lipopigments both in the heart and in neurons of the CNS had curvilinear profiles on electron microscope and reacted immunohistochemically to polyclonal antibodies against subunit c of mitochondrial adenosine triphosphate (ATP) synthase. The degenerative process in this heart muscle disease might be attributable to the same metabolic abnormality as seen in the neuronal degeneration associated with Kufs' disease.

Arrhythmias, Cardiac↗

PubMed1995

[The effect of nicorandil on the neuromuscular block induced by vecuronium].

The effect of nicorandil on the neuromuscular block induced by vecuronium was investigated in vitro with rat phrenic nerve-hemidiaphragm preparation. Both single twitch (ST) and train-of-four ratio (TOFR) elicited with indirect electrical stimulation were used. The administration of nicorandil alone showed no effects on ST and TOFR. The pre-administration of nicorandil exerted significant depressing effect on ST compared with vecuronium alone. High concentration of nicorandil (4 x 10(-6) M) produced more significant effect on ST compared with low concentration of nicorandil (10(-7) M). In this study, the nicorandil showed similar effect as calcium antagonists on neuromuscular blockade.

Animals↗

PubMed1994

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↗

PubMed1994

Extracellular or ghost Pick bodies and their lack of tau immunoreactivity: a histological, immunohistochemical and electron microscopic study.

Histological, immunohistochemical, and electron microscopic evidence of an extracellular, or ghost Pick body has been found in the granular cell layer and, rarely, in the pyramidal cell layer of the hippocampus of an autopsy case of Pick's disease. The ghost Pick body appeared as a blurred, weak argyrophilic mass in the neuropil, and it was composed of accumulated fibrillary structures, 13 nm in diameter, intermingled with glial filament bundles. These ghost Pick bodies did not react with anti-tau and anti-ubiquitin antibodies, but did react weakly with anti-glial fibrillary acidic protein antibody, whereas intracytoplasmic Pick bodies were strongly immunolabeled with anti-tau but only weakly with anti-ubiquitin antibodies. These results suggest that the Pick body is discharged into the neuropil after destruction of the mother neuron, loses its immunoreactivity to certain tau and ubiquitin antibodies during this process (thereby inducing a glial reaction) and remains in the neuropil as a ghost Pick body.

Dementia↗

PubMed1994

A clinical and pathologic study of a large Japanese family with Machado-Joseph disease tightly linked to the DNA markers on chromosome 14q.

The gene locus for Machado-Joseph disease (MJD) has been mapped to chromosome 14q by linkage analysis, mainly using a single large Japanese family. We studied the clinical and neuropathologic findings of this family with MJD, comparing them with those of spinocerebellar ataxia 1 (SCA1) and spinocerebellar ataxia 2 (SCA2) families. The pedigree included 30 affected persons in 125 members of five generations. Neurologic examination of 21 patients revealed that dystonia, difficulty in eyelid opening, slowness of movements, bulging eyes, and facial-lingual fasciculation-like movements or myokymia are characteristic of this MJD family, although these three autosomal dominant spinocerebellar degenerations have several neurologic signs and symptoms in common. In contrast with SCA1 and SCA2, degeneration of the subthalamopallidal system and relative sparing of the olivocerebellar system were the main neuropathologic features of MJD.

Adult↗

PubMed1993

Mechanical instability of Pick bodies and their isolation in an intact form using urea solution.

Isolation of Pick bodies was attempted from an autopsied brain of a patient with Pick's disease following a modified method for isolation of senile plaques cores and neurofibrillary tangles in the brains of Alzheimer's disease. Pick bodies were mechanically less stable than senile plaque cores and neurofibrillary tangles since Pick bodies were destroyed by vigorous homogenization or treatment with 1% sodium dodecyl sulfate solution. Pick bodies were released out of mother neuronal cells in an intact form by treatment with 8 M urea solution for several hours at room temperature. Finally, fine structure of Pick filaments released from Pick bodies was compared with the filaments of neurofibrillary tangles.

Brain↗

PubMed1993

An early cytoplasmic change before Lewy body maturation: an ultrastructural study of the substantia nigra from an autopsy case of juvenile parkinsonism.

Neurons containing a central pale area which may possibly represent an early cytoplasmic change before Lewy body maturation were observed in the substantia nigra from a rare autopsy case of juvenile parkinsonism. Ultrastructurally, such neurons exhibited cytoplasmic swelling along with disappearance of the rough endoplasmic reticulum from the central part of the perikaryon. The pale central cytoplasm was replaced by numerous-cored vesicles, mitochondria, ribosome-like granules and a few Lewy body filaments. The relation of this central pale area to the pale body is discussed.

Brain↗

PubMed1992

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↗

PubMed1992

Ultrastructural and immunohistochemical study of degenerate neurite-bearing ghost tangles.

Some ghost tangles in the brains of Alzheimer patients were accompanied by many small argyrophilic structures which were electron microscopically confirmed to be degenerate neurites. In these ghost tangles, roughly dispersed 15 nm straight and occasional twisted tubules were penetrated by proliferated astrocytic processes. Immunohistochemically, these ghost tangles lost immunoreactivities to anti-NFT, -tau and -ubiquitin antibodies, but were thioflavine-S fluorescent, though antigenicity to beta-protein was not proved. This similarity in composition of degenerate neurite-bearing ghost tangles to senile plaques might be induced by the amyloid nature of tubules, which probably provokes the reaction of neuropils.

Aged↗

PubMed1989

Senile plaque-like structures: observation of a probably unknown type of senile plaque by periodic-acid methenamine silver (PAM) electron microscopy.

Numerous diffuse senile plaque-like structures (SPLSs) were found in the cerebral cortex from cases with dementia of the Alzheimer type by means of the methenamine-Bodian method. SPLSs varied in shape and size. They were never recognized in the original Bodian, PAS and Congo red preparations, but were positive with anti-beta-protein immunostaining and periodic-acid methenamine silver (PAM) methods, which are thought to specifically stain amyloid substance. With PAM electron microscopy, we found sparse aggregations of amorphous, often ramified, structures with fine granular silver deposits in SPLS. Routine electron microscopic examination on the same portion where SPLS were confirmed by PAM electron microscopy revealed amorphous, partially fibrous structures. These structures might be amyloid or amyloid-precursor substance. In SPLSs only a few degenerated neurites and astrocytic processes with glycogen granules were seen. We consider SPLSs to be a kind of senile plaque.

Adult↗