Adult fucosidosis: histochemical and ultrastructural studies of rectal mucosa biopsy.

abstract：:We report the cases of 5 patients from 2 sibships with the "adult" or chronic form of GM2 gangliosidosis and 2 patients from another sibship with the juvenile form. We demonstrated hexosaminidase A deficiency in all cases but in 1 sibship the enzymatic profile was identical to that in Tay-Sachs disease, whereas in the...

journal_title：Neurology

authors： Specola N,Vanier MT,Goutières F,Mikol J,Aicardi J

更新日期：1990-01-01 00:00:00

abstract：:The behavioral and anatomic correlates of pure alexia were analyzed in 16 patients. Right homonymous hemianopia failed to appear in three patients, who had right achromatopsia instead. Color anomia and unilateral optic ataxia were seen in six patients. Memory defects were found in two patients. Visual agnosia was note...

journal_title：Neurology

authors： Damasio AR,Damasio H

更新日期：1983-12-01 00:00:00

abstract：BACKGROUND:Treatment of aneurysmal subarachnoid hemorrhage (SAH) has changed substantially over the last 25 years but there is a lack of reliable population-based data on whether case-fatality or functional outcomes have improved. METHODS:We determined changes in the standardized incidence and outcome of SAH in the sa...

journal_title：Neurology

authors： Lovelock CE,Rinkel GJ,Rothwell PM

更新日期：2010-05-11 00:00:00

abstract：OBJECTIVE:To investigate the relationship between baseline hypertension and severity of white matter hyperintensities (WMH) at 4-year follow-up in a sample of subjects aged 59 to 71 years old at entry. METHODS:Subjects were participants in the Epidemiology of Vascular Ageing study, a longitudinal study on vascular agi...

journal_title：Neurology

authors： Dufouil C,de Kersaint-Gilly A,Besançon V,Levy C,Auffray E,Brunnereau L,Alpérovitch A,Tzourio C

更新日期：2001-04-10 00:00:00

abstract：:Urinary incontinence (UI) after stroke is common and associated with overall poor functional outcomes. There is controversy regarding which factors contribute to incontinence after stroke and which factors may be predictive of recovery of continence. This study investigated consecutive stroke admissions to an inpatien...

journal_title：Neurology

authors： Ween JE,Alexander MP,D'Esposito M,Roberts M

更新日期：1996-09-01 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：:E.C. Seguin was one of the early, influential 19th-century neurologists who participated in the development of neurology as a specialty in the United States. Born in France, but raised from early childhood in the United States, Seguin published widely, developed a high-profile New York City practice, and was named Cli...

journal_title：Neurology

authors： Goetz CG,Harter DH

更新日期：2013-04-30 00:00:00

abstract：:Cerebral cholesterol metabolism has been linked with production of amyloid peptide (Abeta) crucial in AD. The association between use of cholesterol-lowering drugs (statins) and AD disease is currently being intensely discussed. In this case-control study on elderly nondemented subjects, the authors provide the first ...

journal_title：Neurology

authors： Fassbender K,Stroick M,Bertsch T,Ragoschke A,Kuehl S,Walter S,Walter J,Brechtel K,Muehlhauser F,Von Bergmann K,Lütjohann D

更新日期：2002-10-22 00:00:00

abstract：BACKGROUND:PD was associated with increased mortality before levodopa therapy became available. There have been conflicting reports of PD mortality in the modern era. OBJECTIVE:To assess current mortality rates in a large unselected population receiving treatment for parkinsonism (PKM) followed for up to 6 years. MET...

journal_title：Neurology

authors： Guttman M,Slaughter PM,Theriault ME,DeBoer DP,Naylor CD

更新日期：2001-12-26 00:00:00

abstract：BACKGROUND:Biomarker levels in blood after traumatic brain injury (TBI) may offer diagnostic and prognostic tools in addition to clinical indices. This study aims to validate glial fibrillary acidic protein (GFAP) and S100B concentrations in blood as outcome predictors of TBI using cutoff levels of 1.5 μg/L for GFAP an...

journal_title：Neurology

authors： Vos PE,Jacobs B,Andriessen TM,Lamers KJ,Borm GF,Beems T,Edwards M,Rosmalen CF,Vissers JL

更新日期：2010-11-16 00:00:00

abstract：:A 60-year-old man presented with progressive large fiber sensory loss in the right first three fingers and, to a lesser extent, in both fourth and fifth fingers. Electrophysiologic studies were characteristic of chronic sensory demyelinating polyneuropathy, a variant of chronic inflammatory demyelinating polyneuropath...

journal_title：Neurology

authors： Thonnard JL,Detrembleur C,Van den Bergh PY

更新日期：1997-07-01 00:00:00

abstract：:Cholinergic neurons in the nucleus ambiguus (NA) were counted in autopsy tissue obtained from five patients with multiple system atrophy (MSA), four patients with PD, and five controls. The number of neurons in the dorsal NA was not significantly different among the three groups. Neurons in the ventrolateral portion o...

journal_title：Neurology

authors： Benarroch EE,Schmeichel AM,Parisi JE

更新日期：2003-01-14 00:00:00

abstract：OBJECTIVE:To determine whether immunoglobulin G (IgG) from patients with Lambert-Eaton myasthenic syndrome (LEMS) decreases action potential–evoked synaptic vesicle exocytosis,and whether the effect is mediated by P/Q-type voltage-gated calcium channels (VGCCs). METHODS:IgG was obtained from 4 patients with LEMS (3 ma...

journal_title：Neurology

authors： Spillane J,Ermolyuk Y,Cano-Jaimez M,Lang B,Vincent A,Volynski KE,Kullmann DM

更新日期：2015-02-10 00:00:00

abstract：:This study examined the frequency of epilepsy in a consecutive series of patients who received a definitive diagnosis of psychogenic nonepileptic seizures (PNES) after completing inpatient video-EEG (VEEG) monitoring. Of the 1,590 patients receiving definitive diagnosis, 514 (32.3%) were diagnosed with PNES. Twenty-ni...

journal_title：Neurology

authors： Martin R,Burneo JG,Prasad A,Powell T,Faught E,Knowlton R,Mendez M,Kuzniecky R

更新日期：2003-12-23 00:00:00

abstract：:We evaluated the neuroprotective effect of MK-801, a noncompetitive, selective N-methyl-D-aspartate receptor antagonist, in a neonatal hypoxic-ischemic animal model. Seven-day-old rats underwent bilateral ligation of the carotid arteries followed by exposure to an 8% oxygen atmosphere for 1 hr. We sacrificed the anima...

journal_title：Neurology

authors： Hattori H,Morin AM,Schwartz PH,Fujikawa DG,Wasterlain CG

更新日期：1989-05-01 00:00:00

abstract：:We performed a case-control study to investigate the role of recent infection as stroke risk factor and to identify pathogenetic pathways linking infection and stroke. We examined 166 consecutive patients with acute cerebrovascular ischemia and 166 patients hospitalized for nonvascular and noninflammatory neurologic d...

journal_title：Neurology

authors： Grau AJ,Buggle F,Becher H,Zimmermann E,Spiel M,Fent T,Maiwald M,Werle E,Zorn M,Hengel H,Hacke W

更新日期：1998-01-01 00:00:00

abstract：OBJECTIVES:There is currently much interest in biomarkers of disease activity in frontotemporal lobar degeneration (FTLD). We assessed MRI and behavioral measures of progression in a longitudinal FTLD cohort. METHODS:Thirty-two patients with FTLD (11 behavioral variant frontotemporal dementia [bvFTD], 11 semantic deme...

journal_title：Neurology

authors： Gordon E,Rohrer JD,Kim LG,Omar R,Rossor MN,Fox NC,Warren JD

更新日期：2010-02-23 00:00:00

abstract：OBJECTIVE:To develop and evaluate a model for staging cortical amyloid deposition using PET with high generalizability. METHODS:Three thousand twenty-seven individuals (1,763 cognitively unimpaired [CU], 658 impaired, 467 with Alzheimer disease [AD] dementia, 111 with non-AD dementia, and 28 with missing diagnosis) fr...

journal_title：Neurology

authors： Collij LE,Heeman F,Salvadó G,Ingala S,Altomare D,de Wilde A,Konijnenberg E,van Buchem M,Yaqub M,Markiewicz P,Golla SSV,Wottschel V,Wink AM,Visser PJ,Teunissen CE,Lammertsma AA,Scheltens P,van der Flier WM,Boellaard R

更新日期：2020-09-15 00:00:00

abstract：OBJECTIVE:To determine how memory-encoding tasks elicit functional perfusion change in subjects with amnestic mild cognitive impairment (aMCI). METHODS:Twelve subjects with aMCI and 14 age-matched cognitively normal (CN) subjects were recruited for this study. Arterial spin-labeling perfusion MRI (ASL-MRI) was employe...

journal_title：Neurology

authors： Xu G,Antuono PG,Jones J,Xu Y,Wu G,Ward D,Li SJ

更新日期：2007-10-23 00:00:00

abstract：:We report a patient with unusual MRI abnormalities that had the physical characteristics of ferromagnetic artifact. We believe that the MRI artifacts were due to microscopic embolic metal fragments, most likely from a mechanical heart valve prosthesis. Potential sources of metal emboli should be considered in patients...

journal_title：Neurology

authors： Wingerchuk DM,Krecke KN,Fulgham JR

更新日期：1997-11-01 00:00:00

abstract：:Treatment of functional symptoms has a long history, and interventions were often used in soldiers returning from battle. On the 75th anniversary of the end of the Second World War, I review the portrayal of neurology in documentary film. Two documentaries were released in 1946 and 1948 (Let There Be Light and Shades ...

journal_title：Neurology

authors： Wijdicks EFM

更新日期：2020-06-09 00:00:00

abstract：:Information on 1,343 hospitalized patients in the Cornell Neurology Database with final diagnoses of transient ischemic attacks (TIA), reversible ischemic neurologic deficits (RIND), or ischemic stroke was examined in order to determine the duration of ischemic deficits. Episodes resolved within the first 24 hours (cl...

journal_title：Neurology

authors： Levy DE

更新日期：1988-05-01 00:00:00

abstract：OBJECTIVE:To examine whether late-life exposure to PM2.5 (particulate matter with aerodynamic diameters <2.5-µm) contributes to progressive brain atrophy predictive of Alzheimer's disease (AD) using a community-dwelling cohort of women (aged 70-89) with up to two brain MRI scans (MRI-1: 2005-6; MRI-2: 2010-11). METHOD...

journal_title：Neurology

authors： Younan D,Wang X,Casanova R,Barnard R,Gaussoin SA,Saldana S,Petkus AJ,Beavers DP,Resnick SM,Manson JE,Serre ML,Vizuete W,Henderson VW,Sachs BC,Salinas JA,Gatz M,Espeland MA,Chui HC,Shumaker SA,Rapp SR,Chen JC,Wom

更新日期：2020-11-18 00:00:00

abstract：OBJECTIVE:To evaluate the retinal nerve fiber layer (RNFL) thickness and macular volume in neuromyelitis optica (NMO) spectrum patients using optical coherence tomography (OCT). BACKGROUND:OCT can quantify damage to retinal ganglion cell axons and can identify abnormalities in multiple sclerosis and optic neuritis (ON...

journal_title：Neurology

authors： Ratchford JN,Quigg ME,Conger A,Frohman T,Frohman E,Balcer LJ,Calabresi PA,Kerr DA

更新日期：2009-07-28 00:00:00

abstract：BACKGROUND:Adiponectin is a protein secreted by adipose cells that improves insulin sensitivity and possesses antiatherogenic properties. In this study, we investigated the relationship between adiponectin levels and ischemic stroke subtype. METHODS:Using clinical, imaging, and laboratory data, 231 consecutive patient...

journal_title：Neurology

authors： Bang OY,Saver JL,Ovbiagele B,Choi YJ,Yoon SR,Lee KH

更新日期：2007-05-29 00:00:00

abstract：:We recorded interictal spikes with closely spaced scalp electrodes and sphenoidal electrodes in four patients with temporal lobe epilepsy. We used multiple dipole modeling to study the number, three-dimensional intracerebral location, time activity, and functional relationship of the neuronal sources underlying the ep...

journal_title：Neurology

authors： Baumgartner C,Lindinger G,Ebner A,Aull S,Serles W,Olbrich A,Lurger S,Czech T,Burgess R,Lüders H

更新日期：1995-01-01 00:00:00

abstract：:We conducted a case-control study to assess possible factors associated with Alzheimer's disease (AD) with 70 clinically diagnosed AD patients and 140 age- and sex-matched nondemented neighborhood controls in China. Factors significantly associated with AD cases were family history of dementia in first-degree relative...

journal_title：Neurology

authors： Li G,Shen YC,Li YT,Chen CH,Zhau YW,Silverman JM

更新日期：1992-08-01 00:00:00

abstract：:The authors report a double-blind, placebo-controlled, crossover study of talampanel in 49 patients with refractory partial seizures. Three doses of talampanel were investigated based on differences in patients' concomitant antiepileptic drug usage. Talampanel showed efficacy in reducing seizure frequency (p = 0.001) ...

journal_title：Neurology

authors： Chappell AS,Sander JW,Brodie MJ,Chadwick D,Lledo A,Zhang D,Bjerke J,Kiesler GM,Arroyo S

更新日期：2002-06-11 00:00:00

abstract：BACKGROUND AND OBJECTIVE:Posterior cortical atrophy (PCA) is a progressive neurodegenerative syndrome that presents with cortical visual dysfunction and relatively preserved memory. Although higher cortical visual syndromes are well known in PCA, visual field defects detected by computerized visual field (CVF) perimetr...

journal_title：Neurology

authors： Pelak VS,Smyth SF,Boyer PJ,Filley CM

更新日期：2011-12-13 00:00:00

abstract：OBJECTIVE:-To determine whether pre-treatment cerebral microbleeds (CMBs) presence and burden are correlated with an increased risk of intracranial hemorrhage (ICH) or poor functional outcome following endovascular thrombectomy (EVT) for acute ischemic stroke (AIS). METHODS:-Consecutive patients treated by EVT for ant...

journal_title：Neurology

authors： Derraz I,Cagnazzo F,Gaillard N,Morganti R,Dargazanli C,Ahmed R,Pierre-Henri L,Riquelme C,Mourand I,Gascou G,Bonafe A,Arquizan C,Costalat V

更新日期：2021-01-25 00:00:00