Therapeutic trial of isaxonine in Duchenne muscular dystrophy.


:A randomized double-blind therapeutic trial of isaxonine was completed over a 2-year period for 20 ambulant boys with Duchenne muscular dystrophy aged 5 1/2-10 years. The effect of the drug was monitored by measurement of walking times over 28 and 150 ft, motor ability score, MRC score based on 32 muscle groups, and myometry of 7 muscle groups. The drug had no significant effect on the progression of the disease. The trial had statistical power comparable to previous larger-scale multicenter trials. This reflected the low variability in the patients in relation to the magnitude of the overall deterioration. Measurements of muscle force (myometry and MRC score) had much greater statistical power than measurements of function (motor ability score and walking times) as analyzed by our methods. These observations have important implications for the design of future trials.


Muscle Nerve


Muscle & nerve


Heckmatt JZ,Hyde SA,Gabain A,Dubowitz V




Has Abstract


1988-08-01 00:00:00












  • Chronic bilateral T1 denervation in women with macromastia.

    abstract:INTRODUCTION:The objective of this study was to identify the pattern of neurological deficits and document electrophysiological changes in women with macromastia. METHODS:Patients with macromastia and neurological complaints underwent clinical evaluation and electrodiagnostic (EDx) studies of the upper limbs. RESULTS...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Ansell JR,Buchholz MC

    更新日期:2013-02-01 00:00:00

  • Vasomotor dysfunction in carpal tunnel syndrome.

    abstract::Little attention has been paid to small-fiber dysfunction in carpal tunnel syndrome (CTS) although its symptoms are common. This study investigates vasomotor dysfunction, which is controlled by small nerve fibers, in patients with CTS compared with control subjects. Vasomotor function was quantified by measuring, with...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Wilder-Smith EP,Fook-Chong S,Chew SE,Chow A,Guo Y

    更新日期:2003-11-01 00:00:00

  • The electrodiagnostic findings in polyneuropathies associated with IgM monoclonal gammopathies.

    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

    pub_type: 杂志文章


    authors: Kelly JJ Jr

    更新日期:1990-12-01 00:00:00

  • Transgenic overexpression of laminin alpha1 chain in laminin alpha2 chain-deficient mice rescues the disease throughout the lifespan.

    abstract::Several approaches to treat laminin alpha2 chain-deficient congenital muscular dystrophy (MDC1A) in mouse models have been undertaken. Most have shown promising results in young animals. However, older animals have only been characterized to some extent. Herein we analyze the lifespan of laminin alpha2 chain-deficient...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Gawlik KI,Durbeej M

    更新日期:2010-07-01 00:00:00

  • Randomized controlled trial of a clinical decision support system for painful polyneuropathy.

    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

    pub_type: 杂志文章,随机对照试验


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

    更新日期:2020-05-01 00:00:00

  • Electrophysiologic assessment of a median nerve injury in the palm.

    abstract::In median nerve lesions of the hand, electrophysiologic evaluation of the second lumbrical muscle can help to differentiate between lesions of the common digital nerves and the proper digital nerves, as illustrated by this case report. Median nerve conduction studies to the second lumbrical muscle can be performed by ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Busis NA,Logigian EL,Shahani BT

    更新日期:1986-03-01 00:00:00

  • Motor unit action potential topography and its use in motor unit number estimation.

    abstract::High-density multichannel electromyography (EMG) recordings add spatial information to the temporal information content of the surface EMG (sEMG) signal. This study explores the potential value of such multichannel information at a single motor unit level, in particular for the improvement of motor unit number estimat...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Blok JH,Van Dijk JP,Zwarts MJ,Stegeman DF

    更新日期:2005-09-01 00:00:00

  • Cognitive impairment in neuromuscular disorders.

    abstract::Several studies have suggested the presence of central nervous system involvement manifesting as cognitive impairment in diseases traditionally confined to the peripheral nervous system. The aim of this review is to highlight the character of clinical, genetic, neurofunctional, cognitive, and psychiatric deficits in n...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审


    authors: D'Angelo MG,Bresolin N

    更新日期:2006-07-01 00:00:00

  • Changes in Na(+) channel expression and nodal persistent Na(+) currents associated with peripheral nerve regeneration in mice.

    abstract::Patients with peripheral neuropathy frequently suffer from positive sensory (pain and paresthesias) and motor (muscle cramping) symptoms even in the recovery phase of the disease. To investigate the pathophysiology of increased axonal excitability in peripheral nerve regeneration, we assessed the temporal and spatial ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Nakata M,Baba H,Kanai K,Hoshi T,Sawai S,Hattori T,Kuwabara S

    更新日期:2008-06-01 00:00:00

  • Recurrent idiopathic lumbosacral plexopathy.

    abstract::Recurrent idiopathic lumbosacral plexopathy has rarely been reported in the literature. The present report describes a 59-year-old man presenting with recurrent episodes of acute leg pain, followed by weakness. After each episode, symptoms progressed for several months before peaking. Thereafter, gradual recovery ensu...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审


    authors: Yee T

    更新日期:2000-09-01 00:00:00

  • Cardiac repolarization abnormalities in multiple sclerosis: spinal cord MRI correlates.

    abstract::Ventricular repolarization dysfunction has recently been reported in multiple sclerosis (MS). We evaluated ventricular repolarization dysfunction in 52 MS patients and looked for a relationship between corrected QT (QTc) abnormalities (i.e., abnormalities of QT intervals corrected for rate) and spinal cord magnetic re...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: de Seze J,Stojkovic T,Gauvrit JY,Saint Michel T,Ayachi M,Pruvo JP,Vermersch P

    更新日期:2000-08-01 00:00:00

  • Comparing the function of the corticospinal system in different species: organizational differences for motor specialization?

    abstract::An appreciation of the comparative functions of the corticospinal tract is of direct relevance to the understanding of how results from animal models can advance knowledge of the human motor system and its disorders. Two critical functions of the corticospinal tract are discussed: first, the role of descending project...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审


    authors: Lemon RN,Griffiths J

    更新日期:2005-09-01 00:00:00

  • Normal myoblast injections provide genetic treatment for murine dystrophy.

    abstract::A treatment has been developed to alleviate muscle weakness in murine dystrophy. Cultured myoblasts from genetically normal mouse embryos were injected into the right soleus of 20-day-old normal or dystrophic mice. Hosts and donors were immunocompatible but exhibited different genotype markers. Donor cells produced GP...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Law PK,Goodwin TG,Wang MG

    更新日期:1988-06-01 00:00:00

  • A novel electrodiagnostic assessment of the laryngeal closure reflex.

    abstract:INTRODUCTION:We describe a novel, clinically applicable conduction study of the laryngeal nerves. METHODS:Seventeen normal volunteer subjects were included. Activation of the sensory territory of the superior laryngeal nerve was performed by administration of low level brief electrical stimuli. The laryngeal closure r...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Carey B,Sulica L,Wu A,Branski R

    更新日期:2013-03-01 00:00:00

  • Paradoxical sleep deprivation induces muscle atrophy.

    abstract:INTRODUCTION:Because paradoxical sleep deprivation (PSD) induces a catabolic hormone profile, we sought to evaluate the morphology of the tibialis anterior (TA) muscle and testosterone and corticosterone levels of paradoxical sleep-deprived rats. METHODS:Three study groups of rats were established: the first group was...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Dattilo M,Antunes HK,Medeiros A,Mônico-Neto M,Souza Hde S,Lee KS,Tufik S,de Mello MT

    更新日期:2012-03-01 00:00:00

  • A new mutation in the regulatory domain of the myophosphorylase gene affecting protein dimer contact.

    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

    pub_type: 杂志文章


    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

  • Acute Lyme neuropathy presenting with polyradicular pain, abdominal protrusion, and cranial neuropathy.

    abstract::A 53-year-old man developed multifocal radicular pain. The diagnosis of Lyme neuroborreliosis was delayed until bifacial paresis and right lower abdominal wall weakness developed, prompting further evaluation. Cerebrospinal fluid (CSF) examination showed aseptic meningitis. Antibodies directed against Borrelia burgdor...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Krishnamurthy KB,Liu GT,Logigian EL

    更新日期:1993-11-01 00:00:00

  • Comparing the force- and excursion- frequency relationships in human skeletal muscle.

    abstract::We examined the influence of varying stimulation frequency on muscle output during isometric and dynamic contractions. Our findings demonstrate that the predictability of the force- and excursion-frequency relationships is extremely strong across stimulation intensities. There were no differences in the frequency whic...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Gregory CM,Bickel CS,Sharma N,Dixon WE

    更新日期:2008-12-01 00:00:00

  • A population-based survey of risk for cancer in individuals diagnosed with myotonic dystrophy.

    abstract:INTRODUCTION:The risk of cancer in patients diagnosed with myotonic dystrophy (DM) is reported for the homogeneous Utah population. METHODS:Clinical data accessed from the largest Utah healthcare providers have been record-linked to the Utah Population Database, a population-based resource also linked to the Utah Canc...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Abbott D,Johnson NE,Cannon-Albright LA

    更新日期:2016-10-01 00:00:00

  • Anatomic variation of the innervation of the flexor digitorum profundus muscle and its clinical implications.

    abstract::In anterior interosseous nerve syndrome and ulnar neuropathy, paralysis or weakness of the flexor digitorum profundus (FDP) muscles has been shown to vary according to the degree of involvement of the median and ulnar nerves, respectively. We traced these nerves in 50 cadaveric specimens in which each FDP was complete...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Oh CS,Won HS,Lee KS,Chung IH,Kim SM

    更新日期:2009-04-01 00:00:00

  • Magnetic resonance imaging signal hyperintensity of neural tissues in diffuse chronic pain syndromes: a pilot study.

    abstract::In this pilot study we use T2-weighted magnetic resonance imaging (MRI) to identify possible peripheral nerve inflammation in 4 patients with diffuse chronic pain. In all 4 patients, there was an increase in median and ulnar nerve T2 signal intensity at the wrist (P < 0.05 vs. controls). Positive clinical signs of per...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Dilley A,Greening J,Walker-Bone K,Good C

    更新日期:2011-12-01 00:00:00

  • Failure to detect HTLV-I by in situ hybridization in the biopsied muscles of viral carriers with polymyositis.

    abstract::Direct infection of muscle fibers by human T-lymphotropic virus type I (HTLV-I) has recently been reported in a patient with polymyositis infected with both HTLV-I and human immunodeficiency virus (HIV). Coinfections of these viruses are frequently found in the United States. In Kagoshima, Japan, patients with polymyo...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Higuchi I,Nerenberg M,Yoshimine K,Yoshida M,Fukunaga H,Tajima K,Osame M

    更新日期:1992-01-01 00:00:00

  • Somatosensory evoked potentials following stimulation of digital nerves.

    abstract::Cortical and spinal somatosensory evoked potentials (SEP) were recorded in healthy individuals following stimulation of digits I, III, and V with an intensity below discomfort level. Peak latency of the earliest cortical negativity (N1) was found to be the most consistent and easily measured parameter, whereas a spina...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章


    authors: Kwast-Rabben O,Libelius R,Heikkilä H

    更新日期:2002-10-01 00:00:00

  • AAEE case report #19: ischemic monomelic neuropathy.

    abstract::A case study of a 50-year-old man who awakened from aortobifemoral bypass graft surgery with painful burning and numbness of the left foot is reported. Angiography showed embolic occlusion of the left popliteal artery proximal to the knee. Nerve conduction studies performed 1 year after occlusion showed loss or severe...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Levin KH

    更新日期:1989-10-01 00:00:00

  • Postpolio muscular dysfunction: relationships between muscle energy metabolism, subjective symptoms, magnetic resonance imaging, electromyography, and muscle strength.

    abstract::Eleven patients with previous polio were studied. The concentration of energy-related metabolites and energy charge was measured from the vastus lateralis muscle, as was isometric muscle strength of knee extension. Cross-sectional area of the quadriceps femoris muscle was calculated from magnetic resonance imaging. Re...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Nordgren B,Falck B,Stålberg E,Ronquist G,Waldenström A,Ahlström H,Hemmingson A

    更新日期:1997-11-01 00:00:00

  • Frontal plane ankle proprioceptive thresholds and unipedal balance.

    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

    pub_type: 杂志文章


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

    更新日期:2009-02-01 00:00:00

  • The care of patients with Duchenne, Becker, and other muscular dystrophies in the COVID-19 pandemic.

    abstract::The coronavirus disease 2019 (COVID-19) pandemic has resulted in the reorganization of health-care settings affecting clinical care delivery to patients with Duchenne and Becker muscular dystrophy (DBMD) as well as other inherited muscular dystrophies. The magnitude of the impact of this public health emergency on the...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审


    authors: Veerapandiyan A,Wagner KR,Apkon S,McDonald CM,Mathews KD,Parsons JA,Wong BL,Eichinger K,Shieh PB,Butterfield RJ,Rao VK,Smith EC,Proud CM,Connolly AM,Ciafaloni E

    更新日期:2020-07-01 00:00:00

  • Isometric contractions of normal and spastic human skeletal muscle.

    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

    pub_type: 杂志文章


    authors: Ismail HM,Ranatunga KW

    更新日期:1981-05-01 00:00:00

  • Effects of transcranial magnetic stimulation on results of the twitch interpolation technique.

    abstract::To investigate whether transcranial magnetic stimulation (TMS) has an effect on isometric muscle force elicited by maximal voluntary contractions (MVC) and the ability to activate a muscle voluntarily (VA, as a percentage of full muscle activation), a twitch-interpolation technique was applied on the quadriceps femori...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章


    authors: Urbach D,Awiszus F

    更新日期:2000-07-01 00:00:00

  • Microvascular decompression for hemifacial spasm in patients >65 years of age: an analysis of outcomes and complications.

    abstract:INTRODUCTION:Few data are available to quantify the risks and benefits of microvascular decompression (MVD) in elderly patients with hemifacial spasm. METHODS:Twenty-seven patients >65 years of age and 104 younger patients who underwent MVD for hemifacial spasm (HFS) over a 3-year period were analyzed retrospectively ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章


    authors: Sekula RF Jr,Frederickson AM,Arnone GD,Quigley MR,Hallett M

    更新日期:2013-11-01 00:00:00