Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy.

abstract：:The electrical characteristics of myotonia in skeletal muscle appear remarkably similar in a variety of natural disorders and animal models. Recent experimental work has made it clear, however, that the pathophysiologic mechanisms underlying these myotonic discharges can be quite different. A mechanistic classificatio...

journal_title：Muscle & nerve

authors： Barchi RL

更新日期：1982-01-01 00:00:00

abstract：:Both high and low body mass index (BMI) have been reported as risk factors for ulnar neuropathy at the elbow (UNE), and a high BMI as a risk factor for carpal tunnel syndrome (CTS). To determine whether the extremes of BMI are risk factors for UNE or CTS, and whether BMI affects calculation of median and ulnar motor n...

journal_title：Muscle & nerve

authors： Landau ME,Barner KC,Campbell WW

更新日期：2005-09-01 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

authors： Echaniz-Laguna A,Rene F,Marcel C,Bangratz M,Fontaine B,Loeffler JP,Nicole S

更新日期：2009-07-01 00:00:00

abstract：:We prospectively evaluated thalidomide-induced neuropathy using electrodiagnostic studies. Sixty-seven men with metastatic androgen-independent prostate cancer in an open-label trial of oral thalidomide underwent neurologic examinations and nerve conduction studies (NCS) prior to and at 3-month intervals during treatm...

journal_title：Muscle & nerve

authors： Molloy FM,Floeter MK,Syed NA,Sandbrink F,Culcea E,Steinberg SM,Dahut W,Pluda J,Kruger EA,Reed E,Figg WD

更新日期：2001-08-01 00:00:00

abstract：:A common misconception attributes sparing of the flexor carpi ulnaris (FCU) in ulnar neuropathy at the elbow (UNE) to its innervating branch arising "at or above the elbow." We examined the relationship of FCU branches to the medial epicondyle (ME) and humeroulnar aponeurotic arcade (HUA) in 30 cadaver elbows. In only...

journal_title：Muscle & nerve

authors： Campbell WW,Pridgeon RM,Riaz G,Astruc J,Leahy M,Crostic EG

更新日期：1989-12-01 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：:We used peristimulus time histograms (PSTHs) to estimate characteristics of the composite excitatory postsynaptic potentials (EPSPs) generated at the anterior horn cell by a descending cortical volley induced by subthreshold transcranial magnetic stimuli in 11 normal subjects, 9 patients with DNA-confirmed Kennedy's d...

journal_title：Muscle & nerve

authors： Weber M,Eisen A

更新日期：1999-03-01 00:00:00

abstract：INTRODUCTION:The etiology of neuropathy was idiopathic in 20%-30% of patients despite thorough investigation, based on results from the 1980s and 1990s. Since then, new etiologies have been recognized, and skin biopsy has been used to confirm small-fiber neuropathy. METHODS:The authors reviewed the charts of 373 patie...

journal_title：Muscle & nerve

authors： Farhad K,Traub R,Ruzhansky KM,Brannagan TH 3rd

更新日期：2016-06-01 00:00:00

abstract：:A middle-aged male developed a right posterior femoral cutaneous neuralgia that persisted for 10 years without any associated neurologic, electrophysiologic, or radiologic findings. Five years after onset, surgical exploration of the subgluteal area identified a venous malformation surrounding the nerve. The syndrome ...

journal_title：Muscle & nerve

authors： Chutkow JG

更新日期：1988-11-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disorder with complex biology and significant clinical heterogeneity. Many preclinical and early phase ALS clinical trials have yielded promising results that could not be replicated in larger phase 3 confirmatory trials. One reason for the lack o...

journal_title：Muscle & nerve

authors： Goyal NA,Berry JD,Windebank A,Staff NP,Maragakis NJ,van den Berg LH,Genge A,Miller R,Baloh RH,Kern R,Gothelf Y,Lebovits C,Cudkowicz M

更新日期：2020-08-01 00:00:00

abstract：:Daptomycin is a lipopeptide antibiotic with strong bactericidal effects against Gram-positive bacteria and minor side effects on skeletal muscles. The type and magnitude of the early effect of daptomycin on skeletal muscles of rats was quantified by histopathology, examination of contractile properties, Evans Blue Dye...

journal_title：Muscle & nerve

authors： Kostrominova TY,Hassett CA,Rader EP,Davis C,Larkin LM,Coleman S,Oleson FB,Faulkner JA

更新日期：2010-09-01 00:00:00

abstract：INTRODUCTION:Are there electrophysiological findings that predict response to intravenous immunoglobulin (IVIg) in patients with lower motor neuron (LMN) syndromes without multifocal conduction block (MCB)? METHODS:We enrolled 9 patients with LMN syndromes without MCB to receive 18 weeks of IVIg therapy. Response was ...

journal_title：Muscle & nerve

authors： Lange DJ,Nijjar R,Voustianiouk A,Seidel G,Panchal J,Wang AK

更新日期：2011-04-01 00:00:00

abstract：INTRODUCTION:We report preliminary findings of nerve ultrasound in patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy. METHODS:The ultrasound cross-sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compa...

journal_title：Muscle & nerve

authors： Pelosi L,Leadbetter R,Mulroy E,Chancellor AM,Mossman S,Roxburgh R

更新日期：2017-07-01 00:00:00

abstract：:Compound muscle action potentials (CMAPs) evoked by magnetic brain stimuli are larger if the subject provides a steady background voluntary contraction of the target muscle. This facilitation could be due either to cortical or spinal mechanisms, or both. Both magnetic and electrical stimuli given immediately after the...

journal_title：Muscle & nerve

authors： Mills KR,Kimiskidis V

更新日期：1996-08-01 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 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:Diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) remains uncertain when nerve conduction studies (NCS) fail to show demyelination. METHODS:We conducted a retrospective study of patients who presented with clinical criteria of CIDP in whom electrodiagnostic (EDx) criteria of definite o...

journal_title：Muscle & nerve

authors： Devic P,Petiot P,Mauguiere F

更新日期：2016-01-01 00:00:00

abstract：INTRODUCTION:In preparation for clinical trials we examine the validity, reliability, and patient understanding of the Myotonic Dystrophy Health Index (MDHI). METHODS:Initially we partnered with 278 myotonic dystrophy type-1 (DM1) patients and identified the most relevant questions for the MDHI. Next, we used factor a...

journal_title：Muscle & nerve

authors： Heatwole C,Bode R,Johnson N,Dekdebrun J,Dilek N,Heatwole M,Hilbert JE,Luebbe E,Martens W,Mcdermott MP,Rothrock N,Thornton C,Vickrey BG,Victorson D,Moxley R 3rd

更新日期：2014-06-01 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

authors： Meyers EC,Granja R,Solorzano BR,Romero-Ortega M,Kilgard MP,Rennaker RL 2nd,Hays S

更新日期：2017-12-01 00:00:00

abstract：:Absence of α-actinin-3, encoded by the ACTN3 "speed gene," is associated with poorer sprinting performance in athletes and a slowing of relaxation in fast-twitch muscles of Actn3 knockout (KO) mice. Our first aim was to investigate, at the individual-fiber level, possible mechanisms for this slowed relaxation. Our sec...

journal_title：Muscle & nerve

authors： Chan S,Seto JT,Houweling PJ,Yang N,North KN,Head SI

更新日期：2011-01-01 00:00:00

abstract：:Nerve conduction studies (NCS) may be deferred because of a perceived risk of cardiac arrhythmia in the presence of same-limb peripheral intravenous lines. Patients with implanted pacemakers or defibrillators provide a model in whom this risk can be assessed. Twenty patients, seven with pacemakers and 13 with defibril...

journal_title：Muscle & nerve

authors： Mellion ML,Buxton AE,Iyer V,Almahameed S,Lorvidhaya P,Gilchrist JM

更新日期：2010-08-01 00:00:00

abstract：INTRODUCTION:Doppler ultrasonography (DU) has recently been shown to be useful in imaging carpal tunnel syndrome (CTS). In this study, we aim to characterize the changes seen after exercise and electrical stimulation. METHODS:Five patients with CTS were recruited with 5 age-matched subjects. DU was used to visualize t...

journal_title：Muscle & nerve

authors： Ng ES,Ng KW,Wilder-Smith EP

更新日期：2013-01-01 00:00:00

abstract：:We have assessed the reliability of hand-held myometry in 33 patients with spinal muscular atrophy (SMA), testing elbow flexion, handgrip, three-point pinch, knee flexion, knee extension, and foot dorsiflexion, and determining intraclass correlation coefficients (ICC). Interrater reliability was high for upper limbs, ...

journal_title：Muscle & nerve

authors： Merlini L,Mazzone ES,Solari A,Morandi L

更新日期：2002-07-01 00:00:00

abstract：INTRODUCTION:In this study we noninvasively evaluated skeletal muscle mitochondrial capacity, muscle-specific endurance, and energy/fatigue feelings in persons with Friedreich ataxia (FRDA) and able-bodied controls (AB). METHODS:Forearm mitochondrial capacity was measured in FRDA (n = 16) and AB (n = 10) study partici...

journal_title：Muscle & nerve

authors： Bossie HM,Willingham TB,Schoick RAV,O'Connor PJ,McCully KK

更新日期：2017-10-01 00:00:00

abstract：:We have identified a novel missense mutation in the myophosphorylase gene in a Spanish patient with McArdle's disease. The patient was homozygous for a T-to-C transition at codon 115 (L115P) in exon 3, which changed an encoded leucine (CUG) to a proline (CCG). This is the first mutation to be described in exon 3 and i...

journal_title：Muscle & nerve

authors： Gamez J,Fernandez R,Bruno C,Andreu AL,Cervera C,Navarro C,Schwartz S,Dimauro S

更新日期：1999-08-01 00:00:00

abstract：INTRODUCTION:The Accurate Test of Limb Isometric Strength (ATLIS) device can reliably measure the strength of 12 muscle groups using a fixed load cell. The purpose of this study was to analyze ATLIS data from healthy adults to calculate an individual's predicted strength scores. METHODS:ATLIS data were collected from ...

journal_title：Muscle & nerve

authors： Andres PL,English R,Mendoza M,Florence J,Malkus E,Schierbecker J,Siener C,Malspeis S,Schoenfeld DA,Munsat TL,Cudkowicz ME

更新日期：2013-02-01 00:00:00

abstract：:A mathematical model is presented that predicts the electric field induced in the arm during magnetic stimulation of a peripheral nerve. The arm is represented as a homogeneous, cylindrical volume conductor. The electric field arises from two sources: the time-varying magnetic field and the accumulation of charge on t...

journal_title：Muscle & nerve

authors： Roth BJ,Cohen LG,Hallett M,Friauf W,Basser PJ

更新日期：1990-08-01 00:00:00

abstract：:Of 185 patients with myopathy, 22 showed abnormal muscle mitochondria. In 12 of the 22 patients, all of whom had ocular myopathy or the ophthalmoplegia-plus syndrome, muscle biopsies contained 5%-25% "ragged red" fibers. In 4 patients with a facioscapulohumeral distribution of weakness, ragged red fibers were less num...

journal_title：Muscle & nerve

authors： Kamieniecka Z,Schmalbruch H

更新日期：1978-09-01 00:00:00

abstract：:Type 1 myotonic dystrophy or DM1 (Steinert's disease), which is the commonest muscular dystrophy in adults, has intrigued physicians for over a century. Unusual features, compared with other dystrophies, include myotonia, anticipation, and involvement of other organs, notably the brain, eyes, smooth muscle, cardiac co...

journal_title：Muscle & nerve

authors： Machuca-Tzili L,Brook D,Hilton-Jones D

更新日期：2005-07-01 00:00:00

abstract：:The goal of this study was to examine the effects of altered thyroid state and denervation (Den) on skeletal myosin heavy chain (MHC) expression in the plantaris and soleus muscles. Rats were subjected to unilateral denervation (Den) and randomly assigned to one of three groups: (1) euthyroid; (2) hyperthyroid; (3) an...

journal_title：Muscle & nerve

authors： Haddad F,Arnold C,Zeng M,Baldwin K

更新日期：1997-12-01 00:00:00