Age of onset, sex, and cardiomyopathy as predictors of disability and survival in Friedreich's disease: a retrospective study on 119 patients.

abstract：OBJECTIVE:To determine the effect of clinical and radiologic disease activity on the rate of thinning of the ganglion cell/inner plexiform (GCIP) layer and the retinal nerve fiber layer in patients with multiple sclerosis (MS) using optical coherence tomography (OCT). METHODS:One hundred sixty-four patients with MS an...

journal_title：Neurology

authors： Ratchford JN,Saidha S,Sotirchos ES,Oh JA,Seigo MA,Eckstein C,Durbin MK,Oakley JD,Meyer SA,Conger A,Frohman TC,Newsome SD,Balcer LJ,Frohman EM,Calabresi PA

更新日期：2013-01-01 00:00:00

abstract：OBJECTIVE:To describe a new familial association of late-onset dementia, patchy leukoencephalopathy, intracerebral hemorrhages, bilateral occipital calcifications (BOC), and external carotid artery dysplasia (ECAD). METHODS:At age 62, the proband, who was of Spanish descent, had left temporal hemorrhage in a backgroun...

journal_title：Neurology

authors： Iglesias S,Chapon F,Baron JC

更新日期：2000-12-12 00:00:00

abstract：OBJECTIVES:To investigate the relationship between markers of vitamin B(12) status and brain volume loss per year over a 5-year period in an elderly population. METHODS:A prospective study of 107 community-dwelling volunteers aged 61 to 87 years without cognitive impairment at enrollment. Volunteers were assessed year...

journal_title：Neurology

authors： Vogiatzoglou A,Refsum H,Johnston C,Smith SM,Bradley KM,de Jager C,Budge MM,Smith AD

更新日期：2008-09-09 00:00:00

abstract：:A 35-year-old woman had cardiac arrest requiring treatment with a left ventricular assist device. After regaining consciousness, examination revealed upbeating nystagmus, otherwise normal bulbar function, left arm dysmetria, and numbness periorally and in areas she described as "a turtleneck of numbness with a T-shirt...

journal_title：Neurology

authors： Ryman DC,Ju YE

更新日期：2013-12-03 00:00:00

abstract：:The mechanism by which prednisone improves muscle strength and function in Duchenne muscular dystrophy (DMD) is unknown. We addressed the possibility that clinical improvement was related to prednisone-induced alterations in skeletal muscle dystrophin. We performed muscle biopsies on patients at the conclusion of a ra...

journal_title：Neurology

authors： Burrow KL,Coovert DD,Klein CJ,Bulman DE,Kissel JT,Rammohan KW,Burghes AH,Mendell JR

更新日期：1991-05-01 00:00:00

abstract：:A 30-year-old woman was thought to have Friedreich's disease because of progressive ataxia, dysarthria, and titubation from age 3 years. Her diet was normal, and there were neither symptoms nor laboratory evidence of liver disease or fat malabsorption. Serum vitamin E content and the ratio of serum vitamin E to total ...

journal_title：Neurology

authors： Stumpf DA,Sokol R,Bettis D,Neville H,Ringel S,Angelini C,Bell R

更新日期：1987-01-01 00:00:00

abstract：OBJECTIVES:To learn caregivers' preferences for the treatment of AD with a disease-slowing therapy, and to identify relationships between these preferences and the characteristics of caregivers and patients. METHODS:A structured interview with 40 caregivers of patients with AD. Preferences were measured for an AD-slow...

journal_title：Neurology

authors： Karlawish JH,Klocinski JL,Merz J,Clark CM,Asch DA

更新日期：2000-10-10 00:00:00

abstract：BACKGROUND:The prevalence of symptoms suggesting distal symmetric polyneuropathy (DSP) was reported to be higher among deployed veterans (DV) to the Persian Gulf in 1990-1991 than to control non-deployed veterans (NDV). The authors therefore compared the prevalence of DSP by direct examination of DV and their spouses t...

journal_title：Neurology

authors： Davis LE,Eisen SA,Murphy FM,Alpern R,Parks BJ,Blanchard M,Reda DJ,King MK,Mithen FA,Kang HK

更新日期：2004-09-28 00:00:00

abstract：OBJECTIVE:The relationship between relapses and long-term disability in multiple sclerosis (MS) remains to be fully elucidated. Current literature is conflicting and focused on early relapses. We investigated the effects of relapses at different stages on disability progression. METHODS:We conducted a retrospective re...

journal_title：Neurology

authors： Tremlett H,Yousefi M,Devonshire V,Rieckmann P,Zhao Y,UBC Neurologists.

更新日期：2009-11-17 00:00:00

abstract：BACKGROUND:Although gray matter (GM) atrophy is recognized as a common feature of multiple sclerosis (MS), conflicting results have been obtained in patients with clinically isolated syndromes (CIS). Methodologic and clinical constraints may take account for literature discrepancies. METHODS:A total of 105 patients pr...

journal_title：Neurology

authors： Calabrese M,Rinaldi F,Mattisi I,Bernardi V,Favaretto A,Perini P,Gallo P

更新日期：2011-07-19 00:00:00

abstract：:To study the anti-Schwann cell activity of serum from patients with Guillain-Barré syndrome (GBS), we used a 51Cr-release cytotoxicity assay. Specific release of 51Cr mediated by GBS serum did not differ from that with normals or disease controls. Heating sera from GBS patients or controls at 56 or 50 degrees C for 30...

journal_title：Neurology

authors： Lisak RP,Kuchmy D,Armati-Gulson PJ,Brown MJ,Sumner AJ

更新日期：1984-09-01 00:00:00

abstract：:The scanning electronmicroscope was used to study the development of anencephaly in an experimental model. Anencephaly was produced consistently, using vitamin A as the teratogen. Embryos destined to become anencephalic displayed failure of opposing sides of the rostral neural tube to fuse. Subsequently, the neural fo...

journal_title：Neurology

authors： Smith MT,Wood LR,Honig SR

更新日期：1982-09-01 00:00:00

abstract：:We reviewed retrospectively 114 prednisone dose reduction attempts in 63 myasthenic patients. Dose reduction was considered successful if a patient remained asymptomatic for more than 1 year on no prednisone or a stable low dose of prednisone. Successful dose reduction attempts were more common in patients taking azat...

journal_title：Neurology

authors： Miano MA,Bosley TM,Heiman-Patterson TD,Reed J,Sergott RC,Savino PJ,Schatz NJ

更新日期：1991-06-01 00:00:00

abstract：:This nested case-control study assessed the putative protective effect of human herpesvirus-8 (HHV-8) against HIV-1-related dementia (dementia). The HHV-8 seropositivity of 210 male age- and HIV disease stage-matched cases and controls was compared. The overall HHV-8 seropositivity of 66% was similar among demented HI...

journal_title：Neurology

authors： Polk S,Muñoz A,Sacktor NC,Jenkins FJ,Cohen B,Miller EN,Jacobson LP

更新日期：2002-09-24 00:00:00

abstract：OBJECTIVE:To evaluate the integrity of the dorsal striatal dopaminergic innervation in rigid and choreic Huntington disease (HD). BACKGROUND:Some patients with HD have an akinetic-rigid phenotype. It has been suggested that nigrostriatal in addition to striatal pathology is present in this subgroup. The authors sought...

journal_title：Neurology

authors： Bohnen NI,Koeppe RA,Meyer P,Ficaro E,Wernette K,Kilbourn MR,Kuhl DE,Frey KA,Albin RL

更新日期：2000-05-09 00:00:00

abstract：OBJECTIVE:To investigate the prevalence and nature of cognitive changes associated with sporadic amyotrophic lateral sclerosis (ALS) using a large scale study. METHODS:Consecutive patients with sporadic ALS (n = 279) underwent comprehensive neurologic evaluation and neuropsychological testing. Testing data from normal...

journal_title：Neurology

authors： Ringholz GM,Appel SH,Bradshaw M,Cooke NA,Mosnik DM,Schulz PE

更新日期：2005-08-23 00:00:00

abstract：OBJECTIVE:To determine whether correlates of white matter integrity can provide general as well as specific insight into the chronic effects of head injury coupled with depression symptom expression in professional football players. METHOD:We studied 26 retired National Football League (NFL) athletes who underwent dif...

journal_title：Neurology

authors： Strain J,Didehbani N,Cullum CM,Mansinghani S,Conover H,Kraut MA,Hart J Jr,Womack KB

更新日期：2013-07-02 00:00:00

abstract：:Over the last 50 years, there have been many improvements in therapy for individuals with neurologic disorders. Simultaneously, the complexity and cost of care have increased. The delivery of neurologic services is inefficient. The needs of both patients and neurologists are not being optimally addressed. Although gre...

journal_title：Neurology

authors： Ringel SP

更新日期：2015-05-19 00:00:00

abstract：:We report a Jewish family of Yemenite origin in which three brothers born from a consanguineous marriage had juvenile parkinsonism. The DNA samples from three affected brothers and one healthy brother were analyzed for the linkage to markers covering the autosomal-recessive juvenile parkinsonism (AR-JP) locus. A perfe...

journal_title：Neurology

authors： Nisipeanu P,Inzelberg R,Blumen SC,Carasso RL,Hattori N,Matsumine H,Mizuno Y

更新日期：1999-10-22 00:00:00

abstract：:We report a woman with a chronic inflammatory myopathy who developed muscle stiffness and dystonic postures of both upper extremities. Electromyography demonstrated that involuntary complex repetitive discharges of muscle caused the posture. Rare cases of dystonic posturing may be of peripheral, and not central system...

journal_title：Neurology

authors： Preston DC,Finkleman RS,Munsat TL

更新日期：1996-01-01 00:00:00

abstract：:We studied the scalp distribution of auditory event-related potentials (P300), using simultaneously a cephalic (linked ears) and a noncephalic (balanced sternovertebra) reference. The recordings with noncephalic reference showed that infrequent, attended auditory stimuli evoke a negative bitemporal component (N300) as...

journal_title：Neurology

authors： Neshige R,Lüders H

更新日期：1988-11-01 00:00:00

abstract：:The childhood type fo dermatomyositis, which occurs in children and young adults, shows a specific constellation of pathologic changes in muscle. Capillary necrosis leads to capillary loss, generally starting on the periphery of muscle fascicles. Electron microscopy discloses undulating tubules in endothelial cells, l...

journal_title：Neurology

authors： Carpenter S,Karpati G,Rothman S,Watters G

更新日期：1976-10-01 00:00:00

abstract：:Cognitive decline is recognized as a prevalent and debilitating symptom of multiple sclerosis (MS), especially deficits in episodic memory and processing speed. The field aims to (1) incorporate cognitive assessment into standard clinical care and clinical trials, (2) utilize state-of-the-art neuroimaging to more thor...

journal_title：Neurology

authors： Sumowski JF,Benedict R,Enzinger C,Filippi M,Geurts JJ,Hamalainen P,Hulst H,Inglese M,Leavitt VM,Rocca MA,Rosti-Otajarvi EM,Rao S

更新日期：2018-02-06 00:00:00

abstract：:We measured oxygen consumption in the exercising lower limb by using noninvasive tissue oximetry with the near-infrared spectra of hemoglobin in the quadriceps muscle during bicycle ergometer exercise in four normal controls and three patients with chronic progressive external ophthalmoplegia (CPEO) as well as one pat...

journal_title：Neurology

authors： Abe K,Matsuo Y,Kadekawa J,Inoue S,Yanagihara T

更新日期：1997-09-01 00:00:00

abstract：OBJECTIVE:To evaluate the presence of prodromal markers of Parkinson disease (PD) in patients with longstanding idiopathic REM sleep behavior disorder (IRBD), a small subgroup of individuals with IRBD with long-term follow-up thought not to be at risk of developing PD. METHODS:Demographic, clinical, and neuroimaging m...

journal_title：Neurology

authors： Iranzo A,Stefani A,Serradell M,Martí MJ,Lomeña F,Mahlknecht P,Stockner H,Gaig C,Fernández-Arcos A,Poewe W,Tolosa E,Högl B,Santamaria J,SINBAR (Sleep Innsbruck Barcelona) group.

更新日期：2017-07-18 00:00:00

abstract：OBJECTIVE:The authors investigated chronic cerebellar reorganization following unilateral cortical lesions in children and adults using PET to measure benzodiazepine receptor (BZR) binding with [11C]flumazenil (FMZ) and glucose metabolism with 2-deoxy-2[18F]fluoro-D-glucose (FDG). BACKGROUND:Crossed cerebellar diaschi...

journal_title：Neurology

authors： Niimura K,Chugani DC,Muzik O,Chugani HT

更新日期：1999-03-10 00:00:00

abstract：OBJECTIVE:The MS Functional Composite (MSFC), a recently developed outcome measure for clinical trials, was applied to 240 patients with MS to explore its utility in different subgroups of MS and for comparison with the Expanded Disability Status Scale (EDSS). METHOD:Three clinical dimensions were examined: arm/hand f...

journal_title：Neurology

authors： Kalkers NF,de Groot V,Lazeron RH,Killestein J,Adèr HJ,Barkhof F,Lankhorst GJ,Polman CH

更新日期：2000-03-28 00:00:00

abstract：:Rare reflex epilepsies are a clinical curiosity but may also give insight into neurocognitive processes. The authors present a patient whose seizures were precipitated by drawing but not by writing. This dissociation complements previous reports on selective impairment of writing. EEG revealed a focus in the right fro...

journal_title：Neurology

authors： Kho KH,van den Bergh WM,Spetgens WP,Leijten FS

更新日期：2006-03-14 00:00:00

abstract：OBJECTIVE:To determine the role of circulating microvesicles (MV) on long-term cardiovascular outcomes after stroke, we measured them in patients with first-ever stroke with a 3-year follow-up. METHODS:In the Prospective Cohort With Incident Stroke Berlin (PROSCIS-B), patients with first-ever ischemic stroke were foll...

journal_title：Neurology

authors： Huo S,Kränkel N,Nave AH,Sperber PS,Rohmann JL,Piper SK,Heuschmann P,Landmesser U,Endres M,Siegerink B,Liman TGG

更新日期：2020-11-12 00:00:00

abstract：:Factors affecting osmotic fragility were studied in red blood cells of patients with Duchenne muscular dystrophy. The mean osmotic fragility (MOF), operationally defined as the NaCl concentration for 50% hemolysis, was found to be higher by 3.63 +/- 0.51 mM in Duchenne cells than in normal cells having an MOF of 60.1 ...

journal_title：Neurology

authors： Kim HD,Luthra MG,Watts RP,Stern LZ

更新日期：1980-07-01 00:00:00