Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Muscle biopsy specimens from 15 autopsied patients with the isolated form of amyotrophic lateral sclerosis were examined by routine histological and immunocytochemical methods using a panel of monoclonal antibodies directed against differentiation and activation markers of immunocompetent cells. In 12 cases, cellular infiltrates consisting mainly of T-cells and macrophages were seen. Both CD8+ and CD3+ cells, in juxtaposition with OKM1+ macrophages, were particularly seen in the atrophied parts of muscle. The majority of the T-cells appeared to be of the CD4+ T-helper/inducer type, whereas the CD8+ T-suppressor/cytotoxic cells were only rarely and focally present. On the other hand, B-, NK- and K-cells were infrequently seen. Most of the T-cells and macrophages surrounding the atrophied muscle fibers were in an activated state, as indicated by their intense HLA DR expression. In addition, some angulated degenerated fibers showed strong endomysial positivity for HLA DR in the regions where T-cells and macrophages were present in clusters. The immunoreactive changes in ALS-associated muscle atrophy are very similar to those reported for exercise-induced damage and some forms of myositis. The present study shows that the expression of major histocompatibility complex products and the relative numbers of infiltrating immunocompetent cells are closely associated with the extent of destruction of muscle fibers in ALS.
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PMID:Immunohistological alterations in muscle of patients with amyotrophic lateral sclerosis: mononuclear cell phenotypes and expression of MHC products. 161 23

Electrodiagnostic methods play important roles for the diagnosis and evaluation of ALS. They are useful for 1) the early establishment of the diagnosis, 2) the differential diagnosis, and 3) the quantitative evaluation of the progression. Needle electromyography reveals active neurogenic changes over the wide territories of the body. Fibrillation potentials and positive sharp waves indicate presence of denervated fibers. However, they are not specific for ALS or even neurogenic process, and widespread occurrence of fibrillations are also characteristic for myositis and inclusion body myositis (IBM). Fasciculation potentials are spontaneous firing of the lower motor neuron and most of them are supposed to arise from the nerve endings. Fasciculation potentials are seen solely in neurogenic process and sufficiently specific for ALS. Its diagnostic role, especially in the early diagnosis, has been stressed by several researchers, including the present authors (Sonoo 1996), and fasciculation potentials will be given the same significance as fibrillation potentials in the coming Awaji criteria for the diagnosis of ALS. Motor unit potentials (MUPs) in ALS often show polyphasia and instability reflecting the presence of immature sprouts. Unstable MUPs (increased jiggle) are counterparts of instability in SFEMG. Giant MUPs are frequent, but low amplitude MUPs may be also observed in rapidly progressing cases. Observation of the recruitment pattern is crucial for the differential diagnosis from myopathies. Nerve conduction studies are important for the exclusion of other diagnoses, especially multifocal motor neuropathy (MMN). Some degree of slowing and disappearance of F-waves can occur simply due to loss of motor units. The utility of Neurophsiological Index remains to be confirmed. Repetitive nerve stimulation often reveals decremental responses, whose presence supports the diagnosis of ALS. Decremental responses in ALS usually occur in wasted muscles with low CMAP amplitude, and may predict the speed of further deterioration of the CMAP.
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PMID:[Electrodiagnosis of ALS]. 1796 43