Spastic paraplegia, ataxia, mental retardation (SPAR): a novel genetic disorder.

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：OBJECTIVE:To improve clinical recognition and provide research diagnostic criteria for three clinical syndromes associated with frontotemporal lobar degeneration. METHODS:Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed b...

journal_title：Neurology

authors： Neary D,Snowden JS,Gustafson L,Passant U,Stuss D,Black S,Freedman M,Kertesz A,Robert PH,Albert M,Boone K,Miller BL,Cummings J,Benson DF

更新日期：1998-12-01 00:00:00

abstract：:Symptoms in patients with demyelinating disorders are associated with 3 important conduction abnormalities: complete conduction block, rate-dependent conduction block, and slowed impulse conduction. The recent observation that the electrogenic Na/K pump causes the nerve membrane hyperpolarization responsible for rate-...

journal_title：Neurology

authors： Kaji R,Sumner AJ

更新日期：1989-10-01 00:00:00

abstract：:Childhood absence epilepsy (CAE) is defined by absence seizures in a normally developing child, with onset between 4 and 10 years of age.(1) Typical absence seizures consist of behavioral arrest with or without automatisms, usually last 30-60 seconds, and demonstrate a characteristic 3-Hz generalized spike-wave (GSW) ...

journal_title：Neurology

authors： Bernson-Leung ME,Mazumdar M

更新日期：2014-05-06 00:00:00

abstract：:Antibodies to myelin-associated glycoprotein (MAG) are found in patients with both monoclonal gammopathy and sensorimotor peripheral neuropathy but almost never in patients with amyotrophic lateral sclerosis (ALS). Ninety percent of patients with anti-MAG activity also have antibodies to sulfated glucuronic acid parag...

journal_title：Neurology

authors： Rowland LP,Sherman WL,Hays AP,Lange DJ,Latov N,Trojaborg W,Younger DS

更新日期：1995-04-01 00:00:00

abstract：:People with AD have deficient contrast sensitivity and impaired face discrimination. The authors presented photographs of unfamiliar faces of three different sizes to enhance the low, middle, or high facial frequency information (cycles per face). Patients with AD demonstrated normal discrimination of small faces only...

journal_title：Neurology

authors： Cronin-Golomb A,Cronin-Golomb M,Dunne TE,Brown AC,Jain K,Cipolloni PB,Auerbach SH

更新日期：2000-06-27 00:00:00

abstract：:A 20-year-old woman using an oral diet aid preparation containing phenylpropanolamine presented with an intracerebral hemorrhage and angiographic evidence of cerebral vasculitis. Gas chromatography demonstrated phenylpropanolamine in the urine. Even when used within recommended guidelines, phenylpropanolamine usage sh...

journal_title：Neurology

authors： Fallis RJ,Fisher M

更新日期：1985-03-01 00:00:00

abstract：OBJECTIVE:Development of an easy to administer, low-cost test of vestibular function. METHODS:Members of the NIH Toolbox Sensory Domain Vestibular, Vision, and Motor subdomain teams collaborated to identify 2 tests: 1) Dynamic Visual Acuity (DVA), and 2) the Balance Accelerometry Measure (BAM). Extensive work was comp...

journal_title：Neurology

authors： Rine RM,Schubert MC,Whitney SL,Roberts D,Redfern MS,Musolino MC,Roche JL,Steed DP,Corbin B,Lin CC,Marchetti GF,Beaumont J,Carey JP,Shepard NP,Jacobson GP,Wrisley DM,Hoffman HJ,Furman G,Slotkin J

更新日期：2013-03-12 00:00:00

abstract：OBJECTIVES:To compare the degrees to which 8 domains of health are affected across types of cerebrovascular events and to identify factors associated with domain scores in different event types. METHODS:This was an observational cohort study of 2,181 patients with ischemic stroke, intracerebral hemorrhage (ICH), subar...

journal_title：Neurology

authors： Katzan IL,Schuster A,Newey C,Uchino K,Lapin B

更新日期：2018-12-04 00:00:00

abstract：OBJECTIVES:To correlate the two types of early ischemic change on noncontrast CT (NCCT) (parenchymal hypoattenuation [PH] and isolated focal swelling [IFS]) with concurrent assessment of cerebral perfusion and to compare their rates of progression to infarction. METHODS:We assessed cortical regions on NCCT for early i...

journal_title：Neurology

authors： Parsons MW,Pepper EM,Bateman GA,Wang Y,Levi CR

更新日期：2007-03-06 00:00:00

abstract：:This study was undertaken to determine the amount and histologic appearance of the persistent thymic tissue removed post mortem from 20 patients over 60 years of age with myasthenia gravis. One patient died several days after thymectomy. No recognizable thymic tissue on gross examination was seen in any patient. On mi...

journal_title：Neurology

authors： Perlo VP,Arnason B,Castleman B

更新日期：1975-03-01 00:00:00

abstract：OBJECTIVE:To assess the recovery process in patients after an acute optic neuritis (ON) attack, comparing static and dynamic visual functions. METHODS:In this prospective controlled study, 21 patients with unilateral, first-ever ON were followed over the course of 1 year. Standard visual tests, visual evoked potential...

journal_title：Neurology

authors： Raz N,Dotan S,Benoliel T,Chokron S,Ben-Hur T,Levin N

更新日期：2011-06-14 00:00:00

abstract：:Recurrent episodes of oscillopsia, rotational vertigo, and postural imbalance were elicited and modulated by changing the horizontal head positions of a patient with an arachnoid cyst in the right cerebellopontine angle that distorted the vestibulocochlear nerve. Oculomotor analysis revealed two different types of att...

journal_title：Neurology

authors： Arbusow V,Strupp M,Dieterich M,Jäger L,Hischa A,Schulz P,Brandt T

更新日期：1998-11-01 00:00:00

abstract：:We studied the effects of intravenous immunoglobulin (IVIg) in 12 patients with motor neuron syndromes associated with high titers of anti-GM1 antibodies. Five of the patients had conduction blocks. The study design was a double-blind, placebo-controlled, crossover trial with IVIg (0.4 g/kg body weight per day injecte...

journal_title：Neurology

authors： Azulay JP,Blin O,Pouget J,Boucraut J,Billé-Turc F,Carles G,Serratrice G

更新日期：1994-03-01 00:00:00

abstract：:In children, seizures associated with status epilepticus (SE) include a number of types that are age-related. These types of seizures are not associated with SE in older patients. Likewise, etiologies of SE in children are also unique to this patient population, in particular those responsible for SE in the neonate. C...

journal_title：Neurology

authors： Lockman LA

更新日期：1990-05-01 00:00:00

abstract：:We performed an open-label study testing the effects of GM1 ganglioside on 10 Parkinson's disease (PD) patients. Patients received 1,000 mg of GM1 by IV infusion after the last of three baseline functional assessments. Patients then self-administered GM1 at a dose of 200 mg/d, by subcutaneous injection, for 18 weeks. ...

journal_title：Neurology

authors： Schneider JS,Roeltgen DP,Rothblat DS,Chapas-Crilly J,Seraydarian L,Rao J

更新日期：1995-06-01 00:00:00

abstract：:We investigated 185 families with ALS for evidence of anticipation and mitochondrial inheritance. Although initial analysis demonstrated significant anticipation of age at death between generations in patients with familial ALS, further analysis demonstrated features of regression to the mean, suggesting that the perc...

journal_title：Neurology

authors： Bradley M,Bradley L,de Belleroche J,Orrell RW

更新日期：2005-05-10 00:00:00

abstract：OBJECTIVE:To determine whether longitudinal cognitive decline is associated with increased risk of sleep disturbance in older, nondemented, community-dwelling women. METHODS:We studied 2,474 women (mean age 68.9 years) who were part of a prospective study started in 1986; women with baseline or follow-up evidence of p...

journal_title：Neurology

authors： Yaffe K,Blackwell T,Barnes DE,Ancoli-Israel S,Stone KL,Study of Osteoporotic Fractures Group.

更新日期：2007-07-17 00:00:00

abstract：OBJECTIVE:To prospectively investigate the relationships between serum tissue inhibitor metalloproteinase-1 (TIMP-1) and clinical outcomes in patients with acute ischemic stroke. METHODS:We derived data from the China Antihypertensive Trial in Acute Ischemic Stroke. Baseline serum TIMP-1 concentrations were measured i...

journal_title：Neurology

authors： Zhong C,Wang G,Xu T,Zhu Z,Guo D,Zheng X,Wang A,Bu X,Peng H,Chen J,Xu T,Peng Y,Li Q,Ju Z,Geng D,He J,Zhang Y

更新日期：2019-10-29 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：: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：OBJECTIVE:To describe the clinical features and outcome of anti-NMDA receptor (NMDAR) encephalitis in patients ≥45 years old. METHOD:Observational cohort study. RESULTS:In a cohort of 661 patients with anti-NMDAR encephalitis, we identified 31 patients ≥45 years old. Compared with younger adults (18-44 years), older ...

journal_title：Neurology

authors： Titulaer MJ,McCracken L,Gabilondo I,Iizuka T,Kawachi I,Bataller L,Torrents A,Rosenfeld MR,Balice-Gordon R,Graus F,Dalmau J

更新日期：2013-09-17 00:00:00

abstract：OBJECTIVE:(1)H-MRS studies have shown abnormalities in brain levels of myo-inositol (mI) and N-acetyl aspartate (NAA) in AD, but the relation of these abnormalities with dementia severity was not examined. The authors sought to determine whether altered brain levels of mI and other metabolites occur in mild AD and whet...

journal_title：Neurology

authors： Huang W,Alexander GE,Chang L,Shetty HU,Krasuski JS,Rapoport SI,Schapiro MB

更新日期：2001-08-28 00:00:00

abstract：:We describe the clinical, pathologic, and ultrastructural findings in a case of juvenile onset neuronal ceroid-lipofuscinosis without visual symptoms or retinal abnormalities. The histochemical and ultrastructural characteristics of the neuronal lipopigment were similar to those in typical cases of neuronal ceroid-lip...

journal_title：Neurology

authors： Greenwood RS,Nelson JS

更新日期：1978-07-01 00:00:00

abstract：OBJECTIVE:To clinically characterize the temporal relationship between dyskinesia and the antiparkinsonian response when dyskinesia first emerges during long-term levodopa therapy and to determine if it is consistent with the hypothesized mechanism by which dyskinesia develops. METHODS:Dyskinesia and the antiparkinson...

journal_title：Neurology

authors： Nutt JG,Chung KA,Holford NH

更新日期：2010-04-13 00:00:00

abstract：BACKGROUND:Patients with progressive supranuclear palsy (PSP) fall frequently, beginning early in the course of their disease. Abnormal vestibulospinal reflexes are suspected, but the angular vestibulo-ocular reflex, which is mediated by the labyrinthine semicircular canals, survives late into the course of the disease...

journal_title：Neurology

authors： Liao K,Wagner J,Joshi A,Estrovich I,Walker MF,Strupp M,Leigh RJ

更新日期：2008-03-04 00:00:00

abstract：:The authors retrospectively reviewed the efficacy of mycophenolate mofetil (MMF; mean dose, 2 g/day given for an average of 14 months) in 21 patients with immune demyelinating polyneuropathy. There were no significant differences in the median strength, sensory, or Rankin scores before and after treatment. In patients...

journal_title：Neurology

authors： Gorson KC,Amato AA,Ropper AH

更新日期：2004-08-24 00:00:00

abstract：OBJECTIVE:To learn to which portions of a line normal subjects would attend when watching this line actually moving or when perceiving movement even in the absence of actual movement, an illusory movement. METHODS:Twenty normal subjects watched a computer monitor on which either lines or dots moved to the right or lef...

journal_title：Neurology

authors： Jeong Y,Lee BH,Ahn HJ,Park KC,Heilman KM,Na DL

更新日期：2004-04-27 00:00:00

abstract：:We investigated 26 right-handed patients who had aphasia caused by a single infarct in the territory of the left middle cerebral artery (MCA) with cranial CT, positron emission tomography, and a standard language test battery including the Token test. The patients represented an unselected sample of various infarct lo...

journal_title：Neurology

authors： Karbe H,Herholz K,Szelies B,Pawlik G,Wienhard K,Heiss WD

更新日期：1989-08-01 00:00:00

abstract：:We report a patient with striatonigral degeneration in whom T2-weighted MRI imaging revealed low signal and atrophy in the putamen. Neuropathologic studies confirmed putaminal atrophy and iron deposition in the MRI low signal regions. ...

journal_title：Neurology

authors： O'Brien C,Sung JH,McGeachie RE,Lee MC

更新日期：1990-04-01 00:00:00