Early prednisone treatment in Duchenne muscular dystrophy.

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, and muscle strength with a hand-held myometer. After 55 months of treatment, the five patients (mean age 8.3 years) in the prednisone group were still able to get up from the floor, whereas two of the three in the control group had lost this ability. Side effects included a decline in growth rate in the prednisone-treated patients and excessive weight gain in one control and three treated patients. Because steroids are effective in prolonging function, but not in recovering lost function, we propose that treatment be started with low-dose prednisone in DMD patients as soon as the diagnosis is definite.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

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

doi

10.1002/mus.10319

subject

Has Abstract

pub_date

2003-02-01 00:00:00

pages

222-7

issue

2

eissn

0148-639X

issn

1097-4598

journal_volume

27

pub_type

临床试验,杂志文章
  • Effect of thermode application pressure on thermal threshold detection.

    abstract::Studies using quantitative sensory testing (QST) often present incongruent results due to intra- and intersubject as well as interobserver variability which limit widespread use of the technique. Eliminating or reducing the factors responsible for this variability is of great interest, as it increases reliability and ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21120

    authors: Pavlaković G,Klinke I,Pavlaković H,Züchner K,Zapf A,Bachmann CG,Graf BM,Crozier TA

    更新日期:2008-11-01 00:00:00

  • Cytokines and the pathogenesis of myasthenia gravis.

    abstract::Myasthenia gravis (MG) and its animal model experimental autoimmune myasthenia gravis (EAMG) are caused by autoantibodies against nicotinic acetylcholine receptor (AChR) in skeletal muscle. The production of anti-AChR antibodies is mediated by cytokines produced by CD4+ and CD8+ T helper (Th) cells. Emerging investiga...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(199705)20:5<543::aid-mus2>

    authors: Zhang GX,Navikas V,Link H

    更新日期:1997-05-01 00:00:00

  • Magnetic resonance imaging of peripheral nerves: differences in magnetization transfer.

    abstract:INTRODUCTION:Magnetic resonance imaging (MRI) is an excellent imaging modality for soft tissues. Magnetization transfer (MT) imaging is an MRI technique that is sensitive to the bound protons of macromolecules and therefore can aid in the assessment of nerve damage. METHODS:Measurements of the MT ratio (MTR), which pr...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22240

    authors: Gambarota G,Krueger G,Theumann N,Mekle R

    更新日期:2012-01-01 00:00:00

  • Lack of cold sensitivity in hyperkalemic periodic paralysis.

    abstract::The nosologic distinction between paramyotonia congenita and hyperkalemic periodic paralysis is somewhat blurred. Muscle membrane inexcitability induced by cooling seems to be characteristic of paramyotonia congenita. The effect of cooling on the maximal compound muscle action potential (CMAP) in patients with paramyo...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880090804

    authors: Subramony SH,Wee AS,Mishra SK

    更新日期:1986-10-01 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

    doi:10.1002/mus.880040307

    authors: Ismail HM,Ranatunga KW

    更新日期:1981-05-01 00:00:00

  • Distal sensory nerve conduction of the superficial peroneal nerve: new method and its clinical application.

    abstract::The superficial peroneal nerve subserves sensation on the entire surface of the dorsum of the foot, except in small areas. All previously reported techniques for evaluating nerve conduction along this nerve tested a proximal portion of the nerve. We report a new method for evaluating sensory nerve conduction of the fo...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章

    doi:10.1002/mus.1056

    authors: Oh SJ,Demirci M,Dajani B,Melo AC,Claussen GC

    更新日期:2001-05-01 00:00:00

  • Experimental investigation of muscular neurotization in the rat.

    abstract::Reinnervation of a free muscle graft by nerves from an adjacent intact muscle is called muscular neurotization. This paper investigates the mechanisms and stimuli responsible for muscular neurotization in the rat. Sternohyoid or sternomastoid muscles were transplanted as free muscle grafts to the ventral surface of an...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880100607

    authors: Must R

    更新日期:1987-07-01 00:00:00

  • Passive trunk loading influences muscle activation during dynamic activity.

    abstract:INTRODUCTION:Repetitive loading of the low back tissues induces tension relaxation with a corresponding variation in the myoelectric response of the neuromuscular system, which may influence low back health. The purpose of this study was to observe trunk muscle activities before and after a passive cyclic trunk flexion...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22154

    authors: Olson MW

    更新日期:2011-11-01 00:00:00

  • Measurement of intramuscular fat by muscle echo intensity.

    abstract:INTRODUCTION:The aim of this study was to compare ultrasound echo intensity (EI) with high-resolution T1 -weighted MRI and to establish calibration equations to estimate percent intramuscular fat from EI. METHODS:Thirty-one participants underwent both ultrasound and MRI testing of 4 muscles: rectus femoris (RF); bicep...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24656

    authors: Young HJ,Jenkins NT,Zhao Q,Mccully KK

    更新日期:2015-12-01 00:00:00

  • Diagnostic value of nerve biopsy for atypical chronic inflammatory demyelinating polyneuropathy: evaluation of eight cases.

    abstract::The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) relies primarily on clinical and electrophysiologic examination, but the nerve biopsy findings may be supportive, especially in atypical cases. In order to define the usefulness of nerve biopsy in this disease, we retrospectively studied 44 cons...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10348

    authors: Vallat JM,Tabaraud F,Magy L,Torny F,Bernet-Bernady P,Macian F,Couratier P

    更新日期:2003-04-01 00:00:00

  • Motor function-muscle strength relationship in spinal muscular atrophy.

    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

    pub_type: 杂志文章

    doi:10.1002/mus.20018

    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

  • Proximal conduction block in the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome.

    abstract:INTRODUCTION:Conduction block (CB) has been included in the Rajabally criteria for axonal Guillain-Barré syndrome (GBS). Because the nerve roots may be affected early in GBS, detection of proximal CB by the triple stimulation technique (TST) can be useful. METHODS:We describe TST findings in 2 patients who presented w...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24729

    authors: Taieb G,Grapperon AM,Duclos Y,Franques J,Labauge P,Renard D,Yuki N,Attarian S

    更新日期:2015-12-01 00:00:00

  • The thoracic outlet syndromes.

    abstract::The term thoracic outlet syndrome (TOS) refers to a heterogeneous group of disorders, all of which have in common compression of one or more neurovascular elements at some point within the thoracic outlet. Of the five disorders comprising this group, four have all of the features expected of a syndrome-a recognized co...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,评审

    doi:10.1002/mus.23235

    authors: Ferrante MA

    更新日期:2012-06-01 00:00:00

  • Estimated cost of treating myasthenia gravis in an insured U.S. population.

    abstract:INTRODUCTION:In this study we estimated the costs paid by U.S. health plans for treating myasthenia gravis (MG) in 2009 and determined the major cost drivers. METHODS:One hundred thirteen MG patients were matched by propensity scores with 339 non-MG patients from a comprehensive health-care insurance database. The mea...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22327

    authors: Guptill JT,Sharma BK,Marano A,Soucy A,Krueger A,Sanders DB

    更新日期:2012-03-01 00:00:00

  • Myosin light chain components in single muscle fibers of Duchenne muscular dystrophy.

    abstract::Single muscle fibers were prepared from biopsy specimens of Duchenne muscular dystrophy (DMD), normal, and neuromuscular disease controls. Single muscle cells were classified as type 1, type 2, or intermediate by the skinned fiber method. The intermediate fiber was most abundant in DMD, comprising 29% of fibers examin...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880050511

    authors: Takagi A,Ishiura S,Nonaka I,Sugita H

    更新日期:1982-05-01 00:00:00

  • Can we eliminate placebo in ALS clinical Trials?

    abstract::Amyotrophic lateral sclerosis (ALS) is a fatal disease with limited treatment options. Controlled studies are a necessary part of Phase II and Phase III assessments of proposed therapies. Due to the relatively small number of patients with ALS, several study designs have been proposed to improve the efficiency of Phas...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21358

    authors: Simmons Z

    更新日期:2009-06-01 00:00:00

  • Clinical utility of RNA sequencing to resolve unusual GNE myopathy with a novel promoter deletion.

    abstract:INTRODUCTION:UDP N-acetylglucosamine2-epimerase/N-acetylmannosamine-kinase (GNE) gene mutations can cause mostly autosomal-recessive myopathy with juvenile-onset known as hereditary inclusion-body myopathy (HIBM). METHODS:We describe a family of a patient showing an unusual HIBM with both vacuolar myopathy and myositi...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.26486

    authors: Chakravorty S,Berger K,Arafat D,Nallamilli BRR,Subramanian HP,Joseph S,Anderson ME,Campbell KP,Glass J,Gibson G,Hegde M

    更新日期:2019-07-01 00:00:00

  • Near-infrared spectroscopy during exercise and recovery in children with juvenile dermatomyositis.

    abstract:INTRODUCTION:We hypothesized that microvascular disturbances in muscle tissue play a role in the reduced exercise capacity in juvenile dermatomyositis (JDM). METHODS:Children with JDM, children with juvenile idiopathic arthritis (clinical controls), and healthy children performed a maximal incremental cycloergometric ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.23484

    authors: Habers GE,De Knikker R,Van Brussel M,Hulzebos E,Stegeman DF,Van Royen A,Takken T

    更新日期:2013-01-01 00:00:00

  • Response to exercise of patients with idiopathic hyper-CK-emia.

    abstract::Patients with an idiopathic increase in serum creatine kinase (CK) levels (hyper-CK-emia) have a benign prognosis, but symptoms may be disabling in daily life. Previous studies have suggested that physical exercise increases the severity of complaints in these patients. We studied whether maximal and submaximal bouts ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10232

    authors: Reijneveld JC,Te Boekhorst BC,Zonderland ML,Kalmijn S,Notermans NC

    更新日期:2002-12-01 00:00:00

  • Continuous intrathecal baclofen infusions induced a marked increase of the transcranially evoked silent period in a patient with generalized dystonia.

    abstract::We observed a marked prolongation of the transcranially evoked silent period during continuous intrathecal administration of high doses of the gamma-aminobutyric acid (GABA)B receptor agonist baclofen in a patient with generalized dystonia. Size of motor evoked potentials and central conduction time remained unchanged...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(199809)21:9<1209::aid-mus1

    authors: Siebner HR,Dressnandt J,Auer C,Conrad B

    更新日期:1998-09-01 00:00:00

  • Magnetic resonance imaging in Duchenne muscular dystrophy: longitudinal assessment of natural history over 18 months.

    abstract:INTRODUCTION:In Duchenne muscular dystrophy (DMD), fat replacement of muscle may be a useful endpoint in trials of therapy, although progression in different muscle groups is uneven. In this study we assessed the progression of fat replacement with T(1) -weighted imaging over 2 9-month periods. METHODS:Eight ambulant,...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.23879

    authors: Hollingsworth KG,Garrood P,Eagle M,Bushby K,Straub V

    更新日期:2013-10-01 00:00:00

  • AAEE case report #18: Ulnar neuropathy in the distal forearm.

    abstract::A 66-year-old professional golf instructor developed pain, numbness, and weakness in an ulnar distribution, but the lesion could not be localized to either elbow or wrist by routine nerve conduction techniques. Stimulation along serial 1-cm increments in the distal forearm disclosed a point of focal conduction block a...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880120502

    authors: Campbell WW

    更新日期:1989-05-01 00:00:00

  • Thalidomide neuropathy in patients treated for metastatic prostate cancer.

    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

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1002/mus.1109

    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

  • Expression of sodium channel subtypes during development in rat skeletal muscle.

    abstract::This study contrasts the developmental patterns of expression of 2 subtypes of the voltage-dependent sodium channel in rat muscle that are differentiated by their immunoreactivity with monoclonal antibodies raised to the purified muscle sodium channel protein. One subtype is found in the transverse tubular (T) system ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880140209

    authors: Schotland DL,Fieles W,Barchi RL

    更新日期:1991-02-01 00:00:00

  • Electrophysiological studies in a mouse model of Schwartz-Jampel syndrome demonstrate muscle fiber hyperactivity of peripheral nerve origin.

    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

    pub_type: 杂志文章

    doi:10.1002/mus.21253

    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

  • Radial motor nerve conduction studies in the upper arm.

    abstract:INTRODUCTION:The main goal of this study was to determine the contribution of the anterior forearm muscles to the compound muscle action potential (CMAP) recorded from the extensor digitorum (ED) after proximal stimulation. METHODS:Twenty-one healthy volunteers and 114 patients with compressive and traumatic radial ne...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.27006

    authors: Khodulev VI,Nechipurenko NI,Shcharbina NY

    更新日期:2020-09-01 00:00:00

  • Stroke-related effects on maximal dynamic hip flexor fatigability and functional implications.

    abstract:INTRODUCTION:Stroke-related changes in maximal dynamic hip flexor muscle fatigability may be more relevant functionally than isometric hip flexor fatigability. METHODS:Ten chronic stroke survivors performed 5 sets of 30 hip flexion maximal dynamic voluntary contractions (MDVC). A maximal isometric voluntary contractio...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24520

    authors: Kuhnen HR,Rybar MM,Onushko T,Doyel RE,Hunter SK,Schmit BD,Hyngstrom AS

    更新日期:2015-03-01 00:00:00

  • Analysis of force profile during a maximum voluntary isometric contraction task.

    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

    pub_type: 杂志文章

    doi:10.1002/mus.10564

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

    更新日期:2004-03-01 00:00:00

  • Electrical stimulation impairs early functional recovery and accentuates skeletal muscle atrophy after sciatic nerve crush injury in rats.

    abstract::Neuromuscular recovery after peripheral nerve lesion depends on the regeneration of severed axons that re-establish their functional connection with the denervated muscle. The aim of this study was to determine the effects of electrical stimulation (ES) on the neuromuscular recovery after nerve crush injury in rats. E...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21549

    authors: Gigo-Benato D,Russo TL,Geuna S,Domingues NR,Salvini TF,Parizotto NA

    更新日期:2010-05-01 00:00:00

  • Serum CK, calcium, magnesium, and oxidative phosphorylation in mdx mouse muscular dystrophy.

    abstract::Serum creatine kinase (CK) activity, calcium (Ca) and magnesium (Mg) contents of skeletal muscle and isolated mitochondria, as well as oxidative phosphorylation of X-linked muscular dystrophic (mdx) mice were compared with normal control animals at ages 5, 10, and 23 weeks. Serum CK is elevated in mdx mice at all ages...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880110809

    authors: Glesby MJ,Rosenmann E,Nylen EG,Wrogemann K

    更新日期:1988-08-01 00:00:00