摘要:

AbstractPhysical methods of treatment for neuromuscular diseases constitute the mainstay of current management. The overall goals of management are the maintenance of independent ambulation and the optimal functional state consistent with the disease process. Maintenance of muscle strength requires regular daily physical activity. An active exercise program can give limited increases of strength in muscular dystrophy dependent on the severity of the disease. Active exercise programs do not result in overwork weakness when properly supervised. Endurance exercise training does not appear to be suitable for Duchenne muscular dystrophy but may have value in the less rapidly progressive neuromuscular diseases. Contracture development can be retarded by passive stretching and splinting initiated early in the disease course. Weight control is important both for ambulatory and wheelchair‐bound patients. Reliable assessment of the results of physical therapy programs has been improved by the introduction of newer, more objective methods for measuring muscle strengt

ISSN:0148-639X    

DOI:10.1002/mus.880060502

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

年代:1983

数据来源: WILEY

摘要:

AbstractThe application of recombinant DNA techniques applied to the study of genetic neurological diseases will play a major role in the practice of neurology in upcoming years. Strategies are now available to develop useful and relatively simple biochemical diagnostic tests for heterozygous individuals with diseases inherited as autosomal dominant traits. In addition, molecular genetic methods will lead to the delineation of the genomic mutations responsible for these diseases. This review will update the current status of research in several neurological genetic diseases including myotonic muscular dystrophy, Huntington's disease, Charcot‐Marie‐Tooth disease and Duchenne muscular dystrophy (X‐linked). An introduction and overview of the methodology is provided. Specific research strategies including random screening of libraries, chromosome walking, messenger RNA selection, and messenger RNA translation are described. These strategies are designed to provide heterozygote identification, prenatal diagnosis and gestational management, the development of rational therapies, and the understanding of the molecular basis of disease expre

ISSN:0148-639X    

DOI:10.1002/mus.880060503

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

年代:1983

数据来源: WILEY

摘要:

AbstractThe postdenervation changes in glycogen metabolism were explored in fast (extensor digitorum longus, EDL), and slow (soleus, S) muscle of rat. During the first 12–14 hours after denervation, glycogen accumulates to a similar content in the two muscles, an effect reproduced by paralysis. Increase in the molecular weight of glycogen and decrease in the turnover rate of similar degree occur. During the ensuring 12–24 hours the glycogen concentration decreases in EDL (but not in S). This decrease is influenced by factors such as muscle type, longer or shorter nerve stump, age, and previous training of the animal. In both EDL and S, the rate of glucose incorporation into glycogen and the glycogen synthase [l/(l + D)] activity were depressed by denervation. The cAMP concentration increased 36 hours after denervation. This may have consequences on the muscle sensitivity to hormones. In fact, at this time, insulin did not increase glycogen synthase l activity or the glycogen content (with the exception of glycogen in S) and the effects of epinephrine on glycogen metabolism became more signific

ISSN:0148-639X    

DOI:10.1002/mus.880060504

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

年代:1983

数据来源: WILEY

摘要:

AbstractIn vitro oxidation of U‐[14C]glucose‐6‐phosphate, 2‐[14C]pyruvate, and 1‐[14C]pyruvate was significantly reduced in red skeletal muscle from fasting rats. Over the same time interval of fasting, 1‐[14C]palmitate oxidation remained unchanged. Pyruvate dehydrogenase activity, assayed before in vitro activation, was also reduced in red muscle. Long chain acylcarnitine and long chain acyl CoA increased over the same time period in red muscle. Changes in long chain fatty acid derivatives and pyruvate oxidation were much less dramatic in white muscle. We propose that the rise in levels of long chain fatty acid derivatives may be directly related to the inhibition of carbohydrate oxidation during fasting in red skel

ISSN:0148-639X    

DOI:10.1002/mus.880060505

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

年代:1983

数据来源: WILEY

摘要:

AbstractCompound muscle action potential (CMAP) amplitudes, response to 2 Hz nerve stimulation, response to exercise and electromyographic needle electrode examination findings from the thenar muscles of two patients with paramyotonia congenita were compared with those from two patients with dominantly inherited myotonia congenita in warm (34°C) and cold (20°C) states. Cold induced a significant fall in CMAP amplitude, induced/worsened a significant decremental response to 2 Hz stimulation, and virtually abolished myotonia and voluntary recruitment of motor unit potentials in patients with paramyotonia congenita; none of these occurred in myotonia congenita. Though exercise induced a mild fall in CMAP amplitude in both groups, postexercise fibrillations occurred only in patients with paramyotonia congenita. These findings serve to distinguish these two entities in the clinical electromyography laborator

ISSN:0148-639X    

DOI:10.1002/mus.880060506

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

年代:1983

数据来源: WILEY

摘要:

AbstractMuscle changes have been examined in 16 normal subjects (eight female) after both a 20‐minute and a prolonged step test. Stepping differs from most exercise tests in that it involves eccentric contractions (negative work) in which the active muscle is lengthened. Plasma creatine kinase (CK), muscle force, contractile properties, and tenderness in the quadriceps were measured for up to 9 days after the exercise. Muscle tenderness was experienced only in the muscles that had performed eccentric contractions (i.e., stepped down). All subjects showed some early rise in CK (<400 IU/liter) but eight (both male and female) showed a much greater response (up to 34,500 IU/liter) which took a long time to reach peak levels (4–5 days after stepping). It is suggested that eccentric contractions involved in this form of exercise result in some particular form of muscle damage which, in susceptible subjects, may initiate changes giving rise to a large delayed release of muscle enzy

ISSN:0148-639X    

DOI:10.1002/mus.880060507

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

年代:1983

数据来源: WILEY

ISSN:0148-639X    

DOI:10.1002/mus.880060508

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

年代:1983

数据来源: WILEY

ISSN:0148-639X    

DOI:10.1002/mus.880060510

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

年代:1983

数据来源: WILEY

ISSN:0148-639X    

DOI:10.1002/mus.880060512

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

年代:1983

数据来源: WILEY

ISSN:0148-639X    

DOI:10.1002/mus.880060513

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

年代:1983

数据来源: WILEY