A standardized clinical evaluation of patients affected by facioscapulohumeral muscular dystrophy: The FSHD clinical score.

abstract：:Two sisters with a Charcot-Marie-Tooth disease type 1A (CMT1A) duplication, who had an unusual CMT1A clinical phenotype, are described. The 63-year-old proband presented with dysesthesia on the inner side of the right leg. Neurological examination revealed a localized sensory disturbance in the lower extremities and m...

journal_title：Muscle & nerve

authors： Murakami T,Oomori H,Hara A,Uyama E,Mita S,Uchino M

更新日期：1999-11-01 00:00:00

abstract：:Skeletal muscle fibers from dystrophic mice and littermate controls (ReJ-129) were characterized electrically and then injected with an intracellular marker. In this way they could be identified for examination with an electron microscope to correlate the relative time course of electrical and ultrastructural alterati...

journal_title：Muscle & nerve

authors： Kerr LM,Sperelakis N

更新日期：1983-01-01 00:00:00

abstract：:Recent years have seen steady progress in the identification of genetic muscle diseases as well as efforts to develop treatment for these diseases. Consequently, sensitive and objective new methods are required to identify and monitor muscle pathology. Magnetic resonance imaging offers multiple potential biomarkers of...

journal_title：Muscle & nerve

authors： Leung DG

更新日期：2019-10-01 00:00:00

abstract：:The aim of this work was to determine the role of peripheral facial muscle reinnervation in the central reorganization of the blink reflex (BR) after hypoglossal-facial anastomosis (HFA). An electrophysiological study was performed on seven patients who underwent HFA after facial nerve transection during surgery for a...

journal_title：Muscle & nerve

authors： Bernat I,Vitte E,Lamas G,Soudant J,Willer JC,Tankéré F

更新日期：2006-03-01 00:00:00

abstract：:Cerebral palsy (CP) is the most prevalent neurologic disease in children and a leading cause of severe physical disability. Research and clinical experience indicate that children with CP have abnormal neuromuscular junctions (NMJs), and we present evidence that nonapposition of neuromuscular junction components is as...

journal_title：Muscle & nerve

authors： Theroux MC,Oberman KG,Lahaye J,Boyce BA,Duhadaway D,Miller F,Akins RE

更新日期：2005-11-01 00:00:00

abstract：:A 62-year-old woman with axonal Guillain-Barré syndrome developed weakness and urinary retention simultaneously. The retention failed to recover for 10 months even after she regained the ability to walk. The patient exhibited no postural hypotension. Videourodynamics showed that the retention was caused not by the bla...

journal_title：Muscle & nerve

authors： Sakakibara R,Uchiyama T,Tamura N,Kuwabara S,Asahina M,Hattori T

更新日期：2007-01-01 00:00:00

abstract：INTRODUCTION:We determined whether instrumenting timed functional tasks with wireless inertial motion sensors were responsive to facioscapulohumeral muscular dystrophy (FSHD) progression and movement pattern changes. METHODS:Ten individuals who were clinically affected with genetically confirmed FSHD, mean age 54 year...

journal_title：Muscle & nerve

authors： Statland JM,Karanevich A,Bruetsch A,Huisinga J

更新日期：2019-11-01 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

authors： Lacomis D,Petrella JT,Giuliani MJ

更新日期：1998-05-01 00:00:00

abstract：:We report the case of a 27-year-old man treated for bilateral optic neuritis 5 and 3 years before who within a few months developed sensorimotor disorders of the arms and legs characterized by asymmetric distribution and distal prominence. In addition to sensorimotor defects, which were particularly marked in the left...

journal_title：Muscle & nerve

authors： Péréon Y,Jardel J,Guillon B,Guihéneuc P

更新日期：1994-11-01 00:00:00

abstract：:The purpose of the study was to investigate differences in contractile speed, force, and fatigability of the adductor pollicis muscle between 12 patients with multiple sclerosis (MS) and 8 sedentary control subjects matched for age and gender. There were no differences between the patients with MS and control subjects...

journal_title：Muscle & nerve

authors： de Ruiter CJ,Jongen PJ,van der Woude LH,de Haan A

更新日期：2001-09-01 00:00:00

abstract：:Spatial distribution of motor endplates affects the shape of the electrical activity recorded from muscle. In order to provide information for realistic models of action potential propagation within muscles, we assembled three-dimensional maps of the motor endplates of the rat medial gastrocnemius (MGM) and lateral ga...

journal_title：Muscle & nerve

authors： Prodanov D,Thil MA,Marani E,Delbeke J,Holsheimer J

更新日期：2005-09-01 00:00:00

abstract：:It has been suggested that vibration causes small changes in muscle length, but to the best of our knowledge, these have yet to be demonstrated during whole-body vibration (WBV). This was an observational study to determine whether acute WBV would result in muscle lengthening. We hypothesized that acute WBV would incr...

journal_title：Muscle & nerve

authors： Cochrane DJ,Loram ID,Stannard SR,Rittweger J

更新日期：2009-09-01 00:00:00

abstract：:In a reported patient with myopathic carnitine deficiency, addition of exogenous carnitine to muscle homogenates failed to correct palmitate oxidation, and oral carnitine was of no clinical benefit. In a muscle biopsy from this patient, we found that, in contrast to the marked deficiency of free carnitine (3% of norma...

journal_title：Muscle & nerve

authors： Trevisan CP,Reichmann H,DeVivo DC,DiMauro S

更新日期：1985-10-01 00:00:00

abstract：INTRODUCTION:Lower limb strength has never been characterized separately for late-onset and adult myotonic dystrophy type 1 (DM1) phenotypes. METHODS:The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 asse...

journal_title：Muscle & nerve

authors： Petitclerc É,Hébert LJ,Mathieu J,Desrosiers J,Gagnon C

更新日期：2017-07-01 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

authors： Camic CL,Kovacs AJ,Enquist EA,McLain TA,Hill EC

更新日期：2015-12-01 00:00:00

abstract：:Mycophenolate mofetil 1.5 g daily (30 mg/kg body weight) was given to a patient with ankylosing spondylitis, ulcerative colitis, and severe refractory polymyositis after conventional treatment regimes had failed. No severe side effects occurred. Considerable improvement of clinical symptoms and electromyographic findi...

journal_title：Muscle & nerve

authors： Schneider C,Gold R,Schäfers M,Toyka KV

更新日期：2002-02-01 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

authors： Louboutin JP,Noireaud J

更新日期：1998-11-01 00:00:00

abstract：INTRODUCTION:The immuno-microenvironment of injured nerves adversely affects mesenchymal stem cell (MSC) therapy for neurotmesis. Magnetic resonance imaging (MRI) can be used noninvasively to monitor nerve degeneration and regeneration. The aim of this study was to investigate nerve repair after MSC transplantation com...

journal_title：Muscle & nerve

authors： Yang Z,Zheng C,Zhang F,Lin B,Cao M,Tian X,Zhang J,Zhang X,Shen J

更新日期：2020-06-01 00:00:00

abstract：:Weakness is common in both hyper- and hypothyroidism, and skeletal muscle L-carnitine may play a role in this regard, as suggested by studies indicating abnormal levels of carnitine in serum and urine of patients with thyroid dysfunction. Skeletal muscle samples were obtained for carnitine analysis from control subjec...

journal_title：Muscle & nerve

authors： Sinclair C,Gilchrist JM,Hennessey JV,Kandula M

更新日期：2005-09-01 00:00:00

abstract：:To determine the role of personal variables as risk factors for carpal tunnel syndrome (CTS) and their relationship to severity of nerve conduction abnormality, we studied 210 consecutive symptomatic CTS patients and 320 controls subjects without CTS symptomatology or known systemic disorders. The CTS group was classi...

journal_title：Muscle & nerve

authors： Kouyoumdjian JA,Zanetta DM,Morita MP

更新日期：2002-01-01 00:00:00

abstract：:Laser Doppler flowmetry (LDF) was conducted on familial amyloidotic polyneuropathy (FAP) patients and asymptomatic carriers of FAP. Vasoconstrictive responses in the 11 FAP patients tested, induced by deep inspiration, were markedly depressed compared with those of the healthy controls. The responses decreased with th...

journal_title：Muscle & nerve

authors： Ando Y,Araki S,Shimoda O,Kano T

更新日期：1992-04-01 00:00:00

abstract：:We present three new and 14 retrospective cases of polyradiculopathy in sarcoidosis. Of these, 71% had weakness and 59% areflexia of the lower extremities, and 35% had sphincter dysfunction. Cases often were associated with central nervous system sarcoidosis. All cases involved thoracolumbar or lumbosacral roots, exce...

journal_title：Muscle & nerve

authors： Koffman B,Junck L,Elias SB,Feit HW,Levine SR

更新日期：1999-05-01 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

authors： Spires MC,Brown SM,Chang KW,Leonard JA,Yang LJ

更新日期：2017-01-01 00:00:00

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) subjects ≥5 years with nonsense mutations were followed for 48 weeks in a multicenter, randomized, double-blind, placebo-controlled trial of ataluren. Placebo arm data (N = 57) provided insight into the natural history of the 6-minute walk test (6MWT) and other endpoints. ...

journal_title：Muscle & nerve

authors： McDonald CM,Henricson EK,Abresch RT,Florence JM,Eagle M,Gappmaier E,Glanzman AM,PTC124-GD-007-DMD Study Group.,Spiegel R,Barth J,Elfring G,Reha A,Peltz S

更新日期：2013-09-01 00:00:00

abstract：:The aim of this study was to estimate normal jitter in voluntarily activated extensor digitorum communis (EDC) and orbicularis oculi (OOc) muscles using a disposable concentric needle electrode (CNE). The EDC of 67 normal subjects (22 males and 45 females, mean age 35.5 +/- 10.2 years) and the OOc of 50 normal subject...

journal_title：Muscle & nerve

authors： Kouyoumdjian JA,Stålberg EV

更新日期：2008-06-01 00:00:00

abstract：:In spite of extensive studies it is unclear whether impaired fasting glucose (IFG) or impaired glucose tolerance (IGT), i.e., impaired glucose metabolism (IGM), causes diabetic sensorimotor polyneuropathy (DSPN) or chronic idiopathic axonal polyneuropathy (CIAP); the results and conclusions vary considerably in differ...

journal_title：Muscle & nerve

authors： Dyck PJ,Dyck PJ,Klein CJ,Weigand SD

更新日期：2007-10-01 00:00:00

abstract：INTRODUCTION:Previous studies have reported higher wrist ratios (WR) related to carpal tunnel syndrome (CTS) but have not assessed effect modification by obesity and may have inadequately controlled for confounders. METHODS:Baseline data of a multicenter prospective cohort study were analyzed. CTS was defined by nerve...

journal_title：Muscle & nerve

authors： Thiese MS,Merryweather A,Koric A,Ott U,Wood EM,Kapellusch J,Foster J,Garg A,Deckow-Schaefer G,Tomich S,Kendall R,Drury DL,Wertsch J,Hegmann KT,Wistah Study Team.

更新日期：2017-12-01 00:00:00

abstract：INTRODUCTION:Previous studies of limb-girdle muscular dystrophy type 2A (LGMD2A) patients in many countries have suggested a heterogeneous genetic and clinical spectrum, but the genotypes and phenotypes of Chinese LGMD2A patients remain unclear. METHODS:We directly screened calpain-3 (CAPN3) in 18 Chinese Han subjects...

journal_title：Muscle & nerve

authors： Luo SS,Xi JY,Zhu WH,Zhao CB,Lu JH,Lin J,Wang Y,Lu J,Qiao K

更新日期：2012-11-01 00:00:00

abstract：INTRODUCTION:Thymectomy is required for the treatment of thymoma-associated myasthenia gravis (MG). However, MG may develop only after thymectomy, a condition known as post-thymectomy MG. This study aimed to investigate the risk factors for post-thymectomy MG in patients with thymoma. METHODS:We retrospectively identi...

journal_title：Muscle & nerve

authors： Kim A,Choi SJ,Kang CH,Lee S,Son H,Kim JA,Shin JY,Kim SM,Hong YH,Sung JJ

更新日期：2021-01-12 00:00:00

abstract：INTRODUCTION:Despite the existence of guidelines, painful neuropathy is often inappropriately treated. We sought to determine the effectiveness of a clinical decision support system on guideline-recommended medication use. METHODS:We randomized neurology providers, stratified by subspecialty, to a best practice alert ...

journal_title：Muscle & nerve

authors： Reynolds EL,Burke JF,Banerjee M,Callaghan BC

更新日期：2020-05-01 00:00:00