Type 3 (adult) GM1 gangliosidosis: case report.

abstract：OBJECTIVE:To study the safety of off-label IV thrombolysis in patients with very severe stroke (NIH Stroke Scale [NIHSS] scores >25) compared with severe stroke (NIHSS scores 15-25), where treatment is within European regulations. METHODS:Data were analyzed from 57,247 patients with acute ischemic stroke receiving IV ...

journal_title：Neurology

authors： Mazya MV,Lees KR,Collas D,Rand VM,Mikulik R,Toni D,Wahlgren N,Ahmed N

更新日期：2015-12-15 00:00:00

abstract：:Cost-effective approaches for arriving at correct diagnoses should be used. Herein, we compare three approaches for the differential diagnosis of a common neurologic syndrome, peripheral neuropathy. In the "shotgun" approach, a standard battery of hematologic, biochemical, serologic, antibody, enzyme, molecular geneti...

journal_title：Neurology

authors： Dyck PJ,Dyck PJ,Grant IA,Fealey RD

更新日期：1996-07-01 00:00:00

abstract：:The authors report two patients with suspected brain death who required confirmatory tests other than clinical examination because of prolonged barbiturate administration for intracranial hypertension. Absence of intracranial blood flow was documented on CT angiography and confirmed by CT perfusion images. Cerebral an...

journal_title：Neurology

authors： Qureshi AI,Kirmani JF,Xavier AR,Siddiqui AM

更新日期：2004-02-24 00:00:00

abstract：:Original reports on temporal arteritis and polymyalgia rheumatica were reviewed before and after the introduction of steroid therapy to prevent blindness in temporal arteritis. In some cases, the original data were reworked. There is evidence that both diseases have become more benign. As a result, the perceived risk ...

journal_title：Neurology

authors： Turnbull J

更新日期：1996-04-01 00:00:00

abstract：:The author describes a distinct clinical syndrome in six patients with autonomic failure who manifested habitual, voluntary, transiently suppressible, yet irresistible leg movements occurring only in the sitting position. Keeping the legs still brought on vague symptoms of fatigue, lightheadedness, or apprehension. Re...

journal_title：Neurology

authors： Cheshire WP Jr

更新日期：2000-12-26 00:00:00

abstract：BACKGROUND:Previous work has suggested that functional reorganization of cortical motor areas might have a role in limiting the motor deficits in patients with MS. OBJECTIVE:To test whether movement-associated cortical changes in MS might extend beyond the "classic" motor areas and involve sites for multimodal integra...

journal_title：Neurology

authors： Rocca MA,Matthews PM,Caputo D,Ghezzi A,Falini A,Scotti G,Comi G,Filippi M

更新日期：2002-03-26 00:00:00

abstract：:Whether autoimmune mechanisms play a role in the pathogenesis of inclusion body myositis (IBM) is unknown. Human leukocyte antigen (HLA) analysis in 52 patients, including 17 with autoimmune disorders (AIDs), showed that patients were more likely to have antigens from the autoimmune-prone HLA-B8-DR3 ancestral haplotyp...

journal_title：Neurology

authors： Badrising UA,Schreuder GM,Giphart MJ,Geleijns K,Verschuuren JJ,Wintzen AR,Maat-Schieman ML,van Doorn P,van Engelen BG,Faber CG,Hoogendijk JE,de Jager AE,Koehler PJ,de Visser M,van Duinen SG,Dutch IBM Study Group.

更新日期：2004-12-28 00:00:00

abstract：BACKGROUND:Cerebrovascular disease (CVD) may contribute to mild cognitive impairment (MCI). We sought to determine the relation of white matter hyperintensity (WMH) volume and infarcts in brain MRI to MCI in a community-based sample. METHODS:A total of 679 elderly persons without dementia underwent brain MRI. WMH and ...

journal_title：Neurology

authors： Luchsinger JA,Brickman AM,Reitz C,Cho SJ,Schupf N,Manly JJ,Tang MX,Small SA,Mayeux R,DeCarli C,Brown TR

更新日期：2009-08-11 00:00:00

abstract：BACKGROUND:No data exist on whether the syndrome of amnestic mild cognitive impairment occurs in the oldest old, or if the relationships for functional status and neuropsychometric performance based on clinical diagnosis hold true in this age group. DESIGN/METHODS:The authors performed comprehensive neurologic evaluat...

journal_title：Neurology

authors： Boeve B,McCormick J,Smith G,Ferman T,Rummans T,Carpenter T,Ivnik R,Kokmen E,Tangalos E,Edland S,Knopman D,Petersen R

更新日期：2003-02-11 00:00:00

abstract：BACKGROUND AND OBJECTIVE:Human herpesvirus 8 (HHV-8) has been found in association with Kaposi's sarcomas in human immunodeficiency virus (HIV)-positive and -negative patients, primary effusion lymphomas (PELs), multicentric Castleman's disease, and multiple myeloma. The PELs share several features with acquired immuno...

journal_title：Neurology

authors： Corboy JR,Garl PJ,Kleinschmidt-DeMasters BK

更新日期：1998-02-01 00:00:00

abstract：OBJECTIVE:To examine neurofilament (Nf) concentrations according to symptom onset and clinical diagnostic certainty categories of amyotrophic lateral sclerosis (ALS). METHODS:We measured Nf light chain (NfL) and phosphorylated Nf heavy chain (pNfH) CSF and NfL serum levels in patients with ALS with first symptom onset...

journal_title：Neurology

authors： Feneberg E,Oeckl P,Steinacker P,Verde F,Barro C,Van Damme P,Gray E,Grosskreutz J,Jardel C,Kuhle J,Koerner S,Lamari F,Amador MDM,Mayer B,Morelli C,Muckova P,Petri S,Poesen K,Raaphorst J,Salachas F,Silani V,Stuben

更新日期：2018-01-02 00:00:00

abstract：:Although referrals for narcolepsy are increasing, physicians continue to confront obstacles in diagnosing and treating this condition. Patients with narcolepsy also face challenges at work, at school, and at home. One of the more disturbing problems is the lack of public awareness of the disorder and its potential the...

journal_title：Neurology

authors： Douglas NJ

更新日期：1998-02-01 00:00:00

abstract：OBJECTIVE:To evaluate the effect of amyloid imaging on clinical decision making. METHODS:We conducted a retrospective analysis of 140 cognitively impaired patients (mean age 65.0 years, 46% primary β-amyloid (Aβ) diagnosis, mean Mini-Mental State Examination 22.3) who underwent amyloid (Pittsburgh compound B [PiB]) PE...

journal_title：Neurology

authors： Sánchez-Juan P,Ghosh PM,Hagen J,Gesierich B,Henry M,Grinberg LT,O'Neil JP,Janabi M,Huang EJ,Trojanowski JQ,Vinters HV,Gorno-Tempini M,Seeley WW,Boxer AL,Rosen HJ,Kramer JH,Miller BL,Jagust WJ,Rabinovici GD

更新日期：2014-01-21 00:00:00

abstract：:To learn if limb-kinetic apraxia (LKA) is associated with Parkinson disease (PD), participants with PD (on medications) and control subjects performed finger tapping (FT), measuring movement speed, and performed coin rotation (CR), measuring precise coordinated but independent finger movements and speed. There were no...

journal_title：Neurology

authors： Quencer K,Okun MS,Crucian G,Fernandez HH,Skidmore F,Heilman KM

更新日期：2007-01-09 00:00:00

abstract：OBJECTIVE:The NIH Stroke Scale (NIHSS) may not appropriately assess the spectrum of posterior circulation (PC)-related neurologic deficits. We determined the cutoff baseline NIHSS score that predicts independent daily life activity during the chronic stage in anterior circulation (AC) vs PC ischemic strokes. METHODS:A...

journal_title：Neurology

authors： Sato S,Toyoda K,Uehara T,Toratani N,Yokota C,Moriwaki H,Naritomi H,Minematsu K

更新日期：2008-06-10 00:00:00

abstract：OBJECTIVE:To assess alterations in brain metabolites of patients with Pelizaeus-Merzbacher disease (PMD) with the proteolipid protein gene 1 (PLP1) duplications using quantitative proton MRS. METHODS:Five unrelated male Japanese patients with PMD with PLP1 duplications were analyzed using automated proton brain examin...

journal_title：Neurology

authors： Takanashi J,Inoue K,Tomita M,Kurihara A,Morita F,Ikehira H,Tanada S,Yoshitome E,Kohno Y

更新日期：2002-01-22 00:00:00

abstract：OBJECTIVE:Brain changes occurring early in HIV infection are not well characterized. The Chicago Early HIV Infection Study aimed to evaluate the presence and extent of structural brain alterations using quantitative MRI. METHODS:Forty-three HIV and 21 control subjects were enrolled. Mean length of infection was estima...

journal_title：Neurology

authors： Ragin AB,Du H,Ochs R,Wu Y,Sammet CL,Shoukry A,Epstein LG

更新日期：2012-12-11 00:00:00

abstract：:Despite strong evidence of the importance of hereditary factors in the etiology of Tourette's syndrome (TS), research findings have consistently pointed to a role of environmental influences. A recent line of research has suggested that tic disorders and associated behavioral disturbances, such as obsessive-compulsive...

journal_title：Neurology

authors： Kurlan R

更新日期：1998-06-01 00:00:00

abstract：OBJECTIVE:To investigate the temporal relationship between inflammation and cerebral atrophy in a longitudinal study of 19 patients with relapsing-remitting multiple sclerosis (RRMS) using serial monthly contrast enhanced MRI examinations and monthly measurements of brain fractional volume (BFV) for an average of 4 (ra...

journal_title：Neurology

authors： Richert ND,Howard T,Frank JA,Stone R,Ostuni J,Ohayon J,Bash C,McFarland HF

更新日期：2006-02-28 00:00:00

abstract：OBJECTIVE:To test the hypothesis that transcranial magnetic resonance-guided focused ultrasound (tcMRgFUS) thalamotomy is effective, durable, and safe for patients with medication-refractory essential tremor (ET), we assessed clinical outcomes at 3-year follow-up of a controlled multicenter prospective trial. METHODS:...

journal_title：Neurology

authors： Halpern CH,Santini V,Lipsman N,Lozano AM,Schwartz ML,Shah BB,Elias WJ,Cosgrove GR,Hayes MT,McDannold N,Aldrich C,Eisenberg HM,Gandhi D,Taira T,Gwinn R,Ro S,Witt J,Jung NY,Chang JW,Rosenberg J,Ghanouni P

更新日期：2019-12-10 00:00:00

abstract：:The authors describe a novel pathogenic G5540A transition in the mitochondrial transfer RNA (tRNA)Trp gene of a sporadic encephalomyopathy characterized by spinocerebellar ataxia. Clinical features also included neurosensorial deafness, peripheral neuropathy, and dementia. Biochemistry revealed a severe reduction of c...

journal_title：Neurology

authors： Silvestri G,Mongini T,Odoardi F,Modoni A,deRosa G,Doriguzzi C,Palmucci L,Tonali P,Servidei S

更新日期：2000-04-25 00:00:00

abstract：:Fatigue is a common but poorly understood symptom in Parkinson's disease (PD). Using previously validated scales, we asked 58 nondemented PD patients and 58 controls to fill out questionnaires assessing fatigue and depression and found that PD patients were more depressed and more fatigued than age-matched controls. A...

journal_title：Neurology

authors： Friedman J,Friedman H

更新日期：1993-10-01 00:00:00

abstract：:The authors investigated the PRNP Met129Val polymorphism in 1,393 subjects including 482 patients with Alzheimer disease (AD) and two independent control groups. In patients, PRNP Met homozygosity conferred increasing risk with decreasing age at onset (onset: 61 to 70 years, n = 151, p = 0.02, odds ratio [OR] = 1.72, ...

journal_title：Neurology

authors： Riemenschneider M,Klopp N,Xiang W,Wagenpfeil S,Vollmert C,Müller U,Förstl H,Illig T,Kretzschmar H,Kurz A

更新日期：2004-07-27 00:00:00

abstract：OBJECTIVE:To explore the association between postmenopausal hormone therapy (HT) and Alzheimer disease (AD). METHODS:Twenty-year follow-up data from the Kuopio Osteoporosis Risk Factor and Prevention study cohort were used. Self-administered questionnaires were sent to all women aged 47-56 years, residing in Kuopio Pr...

journal_title：Neurology

authors： Imtiaz B,Tuppurainen M,Rikkonen T,Kivipelto M,Soininen H,Kröger H,Tolppanen AM

更新日期：2017-03-14 00:00:00

abstract：:Monocytes are recruited to active sites of multiple sclerosis (MS) demyelination and may promote local tissue injury by generating an inflammatory response, mediated in part by the production of toxic oxygen metabolites. Corticosteroids are frequently and effectively used to ameliorate MS exacerbations, despite inadeq...

journal_title：Neurology

authors： Fisher M,Levine PH,Doyle EM,Arpano MM,Bergeron DA,Cohen RA,Hoogasian JJ

更新日期：1991-02-01 00:00:00

abstract：OBJECTIVE:To determine the incidence of multiple sclerosis (MS) in a longitudinal surveillance over 35 years and to estimate the prevalence rate in Saskatoon, Saskatchewan, on January 1, 2005. METHODS:A population-based registry was established in 1969, and identification of cases continued to 2005, from medical recor...

journal_title：Neurology

authors： Hader WJ,Yee IM

更新日期：2007-09-18 00:00:00

abstract：OBJECTIVES:To test the hypothesis that neuropsychiatric symptomatology is predictive of the success of seizure control in patients newly treated with antiepileptic drugs (AEDs), and that this predictive value adds to that provided by other clinical, imaging, and genomic factors in a multivariate model. METHODS:One hun...

journal_title：Neurology

authors： Petrovski S,Szoeke CE,Jones NC,Salzberg MR,Sheffield LJ,Huggins RM,O'Brien TJ

更新日期：2010-09-14 00:00:00

abstract：OBJECTIVE:To define potential pathogenic mitochondrial DNA (mtDNA) point mutations in a patient with myoclonus epilepsy with ragged-red fibers (MERRF) syndrome. BACKGROUND:MERRF syndrome is typically associated with point mutations in the mtDNA tRNALys gene. METHODS:We performed morphologic, biochemical, and genetic ...

journal_title：Neurology

authors： Arenas J,Campos Y,Bornstein B,Ribacoba R,Martín MA,Rubio JC,Santorelli FM,Zeviani M,DiMauro S,Garesse R

更新日期：1999-01-15 00:00:00

abstract：:Placebo, or sham surgery, as a control condition in surgical clinical trials in Parkinson disease (PD) remains controversial. The authors reviewed the adverse effects reported in double blind, placebo surgery controlled trials for PD. Placebo surgeries were generally safe and well tolerated but the number of subjects ...

journal_title：Neurology

authors： Frank S,Kieburtz K,Holloway R,Kim SY

更新日期：2005-10-11 00:00:00

abstract：:The one-and-a-half syndrome is a clinical disorder of extraocular movements characterized by a conjugate horizontal gaze palsy in one direction plus an internuclear ophthalmoplegia in the other. The syndrome is usually due to a single unilateral lesion of the paramedian pontine reticular formation or the abducens nucl...

journal_title：Neurology

authors： Wall M,Wray SH

更新日期：1983-08-01 00:00:00