Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a 62-year-old man with an 8-month course of sporadic classical amyotrophic lateral sclerosis, many Lewy body-like hyaline inclusions (LI) were observed in spinal anterior horn cells, hypoglossal nuclei, nucleus ambiguus, and motor nuclei of the trigeminal nerve. These motor neurons showed a mild degree of neuronal loss, several Bunina bodies, spheroids and chromatolytic neurons. Tract degeneration was limited to pyramidal tracts. In addition to intensely stained LI, immunoreactive skeins or granules were recognized by a polyclonal anti-ubiquitin antibody. Thick filaments of 15 to 20 nm in diameters with granules formed conglomerated masses with varying amounts of neurofilaments in the anterior horns, corresponding to light microscopically observed LI. More commonly, these thick granulofilamentous profiles were dispersed in small bundles or individually in the cytoplasm. Electron microscopically, there was no close association of filaments with Bunina bodies.
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PMID:Granulofilamentous profiles in lower motor neurons: a sporadic case of amyotrophic lateral sclerosis with many Lewy body-like inclusions. 131 2

The reticular formation of the brain stem of three cases of familial amyotrophic lateral sclerosis (ALS) with posterior column and spinocerebellar tract involvement showed a paucity of large neurons, chromatolytic neurons and Lewy body-like intracytoplasmic inclusions. Some of the remaining neurons were stained focally by antibodies to phosphorylated neurofilaments and ubiquitin; the staining patterns were similar to those seen in anterior horn cells. Within the reticular formation, the medial part of the medulla oblongata was most pronouncedly affected. The reticular formation should be among the systems other than motor neurons that may be involved in this type of familial ALS.
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PMID:Involvement of the brain stem reticular formation in familial amyotrophic lateral sclerosis. 131 4

The pathological alterations in upper motor neurons were investigated in 27 cases of adult-onset sporadic amyotrophic lateral sclerosis (ALS). No significant cytoskeletal alterations were found in the Betz cells of any of the cases except one, although cytoskeletal pathology was consistently present in lower motor neurons. The one case had severe circumscribed atrophy of the precentral gyrus and, microscopically, had argentophilic intracytoplasmic inclusions in Betz cells and other pyramidal neurons in the primary motor area as eell as in the lower motor neurons. Immunocytochemically these inclusions contained the epitope of phosphorylated neurofilament and ubiquitin and ultrastructurally consisted of granule-associated filaments with neurofilaments. This is the first demonstration of alterations of cytoskeleton and ubiquitination in the giant cells of Betz, an established subset of upper motor neurons in ALS. Thus, although uncommon, cytoskeletal changes can be found in upper motor neurons in some ALS cases.
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PMID:Immunocytochemical and ultrastructural studies of upper motor neurons in amyotrophic lateral sclerosis. 132 Mar 22

According to the literature, only minor nonspecific histopathological lesions are present in the motor cortex in up to 90% of the amyotrophic lateral sclerosis (ALS) patients. These observations, however, have so far been based mainly on conventional staining techniques. An exception to this is the focal glial reaction that has been reported following immunocytochemical staining for glial fibrillary protein (GFAP), which is reported to be distinctive for ALS in the cortex. Since perikarya of degenerating motor neurons in the spinal cord of ALS patients have been found to accumulate phosphorylated neurofilaments (PNF), an investigation was conducted to determine whether PNF was also a sensitive marker for alterations in the motor cortex in this condition. On large brain sections from 15 ALS patients, intense PNF immunoreactivity was found in the motor cortex from 11 patients. It was mainly localized in small pyramidal cells and basket cells, whereas only slight staining was observed in Betz cells. PNF-positive basket cells were also present in controls, but the basket cells staining for PNF were less numerous in controls than in ALS specimens. PNF-positive Betz cells were found in 47% of 15 ALS patients and in 10% of the controls. PNF accumulation was also found in swollen, probably degenerating, terminal boutons around perikarya of large pyramidal cells and Betz cells in the motor areas of ALS patients only. These observations suggest that the premotor innervation of the motor system is preferentially affected in ALS. Small brain sections, comprising the motor cortex, from 18 additional ALS patients demonstrated a similar PNF-staining pattern. However, differentiating ALS patients from controls was much easier when studying large brain sections. No ubiquitin-immunoreactive inclusions were found, except for sporadic tangles. The presence of a focal-GFAP positive astrocytosis as reported in the literature in the precentral cortex was confirmed. However, it was found to be nonspecific since it was also present outside the precentral cortex and in the cortex of normal control patients. No spatial relation was found between the distribution of the glial reaction in ALS and the areas containing neurons and boutons accumulating PNF.
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PMID:Neurofilament and glial alterations in the cerebral cortex in amyotrophic lateral sclerosis. 133 2

Amyotrophic lateral sclerosis (ALS) patients with dementia were found to have ubiquitin-immunoreactive (IR) inclusions in the dentate granule cells of the hippocampus. These inclusions were also present in some patients with minor cognitive changes but otherwise typical ALS. Ubiquitin-IR inclusions were also found in neurons of superficial layers of the frontal and temporal cortex and in the entorhinal cortex in patients with ALS and dementia. These ubiquitin-IR inclusions were non-argyrophilic, and were not labelled by antibodies which identify Alzheimer's neurofibrillary tangles and Pick bodies, nor were they typical of cortical Lewy bodies. Our findings indicate that ubiquitin-IR inclusions in small neurons of the hippocampus, entorhinal area and neocortex are a characteristic feature of degeneration of non-motor cortex in ALS, and are particularly associated with cognitive impairment and dementia of frontal lobe type.
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PMID:Hippocampal and neocortical ubiquitin-immunoreactive inclusions in amyotrophic lateral sclerosis with dementia. 137 81

This report deals with a comparative study on the expression of alpha B crystallin, ubiquitin, stress-response protein 27 (srp 27), srp 72 and phosphorylated neurofilament protein (pNFP) by ballooned neurons in Pick's disease, Creutzfeldt-Jakob disease (CJD), amyotrophic lateral sclerosis (ALS), leptomeningeal carcinomatosis, anterior spinal artery syndrome and pellagra. Immunohistochemical techniques were used. alpha B Crystallin was expressed by the majority of ballooned neurons of Pick's disease and CJD, but not by those of the other disorders. Ubiquitin and srp 27 expression was also restricted to abnormal neurons of Pick's disease and CJD, but the proportion of stained cells was less than that expressing alpha B-crystallin. There was no evidence of ballooned neurons expressing srp 72. Except for those of pellagra patients, phosphorylated neurofilament protein (pNFP) was detected in most abnormal neurons. Our results suggest that the mechanisms involved in formation and maintenance of swollen neurons in Pick's disease and CJD may be different than those of ballooned neurons in the other entities studied.
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PMID:Comparative immunohistochemical study on the expression of alpha B crystallin, ubiquitin and stress-response protein 27 in ballooned neurons in various disorders. 138 40

Filamentous, fibronectin-immunopositive structures, previously described in Alzheimer's disease and control brains were negative for neuronal, glial, and macrophage markers. The present study sought to determine the nature of these entities and to further characterize their morphology, immunoreactivity and distribution between neuropathologies. Ultrastructural analysis shows these formations to be filamentous micro-organisms, which may belong to the actinomycetes. Immunohistochemistry for the cell-stress protein ubiquitin is consistently positive in these organisms. They are also present in Down's syndrome, dementia pugilistica, amyotrophic lateral sclerosis with dementia, and Parkinson's disease. The pattern of tissue distribution implies a pre-mortem invasion of the brain, and, as the micro-organism is present at a four to five-fold higher frequency in Alzheimer's disease, it may act pathogenically in this dementing illness.
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PMID:Fibronectin staining detects micro-organisms in aged and Alzheimer's disease brain. 142 Nov 18

We describe two patients with sporadic amyotrophic lateral sclerosis (ALS), who had developed progressive external ophthalmoplegia of a predominantly supranuclear type while they survived on respirators, and displayed histopathological abnormalities both typical and atypical of ALS. Patient 1 was a 43-year-old man with ALS of 5-year duration, who had initially exhibited fulminant ALS, and remained on a respirator for 4 years. Patient 2 was a 51-year-old man with ALS of 13-year duration, who remained on a respirator for 8 years. Both patients died in a "totally locked-in state". Autopsy of both patients revealed not only histopathological abnormalities consistent with ALS, but also multisystem degeneration which involved the pontine tegmentum, substantia nigra, Clarke's dorsal nuclei and spinocerebellar tracts. In addition, Patient 2 displayed intracytoplasmic neuronal basophilic inclusion bodies which exhibited marked immunoreactivity to anti-ubiquitin antibodies. Our case reports indicate that the longer survival which is possible through the use of respirators may make one subgroup of ALS patients prone to develop atypical clinical and neuropathological features which are not observed during the natural course of ALS.
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PMID:Amyotrophic lateral sclerosis with ophthalmoplegia and multisystem degeneration in patients on long-term use of respirators. 144 18

The immunohistochemical and ultrastructural characteristics of spinal cord neurofibrillary tangles (NFTs) were examined in Guamanian amyotrophic lateral sclerosis and in parkinisonism-dementia complex on Guam. The spinal cord NFTs reacted with antibodies to tau protein (tau-2), ubiquitin and paired helical filaments (PHFs). Ultrastructurally, the components of the NFTs were seen as randomly arranged fibrils which were often associated with osmiophilic granules; small bundle-like arrangements were also occasionally observed. Individual NFT fibrils appeared as straight fibrils with a diameter of approximately 15 nm and constricted fibrils with a periodicity of approximately 80 nm. Ultrastructural microscopic examination of specimens stained by the modified Bielschowsky method and with the antibodies revealed silver particles and the products of the tau, ubiquitin and PHF immunoreactions on the NFT fibrils. This is the first demonstration of the fine structure of the spinal cord NFTs.
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PMID:Ultrastructural identification of neurofibrillary tangles in the spinal cords in Guamanian amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam. 155 58

The incidence of amyotrophic lateral sclerosis (ALS) and Parkinsonism-dementia complex (PDC) among the Chamorros in Guam is remarkably high. The patients with ALS have clinical and pathological characteristics similar to those in other parts of the world. The PDC patients display parkinsonism and progressive dementia and show a characteristic neuronal loss in certain parts of the central nervous system such as the hippocampus and substantia nigra. The Guamanian patients with ALS and PDC commonly have widespread Alzheimer's neurofibrillary changes, but without the associated senile plaques. We have applied immunohistochemical procedures to examine the expression of marker substances in Guamanian ALS and PDC. The markers studied include tau protein, ubiquitin, beta proteins, synaptophysin, calcineurin, Met-enkephalin, substance P and tyrosine hydroxylase. The results were compared with the findings in patients with Alzheimer's disease, Parkinson's disease, sporadic ALS and familial ALS.
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PMID:Amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam: immunohistochemical studies. 158 17


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