摘要:

We describe the presence of tau protein‐positive argyrophilic meshwork structures in the cerebral corticomedullary junctions of three patients with progressive supranuclear palsy (PSP). Meshwork structures are composed of fine, winding fibrils, partly connected to coiled inclusions in oligodendrocytes, and are present on neurofilament protein‐positive axons. The novel abnormal structures are numerous in the middle frontal and precentral gyri, and at the ultrastructural level they are present mainly in the outer myelin sheath. Our results suggest that the unusual meshwork structures are formed in processes of oligodendroglia in the cerebral cortex. Further study of well characterized cases is required in order to determine the significance of these structures in cerebral cortical functions in patients with

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00146.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

We observed abundant membranous cytoplasmic bodies in several remaining anterior horn neurons in two patients with sporadic amyotrophic lateral sclerosis (ALS). These bodies were rare in the neurons of the frontal cortex and of the rectum examined in one case. The link between membranous cytoplasmic bodies and ALS is vague; however, pathogenesis of ALS is unknown and there are few reports on the lysosomal system in ALS. Therefore, further study is needed in order to evaluate abnormalities in the lysosomal system to clarify the degenerative processes in ALS.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00147.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

This is an immunohistochemical investigation of the indusium griseum (supracommissural hippocampus) of the brains of control subjects and patients with Alzheimer's disease (AD). Our assays revealed the presence of fine deposits of synaptophysin‐positive dots in the neuropil and around neurons in the indusium griseum of control subjects. By using an antibody to Alzheimer neurofibrillary tangles (NFT) we identified NFT and neuropil thread‐like structures in the indusium griseum of AD patients; no β‐amyloid protein‐containing senile plaques were seen. To our knowledge, this is the first documentation of the occurrence of NFT in the indusium griseum. We also performed immunohistochemical assays on anterior sagittal sulcal lesions of the corpus callosum (ASSLCC), also known as surface lesions of the corpus callosum. These studies revealed a uniformly increased expression of the 200 kDa phosphorylated neurofilament protein by the axons in the pale myelin areas around the zonal depressions caused by the mechanical pressure of the anterior pericallosal artery. By contrast, immunoreactivity with an antibody to glial fibrillary acidic protein was either unchanged or reduced in th

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00148.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Experimental allergic encephalomyelitis (EAE) is an organspecific, cell‐mediated inflammatory autoimmune disease and is regarded as a model of multiple sclerosis (MS). Relapsing EAE was induced in Lewis rats and the effect of mizoribine on the relapsing EAE was examined clinically, histologically and immunohistochemically. Mizoribine (4‐carbamoyl‐1‐β‐D‐ribofuranosyl‐imidazolium‐5‐olate), an immunosuppresive agent, is an imidazole nucleotide isolated from a culture infiltrate of Eupenicillium brefeldianum M‐2166. Most control and treated rats suffered two attacks. Treated rats at a dose of 10mg/kg per day showed clinically significant delay of the disease attacks and histologically reduction of infiltrating cell number. Treated rats at a dose of 20 mg/kg per day showed a further delay in the attacks and attenuation of clinical signs, and a smaller number of inflammatory cells and Ia positive cells were revealed at the first attack. This study suggests that mizoribine suppresses the clinical severity and inflammat

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00149.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Three autopsied cases of pallidonigroluysian atrophy (PNLA) were studied clinicopathologically and 11 previously reported cases were reviewed for the purpose of clarifying the clinicopathological characteristics of the disease. The main clinical symptoms are parkinsonism, predominantly akinesia or pure akinesia with or without effective levodopa therapy. Major lesions approximately were confined to the globus pallidus, subthalamic nucleus and substantia nigra. Gallyas‐Braak method and immunohistochemistry with a monoclonal antibody (mAb) to tau‐2 protein (tau‐2) revealed a small number of neurofibrillary tangles (NFT) and argyrophilic and tau‐2 positive structures within the cytoplasms of the glial cells in the major lesions. Immunohistochemistry with a mAb to Alzheimer NFT (Ab39) demonstrated only a few classical tangles in the globus pallidus in one case. These findings suggest that PNLA can be pathologically differentiated from progressive supranuclea

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00150.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The case of a 6 year old girl with aneurysmal bone cyst (ABC) presenting as a subperiosteal hematoma is reported. Postoperative computerized tomography revealed a new multicystic lesion suggestive of ABC which disappeared spontaneously about 1 month after the operation. The slowly progressing process and spontaneous disappearance of ABC in this case may be noteworthy when considering the surgical indication of this pathology.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00151.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

A cerebral type of Lewy body disease (LBD) is proposed. Lewy body disease was split formerly into three types: brainstem type, transitional type and diffuse type. The diffuse type is now called diffuse Lewy body disease (DLBD). These three types are characterized pathologically by the presence of a large number of Lewy bodies in the CNS. In the brainstem type, Lewy bodies are numerous in the brainstem and diencephalon nuclei, and in DLBD, a vast number are present not only in these nuclei but also in the cerebral cortex and amygdala. In the cerebral type of LBD, as many Lewy bodies are found in the cerebral cortex and in the amygdala as there are in DLBD, but only rarely are they present in the brainstem and diencephalon nuclei. Thus, this type of LBD is different from other types in that it has no parkinson pathology. Therefore, parkinsonism fails to occur throughout the whole clinical course of this disease. The existence of a cerebral type of LBD suggests that Lewy bodies occur in the cerebral cortex earlier than in the brainstem nuclei and that cortical Lewy bodies appear even when the mesocortical dopaminergic system is intact. In addition, this might explain why dementia frequently precedes parkinsonism in DLBD.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00152.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

This report concerns an autopsy case of a unique type of multiple system degeneration of the central nervous system. The patient was a Japanese man who was 57 years old at the time of death. The family history did not reveal hereditary burden. His clinical manifestations included deafness in the latter half of the second decade of life, followed by cerebellar ataxia with disturbance of deep sensation. Slight dementia, including psychiatric signs, appeared at the terminal stage of the illness. The neuropathological examination revealed severe degenerative changes in the subthalamic nucleus, substantia nigra, dentate nucleus, superior olivary nucleus, inferior olive, and posterior column. As far as we are aware there is no report of a patient with multiple system degeneration, whose lesions involved such a wide range of anatomical sites as the present case. Because some of his alterations are seen in other disorders, we will address differences between our case and those of other disorders. It is concluded that our patient did not have optico‐cochleo‐dentate atrophy, Friedreich's ataxia, or Machado‐Joseph di

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00153.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Hereditary dentatorubropallidoluysian atrophy (DRPLA) is an inherited disease with an autosomal dominant trait. Its cardinal symptoms are myoclonus, epileptic seizures, ataxia, choreoathetosis, and dementia. The age of onset ranges from childhood to senescence. There is a particular correlation between the age at onset and clinical symptoms; progressive myoclonus epilepsy is a characteristic feature of DRPLA patients with juvenile onset, whereas those with late adult onset manifest cerebellar ataxia and choreoathetosis usually without myoclonus with or without epilepsy. The clinical forms of DRPLA may be classified into three subtypes: juvenile, early adult and late adult type.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00154.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

We examined 12 autopsy cases of dentatorubropallidoluysian atrophy (DRPLA). We confirmed the commonly or often recognized neuropathological findings in DRPLA as follows: 1, thickening of the skull bone; 2, atrophy of the brain; 3, degeneration of the dentate nucleus and its afferent fibers; 4, degeneration of the globus pallidus‐subthalamic nucleus system; 5, atrophy of the tegmentum of the brain stem especially in the pons; 6, symmetrical necrotic small foci of the ventro‐lateral portion of the pontine tegmentum; 7, degeneration of the striatum; 8, degeneration of the superior colliculus; 9, degeneration of the gracile nucleus; 10, degeneration of the pyramidal tract; 11, mild degeneration of the cerebellar cortex; 12, mild degeneration of the cerebral cortex; and 13, degeneration of the cerebral white matter. From the histopathological point of view, DRPLA is clearly distinguished from Machado‐Joseph disease and myoclonus epilepsy associated with ragged‐red fibers. We also examined the clinico‐pathological correlations in three clinical groups which were classified as juvenile type, early adult type and late adult type. We noted that in juvenile type presenting with progressive myoclonus epilepsy syndromes as the main clinical features of DRPLA, degeneration of the globus pallidus was more severe than that of the dentate nucleus, and atrophy of the brain stem and spinal cord was mild. We also noted that in late adult type presenting with cerebellar ataxia and choreoathetoid involuntary movements without myoclonus or epilepsy or either, degeneration of the dentate nucleus was more severe than that of the globus pallidus, and severe atrophy of the brain stem and sp

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1996.tb00155.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY