ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00229.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00230.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This systematic study concerns the comparison of amygdaloid changes in 16 patients with senile dementia of the Alzheimer type (SDAT) and 16 control subjects. While previous studies have dealt mainly with the distribution of senile plaques (SPs) and neurofibrillary tangles (NFTs), we focused on the degree and distribution of both, neuronal loss and astrocytosis in the amygdala. Our results indicate that brain atrophy was restricted to the medial temporal gyri of most cases, with the anterior portion of the temporal lobes being preferentially involved. With respect to the amygdala, the most pronounced degeneration was in the basolateral subnuclei, i.e., accessory basal, basal, and lateral nuclei. We found that the degree of astrocytosis exceeded that of neuronal loss by far, and that the accessory basal nucleus exhibited the most severe alterations. There was marked neuronal loss with astrocytosis in the cerebral cortex, entorhinal cortex and prosubiculum, and there appears to exist a correlation of the degree of neuronal loss in the amygdala with that in the entorhinal cortex and prosubiculum. By contrast, the presence of SPs and NFTs in the amygdala did not correlate with the extent of neuronal loss, but numerous SPs and NFTs were seen in the corticomedial subnuclei of the SDAT patients. However, the striking loss of neurons and the pronounced astrocytosis in the basolateral subnuclei merit emphasis, since these abnormalities could be responsible for the atrophy of the amygdala.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00231.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This report concerns the immunohistochemical detection of the intercellular adhesion molecule‐1 (ICAM‐1), HLA‐ABC and HLA‐DR in consecutive cryostat sections of skeletal muscle biopsies from 38 patients with inflammatory myopathy. Among these were 12 cases of dermatomyositis, 22 cases of polymyositis, two cases each of inclusion body myositis and sarcoid myopathy. Specimens from eight cases of Duchenne muscular dystrophy, three cases each of alcoholic polyneuropathy and myotonic dystrophy, two cases of motor neuron disease, and three normal individuals were included for comparison. Sarcolemmal ICAM‐1 expression by undamaged muscle fibers was detected in 18/38 cases of inflammatory myopathy who had large numbers of endomysial inflammatory cells, but not in normals or patients with non‐inflammatory myopathy. Sarcolemmal immunostaining was more intense in the vicinity of the inflammatory cells. Some regenerating muscle fibers, irrespective of disease, expressed sarcoplasmic ICAM‐1. Sarcolemmal HLA‐ABC and HLA‐DR immunoreactivity was detected in undamaged muscle fibers of 37/38 and 18/38 cases of inflammatory myopathy, respectively. Sarcolemmal immunostaining for HLA‐DR was observed in cases with large amounts of endomysial inflammatory cells, and was stronger in muscle fibers that were in their vicinity. HLA‐DR and ICAM‐1 immunoreaction product deposits were generally colocalized in the sarcolemma of the same fibers. The specific sarcolemmal expression of ICAM‐1, and its colocalization with HLA‐DR may be important for the recognition of muscle fibers by invading inflammatory cel

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00232.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

With the purpose of achieving a better correlation between the neuropathologic features observed by light microscopy (LM) and those seen by electron microscopy (EM), the authors attempted to use a conventional electron microscope (EMe) to obtain images at the extremely low magnification (mag) of 500x. This goal was successfully accomplished by using the methodology devised for this investigation, for which 100 nm thick, 2×2 mm sections were prepared. These were mounted on 75 hexagonal grids (VECO), double‐stained for a slightly longer period of time than usual, and then examined with a conventional EMe using the low mag mode lens system and a 100 KV accelerating voltage. The technique was used for the examination of three degenerative structures, to wit: grumose degeneration, stellate body and senile plaques. The present method allowed satisfactory viewing at an extremely low mag, as a given degenerative structure readily fit in its entirety on one sheet of film. The structural features were strikingly more distinct when viewed at low mag EM than at a similar LM mag. For instance, by using the former, the central cores of classical senile plaques had the appearance of chestnut burrs or of a herring‐bone pattern with numerous amyloid bundles. Based on their results, the authors believe that the low mag EM technique provides an essential step toward the correlation of LM and high mag EM find

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00233.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This report concerns an autopsy case of a peculiar neurodegenerative disorder associated with abnormal copper metabolism. The patient was a 46‐year‐old woman, who presented with dementia and ataxia, and who died after a progressive clinical course of approximately 19 months. The patient had marked hypocupremia and hypoceruloplasminemia. The neuropathological examination revealed: (1) neuronal loss with remarkable rarefaction of the neuropil of the mammillary body; (2) severe thalamic degeneration; (3) degeneration and softening of the olivary nucleus, accompanied by remarkable capillary proliferation; (4) coarse spongy state or vacuolization of the cerebral white matter, and (5) degeneration of Clarke's column. The cerebral cortex, basal ganglia, and cerebellar cortex were preserved; brain deposition of iron and copper was not detected. The liver showed congestion, parenchymal atrophy and iron deposition, but neither copper deposition nor cirrhotic changes. Although the clinical features of patients with neurological disorders associated with abnormal copper metabolism, but distinct from both Wilson's disease and Menkes' kinky hair disease have been described, no autopsy case report has been published to date. It is of particular interest that the subnuclear distribution pattern of the thalamic lesion and the isolated degeneration of Clarke's column and the posterior spinocerebellar tract of the spinal cord of the present patient closely resembled the lesions in Menkes' kinky hair dise

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00234.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00235.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY