摘要:

AbstractComplement‐fixing antibody response to eleven different viruses were measured in amyotrophic lateral sclerosis (ALS) patients, contacts of ALS patients, neurological controls, and normal controls. The normal controls showed a decreased response to adeno‐associated virus and an increased response to adenovirus when compared to the other groups. Levels of interferon‐like substances also were investigated in sera and cerebrospinal fluids of ALS patients and neurological controls. Responses were of a low titer and were not increased in the ALS group. Explant cultures were established from tissues of 24 ALS autopsy cases. Cultures were investigated directly or following fusion to various indicator cell lines for viral‐like agents. Techniques such as interference assays, 5‐bromodeoxyuridine (BudR) induction, hemadsorption, and fluorescent antibody staining failed to detect virus. By addition of helper adenovirus to primary explant cultures, adeno‐associated virus was isolated from 2 of 1

ISSN:0148-639X    

DOI:10.1002/mus.880050203

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

年代:1982

数据来源: WILEY

摘要:

AbstractMuscles from 13 patients with clinical characteristics of congenital muscular dystrophy and central nervous system involvement (Fukuyama type) (FCMD) were examined using morphometric and fiber type analysis. The muscles from patients aged 5 months to 3 years demonstrated small‐calibered fibers with increased variation in fiber size, connective tissue proliferation, and scattered necrotic and regenerating fibers. Groups of atrophic fibers were absent. Both type 1 and type 2 fibers were affected, though type 1 fibers predominated and type 2B fibers decreased as the disease progressed. The muscle changes were apparently progressive, affecting not only the limbs but also the intercostal, diaphragm, and cardiac muscles. Although there was no qualitative difference in the muscle histochemistry between FCMD and Duchenne muscular dystrophy (DMD), there was a greater proportion of type 2C fibers and fibrosis was present at the early infantile stage of FCM

ISSN:0148-639X    

DOI:10.1002/mus.880050204

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

年代:1982

数据来源: WILEY

摘要:

AbstractDiabetic neuropathy in 180‐day‐old C57BL/Ks inbred mice is characterized by a marked reduction of nerve conduction velocity (NCV) and by axonal atrophy, as suggested by the decreased number of large diameter myelinated fibers. No demyelination or remyelination was observed. Ganglioside treatment from 150 to 180 days of age significantly improved the NCV and returned the fiber size distribution to control val

ISSN:0148-639X    

DOI:10.1002/mus.880050205

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

年代:1982

数据来源: WILEY

摘要:

AbstractA careful monitoring of the accuracy of diagnosis in six cases of Guillain‐Barre syndrome has shown that a substantial proportion of these patients initially diagnosed as having Guillain‐Barre syndrome on the basis of characteristic clinical findings and an elevated level of protein in the spinal fluid had a neuropathy caused by another etiology. The pitfalls in the laboratory and clinical diagnosis of disorders that were confused with Guillain‐Barre syndrome were several: the pattern of neurological dysfunction in the Guillain‐Barre syndrome was not unique to that disorder; no specific laboratory test existed to confirm the diagnosis of Guillain‐Barre syndrome; and the laboratory diagnosis of other causes of similar neurological disorders (especially heavy metal intoxication) depended upon tests that are very u

ISSN:0148-639X    

DOI:10.1002/mus.880050206

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

年代:1982

数据来源: WILEY

摘要:

AbstractIn rats treated biweekly with 20,25‐diazacholesterol (200 mg/kg orally), the desmosterol level in skeletal muscle sarcolemma increased progressively to about 80% of membrane sterol while total sterol levels remained constant. Following a single oral dose of 20,25‐D, the kinetics of desmosterol accumulation and subsequent loss in sarcolemma were more rapid than in whole muscle homogenates. The anisotropy of diphenylhexatriene fluorescence and the calculated microviscosity of the probe's microenvironment decreased significantly with increasing desmosterol levels in a temperature‐dependent manner, although fluorescent lifetimes were not altered. Fluorescent probes which localize in more superficial regions of the membrane detected no change. Studies with erythrocyte ghost membranes yielded results comparable to sarcolemma. Replacement of membrane cholesterol with desmosterol reduced the local microviscosity of the membrane hydrophobic region associated with phospholipid acyl chains and sterol side chains, but had little apparent effect on more superficial, polar regions. These observations can be correlated with the membrane location of the unsaturated side chain in desmos

ISSN:0148-639X    

DOI:10.1002/mus.880050207

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe genetic defect in myotonic muscular dystrophy (MyD) is expressed in several tissues. Evidence of the expression of this defect in MyD fibroblasts was sought by comparing them with normal fibroblasts. Reports describing increased alcianophilia and metachromasia in MyD fibroblasts could not be confirmed. Furthermore, no differences were observed with phase contrast microscopy or scanning and transmission electron microscopy, in histochemical studies, in growth characteristics, or in35S‐sulfate incorporation studie

ISSN:0148-639X    

DOI:10.1002/mus.880050208

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

年代:1982

数据来源: WILEY

摘要:

AbstractA study was made of the frequency of motor neuron disease in Scotland. The frequency of the disease was about the same as in most other countries of the world, but it may be increasing in Scotland. Individuals resident in the north and east of the country and those engaged in agricultural work were found to have an increased risk of developing the disease. It is suggested that motor neuron disease might be related to the use of insecticides or may result from a persistent viral infection.

ISSN:0148-639X    

DOI:10.1002/mus.880050209

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe influence of muscular contraction on the oxidative enzymes and the diameters of muscle fibers was investigated. Soleus muscles of guinea pigs were denervated for four weeks. The denervated fibers showed a reduction in the intensity of staining for β‐hydroxybutyrate dehydrogenase, cytochrome oxidase, succinate dehydrogenase, and NADH‐dependent tetrazolium reductase. Denervation also resulted in a decrease in fiber diameter. Denervated soleus muscles were electrically stimulated to contract over a four‐week period at a frequency normally received by slow contracting muscles. Electrical stimulation caused the stain intensity of histochemical reactions for oxidative enzymes to appear to be normal or greater than normal in 90% of the denervated fibers. Stimulation also caused 69% of the denervated fibers to be of normal or greater than normal size. The results demonstrate that contraction of denervated muscle by electrical stimulation prevents the loss of oxidative enzymes and the atrophy associated with dener

ISSN:0148-639X    

DOI:10.1002/mus.880050210

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

年代:1982

数据来源: WILEY

摘要:

AbstractFluoresceinated alpha‐bungarotoxin (α‐BT) and the Pestronk‐Drachman neuromuscular stain were combined to demonstrate the acetylcholine receptor, the junctional acetylcholinesterase, and the nerve terminal on the same endplate. The method is reliable and rapid. Synapses in both normal muscle and muscle affected by amyotrophic lateral sclerosis (ALS) are equally well dis

ISSN:0148-639X    

DOI:10.1002/mus.880050211

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

年代:1982

数据来源: WILEY

摘要:

AbstractA rapid, gentle technique has been developed for the isolation of sarcoplasmic reticulum (SR) from small amounts of skeletal muscle from normal and dystrophic mice. Assays for mitochondrial and surface membrane marker enzymes revealed only low levels of contamination in the isolated fractions. A small amount of calcium‐insensitive (“basal”) ATPase in the normal preparation and a higher value in the dystrophic were shown to be due to contamination by a lighter membrane fraction of probable surface membrane origin. Isolated SR from normal and dystrophic mice were indistinguishable by thin section and freeze‐fracture electron microscopy. Only small differences in calcium loading rates and capacity, and in calcium‐stimulated ATPase activity, were present. These were attributable to small differences in purity. We conclude that there is no difference in SR from normal and dystrophic mice in the properties

ISSN:0148-639X    

DOI:10.1002/mus.880050212

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

年代:1982

数据来源: WILEY