Abstract:
:In a woman with a slowly progressive adult onset proximal myopathy, muscle biopsy showed storage of PAS positive material in type 1 fibers. This material consisted of a branched chain polysaccharide associated with a mucoprotein. No abnormality of glycogen-pathway enzymes was detected. This suggested that this polysaccharide accumulation occurred because the polysaccharide was laid down in a non-bioavailable form. The clinical and histochemical features in this patient and in the few similar reported cases indicate that polysaccharide storage myopathy is a distinct entity that is allied to the glycogen storage myopathies.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Thompson AJ,Swash M,Cox EL,Ingram DA,Gray A,Schwartz MSdoi
10.1002/mus.880110411subject
Has Abstractpub_date
1988-04-01 00:00:00pages
349-55issue
4eissn
0148-639Xissn
1097-4598journal_volume
11pub_type
杂志文章相关文献
MUSCLE & NERVE文献大全abstract::Neuromuscular recovery after peripheral nerve lesion depends on the regeneration of severed axons that re-establish their functional connection with the denervated muscle. The aim of this study was to determine the effects of electrical stimulation (ES) on the neuromuscular recovery after nerve crush injury in rats. E...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.21549
更新日期:2010-05-01 00:00:00
abstract:INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by progressive muscle degeneration leading to immobility, respiratory failure, and premature death. As chronic inflammation and stress are implicated in DMD pathology, the efficacy of an anti-inflammatory and anti-stress intervention strategy in ameliorati...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25644
更新日期:2017-12-01 00:00:00
abstract:INTRODUCTION:The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population. METHODS:Clinical data accessed from the largest Utah healthcare providers have been record-linked to the Utah Population Database, a population-based resource also linked to the Utah Canc...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25145
更新日期:2016-10-01 00:00:00
abstract::The spectrum of neuromuscular disorders among intensive care unit (ICU) patients has shifted toward disorders acquired within the ICU and away from "traditional" neuromuscular disorders that lead to ICU admission. We sought to assess this spectrum by determining the causes and relative frequencies of neuromuscular dis...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199805)21:5<610::aid-mus7>
更新日期:1998-05-01 00:00:00
abstract::Autosomal dominant oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disease caused by (GCG) repeat expansions in exon 1 of the poly(A) binding protein 2 gene (PABP2). To elucidate the molecular mechanism underlying the disease, we raised an antiserum against a synthetic peptide fragment predicted from PABP2...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/1097-4598(200010)23:10<1549::aid-mus11>3.0
更新日期:2000-10-01 00:00:00
abstract:INTRODUCTION:Peripheral nerve injuries (PNI) are among the leading causes of physical disability in the United States. The majority of injuries occur in the upper extremities, and functional recovery is often limited. Robust animal models are critical first steps for developing effective therapies to restore function a...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25590
更新日期:2017-12-01 00:00:00
abstract:INTRODUCTION:Intensive care unit (ICU) patients often develop weakness. Rehabilitation is initiated early to prevent physical deterioration, but knowledge of optimal training schedules is lacking. A reliable method to assess muscle activity during exercise is needed. In this study we explored the feasibility of electri...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26330
更新日期:2018-11-01 00:00:00
abstract::During postnatal development of extensor digitorum longus (EDL) muscle, sodium withdrawal contractures were observed during the first 6 days after birth, and not after this time. In regenerating EDL muscles, zero-Na contractures were demonstrated: (1) 7 days after bupivacaine injection, but not 14 or 90 days after thi...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199811)21:11<1530::aid-mus
更新日期:1998-11-01 00:00:00
abstract::Neuroinflammation is a characteristic of pathologically affected tissue in several neurodegenerative disorders. These changes can be observed in the brainstem and spinal cord of amyotrophic lateral sclerosis (ALS) cases and in mouse models of the disease. They include an accumulation of large numbers of activated micr...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.10191
更新日期:2002-10-01 00:00:00
abstract::The F-wave was used to estimate motor nerve conduction velocity (MNCV) along the proximal segment of the fastest axons of the deep peroneal nerve (anterior-horn cells to knee) in 13 patients with amyotrophic lateral sclerosis (ALS) and in 21 age- and sex-matched control subjects. Measurements were based on the shortes...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880010607
更新日期:1978-11-01 00:00:00
abstract:INTRODUCTION:RASopathies are a group of genetic conditions due to alterations of the Ras/MAPK pathway. Neurocutaneous findings are hallmark features of the RASopathies, but musculoskeletal abnormalities are also frequent. The objective was to evaluate handgrip strength in the RASopathies. METHODS:Individuals with RASo...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23324
更新日期:2012-09-01 00:00:00
abstract:INTRODUCTION:The 6-minute walk test (6MWT) is a well-established clinical assessment of functional endurance, validated as a measure of walking ability in spinal muscular atrophy (SMA). The current availability of disease-modifying therapies for SMA indicates a growing need for normative reference data to compare SMA p...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26794
更新日期:2020-03-01 00:00:00
abstract:INTRODUCTION:An enlarged tongue (macroglossia) has been reported in advanced-stage patients with amyotrophic lateral sclerosis (ALS). METHODS:In this study we examined the prevalence of macroglossia and analyzed clinical correlations in 65 ALS patients on tracheostomy-invasive ventilation (TIV). RESULTS:Macroglossia ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25058
更新日期:2016-09-01 00:00:00
abstract::Hindlimb unloading (HU) results in oxidative stress, skeletal muscle atrophy, and increased damage upon reloading. Heat shock proteins (HSPs) protect against oxidative stress. However, it is unknown whether HSPs are depressed with long-term unloading (28 days) or reloading. We tested the hypotheses that long-term HU w...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20454
更新日期:2006-02-01 00:00:00
abstract:INTRODUCTION:We investigated interrater reliability of overall assessment of nerve root lesions by electrodiagnostic testing (EDX) in neonatal brachial plexus palsy (NBPP). METHODS:Two blinded, board-certified reviewers retrospectively reviewed de-identified EDX data from 37 infants with NBPP for 2005-2012. Only nerve...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25193
更新日期:2017-01-01 00:00:00
abstract::Multifocal motor neuropathy (MMN) is now a well-defined purely motor multineuropathy characterized by the presence of multifocal partial motor conduction blocks (CB), frequent association with anti-GM1 IgM antibodies, and usually a good response to high-dose intravenous immunoglobulin (IVIg) therapy. However, several ...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.20296
更新日期:2005-06-01 00:00:00
abstract::Sialorrhea is frequently a socially disabling symptom in patients with bulbar amyotrophic lateral sclerosis (ALS). In this open-label prospective study, we report the effect of botulinum toxin A (Botox) injection into the parotid glands in 10 patients with bulbar ALS and socially disabling sialorrhea. We applied three...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章
doi:10.1002/mus.20545
更新日期:2006-08-01 00:00:00
abstract:INTRODUCTION:Patients with DMD have low bone mineral density (BMD) and are at high risk for fractures. We examined changes in BMD and the effects of alendronate in DMD patients treated at our institution in the last decade. METHODS:Retrospective cohort study of 39 DMD patients. RESULTS:Patients had screening dual ene...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23948
更新日期:2014-04-01 00:00:00
abstract::Intravascular lymphomatosis, also known as endovascular lymphoma or angiotropic large cell lymphoma, is a rare malignancy typically diagnosed at autopsy. We describe a case in which the diagnosis was made at an early stage. Specific electrodiagnostic findings suggested concomitant polyneuropathy and myopathy. Suspicio...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.10091
更新日期:2002-05-01 00:00:00
abstract:INTRODUCTION:An environmental trigger of sporadic amyotrophic lateral sclerosis (ALS) is supported by geographic disparities in ALS incidence and development of the disease in conjugal couples. This study aims to investigate the incidence of ALS in the Northern New England states of New Hampshire (NH), Vermont (VT), an...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23761
更新日期:2013-08-01 00:00:00
abstract::Repetitive nerve stimulation (RNS) is a standard diagnostic procedure in myasthenia gravis (MG). Although RNS sensitivity is highest in weak muscles, RNS is easier to perform in distal muscles that are often not affected. Twenty-five patients with MG were assessed to compare the sensitivity of RNS of the nasalis muscl...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章
doi:10.1002/mus.10411
更新日期:2003-08-01 00:00:00
abstract:INTRODUCTION:Meralgia paresthetica is a focal neuropathy caused by compression of the lateral femoral cutaneous nerve (LFCN). The disease can be difficult to assess by neurophysiological or imaging studies. METHODS:We studied 5 patients who presented to our neuromuscular clinic from April 2012 to December 2014 with a ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25044
更新日期:2016-04-01 00:00:00
abstract:INTRODUCTION:Immune-mediated myopathies are a heterogeneous group of chronic autoimmune disorders. Autoantibodies associated with this disease complex are classified into myositis-associated and myositis-specific. Anti-tRNA synthetase antibodies are the most well known of the myositis-specific antibodies. Previous repo...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23383
更新日期:2012-08-01 00:00:00
abstract::Obese individuals have slowed conduction in the median nerve across the wrist, but the mechanism for this is not established. This case-control study of 27 obese subjects and 16 thin subjects was designed to test the hypothesis that obese individuals have higher carpal canal pressures and more median nerve swelling th...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20125
更新日期:2004-10-01 00:00:00
abstract:INTRODUCTION:The aim of this study was to determine the patterns of responses for the electromyographic (EMG) amplitude vs. oxygen uptake ( V̇O2 ) relationships from muscles of the quadriceps femoris and hamstrings during incremental treadmill running. METHODS:Twelve men volunteered to perform an incremental test to e...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24901
更新日期:2015-12-01 00:00:00
abstract::High-density multichannel electromyography (EMG) recordings add spatial information to the temporal information content of the surface EMG (sEMG) signal. This study explores the potential value of such multichannel information at a single motor unit level, in particular for the improvement of motor unit number estimat...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20357
更新日期:2005-09-01 00:00:00
abstract:INTRODUCTION:The aim of this study was to assess whether peripheral neuropathy is a feature of glycogen storage disease type IIIa (GSD IIIa) in adult patients. METHODS:Medical records of a cohort of adult GSD IIIa patients who underwent electromyography (EMG) and nerve conduction studies (NCS) were reviewed, and the r...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24977
更新日期:2016-02-01 00:00:00
abstract::Ultrasound (US) technique was applied to measure the thickness, cross-sectional area (CSA), and internal structure of the quadriceps muscle in 70- to 80-year-old endurance and power athletes and untrained men. Isometric knee extension strength was higher in the power athletes than in the other groups. The mean values ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880140607
更新日期:1991-06-01 00:00:00
abstract::The present study was conducted to determine the effects of acute overload exercise on mitochondrial DNA and the structure of skeletal muscles. Rats were forced to run for 20 min until reaching complete exhaustion. We detected the large-scale deletion (7052 bp) of mitochondrial DNA by the nested polymerase chain react...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199902)22:2<258::aid-mus15
更新日期:1999-02-01 00:00:00
abstract::The aim of this study was to investigate in control subjects the effect of imagery training on the torque of plantar-flexor muscles of the ankle. Twenty-nine subjects were allocated to one of three groups that performed either imagery training, low-intensity strength training, or no training (only measurements). The l...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章
doi:10.1002/mus.10406
更新日期:2003-08-01 00:00:00