Neurogenic muscle involvement in myasthenia gravis. A clinical and histopathological study.
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 II-fibre atrophy (Table 1). No relationship was demonstrable between the presence of clilnical muscular atrophy and age, sex, duration of the disease, severity of the disease, presence of a thymoma, or drug resistant ophthalmoplegia. (2) In this group of patients 61 biopsies were examined from 46 individuals; 40 of these biopsies were taken from the quadriceps muscle. A thymoma was present in 17 patients. Examination disclosed neurogenic changes in 17 biopsies, lymphocytic infiltrates in 21, and myositis in one biopsy (Table 2). A distinct correlation was established between the presence of a thymoma and lymphocytic infiltrates, but none was demonstrable between thymoma and neurogenic changes (Table 3). (3) An enzyme-histochemical study was carried out in 35 cases, including 12 with neurogenic changes. A normal differentiation of type I- and type II-fibres was observed in eight instances, type grouping of type II-fibres in three, and type II-fibre atrophy in two cases. (4) In 21 patients and 19 controls, the smallest mean diameter was determined in the quadriceps muscle. Both type I- and type II-fibres proved to have a smaller mean 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.
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003401:Creatine / Q000652:urine; D003402:Creatine Kinase / Q000097:blood; D004576:Electromyography; D005152:Facial Muscles / Q000503:physiopathology; D005260:Female; D005634:Fructose-Bisphosphate Aldolase / Q000097:blood; D006801:Humans; D008214:Lymphocytes; D008297:Male; D008410:Masticatory Muscles / Q000503:physiopathology; D008875:Middle Aged; D009132:Muscles / Q000473:pathology* / Q000503:physiopathology; D009133:Muscular Atrophy / Q000209:etiology* / Q000473:pathology; D009157:Myasthenia Gravis / Q000150:complications* / Q000201:enzymology / Q000473:pathology / Q000503:physiopathology; D009431:Neural Conduction; D009886:Ophthalmoplegia / Q000209:etiology; D013945:Thymoma / Q000209:etiology; D013953:Thymus Neoplasms / Q000209:etiology
D003402:Creatine Kinase; D005634:Fructose-Bisphosphate Aldolase; D003401:Creatine