Genetic variability and clinical spectrum of Chinese patients with limb-girdle muscular dystrophy type 2A.

abstract：INTRODUCTION:Livedoid vasculopathy is a rare dermatological condition characterized by painful ulceration, atrophic scarring, and persistent livedo reticularis. The pathogenesis is unclear. METHODS:We report a patient with biopsy-proven livedoid vasculopathy who developed a progressive sensory ganglionopathy with prof...

journal_title：Muscle & nerve

authors： Alix JJ,Hadjivassiliou M,Ali R,Slater D,Messenger AG,Rao DG

更新日期：2015-02-01 00:00:00

abstract：:Reliable unipedal balance is fundamental to safe ambulation. Accordingly, older persons with peripheral neuropathy (PN), who are at increased risk for falls, demonstrate impaired unipedal balance. To explore the relationship between afferent function and unipedal balance, frontal plane proprioceptive thresholds at the...

journal_title：Muscle & nerve

authors： Son J,Ashton-Miller JA,Richardson JK

更新日期：2009-02-01 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

authors： Argyropoulos CJ,Panayiotopoulos CP,Scarpalezos S

更新日期：1978-11-01 00:00:00

abstract：:We compared the compound motor action potentials (CMAPs) evoked in the biceps, triceps, and abductor digiti minimi (ADM) muscles by conventional electrical stimulation at Erb's point (EP), and by magnetic coil stimulation of the supraclavicular region in 11 normal subjects. We found that magnetic coil stimulation was ...

journal_title：Muscle & nerve

authors： Cros D,Gominak S,Shahani B,Fang J,Day B

更新日期：1992-05-01 00:00:00

abstract：:A large Swedish family with members affected by progressive external ophthalmoplegia with hypogonadism were followed-up and reviewed. Hypogonadism included delayed sexual maturation, primary amenorrhea, early menopause, and testicular atrophy. Cataracts, cerebellar ataxia, neuropathy, hypoacusia, pes cavus, tremor, pa...

journal_title：Muscle & nerve

authors： Melberg A,Arnell H,Dahl N,Stålberg E,Raininko R,Oldfors A,Bakall B,Lundberg PO,Holme E

更新日期：1996-12-01 00:00:00

abstract：INTRODUCTION:Information related to the long-term follow-up of neuropathy in patients with familial amyloid polyneuropathy after liver transplantation is still scarce. METHODS:We describe the neuropathic features of 3 patients with the transthyretin Val30Met mutation. Each patient underwent liver transplantation at an...

journal_title：Muscle & nerve

authors： Koike H,Hashimoto R,Tomita M,Kawagashira Y,Iijima M,Nakamura T,Watanabe H,Kamei H,Kiuchi T,Sobue G

更新日期：2012-12-01 00:00:00

abstract：:To determine which sensory nerve conduction studies (S-NCS) are helpful in detecting supraclavicular axon loss brachial plexopathies, we selected 53 cases (of 417 reviewed) in whom complicating factors were absent and which, by needle electrode examination findings, involved only a single "truncal" element (upper, mid...

journal_title：Muscle & nerve

authors： Ferrante MA,Wilbourn AJ

更新日期：1995-08-01 00:00:00

abstract：:We investigated 16 patients with Fabry's disease (eight hemizygous men and eight heterozygous women) in one family. We used constant current perception threshold (CPT) testing, which evaluated three major sensory nerve fiber populations, to assess subjective complaints of pain and paresthesias. We also examined clinic...

journal_title：Muscle & nerve

authors： Ro LS,Chen ST,Tang LM,Hsu WC,Chang HS,Huang CC

更新日期：1999-11-01 00:00:00

abstract：:The loss of muscle strength with aging appears to be greater in the lower than upper limbs, but strength and its neural control have never been compared in the same population of individuals in both upper and lower limbs. The aim of this study was to investigate differences between eight young (20-31 years) and eight ...

journal_title：Muscle & nerve

authors： Bazzucchi I,Felici F,Macaluso A,De Vito G

更新日期：2004-11-01 00:00:00

abstract：INTRODUCTION:The morphological characteristics of skeletal muscles innervated caudal to a spinal cord injury (SCI) undergo dramatic phenotypic and microvascular changes. METHOD:Female Sprague-Dawley rats received a severe contusion at thoracic level 9/10 and were randomly assigned to locomotor training, epidural stimu...

journal_title：Muscle & nerve

authors： Kissane RWP,Wright O,Al'Joboori YD,Marczak P,Ichiyama RM,Egginton S

更新日期：2019-03-01 00:00:00

abstract：:In order to further characterize and provide a possible mechanism for the asymmetrical involvement of forearm muscles in inclusion body myositis (IBM), we measured isometric hand and pinch grip strength, and forearm muscle girth on 15 IBM patients. Forearm muscle strength and girth were significantly greater on the do...

journal_title：Muscle & nerve

authors： Felice KJ,Relva GM,Conway SR

更新日期：1998-05-01 00:00:00

abstract：:The loss of contractile protein in Duchenne muscular dystrophy could result from low rates of synthesis, abnormally high rates of protein degradation, or a combination of both. We measured overall protein degradation rates in cultured human muscle cells obtained at biopsy from patients with Duchenne dystrophy or vario...

journal_title：Muscle & nerve

authors： Neville HE,Harrold S

更新日期：1985-03-01 00:00:00

abstract：INTRODUCTION:Factors determining response to intravenous immunoglobulin (IVIg) and plasmapheresis in myasthenia gravis (MG) have not been evaluated systematically. METHODS:This study included patients treated with IVIg (n = 63) or plasmapheresis (n = 42) from two trials evaluating IVIg vs. placebo or plasmapheresis in...

journal_title：Muscle & nerve

authors： Katzberg HD,Barnett C,Bril V

更新日期：2012-05-01 00:00:00

abstract：:Electromyographic examination of the deeper external anal sphincter (EAS) muscle is far more uncomfortable than of the subcutaneous muscle, so we tested the need for its examination. We compared the findings in 85 paired examinations of ipsilateral subcutaneous and deeper EAS muscles in 67 patients with a cauda equina...

journal_title：Muscle & nerve

authors： Podnar S

更新日期：2003-09-01 00:00:00

abstract：:Electrodiagnostic studies were analyzed in patients with neuropathy associated with IgM monoclonal proteins, 7 with anti-myelin associated glycoprotein reactivity (MAG) and 7 nonreactive to MAG. The findings were distinctly different in the two groups. The electrodiagnostic studies of all the MAG-reactive patients had...

journal_title：Muscle & nerve

authors： Kelly JJ Jr

更新日期：1990-12-01 00:00:00

abstract：:This study analyses maximum voluntary isometric contraction (MVIC) and its measurement by recording the force profile during maximal-effort, 7-s hand-grip contractions. Six healthy subjects each performed three trials repeated at short intervals to study variation from fatigue. These three trials were performed during...

journal_title：Muscle & nerve

authors： Househam E,McAuley J,Charles T,Lightfoot T,Swash M

更新日期：2004-03-01 00:00:00

abstract：:The compound muscle action potential (M(MAX)) during a maximal voluntary contraction (MVC) may be measured to determine if the motor nerve has been supramaximally stimulated during the interpolated twitch technique (ITT). Ten males performed isometric knee extension MVCs. M(MAX) for the vastus medialis was recorded du...

journal_title：Muscle & nerve

authors： Berger MJ,Watson BV,Doherty TJ

更新日期：2010-10-01 00:00:00

abstract：INTRODUCTION:Neuromuscular activation can be influenced by both muscle temperature (Tm) and fatigue. METHODS:To assess the effects of Tm and fatigue on the electromechanical delay (EMD), 15 participants performed voluntary isometric contractions of different intensities under neutral (TmN), low (TmL), and high (TmH) T...

journal_title：Muscle & nerve

authors： Cè E,Rampichini S,Agnello L,Limonta E,Veicsteinas A,Esposito F

更新日期：2013-04-01 00:00:00

abstract：:The latency fluctuation of single motor unit potentials (MUPH) in the H-reflex is greater than the latency fluctuation of MUPs in the direct (MUPM) and recurrent (MUPF) responses. This has been attributed to the variability in the impulse generation at the site of nerve stimulation, and to the variation in the synapti...

journal_title：Muscle & nerve

authors： Soliven B,Maselli RA

更新日期：1992-06-01 00:00:00

abstract：:Skeletal muscle involvement can occur at all stages of human immunodeficiency virus (HIV) infection, and may represent the first manifestation of the disease. Myopathies in HIV-infected patients are classified as follows: (1) HIV-associated myopathies and related conditions, including HIV polymyositis, inclusion-body ...

journal_title：Muscle & nerve

authors： Authier FJ,Chariot P,Gherardi RK

更新日期：2005-09-01 00:00:00

abstract：:We investigated whether the modulatory effects of segmental and descending inputs on the soleus H reflex are modified by postural conditions. Fourteen healthy volunteers received a transcranial magnetic stimulus (TMS) or percutaneous electrical stimulation of the posterior tibial nerve (PTN), preceding by 0 to 400 ms ...

journal_title：Muscle & nerve

authors： Goulart F,Valls-Solé J,Alvarez R

更新日期：2000-06-01 00:00:00

abstract：BACKGROUND:Limited data exist regarding myopathies with early or prominent dysphagia. METHODS:A retrospective study was performed (January 2003 to August 2019) to identify myopathy patients in whom dysphagia was the initial symptom or was disproportionately severe compared with limb weakness. RESULTS:Thirty-two patie...

journal_title：Muscle & nerve

authors： Triplett JD,Pinto MV,Hosfield EA,Milone M,Liewluck T

更新日期：2020-09-01 00:00:00

abstract：INTRODUCTION:Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. METHODS:We assessed: (1) Proficiency of grading ...

journal_title：Muscle & nerve

authors： Dyck PJ,Kincaid JC,Dyck PJB,Chaudhry V,Goyal NA,Alves C,Salhi H,Wiesman JF,Labeyrie C,Robinson-Papp J,Cardoso M,Laura M,Ruzhansky K,Cortese A,Brannagan TH 3rd,Khoury J,Khella S,Waddington-Cruz M,Ferreira J,Wang AK,

更新日期：2017-11-01 00:00:00

abstract：:Calmodulin levels have been assessed in whole muscle and primary culture extracts in order to examine the relationship between calmodulin and the accumulation of calcium in dystrophic hamster muscle tissues. Significant decreases in both normal and dystrophic skeletal muscle, tongue, and heart calmodulin levels were o...

journal_title：Muscle & nerve

authors： Klamut HJ,Kotarba JA,Strickland KP

更新日期：1987-01-01 00:00:00

abstract：:To obtain normative muscle morphology data on a healthy population recruited from a population survey, we examined vastus lateralis biopsies from 58 men and 33 women, aged 26-67 years. Biopsies were measured with automated, computer-aided techniques. Data were analyzed according to gender and age, and the influence of...

journal_title：Muscle & nerve

authors： Toft I,Lindal S,Bønaa KH,Jenssen T

更新日期：2003-07-01 00:00:00

abstract：:The presence of differences in motor unit activity across contraction types and between hands suggests that the magnitude of fluctuations in motor output is only weakly related when these conditions are compared. Twenty right-handed young (24.1 +/- 5.3 years) and old (72.5 +/- 4.9 years) adults performed three levels ...

journal_title：Muscle & nerve

authors： Shinohara M,Keenan KG,Enoka RM

更新日期：2005-06-01 00:00:00

abstract：:Isoproterenol (ISO), a potent beta-adrenoreceptor agonist, was found to interfere with the development and progression of hamster hereditary polymyopathy. Cytoprotection involved both skeletal and heart muscles with reduced myofibrillar degeneration, phagocytosis, and an unusual scarring process rarely seen at this st...

journal_title：Muscle & nerve

authors： Jasmin G,Proschek L

更新日期：1983-07-01 00:00:00

abstract：:The development of the personal computer has simplified the process of quantitating sensory thresholds using various testing algorithms. We reviewed the technical aspects and reproducibility of different methods to determine threshold for light touch-pressure, vibration, thermal, and pain stimuli. Clinical uses and li...

journal_title：Muscle & nerve

authors： Chong PS,Cros DP

更新日期：2004-05-01 00:00:00

abstract：:Many post-polio patients develop new muscle weakness decades after the initial illness. However, its mechanism and treatment are controversial. The purpose of this study was to test the hypotheses that: (1) after strength training, post-polio patients show strength improvement comparable to that seen in the healthy el...

journal_title：Muscle & nerve

authors： Chan KM,Amirjani N,Sumrain M,Clarke A,Strohschein FJ

更新日期：2003-03-01 00:00:00

abstract：:Morphological changes of the subneural apparatus (SNA) during denervation and reinnervation are demonstrated using the hamster peroneus longus muscle. One week after nerve section, synaptic grooves (SGs) were more shallow, with lowered sarcoplasmic ridges. After 4 weeks, the whole SNA area was elevated above the sarco...

journal_title：Muscle & nerve

authors： Matsuda Y,Oki S,Kitaoka K,Nagano Y,Nojima M,Desaki J

更新日期：1988-12-01 00:00:00