ISSN:0148-639X    

DOI:10.1002/mus.880010102

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractChanges of the terminal innervation pattern in polymyositis were studied in nine patients using single fiber electromyography (SFEMG) and histochemical investigations of muscle biopsies. In all patients, and in 16 out of 18 muscles studied, reinnervation was found. The preceding denervation could be caused either by segmental necrosis of the muscle fiber that disconnects one part of the fiber from the end‐plate region or by direct intramuscular nerve involvemen

ISSN:0148-639X    

DOI:10.1002/mus.880010103

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractA slowly progressive autosomal dominant neuromuscular disease–termedspheroid body myopathy–is described in four successive generations and documented by muscle biopsies in five patients of two generations. With an onset in adolescence, the disease proceeds to some motor incapacitation, but life span is apparently not shortened. The salient morphologic feature is the presence of spheroid bodies, chiefly occurring in type 1 myofibers. Ultrastructurally, these spheroid bodies are composed of fine filaments but are devoid of organelles; in some cases they resemble cytoplasmic bodies. “Smearing in the l‐band” is a frequent and early finding. At a later age, signs of denervation are also present, both clinically and in muscle biopsies. The clinical and morphologic features justify the designation of this neuromuscular condition as a distin

ISSN:0148-639X    

DOI:10.1002/mus.880010104

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractIn a postmortem study of a patient with adult‐onset acid maltase deficiency (AMD), morphological abnormalities were confined to skeletal muscle and consisted of a vacuolar myopathy. Acid maltase activity, however, was approximately 6% of normal in muscle, liver, and brain, and 3% of normal in heart. Kinetic characteristics, and inhibition by antibodies and Zn++, showed that the residual activity was “authentic” acid maltase. Neutral maltase activity was normal in muscle and liver, but decreased in brain (55% of normal) and heart (19% of normal). Although the relative decrease of acid maltase was similar in different tissues, absolute residual activity was lowest in skeletal muscle: this may explain the selective involvement of this tissue in late‐on

ISSN:0148-639X    

DOI:10.1002/mus.880010105

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractThe F‐wave has been used to estimate the motor nerve conduction velocity (MNCV) along the proximal segment (spinal cord to knee) of the axons of the deep peroneal nerve in patients with Charcot‐Marie‐Tooth disease and those with dystrophia myotonica. A new, modified method has been applied to estimate proximal MNCV in patients in whom F‐waves or M‐responses cannot be obtained from the small muscles of the foot. Terminal latencies and MNCV along the distal nerve segment (knee to ankle) have also been estimated using conventional techniques. The results have been compared with those obtained for control subjects. Proximal MNCV was severely slowed in every patient with Charcot‐Marie‐Tooth disease; the degrees of proximal and distal MNCV decreases were related. In patients with dystrophia myotonica, distal and proximal MNCVs were significantly reduced in comparison with control subjects; the MNCV slowing was not related to the degree of muscle atrophy. This is consistent with the hypothesis that the nerves and muscles are independently affected in dystrophia myotonica. It is concluded that the F‐wave MNCV technique is as useful as, and may be more sensitive than, the conventio

ISSN:0148-639X    

DOI:10.1002/mus.880010106

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractA review of our current knowledge of the etiology and pathogenesis of myasthenia gravis is presented, with particular emphasis on the immunological aspects of the disease. Part 1, published in this issue, deals with the clinical and genetic features of myasthenia gravis which led to the autoimmune theory of the etiology of this disease. Various theories in this field are reviewed, and recent advances in our knowledge of the acetylcholine receptor protein, and its immunology, are examined. Part 2, which will appear in the March/April issue, provides a review of the dysfunction of physiology, pharmacology, and structure of the neuromuscular junction in myasthenia gravis, and the part played by the autoimmune process.

ISSN:0148-639X    

DOI:10.1002/mus.880010107

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractSeveral studies have suggested that muscle lactic acid production is defective in alcoholic myopathy. However, normal controls have not been evaluated. To study the effects of ethanol on the production of lactic acid in normal exercised muscle, oral ethanol (1 g per kg body weight, 20% v/v) was given to eight nonalcoholic subjects. Forearm ischemic exercise with a total work load of 7.2 kg‐m/min was performed just before and 90 min after ingestion of ethanol. At the time of exercise, the serum ethanol level was 95.7 ± 15.1 mg% (mean ± SD). Resting serum lactic acid was significantly higher after ingestion of ethanol (15.7 ± 4.5 mg%) than before (8.5 ± 4.7 mg%). Lactic acid generation with ischemic exercise was significantly lower after ethanol ingestion. The mean peak serum lactic acid level (expressed as percentage elevation above resting) was 330.5 ± 118.2% before and 127.9 ± 75.1% after (p<.05). These results indicate that a single oral dose of ethanol decreases lactic acid production by exercised muscle in normal s

ISSN:0148-639X    

DOI:10.1002/mus.880010108

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractThe authors describe a case of myopathy characterized physically by limb weakness, eyelid ptosis, voluntary and reflex paralysis of vertical movements of gaze, and loss of deep tendon reflexes; and morphologically by the abnormal presence of centrally located nuclei in muscle fibers and type 1 fiber hypotrophy. The establishment in this case study of two particular findings–the probably nuclear or supranuclear ophthalmoplegia and the apparently impaired nuclear migration from the center of the muscle fiber toward its periphery–supports the hypothesis of a neuromuscular disorder whose level of severity depends on the degree of difficulty in the nuclear migration itself. This would be linked to a reduction in central nervous system influe

ISSN:0148-639X    

DOI:10.1002/mus.880010109

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractA new combined stain for the simultaneous demonstration of motor nerve terminals and cholinesterase at neuromuscular junctions is described. It employs bromoindoxyl acetate dye‐staining for cholinesterase and silver‐gold impregnation for nerve terminals. The clarity and reliability of the stain permit quantitative measurements of neuromuscular junctions in order to evaluate nerve terminal sprouting as well as other pathological changes. The method is rapid, reproducible, and simple, and it is well suited for the processing of large numbers of frozen secti

ISSN:0148-639X    

DOI:10.1002/mus.880010110

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY

摘要:

AbstractMuscle biopsy and computerized tomography indicated that unilateral enlargement of the gastrocnemius muscle in a 12‐year‐old girl was the result of an infiltrating lipoma. This rare condition may cause diagnostic confusion before EMG and muscle biopsy studies are performed, but it can be readily distinguished from other causes of unilateral muscle enlargement using these meth

ISSN:0148-639X    

DOI:10.1002/mus.880010111

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1978

数据来源: WILEY