Abstract:
:Exertional muscle pain, contractures, recurrent rhabdomyolysis, and pigmenturia are common in certain muscle glycolytic disorders. However, the frequency, distribution, and long-term significance of these findings are poorly understood. First we performed magnetic resonance imaging (MRI) of the extremities as a screening test for the detection of muscle abnormalities incurred in activities of daily living in four patients with myophosphorylase deficiency (MPD) and three with muscle phosphofructokinase deficiency (PFKD). MRI findings of abnormal muscles detected upon screening were next compared with changes observed in a prospective study of muscle contractures involving the forearms of four of the patients (two MPD, two PFKD). Screening revealed abnormalities of proximal thigh muscles in three of seven patients, in two of whom (one MPD, one PFKD) a recent history of exertional myalgia coincided with increases in T1 and T2 estimates of isolated thigh muscles. In the third patient (PFKD), focal atrophy of the adductor magnus was present bilaterally. In prospective studies, focal areas of prolonged T1 and T2 appeared in the flexor digitorum superificalis in all four cases and in the flexor digitorum profundus in two cases. Serial imaging suggested that the onset of MRI abnormalities begins within 24 hours of contracture and persists for at least several days and possibly for much longer, with complete recovery apparently the rule. These cases suggests a high prevalence of focal muscle abnormalities in patients with glycolytic myopathies and show the potential of MRI to detect them.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Fleckenstein JL,Peshock RM,Lewis SF,Haller RGdoi
10.1002/mus.880121011subject
Has Abstractpub_date
1989-10-01 00:00:00pages
849-55issue
10eissn
0148-639Xissn
1097-4598journal_volume
12pub_type
杂志文章相关文献
MUSCLE & NERVE文献大全abstract::Motor involvement in acute herpes zoster is considered rare, but its incidence is unknown. In a sample of 40 patients with acute herpes zoster of varying severity, an abnormal electromyogram (EMG) (fibrillation, positive waves, high-frequency discharges) was found in 21 (53%), suggesting extension of inflammation to t...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199711)20:11<1433::aid-mus
更新日期:1997-11-01 00:00:00
abstract::To better define electrophysiological abnormalities in myasthenia gravis (MG) patients with muscle-specific tyrosine kinase (MuSK) antibodies (Ab), we compared electrophysiological features of 14 MuSK Ab-positive, 73 acetylcholine receptor antibody (AChR Ab)-positive, and 22 MuSK and AChR Ab-negative (seronegative) pa...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20498
更新日期:2006-04-01 00:00:00
abstract:INTRODUCTION:We assessed antibodies against low-density lipoprotein receptor-related protein 4 (LRP4-Ab) in a Chinese population with myasthenia gravis (MG). METHODS:Serum samples from 116 patients and 80 controls were collected. Acetylcholine receptor antibodies(AChR-Ab) and muscle-specific receptor tyrosine kinase a...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25591
更新日期:2017-11-01 00:00:00
abstract::Recent studies on the distribution of labeled endogenous proteins in the experimental neuropathies induced by streoptozotocin diabetes, galactose feeding, zinc pyridinethione, 2,5-hexanedione, acrylamide, and p-bromophenylacetylurea (BPAU) have demonstrated an impaired build up of retrogradely transported material der...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880060214
更新日期:1983-02-01 00:00:00
abstract::The purpose of this study was to assess turns/amplitude analysis (TAA) as an objective alternative to conventional qualitative electromyography (EMG) for detection of myopathy in facial muscles. Normal values of TAA parameters were calculated in the frontalis and mentalis muscles of 26 control subjects. We estimated t...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.21876
更新日期:2011-03-01 00:00:00
abstract::The intramembrane particle (IMP) profile of control and dystrophic (Bio 14.6) hamster cardiac muscle plasma membrane was assessed in freeze-fracture replicas to determine whether this animal model of muscular dystrophy exhibits the same membrane characteristics found in skeletal muscle from other more thoroughly studi...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880070702
更新日期:1984-09-01 00:00:00
abstract::Statins can induce necrotizing or inflammatory myopathies in some patients. Increased major histocompatibility complex class I (MHC I) expression has been shown in muscle biopsies of statin-induced myopathy. Therefore, we investigated the effect of statins on the expression of MHC I in muscle cells. Using flow cytomet...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.21479
更新日期:2010-02-01 00:00:00
abstract::We investigated the possible role of extracellular matrix in specifying the expression of superfast myosin during cat jaw muscle regeneration. Equal proportions of muscle tissue from jaw and limb were minced together after killing cellular elements from one source. We allowed the mince to regenerate in the bed of a fa...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880140503
更新日期:1991-05-01 00:00:00
abstract::Schwartz-Jampel syndrome (SJS) is an autosomal-recessive condition characterized by muscle stiffness and chondrodysplasia. It is due to loss-of-function hypomorphic mutations in the HSPG2 gene that encodes for perlecan, a proteoglycan secreted into the basement membrane. The origin of muscle stiffness in SJS is debate...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.21253
更新日期:2009-07-01 00:00:00
abstract::Factors that affect the shape of the so-called "normal cloud" of the turns and amplitude measurements of the electromyographic interference pattern are investigated. As the force of voluntary contraction increases from low to moderate levels, the number of turns in the signal increase faster than does the mean amplitu...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880140103
更新日期:1991-01-01 00:00:00
abstract::To enable the quantitative assessment of peripheral nerve structure and function, we determined the normal values for sural nerve hydration structure as measured by magnetic resonance proton imaging, and for neurological function with scores for neuropathic symptoms, signs, and nerve conduction properties. Normal huma...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880160311
更新日期:1993-03-01 00:00:00
abstract::Ten adult myotonic dystrophy patients underwent measurements of lung function, maximal dynamic and static ventilatory efforts, and respiratory muscle electromyography (EMG). EMG studies were performed during spontaneous breathing or when subjects breathed through high inspiratory or expiratory resistive loads. Present...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880080708
更新日期:1985-09-01 00:00:00
abstract::Repetitive nerve stimulation (RNS) of the median nerve is rarely studied in myasthenia gravis (MG). We performed a retrospective analysis of RNS studies performed on 448 patients at our center between 2010 and 2016. Among 95 patients with MG, an abnormal decrement of the compound muscle action potential amplitude was ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26713
更新日期:2019-12-01 00:00:00
abstract::Limb-girdle muscular dystrophy 2D (LGMD2D) is caused by mutations in the alpha-sarcoglycan gene (SGCA). The most frequently reported mutation, 229CGC>TGC (R77C) in exon 3 of SGCA, results in the substitution of arginine by cysteine. We present here the clinical, immunohistochemical, and genetic data of 11 Finnish pati...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20267
更新日期:2005-02-01 00:00:00
abstract::The objective of this study was to determine the self-reported prevalence of needlestick injuries among practicing electromyographers. In January 2008, an anonymous electronic survey was sent to all active members of the American Association for Neuromuscular and Electrodiagnostic Medicine (AANEM) who provided e-mail ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.21118
更新日期:2008-12-01 00:00:00
abstract::Six members originating from two families with hereditary motor and sensory neuropathy (hypertrophic and neuronal types) were noted to have enlarged calf muscles. Muscle computed tomography revealed that muscle enlargement in the propositus of the family with the hypertrophic type of HMSN was due to an increase in mus...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880130109
更新日期:1990-01-01 00:00:00
abstract::This study compared the force output produced by variable frequency, short-duration trains (VFTs) of electrical pulses with the forces produced by constant frequency, short-duration trains (CFTs). Human quadriceps femoris muscle was stimulated with a 300-msec train of pulses once every second for 180 seconds. Each sub...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/mus.880140909
更新日期:1991-09-01 00:00:00
abstract:INTRODUCTION:We sought to determine which muscles to choose for better assessment of the craniobulbar region in establishing the diagnosis of amyotrophic lateral sclerosis (ALS). METHODS:We studied the frontalis muscle in 83 controls and compared it with the tongue, sternocleidomastoid (SCM), and trapezius muscles in ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25236
更新日期:2016-12-01 00:00:00
abstract::We report two female patients with a history of alcohol abuse presenting with proximal painful muscle weakness following aversion therapy with emetine hydrochloride. Muscle biopsy of Case 1 showed a reversible floccular-shaped loss of myosin ATPase and dehydrogenase, an accumulation of PAS positive material, and a nor...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880070109
更新日期:1984-01-01 00:00:00
abstract:INTRODUCTION:Weakness in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) may be caused by decreases in muscle quantity and quality, but this has not been explored. METHODS:Twelve patients with CIDP (mean age 61 years) and 10 age-matched (mean age 59 years) control subjects were assessed for ankl...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26159
更新日期:2018-09-01 00:00:00
abstract::Calpains are Ca2+ -dependent cytosolic cysteine proteases that participate in the pathology of Duchenne muscular dystrophy (DMD). Utrophin is a functional homolog of dystrophin that partially compensates for dystrophin deficiency in myofibers of mdx mice. In this study, we investigated the susceptibility of utrophin t...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20549
更新日期:2006-06-01 00:00:00
abstract:INTRODUCTION:Tarsal tunnel syndrome (TTS) arises from tibial nerve damage under the flexor retinaculum of the fibro-osseus tunnel at the medial malleolus. It is notoriously difficult to diagnose, as many other foot pathologies result in a similar clinical picture. We examined the additional value of nerve ultrasound in...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24963
更新日期:2016-06-01 00:00:00
abstract::Diseases involving chronic inflammation can lead to prolonged exposure of skeletal muscle to inflammatory cytokines such as tumor necrosis factor alpha (TNFalpha), which may contribute to the skeletal muscle weakness seen in these conditions. In this study we examined the effect of a prolonged exposure to TNFalpha on ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20635
更新日期:2007-02-01 00:00:00
abstract:INTRODUCTION:C8-root impingement by C7/T1 lesions on neuroimaging studies is not consistently observed in C8 radiculopathy. We hypothesized that C7 or T1 root lesions (with a pre- or postfixed plexus) or cervical myelopathy might explain some "C8 radiculopathies" without C8 root compression. METHODS:Retrospective anal...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23319
更新日期:2012-06-01 00:00:00
abstract::We studied the mechanism underlying acute organophosphate intoxication (OPI) through in-vivo and in-vitro electrophysiologic studies in rats injected with diisopropylfluorophosphate. Intoxicated rats showed weakness, repetitive compound muscle action potentials (CMAPs) in response to a single stimulus, and decremental...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880141207
更新日期:1991-12-01 00:00:00
abstract:INTRODUCTION:The aim of this work was to determine the effect of chronic alcohol exposure on peripheral nerves in a nutritionally balanced rat model of alcoholism. METHODS:Three different strains of adult male rats were pair-fed for 8 weeks with isocaloric liquid diets containing 0% or 37% ethanol. Nerve conduction st...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23744
更新日期:2013-08-01 00:00:00
abstract::Exercise intolerance and undue fatigue are common complaints in patients with Charcot-Marie-Tooth (CMT) disease. Reduced physical ability is due directly to the disease, but it is also due to physical deconditioning. The aim of this study was to test whether 24 weeks of interval-training exercise (ITE) cycling can sig...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章
doi:10.1002/mus.20959
更新日期:2008-05-01 00:00:00
abstract:UNLABELLED:Contemporary natural history data in Duchenne muscular dystrophy (DMD) is needed to assess care recommendations and aid in planning future trials. METHODS:The Cooperative International Neuromuscular Research Group (CINRG) DMD Natural History Study (DMD-NHS) enrolled 340 individuals, aged 2-28 years, with DM...
journal_title:Muscle & nerve
pub_type: 杂志文章,多中心研究
doi:10.1002/mus.23807
更新日期:2013-07-01 00:00:00
abstract::Intravenous immunoglobulin (IVIg) is a useful tool in the treatment of a variety of neuromuscular disorders. Though IVIg therapy is generally safe, hemolytic anemia is a potentially serious complication that is often overlooked, and is currently not listed in product inserts. We analyzed 45 patients who received IVIg ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199709)20:9<1142::aid-mus8
更新日期:1997-09-01 00:00:00
abstract:INTRODUCTION:In this study we compare the ultrasound features in the median nerve in patients with different types of Charcot-Marie-Tooth (CMT) disease and hereditary neuropathies with liability to pressure palsies (HNPP) as a typical entrapment neuropathy. METHODS:Median nerve ultrasound and conduction studies were p...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23681
更新日期:2013-03-01 00:00:00