ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00249.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

The presentation and distribution of neurofibrillary tangles (NFT), neuropil thread‐like structures (T‐LS) and smaller structures (dots) in the spinal cord of two patients with progressive supranuclear palsy (PSP) is discussed. Monoclonal antibodies to Alzheimer neurofibrillary tangles (ANT) and tau protein were used. Immunoreactive NFT were detected in the spinal cord gray matter. The number of tau‐positive NFT was significantly smaller than that of NFT stained by the anti‐ANT antibody. However, T‐LS and dots immunolabeled by the latter were detected in the gray and white matter. Most white matter T‐LS and immunoreactive dots were found in the anterior funiculus. These findings provide immunohistochemical evidence for the presence of these abnormal structures in the spinal cord white matter of patien

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00250.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Numbers of corpora amylacea (CA) were observed among 1407 autopsied and/or biopsied cases where diabetes mellitus had been diagnosed clinically and in those where the diabetes mellitus did not exist. A comparative examination was carried out by a semi‐quantitative method which revealed a significant difference in the incidence of CA between 100 diabetic and 100 non‐diabetic cases. However, there was not a significant difference in the incidence of CA between the diabetic and ischemic cases who have also shown a tendency for developing CA. The results were evaluated statistically by homogeneity examination. Diabetes may enhance the tendency for forming CA by the hyperglycemia increasing the quantity of unused carbohydrate polymers, which are the main components of CA. Changes in the metabolism of glucose by astrocytes caused by aging or by some pathological processes may play a primary role in the developing of CA. Other factors such as the greater volume of cerebrospinal fluid, barrier disturbances and pathological changes which are associated with the appearance of heat stroke protein positivity in CA, also have a prominent role in this type of astrocytic degeneration. The presence of CA does not have any pathognostic significance but some conditions favor its development. A theory is also suggested for the formation of CA similar to other types of biocrystalloids in the central nervous sys

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00251.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

A quantitative study of the distribution and incidence of Lewy bodies (LB) and senile changes was performed in seven subnuclei of the amygdala in 12 cases with diffuse Lewy body disease (DLBD). The results were compared with those in six cases of idiopathic Parkinson's disease (PD), six cases of Alzheimer‐type dementia (ATD) and six cases of non‐demented controls. Ubiquitin immunohisto‐chemistry for LB, methenamine‐silver stain for senile plaques (SP) and Gallyas stain for neurofibrillary tangles (NFT) were used in this study. In all DLBD cases and four PD cases, cortical LB occurred in all subnuclei with a tendency for their preferential occurrence in certain sub‐nuclei. The probable dysfunction in these subnuclei may contribute to the memory impairment of DLBD. Senile plaques and NFT occurred significantly more frequently in the baso‐lateral nuclear group than in the cortico‐medial nuclear group of the amygdala in DLBD cases as well as in ATD cases, although a tendency for these to occur predominantly in certain subnuclei was not observed. In the central amygdaloid nucleus (CE), many characteristic spherical or fusiform structures were stained positively with ubiquitin immunohistochemistry in all DLBD and PD cases, although the number was less in PD cases. These immunohistochemically ubiquitin positive structures were densely packed electronmicroscopically with ubiquitin‐positive granular and vesicular components. They might have arisen from selective degeneration of the terminal or distal axons of noradrenergic and dopaminergic afferents from the locus ceruleus and substantia nigra, respecti

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00252.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

An 88 year old female had an epileptic attack, followed by fever, vomiting and stupor. A computerized tomogram disclosed bilateral periventricular lucency and a low density area in the right occipital lobe. Antibiotics, hypertonic sodium chloride, steroids, and restricted water intake were ineffective. She died 6 weeks after admission. Autopsy showed that there were extensive irregular necroses in both cerebral hemispheres. Microscopic study disclosed diffuse meningitis and non‐caseating granulomatous inflammation with macrophages and giant cells of Langhans' type in the small‐ and medium‐sized meningeal vessel walls. Some giant cells engulfed elastic membranes in their cytoplasms. The lumina were often thrombosed and her neurological progression was due to diffuse cerebral infar

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00253.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

An 88 year old woman with slowly progressive dementia of 5 years' duration experienced rapidly progressive consciousness disturbance, myoclonus and periodic synchronous discharge on electro‐encephalography from 3 months before her death. Post‐mortem examination revealed numerous senile plaques reactive with antibody against p‐peptide and widespread occurrence of spongiform change, astrocytosis and marked neuronal loss in the entire cerebral cortex and amygdaloid nucleus. These findings indicate that Creutzfeldt‐Jakob disease (CJD) and senile dementia of the Alzheimer type (SDAT) occurred in the same patient. As the number of aged patients with dementia in care homes and hospitals for the elderly is increasing, it is important to know that CJD can occur late in life during the course of SDAT, as shown in the prese

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00254.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

An autopsy case of progressive myoclonic epilepsy (PME) with unusual neuropathologic features is discussed. The patient was a 46 year old man who presented with prominent cerebellar ataxia and an intention tremor, in addition to myoclonic movements and grand mal seizures. He was clinically diagnosed as having PME, in particular dys‐synergia cerebellaris myoclonica (DCM; Ramsay Hunt syndrome). A neuropathologic examination revealed severe degeneration of the cerebellar cortex, mild nerve cell loss with marked fibrillary gliosis of the dentate nucleus, and focal laminar necrosis and mild diffuse nerve cell loss in the frontal cortex. Other recent clinicopathologic studies have revealed that most patients with Ramsay Hunt syndrome are diagnosed pathologically as having either mitochondrial encephalomyopathy, especially myoclonus epilepsy with ragged red fiber (MERRF), or dentatorubral or dentatorubropallidoluysian atrophy (DRPLA). The present case is thought to be unusual in that no pathologic features of MERRF or DRPLA were demonstrate

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00255.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

A 58 year old woman presented with a right frontal lobe cystic mass diagnosed as pleomorphic xanthoastrocytoma (PXA), in which a reticulin fiber network was not evident but prominent vasculature was noted (angiomatous variant). The patient had a 36 year history of epileptic seizure which had been relatively well controlled with anticonvulsants. An ultrastructural study revealed that the neoplastic astrocytes occasionally contained filamentous inclusions identical to Hirano bodies in their cytoplasm and that, at the periphery of the tumor, pre‐existing axon terminals and synaptic junctions were found in close proximity to neoplastic astrocytes. The former may represent a degenerative process in the constituent neoplastic astrocytes and the latter appears to be of interest when considering the epileptogenic lesions associated with this type of benign tumo

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00256.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

The discovery of the abnormalities of CAG codon repeats in dentatorubropallidoluysian atrophy (DRPLA) by gene analysis has had a strong impact on human neuropathology. Some believe that the new method can show all aspects of this disease. This paper highlights many gaps which exist between the genotype and the phenotype, and consider that human Neuropathology could fill these gaps. It is emphasized that pathogenetical investigation, which is needed to link gene abnormalities, has been inadequate to date. It is also stressed that a clinico‐anatomical approach is still necessary for progress in pathogenetical research and human neuro‐anat

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00257.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

This paper reports a clinicopathological study of eight Japanese patients with dentatorubropallidoluysian atrophy (DRPLA) with special reference to the clinicopathological correlation between the pyramidal sign and involvement of the pyramidal tract. None of the patients were related. There were five males and three females with age at disease onset ranging from 8 to 43 years, and the length of clinical course from 12 to 28 years. In each case the pattern of inheritance of the disease was consistent with that of an autosomal dominant trait. The neurological examination revealed that all eight individuals had dementia and showed cerebellar signs. Seven patients had epilepsy and choreoathetoid involuntary movement; myoclonus was evident in six cases. Degeneration of the globus pallidus (the lateral segment in particular) and of the dentate nucleus were the principal pathological features of the DRPLA patients. Pyramidal signs were noted in four patients. Evidence of pyramidal tract involvement was found in three cases. However, the clinicopathological correlation between pyramidal sign and pyramidal tract involvement was weak as there was concurrence of both in only one case. We suggest that pyramidal signs are being overlooked in DRPLA.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1995.tb00258.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY