摘要:

AbstractWe review the evidence that a motor nerve growth factor released from muscle has wide ranging effects on the development and maintenance of muscle innervation. The actions of this putative factor on motor neurons are analogous to the actions of the well known nerve growth factor (NGF) on sympathetic and sensory neurons.

ISSN:0148-639X    

DOI:10.1002/mus.880060402

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

年代:1983

数据来源: WILEY

摘要:

AbstractWithin the Campania region of southern Italy a prospective study on X‐linked progressive muscular dystrophy was conducted over a period of 12 years from 1969 to 1980, inclusive. The mean incidence rate was 21.7 per 100,000 male livebirths for Duchenne muscular dystrophy (DMD) cases and 3.2 per 100,000 male livebirths for Becker muscular dystrophy (BMD) cases. The familial cases were 38.5% among the DMD patients and 50% among the BMD patients. Myocardial involvement appeared in DMD patients at about 6 years of age in a high percentage of cases and increased progressively until the last years of life, when cardiac damage occurred in 95% of cases. The percentage of myocardial involvement in BMD patients was very low before 13 years of age, but increased progressively until 20 years, when cardiac damage occurred in 80% of cases studied; severe cardiomyopathy did not occur before the age of 21. The data reported also include the effects of age on physical performance, serum creatine kinase activity and serum myoglobin levels, the types of cardiac damage, and the causes of deat

ISSN:0148-639X    

DOI:10.1002/mus.880060403

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

年代:1983

数据来源: WILEY

摘要:

AbstractElectromechanical properties of skeletal muscle were compared between malignant hyperthermia (MH)‐susceptible and control pigs. These muscle properties were studied both in vivo and in vitro. The in vivo time interval from nerve stimulation to onset of mechanical tension was longer in muscle of pigs susceptible to MH. Kinetics of formation of the in vivo compound muscle action potential did not differ between pig groups, whereas the time from peak of the muscle action potential to tension onset was longer for MH muscle. In vitro the time from direct stimulation to tension onset was also longer for MH muscle. These results suggest that the mechanism causing longer electromechanical time intervals in MH skeletal muscle is distal to the myoneural junction. Data comparing contraction kinetics between muscle from MH and control pigs indicates that the mechanism contributing to the longer time interval involves the transfer of signal from the transverse tubule to the sarcoplasmic reticulum in the pigs studie

ISSN:0148-639X    

DOI:10.1002/mus.880060404

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

年代:1983

数据来源: WILEY

摘要:

AbstractDaily passive stretch for six weeks ranging from 30 minutes to 24 hours per day was studied in the patagialis (PAT) muscle of normal and dystrophic chickens. Significant increases in wet weight, cross‐sectional area, and mean fiber cross‐sectional area occurred in both normal and dystrophic PAT in response to stretch of all daily durations tested. More than 50% of the growth occurring in response to continuous stretch was elicited by as little as 30 minutes of stretch per day. Oxidative enzyme capacity increased proportionately with increasing durations of stretch in the normal PAT. Similarly, increasing duration of stretch progressively retarded the onset of histopathological signs in the dystrophic PAT. We conclude that daily stretching for as little as 30 minutes per day is a powerful inducer of growth in normal and dystrophic muscle and that the progress of the histopathology in dystrophic muscle is delayed in proportion to the daily duration of stre

ISSN:0148-639X    

DOI:10.1002/mus.880060405

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

年代:1983

数据来源: WILEY

摘要:

AbstractA 4‐year‐old boy with nonprogressive myopathy since 2 years of age had “reducing bodies” in muscle biopsy specimens. Clinical and morphological findings are presented, which support the existence of a benign form of reducing body m

ISSN:0148-639X    

DOI:10.1002/mus.880060406

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

年代:1983

数据来源: WILEY

摘要:

AbstractCharacteristics of acetylcholine receptors were evaluated in G8‐1, a continuous skeletal muscle line. Peak binding of125I‐α‐bungarotoxin was in 10‐day‐old contractile myotubes at 4‐8 nm. Turnover was studied using two different methods; both indicated half‐times as little as half as long as previously reported for primary cultures. The effects of a variety of protease inhibitors on receptor turnover were assessed to determine if G8‐1 receptors were less stable or turned over faster because of increased neutral protease activity. Leupeptin, antipain, and chloroquine markedly slowed receptor degradation. Inhibitors of plasmin or plasminogen activator had definite but less dramatic effects on receptor turnover. Results from studies in which plasmin was increased in the tissue culture media indicated that a small but definite acceleration of receptor turnover occurred. In clonal G8‐1 cells, total number of acetylcholine receptors is controlled by negative feedback and although the major pathway for receptor degradation is lysosomal, plasmin may play a role in initiating recept

ISSN:0148-639X    

DOI:10.1002/mus.880060407

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

年代:1983

数据来源: WILEY

摘要:

AbstractPeripheral nerve dysfunction was a prominent clinical feature in each of three patients with the hypereosinophilic syndrome (HES). The neuropathy, occurring at the onset of marked eosinophilia or at a time of its worsening, had a painful onset, evolved over 1–4 weeks, and affected both sensory and motor function. Electrodiagnostic studies demonstrated both multifocal and generalized nerve involvement, with the former predominating (multiple mononeuropathy). Nerve and muscle biopsies from two patients revealed severe axonal degeneration with neurogenic atrophy of muscle. The eosinophilia decreased dramatically with corticosteroid treatment and the neuropathy gradually improved, but all three patients manifested residual neurologic disability 17–22 months after onset. The neuropathy may be on an ischemic basis or may be related to neurotoxicity of eosinophil produ

ISSN:0148-639X    

DOI:10.1002/mus.880060408

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

年代:1983

数据来源: WILEY

摘要:

AbstractMotor neuron abnormalities have been implicated in the pathogenesis of Duchenne muscular dystrophy. Evidence concerning the effect of injury on motor neurons of human Duchenne muscular dystrophy (DMD) is lacking. We report a DMD patient having, in addition, an obstetric paresis on his left arm. EMG and muscle histochemistry showed signs of reinnervation superimposed on myopathy in his left arm. This suggests that sprouting is preserved in DMD motor neurons and that muscle fibers retain the capability of accepting reinnervation.

ISSN:0148-639X    

DOI:10.1002/mus.880060409

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

年代:1983

数据来源: WILEY

摘要:

AbstractSeveral monoclonal antibodies (mcAbs) elicited against the nicotinic acetylcholine receptor (AChR) fromTorpedoreact also with skeletal muscle AChR. Such mcAbs were used to define antigenic determinants on muscle AChR and to elucidate their effect on muscle AChR functions. Primary chick muscle cultures were used as a model for skeletal muscle. Of the four mcAbs studies only mcAb 5.5, which is directed against the cholinergic site inTorpedoAChR, blocks the binding of α‐bungarotoxin (α‐β) to AChR in chick muscle cultures and inhibits carbamylcholine‐induced sodium transport in these cells. The interaction of mcAb 5.5 with the cholinergic site on muscle AChR demonstrates the conservation of this site. Two mcAbs, 5.5 and 5.34, each of a different antigenic specificity but both directed against conformation‐dependent antigenic determinants, accelerate the degradation of AChR in muscle cultures. From the reactivity of the various mcAbs with Triton‐solubilized and membranous AChR it appears that there are some antigenic differences between the detergent solubilized and membranous forms of

ISSN:0148-639X    

DOI:10.1002/mus.880060410

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

年代:1983

数据来源: WILEY

摘要:

AbstractA combination of multiple sclerosis (MS) and hypertrophic demyelinating neuropathy has been reported in a few autopsy studies. We are reporting a unique case of such a combination, which was proved by the sural nerve biopsy. A patient with classical MS on history and findings had areflexia and sensory abnormalities in stocking distribution. The nerve conduction study showed marked abnormalities indicative of demyelinating neuropathy, and sural nerve biopsy was typical of hypertrophic demyelinating neuropathy.

ISSN:0148-639X    

DOI:10.1002/mus.880060411

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

年代:1983

数据来源: WILEY