摘要:

Amyotrophic lateral sclerosis (ALS) is an age‐associated neurodegenerative disease that primarily involves the motor neuron system. Despite this disease having been studied for over 100 years, its etiology is still unknown and no specific diagnostic laboratory test is available. The diagnosis of ALS is therefore based on clinical and/or neuropathological findings with loss and degeneration of the large anterior horn cells of the spinal cord being the principal and most salient features. Several symposia and workshops on the cytopathology of ALS were held within the last 2 year period, which reflects the many new and important developments taking place. Although loss of motor neurons has been well‐known for many years, cytoplasmic alterations of the lower motor neurons have been studied in detail only in recent years by applying immunohistochemical and electron microscopic procedures. The purpose of this review is to introduce some of the novel findings associated with lower motor neuron alterati

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00277.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Double immunofluorescent stainings of neurofibrillary tangles (NFT) were performed using anti‐tau, ubiquitin, amyloid P component and neurofilament antibodies. A confocal laser scan microscope was used to investigate the distribution and the relationship of these components. Tau‐ and ubiquitin‐immunoreactivities (IR) were observed in the same NFT. Tau‐ and neurofilament‐IR were observed in the same neuron. Tau‐ and amyloid P com‐ponent‐IR were not colocalized in the same NFT. These findings may suggest a process of NFT‐formation which will help in the understanding of pathophysiology of Al

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00278.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Cerebellar medulloblastoma has been recognized as a distinct clinicopathological entity; however, its origin and its differentiating capacity are still disputed because of the primitive nature of the tumor cells. To assess the nature of so‐called medulloblastoma, 12 cases of medulloblastoma‐neuroblastomas, two primitive neuroectodermal tumors (Rorke's NOS category) and two cerebral neuroblastomas were studied. Moreover, some mature neuronal tumors, and some glial and mesenchymal tumors as controls, were examined by electron microscopy and immunohistochem‐istry using a panel of the antibodies. The relatively high frequencies of neuronal differentiation in the so‐called medulloblastoma and the paucity of glial differentiation, albeit with the absence of similar neurofilament immuno‐reactivity in the neoplastic cells, suggest the histogenesis of these neoplasms is more closely aligned with the histogenesis of neuronal elements in the central nervous system.Human β4 tubulin isotype (TuJl) first becomes positive when primitive neuronal neoplastic cells start to form neuritic processes from the cytoplasm. A maturation‐associated switch from vimentin filament synthesis to (34 tubulin may occur in the primitive neuronal neoplastic cells. TuJl is a reliable marker of neu

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00279.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

The densities of senile plaques (SP) and neurofibrillary tangles (NFT) were studied in the cerebral cortex in nine autopsy cases of Alzheimer's disease (AD) and seven cases of senile dementia of Alzheimer type (SDAT). The number of SP and NFT were counted in 14 selected areas of the cerebral cortex using methenamine‐silver and Gallyas‐Braak stains, respectively. The ratio of cortical atrophy in each AD or SDAT case to the mean value in normal age‐matched cases was estimated, and the results were corrected for cortical atrophy using this ratio. The SP density in AD after correction was significantly higher than that in SDAT in the superior and inferior frontal, rectal, anterior cingulate, superior temporal and suprapar‐ietal cortices. The NFT density in AD was also significantly higher in all of these areas and also in the medial temporo‐occipital cortex. The significant difference between AD and SDAT for NFT density after correction was greater than that for SP density in the superior and inferior frontal, anterior cingulate and superior temporal cortices. These results suggest that the higher densities in AD of SP and NFT in these areas are useful in distinguishing AD from SDAT. The NFT density was a more useful indicator than the SP density in differentiating between the two conditions. The greater density in AD of SP and NFT in the cerebral neocortex may reflect the focal clinical symptoms, frequently observed in AD. The quantitative difference between AD and SDAT in the two changes observed in this study was not due only to the difference in the duration of illness, because the SDAT cases with long survival showed significantly lower NFT density than AD cases with similar disease duration and brain weight. It is suggested that degeneration process in AD progresses more rapidly than tha

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00280.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

We identified 19 cases of dementia characterized by cerebral atrophy with non‐specific cortical degeneration, but without pathologic features of Alzheimer's disease or other known neurodegenerative disorders. The cases were selected by a review of 14 000 autopsies and from the clinical records of the Mayo Clinic from 1972 to 1992. Microscopically, four general topographic patterns of atrophy were identified: frontal (n=6), frontotemporal (n=4), temporal (n=3), and mesiotemporal (n=4). The characteristic microscopic findings in the atrophic cortex included neuronal loss with astrocytosis, which predominantly involved the upper cortical layers, and superficial vacuolar changes. A focal or regional accentuation of involvement with different extents and severities of change was common. Neuronal achromasia was present in seven cases. Rare to sparse ubiquitin‐positive linear structures were identified in small neurons in the second cortical layer in nine cases. Argentophilic cortical grains, predominantly in the subiculum, were detected in only three cases. Involvement of the cerebral white matter corresponded to the cortical involvement. The striatum, thalamus, or substantia nigra was affected in all cases. Hippocampal involvement was evident only in the mesiotemporal type; the prosubiculum was the most severely affected area. After analyzing similar cases which had previously been reported under diverse diagnostic terms, we propose the term ‘simple cerebral atrophy of non‐Alzheimer type’ as a tentative comprehensive category for direct correlation, encompassing a spectrum of non‐Alzheimer ty

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00281.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Examination of spinal ganglia of ageing and old Japanese monkeys,Macaca fuscata, by light microscopy and electron microscopy revealed unidentified pathology of the axon initial segment. Although the incidence of this change is infrequent in any one histological section of ganglion, its morphology is quite remarkable. The change is characterized by a progressive swelling of the unmyelinated portion of axon followed by continuous involvement of the first myelin segment. Neurofilaments proliferate in rather randomly oriented bundles within the affected axons accompanied with a considerable amount of mitochondria. The axons, originally 3–5 um in diameter, thus become nodularly varicose and become as thick as 20 um or more in diameter. The second stage of the axonal pathology is characterized by massive dense accumulation of tiny vesicular granules which infiltrates and/or replaces the neurofilament mass, almost fills the whole axon and the affected axons could result in a thickness of 70 um or more in diameter (i.e. larger than the mother perikaryon itself). The third stage of the axonal pathology is characterized by a catabolic transformation of those dense massive aggregates of the vesicular granules into irregular patches of amorphous electron dense substance. Smooth endoplasmic reticulum may make a focus of proliferation within the affected axon, but its development is relatively poor and restricted. It is surprising that the mother perikaryon of the affected axon initial segment remains as a rule quite normal in appearance. It is also remarkable that the part of the axon which is distal to the swelling remains viable, apparently for a long time. The significance of this axonal pathology is discussed in relation to ageing and diabetic axonal dystrophy in the sympathetic ganglia, and to ageing axonal dystrophy in the gracile nucleu

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00282.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

In order to elucidate the enhancement mechanism of amyloid staining by formic acid (FA) pretreatment on a tissue section, we investigated changes of immunoreactivity of amyloid P component (AP) in senile plaques of Alzheimer‐type dementia (ATD) brains. Although immunoreactivity of various amyloidogenic proteins is enhanced by FA treatment, the immunoreactivity of AP was reduced by the treatment in this study. Amyloid P is thought to deposit to amyloid structure by leakage from blood vessels. Therefore, this result suggests that the enhancement effect depends not only on the property of amyloidogenic protein itself but also on the binding form to amyloid structur

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00283.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

An autopsy case of an anaplastic xanthomatous tumor involving the pharynx and clivus in a 2 year old girl was investigated ultrastructurally and immunohistochemi‐cally. The tumor was manifested as an epi‐ and subdural mass in the posterior cranial fossa involving the pharynx and clivus. The tumor showed an interlacing fascicular and pleomorphic pattern. The tumor cells contained numerous intermediate filaments, which were mainly tonofilaments, and lipid droplets, but did not have junctional complex. Immunohistochemically, most of the tumor cells were positive for cytokeratin, epithelial membrane antigen and vimentin, and some tumor cells were positive for myoglobin and a‐1 antichymotrypsin, but negative for S‐100, CD‐68 and glial fibrillary acidic protein. These findings suggest that this tumor was derived from epithelial cells, and showed xanthomatous appearance. A discussion of its differential diagnosis was

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00284.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00285.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00286.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY