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1.
Argyrophilic grain disease (AgD), a frequent type of late onset dementia, is characterized by the occurrence of Gallyas-stained neuropil grains in the hippocampus, entorhinal cortex, amygdala and hypothalamus. High numbers of neurons containing hyperphosphorylated tau protein, but devoid of tangles, are encountered in areas rich in argyrophilic grains (ArGs). A third type of change consists of slender argyrophilic and tau-immunoreactive cytoplasmic inclusions in white matter oligodendrocytes, the coiled bodies. We now extend earlier studies on glial pathology in AgD (20 cases) and compare the results with glial changes in old age (10 cases) and Alzheimer’s disease (AD; 7 cases). Numerous non-argyrophilic, non-neuronal tau-positive stellate cells in the amygdala and anterior entorhinal cortex were consistently found in all of the 20 AgD cases but not in AD cases. Double-labelling experiments performed on paraffin sections with phosphorylation-dependent anti-tau antibody AT8, anti-glial fibrillary acidic protein and anti-CD44, revealed coexpression of these markers in stellate cells. The high expression of CD44 indicate that they probably correspond to reactive astrocytes. Unlike astrocytic plaques in corticobasal degeneration (CBD), where AT8 reactivity is accumulating in distal astrocytic processes, tau reactivity in AgD was found in all astrocytic cell compartments. The absence of glial fibrillary tangles further distinguished tau-labelled astrocytes in AgD from astrocytic plaques in CBD and tufted astrocytes in progressive supranuclear palsy (PSP). In contrast to AD and aged non-demented control cases tau-positive non-argyrophilic astrocytes represent a consistent finding in anterior limbic structures in AgD. Our findings point to a more widespread pathology of the glial cell population in AgD than previously supposed, and will be of further help in differentiating AgD from other neurodegenerative disorders, including AD, PSP, CBD and Pick’s disease. Received: 2 November 1998 / Revised, accepted: 27 January 1999  相似文献   

2.
Recent knowledge on tau-positive glial abnormal structures and their clinico-pathological significance were introduced. The structures outlined here, which are strikingly visualized by the modified Gallyas-Braak method and have tauepitopes, included (i) tuft-shaped astrocytes, (ii) thorn-shaped astrocytes, (iii) glial coiled bodies, (iv) argyrophilic threads, (v) firework-like structures and (vi) argyrophilic perivascular astrocytic feet. This study concludes that the tuft-shaped astrocytes are favored in cases of progressive supranuclear palsy (PSP), and that there is a massive appearance of both the argyrophilic threads and the firework-like structures characteristic in prototype of corticobasal degeneration (CBD). Unlike these structures, glial coiled bodies were observed in a wider range of neurological diseases such as PSP, CBD and some other cases with precocious appearance of the neurofibrillary tangles, although the glial coiled bodies were rare in routine laboratory examination and were not always uniform in their configuration. Thus, the modified Gallyas-Braak method is useful for the diagnosing of some neurodegenerative diseases with tau abnormalities, especially of PSP and CBD. It must be noted, however, that this method could also impregnate tau-negative structures such as glial cytoplasmic inclusions (oligodendroglial microtubular tangles) in multiple system atrophy or grumose or foamy spheroid body in various conditions, especially in PSP and CBD.  相似文献   

3.
An aged albino male cynomolgus monkey (Macaca fascicularis) more than 35 years old died after showing neurological signs including gait disturbance, trembling, drowsing tendency and a decrease in activity. Neuropathological examination revealed glial fibrillary tangles (GFTs) mainly distributed in the putamen, caudate nucleus, thalamic nuclei, substantia nigra, red nucleus, globus pallidus, trapezoid body, pyramid, pons and medulla oblongata of the brain, and neurofibrillary tangles (NFTs) in the thalamic nuclei. These structures were positively stained by the modified Gallyas-Braak (GB) method and immunostained for tau. The tau-positive argyrophilic GFTs were morphologically classified into four types, as in human cases, i.e., tufts of abnormal fibers (TAFs), thorn-shaped astrocytes (TSAs), glial coiled bodies (GCBs) and argyrophilic threads (ATs) depending on their GB profiles, and GCBs were the major structures in this case. Some of these structures were also immunoreactive for α-synuclein. The glial cells possessing the structures were negative for glial fibrillary acidic protein, a marker for astrocytes, indicating that the argyrophilic GFTs were present in oligodendroglia. In addition, marked neuronal loss and ubiquitin-positive spheroid bodies were observed in the substantia nigra and globus pallidus. According to the characteristic distribution of the argyrophilic structures in neurons and glial cells as well as clinical signs, the monkey might have suffered from a neurodegenerative disease such as progressive supranuclear palsy (PSP). This is the first report of the occurrence of a neurodegenerative disease in a nonhuman animal. Received: 16 November 1999 / Revised, accepted: 31 January 2000  相似文献   

4.
We conducted an immunohistochemical and ultrastructural examination of the spinal cords from 11 cases of cervical spondylotic myelopathy (CSM), together with those from 11 age- and sex-matched control subjects. Immunostaining with AT8 antibody revealed various numbers of tau-positive neuropil thread-like structures (NTSs), often demonstrating a conspicuous astrocytic foot-like perivascular or subpial arrangement, and glial cells with short and thick processes, so-called thorn-shaped astrocytes (TSAs), in the affected cervical cords in 8 of the 11 CSM cases (73%). A number of tau-positive neuronal cytoplasmic pretangles/tangles were also found in the gray matter in all the CSM cases (100%). No such astrocytic or neuronal tau lesions were found in the control subjects. The tau deposited in the NTSs and TSAs was predominantly 4-repeat tau, whereas the neuronal cytoplasmic pretangles/tangles contained both 3-repeat and 4-repeat tau. Ultrastructurally, paired helical filaments about 20 nm wide, together with glial filaments, were detected occasionally in the astrocytic processes. In conclusion, the present findings indicate that astrocytic and neuronal tau lesions appear in the affected cervical cord during the disease process of CSM.  相似文献   

5.
Corticobasal degeneration CBD) bears some resemblance to progressive supranuclear palsy (PSP) with regard to its widespread neuroal and glial cytoskeletal abnormalities. In addition, CBD and Pick's disease (PD) are both characterized by circumscribed cerebral atrophy and cortical ballooned neurons. Clinically, all three disease can present with a dementia and may be confused with each other. We examined the morphology and differential distribution of glial fibrillary tangles in PSP (n= 10), CBD (n= 3) and PD with Pick bodies (n= 2). Ballooned neurons were found in two cases of PSP but were much fewer than those found in the CBD or PD. Althought the subcortical lesions in PSP and CBD were similar, the involvement of the subthalamic and dentate nuclei was less severe in the latter. Gallyas-Breaak method revealed that tuft-shaped astrocytes were found almost exclusively in PSP, whereas astrocytic plaques were specific for CBD. None or few argyrophilic structures were visualized in PD. These findings indicate that each disorder is a distinct pathological entity. It is possible that a subpopulation of previous cases reported as unusual case of PD without Pick bodies may be additional examples of CBD.  相似文献   

6.
The immunoreactivity to the free radical-related enzymes, nitric oxide synthase (NOS) and superoxide dismutase (SOD), was examined in brain tissue in progressive supranuclear palsy (PSP). To determine the relationship between the immunoexpression of these enzymes and tau-positive, argyrophilic cytoplasmic inclusions, which are constantly present in PSP brains, double-label immunohistochemistry was applied. We demonstrated for the first time that strong inducible NOS-like immunoreactivity (iNOS-ir) was detected in tau-positive astrocytes that bore tufts of abnormal fibers (TAF), but not in oligodendrocytes containing argyrophilic/tau-positive coiled bodies nor in microglia. No brain NOS-ir was detected in neurons with neurofibrillary tangles. MnSOD-ir was also detected in tau-positive astrocytes and oligodendrocytes. Nitrotyrosine-ir of variable intensity was observed in astrocytes, oligodendrocytes and neurons. Our results indicate: (1) that TAF-bearing astrocytes may be a major source of excessive NO in PSP brains; (2) that after the induction of iNOS by unknown stimulating factors, TAF-bearing astrocytes produce an excessive amount of NO that exceeds the detoxification capability of SOD; and (3) that peroxynitrite and excessive NO, both cytotoxic, may be present in astrocytes, oligodendrocytes and neurons. Although the precise relationship between NO production and neuronal cell death in PSP remained uncertain, based on the specificity of TAF for PSP brains, our results indicated a possible mechanism of NO-mediated cytotoxicity that may contribute to the neuronal and glial cell damage followed by abnormal tau accumulation in this disease. Received: 8 August 1997 / Revised, accepted: 27 October 1997  相似文献   

7.
In the present study the occurrence and distribution of glial fibrillary tangles (GFT) and their related structures in diffuse neurofibrillary tangles with calcification (DNTC) were investigated using Gallyas-Braak (GB) stain. Six cases neuropathologically diagnosed as DNTC were studied (two males and four females). The age at death ranged from 56 to 73 years, with an average of 63.5+/-7.5 years. GFT were classified morphologically, and their immunoreactivites for tau and ubiquitin were examined. Glial cells with GFT were identified with astrocytes and oligodendrocytes by immunostain for glial fibrillary acidic protein and transferrin, respectively. A small number of coiled bodies detected within the oligodendrocytes in the white matter of the cerebrum were positive for tau and ubiquitin. Cell clusters of thorn-shaped astrocytes were detected in the subcortical and subpial regions where gliosis occurred. Thorn-shaped astrocytes were positive for tau, but negative for ubiquitin. A small number of tuft-shaped astrocytes detected prominently in the temporal cortex and amygdala with numerous neurofibrillary tangles were positive for tau and ubiquitin. All three types of GFT were detected, especially in the temporal and limbic lobes, which were the most severely affected sites in DNTC. Moreover, various-shaped neurofibrillary tangles, aggregated rods and some argyrophilic threads were differentiated from GFT. They were positive for GB, but not detected within the glial cells.  相似文献   

8.
We report a 67-year-old man with 4-repeat (4R) tauopathy sharing both features of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Although CBD and PSP have a common pathological feature that 4R tau accumulates in neurons and glia, recent pathological studies have confirmed differences between the two disorders. Clinical features of the present case were asymmetrical apraxia, parkinsonism, memory disturbance, disorientation and left limb myoclonus with a 5-year history. Pathological features were the widespread occurrence of 4R tau-positive structures including pre-tangles, neurofibrillary tangles, astrocytic plaques, tufted astrocytes, coiled bodies and argyrophilic threads. Biochemically, immunoblotting of insoluble tau demonstrated the low molecular fragments of 37 kDa and 33 kDa observed in typical CBD and PSP, respectively, in addition to the presence of 4R tau isoforms. The present case shared tau-related pathological and biochemical features of CBD and PSP. These findings support that CBD and PSP are closely associated disorders having a pathogenesis common to 4R tauopathy.  相似文献   

9.
The microtubule‐associated protein tau aggregates into filaments in the form of neurofibrillary tangles, neuropil threads and argyrophilic grains in neurons, in the form of variable astrocytic tangles in astrocytes and in the form of coiled bodies and argyrophilic threads in oligodendrocytes. These tau filaments may be classified into two types, straight filaments or tubules with 9–18 nm diameters and “twisted ribbons” composed of two parallel aligned components. In the same disease, the fine structure of tau filaments in glial cells roughly resembles that in neurons. In sporadic tauopathies, individual tau filaments show characteristic sizes, shapes and arrangements, and therefore contribute to neuropathologic differential diagnosis. In frontotemporal dementias caused by tau gene mutations, variable filamentous profiles were observed in association with mutation sites and insoluble tau isoforms, including straight filaments or tubules, paired helical filament‐like filaments, and twisted ribbons. Pre‐embedding immunoelectron microscopic studies were carried out using anti‐3‐repeat tau and anti‐4‐repeat tau specific antibodies, RD3 and RD4. Straight tubules in neuronal and astrocytic Pick bodies were immunolabeled by the anti‐3‐repeat tau antibody. The anti‐4‐repeat tau antibody recognized abnormal tubules comprising neurofibrillary tangles, coiled bodies and argyrophilic threads in progressive supranuclear palsy (PSP) and corticobasal degeneration. In the pre‐embedding immunoelectron microscopic study using the phosphorylated tau AT8 antibody, tuft‐shaped astrocytes of PSP were found to be composed of bundles of abnormal tubules in processes and perikarya of protoplasmic astrocytes. In this study, the 3‐repeat tau or 4‐repeat tau epitope was detected in situ at the ultrastructural level in abnormal tubules in representative pathological lesions in Pick’s disease, PSP and corticobasal degeneration.  相似文献   

10.
Kenji Ikeda 《Neuropathology》1997,17(2):127-133
A survey of 32 clinically and pathologically evaluated cases of corticobasal degeneration (CBD) revealed three subtypes of cortical lesions. Besides the main subgroup with frontoparietal atrophy, especially with degeneration around the central sulcus, there are at least two subtypes in which the degenerating focus shifts from the central region to the frontal or superior temporal region. Such diversity of degenerating areas of the cerebrum results in the clinical heterogeneity of CBD. In contrast to the diversity of cerebral lesions, affected nuclei in the subcortical regions are rather uniform and their distribution is of diagnostic value. Cytopathologically, CBD is characterized by several types of abnormal cytoskeletal structures in both neurons and glia. They have common epitopes with hyperphosphorylated tau except for ballooned neurons, which are regarded as a pathological hallmark of this disease. Massive Gallyas/tau-positive structures consist of neurofibrillary tangles and pre-tangles in neurons as well as astrocytic plaques, coiled bodies and argyrophilic threads in glia. A part of argyrophilic threads are proved to originate from neuronal element. There is discrepancy between poor formation of solid tangles and massive appearance of Gallyas/tau-positive structures in CBD. The former is detectable by Bodian method whereas the latter is scarcely seen by conventional silver impregnations. This indicates that almost all of Gallyas/tau-positive structures have poor inclination to form solid structures in CBD. Immunohistochemical study with an antibody to the exon 3-derived epitope of tau indicates that the majority of Gallyas/tau-positive structures in CBD originate from glial cells. Differences as well as overlaps between CBD and progressive supranuclear palsy are discussed.  相似文献   

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