Randomized, double-blind trial of mazindol in Duchenne dystrophy.

abstract：INTRODUCTION:Although there are numerous protocols to adjust the amplitude of the Hoffmann reflex (H-reflex) relative to the size of the direct motor response (M-response), the optimal stimulating location has not been described. We sought to determine the optimal positioning of the stimulating cathode when evoking the...

journal_title：Muscle & nerve

authors： Özyurt MG,Shabsog M,Dursun M,Türker KS

更新日期：2018-12-01 00:00:00

abstract：:Danon disease is a rare X-linked dominant disorder caused by lysosomal-associated membrane protein 2 (LAMP2) deficiency and is characterized by hypertrophic cardiomyopathy, cardiac conduction abnormalities, skeletal vacuolar myopathy, variable degree of mental retardation, and peripheral pigmentary retinopathy. We des...

journal_title：Muscle & nerve

authors： Nadeau A,Therrien C,Karpati G,Sinnreich M

更新日期：2008-03-01 00:00:00

abstract：INTRODUCTION:Diaphragm excursion measured via ultrasound may be an important imaging outcome measure of respiratory function. We developed a new method for measuring diaphragm movement and compared it to the more traditional M-mode method. METHODS:Ultrasound images of the right and left hemidiaphragms were collected t...

journal_title：Muscle & nerve

authors： Goutman SA,Hamilton JD,Swihart B,Foerster B,Feldman EL,Rubin JM

更新日期：2017-01-01 00:00:00

abstract：INTRODUCTION:Chorea-acanthocytosis (ChAc) is a rare autosomal recessive disease characterized by involuntary movements, seizures, cognitive changes, myopathy, and axonal neuropathy. METHODS:We report a patient who presented with gait impairment and dysarthria. Clinical and neurophysiological assessment disclosed upper...

journal_title：Muscle & nerve

authors： Neutel D,Miltenberger-Miltenyi G,Silva I,de Carvalho M

更新日期：2012-02-01 00:00:00

abstract：:The purpose of this long-term, open parallel-group, double-consent study of alternate-day, low-dose prednisone in 2-4-year-old patients with Duchenne muscular dystrophy (DMD) was to determine whether prednisone produces a beneficial effect when given earlier than usual. Muscle function was evaluated by timed tests, an...

journal_title：Muscle & nerve

authors： Merlini L,Cicognani A,Malaspina E,Gennari M,Gnudi S,Talim B,Franzoni E

更新日期：2003-02-01 00:00:00

abstract：:The relationship between motor function and muscle strength in patients with spinal muscular atrophy (SMA) is still controversial. In 120 genetically proven SMA patients, aged 5 years or older, we measured muscle strength in the arms and legs by a hand-held dynamometer, forced vital capacity by a spirometer, and the t...

journal_title：Muscle & nerve

authors： Merlini L,Bertini E,Minetti C,Mongini T,Morandi L,Angelini C,Vita G

更新日期：2004-04-01 00:00:00

abstract：:Dystrophic changes of Schwann cells and demyelination occurred in rats with chronic nerve edema induced by feeding a galactose-rich diet for two years. The mechanism for edema is the sorbitol pathway which generates osmotically active polyols from galactose or glucose. The blood-nerve barrier impedes diffusion of macr...

journal_title：Muscle & nerve

authors： Powell HC,Myers RR

更新日期：1983-03-01 00:00:00

abstract：:Isometric contractile properties of human elbow flexor muscles were examined in 27 normal subjects (7 females and 20 males). Contractions of elbow flexors were elicited by application of electrical pulses to the biceps motor points. The tension development was recorded at the wrist with the elbow angle fixed at 90 deg...

journal_title：Muscle & nerve

authors： Ismail HM,Ranatunga KW

更新日期：1981-05-01 00:00:00

abstract：:Ion channels are complex proteins that span the lipid bilayer of the cell membrane, where they orchestrate the electrical signals necessary for normal function of the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. The role of ion channel defects in the pathogenesis of numerous disorder...

journal_title：Muscle & nerve

authors： Kleopa KA,Barchi RL

更新日期：2002-09-01 00:00:00

abstract：:A 30-year-old man with essential cryoglobulinemia presented with an axonal neuropathy and was found to have vasculitis at nerve biopsy. After 44 months, in accord with clinical deterioration, motor conduction studies showed excessive temporal dispersion multifocally, with partial conduction block persisting for 3 year...

journal_title：Muscle & nerve

authors： Notturno F,Caporale CM,Di Muzio A,Uncini A

更新日期：2007-10-01 00:00:00

abstract：:Muscle makes up the largest tissue volume of the body, yet its size makes muscle-specific therapy difficult. This becomes particularly relevant when approaches to gene therapy for inherited myopathies are evaluated. Thus, a mechanism to target constructs or pharmaceuticals to muscle following intravenous injection wou...

journal_title：Muscle & nerve

authors： Samoylova TI,Smith BF

更新日期：1999-04-01 00:00:00

abstract：:Neurotrophic factors are growth factors or cytokines that are inducible polypeptides and permit intercellular communication. An explosion of information about neurotrophic factors is setting the stage for significant advances in neural disease therapy in the next century. The effects of these trophic factors are overl...

journal_title：Muscle & nerve

authors： Mitsumoto H,Tsuzaka K

更新日期：1999-08-01 00:00:00

abstract：:Intraoperative electrophysiological monitoring of the spinal cord has traditionally been done by recording somatosensory evoked potentials (SEP). There is a risk that SEPs can be unaltered when significant injury to the anterior spinal cord has occurred. The purpose of this report is to describe a simple technique for...

journal_title：Muscle & nerve

authors： Phillips LH 2nd,Blanco JS,Sussman MD

更新日期：1995-03-01 00:00:00

abstract：:This study aimed to clarify the morphologic variations of the Martin-Gruber anastomosis (MGA) by tracing the anastomotic fascicles. We used 102 upper limbs, and MGA was found in 39.2%. Among 12 instances of MGA between the branches innervating the flexor digitorum profundus muscle, eight anastomotic branches solely in...

journal_title：Muscle & nerve

authors： Lee KS,Oh CS,Chung IH,Sunwoo IN

更新日期：2005-01-01 00:00:00

abstract：:Myasthenia gravis (MG) exacerbations may be triggered by infections.In the winter of 2009/2010, the Israeli Ministry of Health recommended that each individual with a chronic condition be vaccinated against both the seasonal influenza and the H1N1 virus. We examined vaccination practices in 74 MG patients. Only 38 pat...

journal_title：Muscle & nerve

authors： Auriel E,Regev K,Dori A,Karni A

更新日期：2011-06-01 00:00:00

abstract：INTRODUCTION:Muscle fatigue could have a greater impact on position sense when antagonists of the movement are fatigued. Hence, this study aimed to compare the effects of antagonist and agonist exercise-induced muscle fatigue on knee joint position sense. METHODS:This within-subjects repeated-measures study included 4...

journal_title：Muscle & nerve

authors： Ribeiro F,Venâncio J,Quintas P,Oliveira J

更新日期：2011-08-01 00:00:00

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

authors： Haanpää M,Häkkinen V,Nurmikko T

更新日期：1997-11-01 00:00:00

abstract：INTRODUCTION:Muscle force generation involves recruitment and firing rate modulation of motor units (MUs). The control of MUs in producing multidirectional forces remains unclear. METHODS:We studied MU recruitment and firing properties, recorded from the first dorsal interosseous muscle, for 3 different directions of ...

journal_title：Muscle & nerve

authors： Lei Y,Suresh NL,Rymer WZ,Hu X

更新日期：2018-01-01 00:00:00

abstract：INTRODUCTION:Our aim in this study was to determine whether intravenous immunoglobulin (IVIg) or plasma exchange (PLEx) for treatment of neurologic disease is a trigger for thrombotic events. METHODS:Using administrative data from 2005 to 2014, we identified index admissions for thrombotic events. We performed case-cr...

journal_title：Muscle & nerve

authors： Jin PH,Shin SC,Dhamoon MS

更新日期：2020-09-01 00:00:00

abstract：INTRODUCTION:Slowly progressive, genetic neuromuscular diseases (gNMDs) often lead to important motor deficiencies and functional limitations. The Quality of Life in Genetic Neuromuscular Disease Questionnaire (QoL-gNMD) is a new health-related quality-of-life questionnaire developed for these patients. The purpose of ...

journal_title：Muscle & nerve

authors： Dany A,Rapin A,Lavrard B,Saoût V,Réveillère C,Bassez G,Tiffreau V,Péréon Y,Sacconi S,Eymard B,Dramé M,Jolly D,Novella JL,Hardouin JB,Boyer FC

更新日期：2017-12-01 00:00:00

abstract：:Muscle sodium-channel disorders cover a spectrum of rare myotonic diseases. In a German family with 17 affected individuals in four generations, we identified a heterozygous missense mutation in exon 24 A1481D (c.4442 C>A) of the voltage-gated sodium channel gene (SCN4A) alpha subunit. Phenotypes of 12 family members ...

journal_title：Muscle & nerve

authors： Schoser BG,Schröder JM,Grimm T,Sternberg D,Kress W

更新日期：2007-05-01 00:00:00

abstract：:KANDID is an advanced EMG decision support system dedicated to the support of the clinical neurophysiologist during EMG examinations. It has facilities for test planning, automatized and structured data interpretation, EMG diagnosis, explanation, and reporting. In a prospective European multicenter field trial, the ag...

journal_title：Muscle & nerve

authors： Vingtoft S,Fuglsang-Frederiksen A,Rønager J,Petrera J,Stigsby B,Willison RG,Jarratt JA,Fawcett PR,Schofield IS,Otte G

更新日期：1993-05-01 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：INTRODUCTION:Two previously reported Norwegian patients with painful muscle cramps and giant myotonic discharges were genotyped and compared with those of members of 21 families harboring the same mutation. METHODS:Using primers specific for SCN4A and CLCN1, the DNA of the Norwegian family members was amplified and bi...

journal_title：Muscle & nerve

authors： Torbergsen T,Jurkat-Rott K,Stålberg EV,Løseth S,Hødneø A,Lehmann-Horn F

更新日期：2015-10-01 00:00:00

abstract：:Several monoclonal antibodies (mcAbs) elicited against the nicotinic acetylcholine receptor (AChR) from Torpedo react also with skeletal muscle AChR. Such mcAbs were used to define antigenic determinants on muscle AChR and to elucidate their effect on muscle AChR functions. Primary chick muscle cultures were used as a...

journal_title：Muscle & nerve

authors： Souroujon MC,Mochly-Rosen D,Gordon AS,Fuchs S

更新日期：1983-05-01 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:Despite use of qualitative laryngeal electromyography (LEMG) guided botulinum toxin A (BoNT-A) injection for treatment of adductor spasmodic dysphonia (AdSD), unsatisfactory injections and complete "misses" remain problematic. We aimed to determine if the quantitative LEMG measure of number of small segments...

journal_title：Muscle & nerve

authors： Dwyer CD,Leclerc AA,Nandedkar SD,Young VN,Rosen CA

更新日期：2020-12-31 00:00:00

abstract：INTRODUCTION:The minimal clinically important difference (MCID) is the smallest outcome change that has clinical significance. Its use has not been established in the study of myasthenia gravis (MG). METHODS:Patients from a published intravenous immunoglobulin (IVIg) vs. placebo study were studied. One anchor-based an...

journal_title：Muscle & nerve

authors： Katzberg HD,Barnett C,Merkies IS,Bril V

更新日期：2014-05-01 00:00:00

abstract：:Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder characterized by autoantibodies against presynaptic voltage-gated calcium channels that impair neuromuscular transmission. Malignancies, especially small cell lung cancer (SCLC), have been associated with LEMS and account for approximately 60% of cases...

journal_title：Muscle & nerve

authors： Wang C,Chen S,Feng B,Guan Y

更新日期：2014-03-01 00:00:00

abstract：:A stand-alone ultrasound shear wave imaging technology has been developed to quantify and visualize Young's modulus distribution by remotely applying ultrasound radiation force and tracking the resulting microvibrations in soft tissues with ultrafast ultrasound imaging. We report the first preliminary data that detect...

journal_title：Muscle & nerve

authors： Shinohara M,Sabra K,Gennisson JL,Fink M,Tanter M

更新日期：2010-09-01 00:00:00