Muscle pathology, thymoma, and immunological abnormalities in patients with myasthenia gravis.

Oosterhuis, H.J.G.H.; Bethlem, J.; Feltkamp, T. E. W.

doi:10.1136/jnnp.31.5.460

Cited by 16 publications

(5 citation statements)

References 16 publications

(14 reference statements)

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“…Other features have also been described in muscles of patients with MG, such as neurogenic muscular atrophy and focal myositis: focal myositis seems to occur chiefly in patients with thymoma [49], whereas neurogenic abnormalities are not linked to thymoma [50]. A relation was also established between lymphorrhages and anti-muscle antibodies [48]. Despite this strong relation between lymphorrhages and thymoma, our two patients presented no thymoma.…”

Section: Discussionsupporting

confidence: 52%

“…Carl Weigert first described the relationship between hypertrophy of the thymus and MG but also observed lymphocytic infiltrations (“lymphorrhages”) in muscles of patients with MG (that he considered as “metastases of the thymoma”) [47]. Lymphorrhages are found more frequently in patients with thymoma [48]. Other features have also been described in muscles of patients with MG, such as neurogenic muscular atrophy and focal myositis: focal myositis seems to occur chiefly in patients with thymoma [49], whereas neurogenic abnormalities are not linked to thymoma [50].…”

Section: Discussionmentioning

confidence: 99%

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Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome

Mathis

Magy²,

Corcia

et al. 2016

Case Reports in Neurological Medicine

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Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders) are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy, myositis, and myasthenia gravis (with positive acetylcholine receptor antibodies). For each patient, we carried out a pathological analysis (nerve and muscle) and an electrophysiological study (and follow-up). To our knowledge, this is the first description of such a triple immune-mediated neuromuscular syndrome. We compared our observations with a few other cases of simultaneous diagnosis of two inflammatory neuromuscular disorders.

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Section: Discussionsupporting

confidence: 52%

Section: Discussionmentioning

confidence: 99%

Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome

Mathis

Magy²,

Corcia

et al. 2016

Case Reports in Neurological Medicine

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“…Fenichel and Shy (1963) described neurogenic histopathological changes in 11 out of 37 patients with myasthenia, but presented no data on clinical muscular atrophy. Neurogenic features in biopsies were also mentioned by Oosterhuis (1964), Engel and McFarlin (1966), Fenichel (1966, and Oosterhuis, Bethlem, and Feltkamp (1968).…”

mentioning

confidence: 76%

Neurogenic muscle involvement in myasthenia gravis: A clinical and histopathological study

Oosterhuis

Bethlem

1973

Journal of Neurology, Neurosurgery & Psychiatry

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SUMMARY An investigation was made into the occurrence of muscular atrophy and muscular pathology in a series of 170 patients with myasthenia gravis. The results can be summarized as follows: (1) Of the 148 patients with generalized myasthenia gravis, 14 showed local muscular atrophies. Of 10 biopsies from atrophic muscles, eight showed neurogenic changes, with or without lymphocytic infiltrations. One biopsy showed lymphocytic infiltrations only, and one showed type IL-fibre atrophy ( diameter in the female patients than in the controls. In the male patients this could not be proven. (5) Of the eight patients who had died without disorders of ventilation, 90 muscle specimens were examined postmortem. Four of these patients had a thymoma. Lymphocytic infiltrations, found in 32 biopsy specimens, were mostly observed in the presence of a thymoma. Neurogenic changes were apparently unrelated to the presence of a thymoma (Tables 5 and 6). The post mortem examination included the spinal cord in five, and peripheral nerves in three cases. No abnormalities were found. (6) The muscular atrophy found in patients with myasthenia is not a myopathy but an affection of the lower motor neurone. Neurogenic changes were regularly found in the muscles of patients with myasthenia, even without muscular atrophy. The finding of these changes is no reason to reject the diagnosis. It is postulated that denervation occurs at the neuromuscular junction as a result of permanent absence of acetylcholine.

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“…It is of interest to note that lymphorrhagia did not occur in any of our cases. According to OOSTERHUIS et al [1968], lymphocytic infiltration in the proximity of necrotic muscle fibres may be observed most frequently in thymoma patients.…”

Section: Discussionmentioning

confidence: 99%

Fine Structural Studies in Myasthenia gravis

et al. 1973

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The authors carried out electron microscopic studies on muscle biopsy material from 23 patients suffering from myasthenia gravis, in order to show the fine structural changes occurring in the muscle fibres. Splitting of the central part of the sarcomeres and hyperplasia of Z formations were observed in some cases. Formations of concentrically arranged thin filaments were wide-spread. Pleioconial mitochondria containing various inclusions were noted, the lamellae of the crystalline inclusions being composed of granules of double helix configuration. No succino-dehydrogenase activity could be detected in crystalline-containing mitochondria by enzyme-cytochemical tests using the electron microscope. The nonspecificity of these alterations occurring in the muscle fibre organelles is stressed. The general picture reveals the biochemical nature and extent of the lesions, and gives an explanation of the structural changes associated with them.

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Muscle pathology, thymoma, and immunological abnormalities in patients with myasthenia gravis.

Cited by 16 publications

References 16 publications

Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome

Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome

Neurogenic muscle involvement in myasthenia gravis: A clinical and histopathological study

Fine Structural Studies in Myasthenia gravis

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