Abstract:
OBJECTIVE:To perform a double-blind, randomized study comparing efficacy and safety of daily and weekend prednisone in boys with Duchenne muscular dystrophy (DMD). METHODS:A total of 64 boys with DMD who were between 4 and 10 years of age were randomized at 1 of 12 centers of the Cooperative International Neuromuscular Research Group. Efficacy and safety of 2 prednisone schedules (daily 0.75 mg/kg/day and weekend 10 mg/kg/wk) were evaluated over 12 months. RESULTS:Equivalence was met for weekend and daily dosing of prednisone for the primary outcomes of quantitative muscle testing (QMT) arm score and QMT leg score. Secondary strength scores for QMT elbow flexors also showed equivalence between the 2 treatment groups. Overall side effect profiles of height and weight, bone density, cataract formation, blood pressure, and behavior, analyzed at 12 months, did not differ between weekend and daily dosing of prednisone. CONCLUSIONS:Weekend dosing of prednisone is equally beneficial to the standard daily dosing of prednisone. Analysis of side effect profiles demonstrated overall tolerability of both dosing regimens. CLASSIFICATION OF EVIDENCE:This study provides Class I evidence that weekend prednisone dosing is as safe and effective as daily prednisone in preserving muscle strength and preventing body mass index increases in boys with DMD over a 12-month period.
journal_name
Neurologyjournal_title
Neurologyauthors
Escolar DM,Hache LP,Clemens PR,Cnaan A,McDonald CM,Viswanathan V,Kornberg AJ,Bertorini TE,Nevo Y,Lotze T,Pestronk A,Ryan MM,Monasterio E,Day JW,Zimmerman A,Arrieta A,Henricson E,Mayhew J,Florence J,Hu F,Connolly Adoi
10.1212/WNL.0b013e318227b164subject
Has Abstractpub_date
2011-08-02 00:00:00pages
444-52issue
5eissn
0028-3878issn
1526-632Xpii
WNL.0b013e318227b164journal_volume
77pub_type
杂志文章,随机对照试验相关文献
NEUROLOGY文献大全abstract:OBJECTIVE:To determine the contribution of sequence variations in PHOX2B to sudden unexpected death in epilepsy (SUDEP). METHODS:Patients who died of SUDEP were identified in 2 major Australian cohorts, the Epilepsy Genetics research program in Melbourne and postmortem cases at the Department of Forensic Medicine in S...
journal_title:Neurology
pub_type: 杂志文章,多中心研究
doi:10.1212/WNL.0000000000000781
更新日期:2014-09-09 00:00:00
abstract::We evaluated the ability of nurse clinicians to assess parkinsonian signs in older persons with a modified version of the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS). After completing a structured training protocol, three nurse clinicians and a neurologist with expertise in movement disorders...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.49.6.1580
更新日期:1997-12-01 00:00:00
abstract::Brainstem auditory evoked responses were recorded after acute asphyxia in 126 infants, ages birth through 18 months. Of these, 21 had markedly abnormal amplitude ratios, and all infants with abnormal amplitude ratios had severe neurologic handicaps. An abnormal amplitude ratio predicts long-term neurologic sequelae of...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.31.11.1429
更新日期:1981-11-01 00:00:00
abstract:BACKGROUND:The 1875 publications of Erb and Westphal brought widespread attention to the percussion of muscle stretch reflexes. Although the maneuver may have been observed before 1875, there is little concrete evidence of this. OBJECTIVES:To bring to light an early report of percussion eliciting the muscle stretch re...
journal_title:Neurology
pub_type: 传,历史文章,杂志文章
doi:10.1212/01.wnl.0000305968.03898.fe
更新日期:2008-03-18 00:00:00
abstract:BACKGROUND:In a recent study, the authors found that blinks in healthy volunteers always triggered ocular torsion quick phases during dynamic roll movements of the head. On the basis of this observation, they hypothesized that blinks in patients with a vestibular tone imbalance would also trigger torsional quick phases...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000141144.02666.8c
更新日期:2004-10-12 00:00:00
abstract::Using an in vitro blood-nerve barrier (BNB) model, the authors tested the effect of various monoclonal antiganglioside antibodies on BNB function. Only anti-GM1 antibody significantly facilitated BNB leakage in a concentration-dependent, complement-independent manner. This study provided evidence that anti-GM1 antibod...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.55.4.585
更新日期:2000-08-22 00:00:00
abstract::This review summarizes evidence, primarily from recent human studies, indirectly supporting a novel hypothesis: that the assessment of healthy individuals' responses to standardized noxious stimuli in a controlled laboratory environment has important implications for the later risk of developing a broad spectrum of ch...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/01.wnl.0000171862.17301.84
更新日期:2005-08-09 00:00:00
abstract::Many studies of monozygotic (MZ) twins have revealed evidence of genetic influences on intellectual functions and their derangement in certain neurologic and psychiatric diseases afflicting the forebrain. Relatively little is known about genetic influences on the size and shape of the human forebrain and its gross mor...
journal_title:Neurology
pub_type: 临床试验,杂志文章
doi:10.1212/wnl.50.5.1246
更新日期:1998-05-01 00:00:00
abstract::We investigated the possible role of potassium deficiency, a recognized cause of myopathy, in the pathogenesis of experimental acute alcoholic myopathy (EAM) in the rat. Alcohol-treated animals receiving the same dietary potassium as controls developed mild hypokalemia; however, muscle potassium was preserved or eleva...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.4.529
更新日期:1984-04-01 00:00:00
abstract::Children with epilepsy have more behavioral and cognitive problems than children with other chronic illnesses and children in the general population. Risk factors are multiple, probably involving a combination of neurological, seizure, family, and child variables. Problems with attention and symptoms of depression occ...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:
更新日期:1999-01-01 00:00:00
abstract:OBJECTIVE:To establish a variant of sporadic prion disease as the sporadic form of fatal familial insomnia (FFI). BACKGROUND:FFI is a recently described prion disease characterized clinically by severe sleep impairment, dysautonomia, and motor signs, and pathologically by atrophy of thalamic nuclei, especially the med...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.52.9.1757
更新日期:1999-06-10 00:00:00
abstract:OBJECTIVES:We evaluated the frequency and predictive value of ocular fundus abnormalities among patients who presented to the emergency department (ED) with focal neurologic deficits to determine the utility of these findings in the evaluation of patients with suspected TIA and stroke. METHODS:In this cross-sectional ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000001759
更新日期:2015-07-21 00:00:00
abstract::We transplanted autologous adrenal medullary tissue into the caudate nucleus of 3 patients with advanced Parkinson's disease. The 1st patient, a 59-year-old man with parkinsonian symptoms for 15 years, had mild improvement in his motor functioning after the operation. However, his postoperative course was characterize...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.39.9.1227
更新日期:1989-09-01 00:00:00
abstract::Since cytomegalovirus (CMV) has been implicated in the pathogenesis of Rasmussen's syndrome, we treated four patients with ganciclovir, a potent anti-CMV drug. A 7-year-old girl with seizures escalating to 60/day over 3 months despite triple antiepileptic drug therapy became seizure-free 5 days after initiation of tre...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.47.4.925
更新日期:1996-10-01 00:00:00
abstract::Serum from 35 cases of naturally occurring acquired canine myasthenia gravis (MG) were assayed for patterns of autoantibody specificities against canine acetylcholine receptor (AChR) using monoclonal antibodies (mAbs) and antiserum against defined regions of the AChR as competitive inhibitors of autoantibody binding. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.38.9.1417
更新日期:1988-09-01 00:00:00
abstract:OBJECTIVE:To identify the genetic cause of a familial form of late-onset action myoclonus in 2 unrelated patients. Both probands had 2 siblings displaying a similar disorder. Extensive laboratory examinations, including biochemical assessment for urine sialic acid in the 2 probands, were negative. METHODS:Exome sequen...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/WNL.0000000000000482
更新日期:2014-06-03 00:00:00
abstract:OBJECTIVE:Learning and memory impairments are prevalent among persons with multiple sclerosis (MS); however, such deficits are only weakly associated with MS disease severity (brain atrophy). The cognitive reserve hypothesis states that greater lifetime intellectual enrichment lessens the negative impact of brain disea...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181e396be
更新日期:2010-06-15 00:00:00
abstract:PURPOSE:To assess the extent of demyelination in cervical spondylotic myelopathy (CSM) using myelin water imaging (MWI) and electrophysiologic techniques. METHODS:Somatosensory evoked potentials (SSEPs) and MWI were acquired in 14 patients with CSM and 18 age-matched healthy controls. MWI was performed on a 3.0T whole...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000004197
更新日期:2017-08-08 00:00:00
abstract::Visual hallucinations (VHs) occur frequently in Parkinson's disease (PD). VHs occur more frequently in elderly patients with longer duration of illness, cognitive impairment, and sleep disturbances. The relationship between the use of antiparkinsonian drugs and VHs is complicated, but most drugs used to treat parkinso...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.63.8_suppl_3.s28
更新日期:2004-10-26 00:00:00
abstract:BACKGROUND:Only a small percentage of patients with acute stroke are treated with recombinant tissue plasminogen activator (rt-PA). OBJECTIVE:To investigate why patients with right-hemisphere strokes seem at high risk of not receiving rt-PA. METHODS:This study includes two phases. Phase 1: the authors compared demogr...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000167608.94237.aa
更新日期:2005-07-12 00:00:00
abstract::Creutzfeldt-Jakob disease was studied among Libyan-born Israelis, in whom the disease appears with unusual frequency. Interviews with relatives of deceased victims revealed statistically significant clustering within families. The results suggest either a common source of exposure or a genetic influence on susceptibil...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.29.2.225
更新日期:1979-02-01 00:00:00
abstract::Functional brain imaging has been a standard tool in the basic and clinical neurosciences for at least 2 decades, and structural brain imaging can claim an even longer track record of successful use for diagnosis of neurodegenerative disease. It is instructive to recall the ways in which brain imaging has been used to...
journal_title:Neurology
pub_type: 评论,社论
doi:10.1212/WNL.0b013e31827b9305
更新日期:2013-01-08 00:00:00
abstract::We performed a prospective clinical and electrophysiologic study of common peroneal (CP) neuropathy to evaluate the extent of involvement of the muscles and cutaneous areas supplied by this nerve. In 22 patients, seven had more weakness clinically in muscles innervated by the deep peroneal nerve than in those innervat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.41.7.1029
更新日期:1991-07-01 00:00:00
abstract:OBJECTIVE:To determine whether there are distinct thalamic regions statistically associated with either contraversive or ipsiversive disturbance of verticality perception measured by subjective visual vertical (SVV). METHODS:We used modern statistical lesion behavior mapping on a sample of 37 stroke patients with isol...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000002238
更新日期:2016-01-12 00:00:00
abstract:OBJECTIVE:To evaluate relationships among cognitive, behavioral, and psychiatric/psychosocial measures assessed in a multicenter cohort of patients with amyotrophic lateral sclerosis (ALS). METHODS:Recently diagnosed patients with definite or probable ALS diagnosis were administered 7 standardized psychiatric/psychoso...
journal_title:Neurology
pub_type: 杂志文章,多中心研究
doi:10.1212/WNL.0000000000003035
更新日期:2016-09-27 00:00:00
abstract::A number of high-quality studies have recently been published that examine the association between neuropathic pain and health-related quality of life (HRQoL). The current review identified 52 such studies in patients with six neuropathic pain conditions associated with lesions of either the peripheral (postsurgical n...
journal_title:Neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1212/01.wnl.0000259085.61898.9e
更新日期:2007-04-10 00:00:00
abstract:OBJECTIVE:In the North Dublin Population Stroke Study, we investigated the risk of recurrent stroke within the 14-day time window recommended for endarterectomy. METHODS:In a population-based prospective cohort study, all ischemic stroke patients were identified over 1 year and categorized into those with (CS-positive...
journal_title:Neurology
pub_type: 杂志文章,随机对照试验
doi:10.1212/WNL.0b013e31822b00cf
更新日期:2011-08-23 00:00:00
abstract:OBJECTIVES:This study examines the anatomical correlates of naming vs recognizing faces using a novel measure that utilizes culturally relevant and age-appropriate items, the Northwestern University Famous Faces (NUFFACE) Test, in primary progressive aphasia (PPA), a syndrome characterized by progressive language defic...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3182a08f83
更新日期:2013-08-13 00:00:00
abstract:BACKGROUND:The head impulse test (HIT) is a useful bedside test to identify peripheral vestibular deficits. However, such a deficit of the vestibulo-ocular reflex (VOR) may not be diagnosed because corrective saccades cannot always be detected by simple observation. The scleral search coil technique is the gold standar...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181bacf85
更新日期:2009-10-06 00:00:00
abstract:BACKGROUND:Attempts at cell-based dopamine replacement therapy in Parkinson disease (PD) have included surgical implantation of adrenal medullary, fetal mesencephalic, and cultured human mesencephalic tissue grafts. Trials involving putamenal implantation of human retinal pigment epithelial (RPE) cells in PD have also ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181bbff1c
更新日期:2009-10-06 00:00:00