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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

It is believed that one muscle fiber consists of one fiber type determined by its innervating neuron. In biopsied muscles of Duchenne muscular dystrophy (DMD), however, the author has incidentally found a double-typed fiber which is divided into inner and outer parts. The author termed it a "boiled-egg fiber". The author has examined the appearance rate of the boiled-egg fibers on 682 biopsied muscles obtained from patients with various neuromuscular disorders, and classified the types of the inner and outer parts of the boiled-egg fibers by ATPase staining. Boiled-egg fibers were recognized in 17 cases out of 60 with DMD, 5 out of 146 with other types of muscular dystrophy and 6 out of 94 with myositis. No boiled-egg fiber was found in the remaining 382 cases with other disorders which did not represent necrosis with regeneration of muscle fibers. The total number of boiled-egg fibers was 235 with 192 in DMD and 43 in other disorders. 197 of 235 (83.8%) had the same type for both inner and outer parts and remaining 38 (16.2%) had different types for their inner parts. In 133 of 235 (56.6%), the inner parts were type 2C fibers. Boiled-egg fibers were segmentally found with the length of several hundred micrometers. The above findings suggest that boiled-egg fibers reflect an abnormal regenerating process. It remains to be clarified whether or not inner and outer parts of boiled-egg fibers are double-innervated respectively.
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PMID:[The significance of boiled-egg fibers in biopsied muscles of neuromuscular disorders]. 173 23

Various types of the distal myopathy except Welander's late distal myopathy of Swedish type were described. There were many reports in the past concerning the varieties of the distal myopathy. Distal myopathy is a rather rare disorder and it may be difficult to diagnose these cases. Among these various distal myopathy, an emphasis was made upon the clinical and pathological characteristics of the diseases, particularly distal muscular dystrophy of Miyoshi, Distal myopathy with rimmed vacuole formation of Nonaka and ours, and Oculo-pharyngo-distal myopathy. All these diseases show the distal muscle involvement but the clinical features, heredity, course and pathological features are quite different, and prognosis is also different. Accordingly when we examined these distal myopathy cases, clinical characteristics and histo-pathological findings should be carefully studied. It should be also emphasized that the level of serum CPK dose not indicate the severity of the myopathy. In muscular dystrophy or myositis, serUm CPK elevates remarkably and we can consider the level of CPK as a sign of the severity or condition of the disease. However, in myopathy as "rimmed vacuole distal myopathy" serum CPK remains in normal level even though weakness is severe. This is due to absence of the membrane abnormalities of muscle cells. Leakage of the CPK from muscle cells, therefore, does not reflect the degree of the cell destruction. In this point we have to remember the fact that serum CPK is not a indicator of all muscle diseases and even if the CPK is normal, we have to consider the presence of myopathy.
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PMID:Distal myopathy. 208 93

Samples of paravertebral muscles were obtained from the lesion sites of forty patients operated on for recurrent lumboischiadic syndrome of discogenic etiology. None of the specimens was physiological. All of them were pathologically altered in various grades of involvement. The histopathological changes were highly varied. The findings included muscle fibre atrophy of a nonspecific type, neurogenic atrophy of typical fascicular distribution. Apart from atrophic fibres there were hypertrophic ones either at the same time or in connection with another histopathological process. Changes of myopathic nature were also present with rounded up muscle fibres and shift of nuclei from the subsarcolemnic spaces into the centre of the muscle fibre, multiplication of the connective tissue and vicarious growth of adipose tissue. The visible neuromuscular spindles contained thickened connective tissue capsules and atrophied intrafusal fibres. The present authors' conclusion of their interpretation of these histopathological changes is that they are not produced by one but by a whole set of factors. What results is a cyclic nature of the changes, when it is hard to decide whether we are facing a primary cause or its sequela. The regular company of discopathies are repeated microtraumas during recurrent paravertebral contractures and spinal blocks. A not negligible part can be played also by primary muscle diseases affecting, among others, back muscles, e.g. progressive muscular dystrophy of Duchenne's and Becker's type, various myopathies, nonspecific myositis and paravertebral polymyositis, and other rheumatological involvement. These muscular diseases with disturbed proprioception causing unphysiological posture and loading the spine can share in the origin degenerative processes on intervertebral disks.
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PMID:Histopathological changes in paravertebral muscles by chronic discopathies. 215 Feb 77

Intracellular staining for calcium was studied in muscle biopsies from 15 dogs by the alizarin red S (ARS) stain. Rare positive fibres were present in normal muscle and in denervation atrophy. The percentage of positive fibres was slightly increased in polymyositis, dermatomyositis and canine temporal/masseter myositis and markedly increased in progressive muscular dystrophy. Calcium-positive fibres were usually so-called large-dark (hypercontracted) fibres or necrotic fibres, although there was occasional staining of normal and atrophied fibres. These results indicate the probable involvement of calcium in muscle injury in canine inflammatory myopathies and in canine muscular dystrophy. In addition, use of the ARS stain appears to be useful for detecting the earliest lesions of acute muscle fibre injury.
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PMID:Intracellular calcium in canine muscle biopsies. 247 Jul 89

The distribution of HLA ABC class I antigens in human skeletal muscle obtained by needle biopsy was investigated by means of a monoclonal antibody (W6/32) and an immunoperoxidase technique. Five samples from normal individuals and twenty-nine from patients with various neuromuscular disorders were examined. Normal muscle fibres and those from patients with congenital muscular dystrophy expressed little or no class I antigens, whereas muscle fibres of patients with myositis and various X-linked muscular dystrophies showed consistently strong expression. In other neuromuscular diseases expression was more variable. The presence of class I antigens on diseased muscle fibres may render them susceptible to cytotoxic T cells; these antigens may thus have an important role in the destruction of muscle fibres.
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PMID:Increased expression of HLA ABC class I antigens by muscle fibres in Duchenne muscular dystrophy, inflammatory myopathy, and other neuromuscular disorders. 285 18

Paraspinal muscle biopsy was done on the erector spinae muscles in sixty consecutive scoliotic patients. Together with the clinical and muscle biopsy findings, these sixty patients were classified into thirty-two patients with idiopathic scoliosis, eleven patients with myopathic scoliosis, nine patients with neuropathic scoliosis, and eight patients with miscellaneous scoliosis. Of the thirty-two patients with idiopathic scoliosis, nineteen cases displayed neurogenic changes in the grouping of the Type 1 fibers and target fiber formation more common on the convex side back muscles. The myopathies consisted of central core disease, nemalin myopathy, congenital fiber type disproportion, mitochondrial myopathy, congenital muscular dystrophy, and myositis. Patients with neuropathic scoliosis all showed loss of the normal mosaic pattern and grouping of both Type 1 and 2 fibers in their muscle biopsies. This result indicates that there is a variety of neuromuscular diseases in scoliotic patients. The neurogenic changes observed more commonly on the convex side muscle of idiopathic scoliotic patients deserve further investigation.
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PMID:Morphological studies on the erector spinae muscle in sixty consecutive scoliotic patients. 324

Thirty-six biopsy specimens of human biceps and vastus lateralis muscles were examined by histometric analysis and determination of enzyme activities (phosphorylase, triosephosphate dehydrogenase, 3-hydroxacyl-CoA-dehydrogenase, lactate dehydrogenase, hexose isomerase, citrate synthetase, 6-phosphogluconate dehydrogenase). The series included 13 specimens from patients suffering from a benign form of muscular dystrophy (limb girdle and Becker type of muscular dystrophy) and 12 specimens from patients with an acute (n = 5) or chronic (n = 7) form of myositis. Muscle fibres were atrophic in myositis and hypertrophic (with an increased variation of fibre diameters) in muscular dystrophies, as has been shown previously. When myositis samples were compared with either normal or dystrophic muscles, a highly significant lowering of glycolytic enzyme activity was found in chronic myositis, while the activity of 6-phosphogluconate dehydrogenase was elevated to highly significant levels. Measurements of the latter enzyme's activity might be of additional value in differentiating chronic forms of myositis from benign muscular dystrophies.
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PMID:Additional biochemical criteria in the differential diagnosis of myositis. 343 Jan 87

Several case reports have suggested an association between acquired toxoplasmosis and polymyositis-dermatomyositis. Because the presence of anti-Toxoplasma IgM antibodies suggests recent infection, 58 patients with polymyositis-dermatomyositis (from two medical centers) were examined for the presence of IgM antibodies using a specific indirect immunofluorescent technique. Serum samples were also examined for antibodies using the Sabin-Feldman dye test and complement fixation methods. Of 58 patients with polymyositis-dermatomyositis, 29 (50 percent) had positive Sabin-Feldman dye test results and 14 (24 percent) had positive IgM immunofluorescent findings. This is higher than the expected frequency. None of the patients with negative Sabin-Feldman dye test results had IgM immunofluorescent antibodies. Furthermore, IgM immunofluorescent antibodies were associated with the presence and titer of both Sabin-Feldman dye test and complement fixation antibodies. Evidence that the presence of antinuclear antibody and rheumatoid factor did not influence these results is presented. Patients with muscular dystrophy and systemic lupus erythematosus (with or without myositis) did not have an increased frequency of anti-Toxoplasma IgM immunofluorescent antibodies.
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PMID:Serologic evidence for acute toxoplasmosis in polymyositis-dermatomyositis. Increased frequency of specific anti-toxoplasma IgM antibodies. 634 49

The clinical diagnosis of a 34-year-old man was muscular dystrophy of the ascending limb-girdle type. There were no detectable signs of myotonia. Histological and biochemical investigations confirmed an acid maltase deficiency (AMD). Electromyography demonstrated a myopathic pattern, fibrillation potentials, normal nerve conduction velocity, and so-called pseudomyotonic or bizarre high frequency discharges. In the literature this EMG activity has been described as characteristic for AMD. But it seems to be a nonspecific EMG sign, which occurs in myogenic and neurogenic lesions such as myositis, metabolic and dystrophic myopathy, nuclear damage, radiculopathy, and neuropathy. It is therefore of little diagnostic value.
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PMID:[High frequency discharges as a non-specific EMG activity in adult acid maltase deficiency (author's transl)]. 693 Jan 99

This report deals with morphometric investigations of muscle biopsies using a semiautomatic manual analysis system. Examples for differential diagnostic value of metric results are described: 1) Different distribution curves of muscle fibers are helpful in differentiating between two important groups of muscle diseases, chronic polymyositis and muscular dystrophy. 2) Some groups of disorders (e.g. interstitial myositis, neurogenic atrophy) may be subtyped by metric data. 3) Several diseases (e.g. myotonic dystrophy) are characterized by selective atrophy of one fiber type only in distinct muscle groups.
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PMID:[Value of myometric findings in the morphological differential diagnosis of neuromuscular disorders (author's transl)]. 693 Dec 79


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