A distinctive autosomal dominant vacuolar neuromyopathy linked to 19p13.

abstract：:A 60-year-old woman suffered from recurrent episodes of fever, hypertension, facial flushing, vomiting, stridor, slowly progressive symptoms of hypohidrosis, and orthostatic hypotension. The episodes were synchronous with elevated catecholamine concentration in plasma and urine. This is an example of paroxysmal autono...

journal_title：Neurology

authors： Mitsui T,Kawai H,Taguchi E,Miyamoto H,Saito S

更新日期：1993-09-01 00:00:00

abstract：:A man with onset of obsessive-compulsive disorder (OCD) at age 62 had a large right posterior frontal infarct and occlusion of the ipsilateral internal carotid artery. We review additional cases from the literature with obsessive or compulsive behaviors and structural lesions. OCD may have a structural correlate, and ...

journal_title：Neurology

authors： Swoboda KJ,Jenike MA

更新日期：1995-12-01 00:00:00

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 Neuromuscula...

journal_title：Neurology

authors： 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 A

更新日期：2011-08-02 00:00:00

abstract：OBJECTIVE:To evaluate the validity of data derived from magnetic source imaging (MSI) regarding cerebral dominance for language in patients with intractable seizure disorder. METHOD:The authors performed functional imaging of the receptive language cortex using a whole-head neuromagnetometer in 26 consecutive epilepsy...

journal_title：Neurology

authors： Breier JI,Simos PG,Zouridakis G,Wheless JW,Willmore LJ,Constantinou JE,Maggio WW,Papanicolaou AC

更新日期：1999-09-22 00:00:00

abstract：OBJECTIVE:We determined whether Gram-negative bacterial molecules are associated with Alzheimer disease (AD) neuropathology given that previous studies demonstrate Gram-negative Escherichia coli bacteria can form extracellular amyloid and Gram-negative bacteria have been reported as the predominant bacteria found in no...

journal_title：Neurology

authors： Zhan X,Stamova B,Jin LW,DeCarli C,Phinney B,Sharp FR

更新日期：2016-11-29 00:00:00

abstract：:About 9% of patients presenting to a Huntington disease (HD) clinic for evaluation of possible HD lacked a family history of the disorder. HD was the final diagnosis in 53 to 83% of these patients. As a group, HD-affected individuals without a family history of HD were older and had fewer CAG repeats than the average ...

journal_title：Neurology

authors： Nance MA,Westphal B,Nugent S

更新日期：1996-12-01 00:00:00

abstract：BACKGROUND:Periodic paralyses and paramyotonia congenita are rare disorders causing disabling weakness and myotonia. Mutations in sodium, calcium, and potassium channels have been recognized as causing disease. OBJECTIVE:To analyze the clinical phenotype of patients with and without discernible genotype and to identif...

journal_title：Neurology

authors： Miller TM,Dias da Silva MR,Miller HA,Kwiecinski H,Mendell JR,Tawil R,McManis P,Griggs RC,Angelini C,Servidei S,Petajan J,Dalakas MC,Ranum LP,Fu YH,Ptácek LJ

更新日期：2004-11-09 00:00:00

abstract：:Patients with symptomatic > or = 60% (n = 134), asymptomatic > or = 80% (n = 143), and asymptomatic progressive > or = 60% (n = 25) internal carotid artery stenosis underwent stenting and were followed clinically and by Doppler-assisted duplex imaging for 27.1 +/- 15.6 months. Stroke and death from stroke occurred wit...

journal_title：Neurology

authors： Bibl D,Lampl C,Biberhofer I,Kerschner K,Kypta A,Bergmann J,Kaindlstorfer A,Röper C,Yazdi K,Engleder C,Hofmann R,Deibl M,Leisch F,Ransmayr G

更新日期：2005-07-12 00:00:00

abstract：:We administered Ro 40-7592, an inhibitor of the enzyme catechol-O-methyltransferase (COMT) that crosses the blood-brain barrier, to rats and monitored extracellular catecholamine levels in the corpus striatum before and after the intraperitoneal administration of a bolus of l-dopa. Acute administration of Ro 40-7592 i...

journal_title：Neurology

authors： Brannan T,Martínez-Tica J,Yahr MD

更新日期：1992-03-01 00:00:00

abstract：OBJECTIVE:The objective of this study was to determine whether environmental and genetic alterations of neuronal aldehyde dehydrogenase (ALDH) enzymes were associated with increased Parkinson disease (PD) risk in an epidemiologic study. METHODS:A novel ex vivo assay was developed to identify pesticides that can inhibi...

journal_title：Neurology

authors： Fitzmaurice AG,Rhodes SL,Cockburn M,Ritz B,Bronstein JM

更新日期：2014-02-04 00:00:00

abstract：:Self-reported versus informant-reported memory problems in nondemented elderly adults and in individuals with very mild and mild dementia of the Alzheimer type (DAT) were correlated with cognitive outcomes. No significant correlations were found between self-reported memory complaints and cognitive performance or (in ...

journal_title：Neurology

authors： Carr DB,Gray S,Baty J,Morris JC

更新日期：2000-12-12 00:00:00

abstract：:This cross-sectional mail survey evaluated the prevalence and predictors of unconventional therapy (UT) utilization among 569 community-based people with MS. One-third reported having visited a UT practitioner in the last 6 months. Utilizers tended to report at least one medical comorbidity, earn at least $50,000 a ye...

journal_title：Neurology

authors： Schwartz CE,Laitin E,Brotman S,LaRocca N

更新日期：1999-02-01 00:00:00

abstract：:In 10 patients with chronic autonomic failure, the sympathetic perivascular nerve plexuses from quadriceps muscle biopsies were studied by catecholamine fluorescence and electronmicroscopy. There was almost complete absence of catecholamine fluorescence and fewer than normal numbers of small granular (noradrenergic) v...

journal_title：Neurology

authors： Bannister R,Crowe R,Eames R,Burnstock G

更新日期：1981-12-01 00:00:00

abstract：OBJECTIVE:We aimed to assess sensitivity and specificity of the updated criteria for behavioral variant frontotemporal dementia (bvFTD) based on a large autopsy-confirmed cohort of patients with dementia. METHODS:Two hundred thirty-nine consecutive pathologically confirmed dementia patients, clinically assessed in a s...

journal_title：Neurology

authors： Harris JM,Gall C,Thompson JC,Richardson AM,Neary D,du Plessis D,Pal P,Mann DM,Snowden JS,Jones M

更新日期：2013-05-14 00:00:00

abstract：:Patients with mild cognitive impairment (MCI) were assessed, and a metabolic profile associated with conversion to AD at 18-month follow-up was sought. As compared with nonconverters (n = 10), converters (n = 7) had lower fluorodeoxyglucose uptake in the right temporoparietal cortex (p = 0.02, corrected for cluster si...

journal_title：Neurology

authors： Chételat G,Desgranges B,de la Sayette V,Viader F,Eustache F,Baron JC

更新日期：2003-04-22 00:00:00

abstract：OBJECTIVE:To compare the efficacy, tolerability, and safety of IV methylprednisolone (IV MP) vs oral methylprednisolone (oMP) at equivalent high doses in patients with multiple sclerosis (MS) experiencing a recent relapse. METHODS:Patients with a clinical relapse within the previous 2 weeks and at least 1 gadolinium (...

journal_title：Neurology

authors： Martinelli V,Rocca MA,Annovazzi P,Pulizzi A,Rodegher M,Martinelli Boneschi F,Scotti R,Falini A,Sormani MP,Comi G,Filippi M

更新日期：2009-12-01 00:00:00

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：:Titus Lucretius Carus was an ancient Roman philosopher of the Epicurean school whose epic poem On the Nature of Things described numerous aspects of the natural world. In fact, much contemporary scientific understanding is consistent with or inspired by his work. Among Lucretius's contributions to neurology were his d...

journal_title：Neurology

authors： Hyam JA,Paterson DJ,Aziz TZ,Green AL

更新日期：2011-09-06 00:00:00

abstract：:The authors describe the outcome of five patients with a cardiac thrombus selected among 183 patients with stroke (2.7 %) who were given IV tissue plasminogen activator (tPA). No early systemic or cerebral embolism occurred. Two patients made a complete recovery at 3 months. Two patients had a moderate outcome. One pa...

journal_title：Neurology

authors： Derex L,Nighoghossian N,Perinetti M,Honnorat J,Trouillas P

更新日期：2001-12-11 00:00:00

abstract：:Relating the onset of clinical attacks as recorded on a videotape to surface and depth electrographic events improves our ability to define the local or diffuse origin of seizures. In two patients with complex partial seizures, all 14 attacks appeared 3 to 6 seconds after focal discharges began in the amygdala or hipp...

journal_title：Neurology

authors： Delgado-Escueta AV,Nashold B,Freedman M,Keplinger C,Waddell G,Miller P,Carwille S

更新日期：1979-04-01 00:00:00

abstract：OBJECTIVE:To report 5-year outcomes from the subthalamic nucleus (STN) deep brain stimulation (DBS) in early-stage Parkinson disease (PD) pilot clinical trial. METHODS:The pilot was a prospective, single-blind clinical trial that randomized patients with early-stage PD (Hoehn & Yahr II off medications) to receive bila...

journal_title：Neurology

authors： Hacker ML,Turchan M,Heusinkveld LE,Currie AD,Millan SH,Molinari AL,Konrad PE,Davis TL,Phibbs FT,Hedera P,Cannard KR,Wang L,Charles D

更新日期：2020-07-28 00:00:00

abstract：:Recurrent stroke is a major public health concern and new treatment strategies are needed. While modulation of the renin angiotensin aldosterone system (RAAS) has proven effective in reducing recurrent cardiac events, its role in preventing recurrent cerebrovascular events remains unclear. RAAS is both a circulating a...

journal_title：Neurology

authors： Sokol SI,Portnay EL,Curtis JP,Nelson MA,Hebert PR,Setaro JF,Foody JM

更新日期：2004-07-27 00:00:00

abstract：:We analyzed the frequency of cardiac embolic sources in 62 patients with acute lacunar stroke vs 50 controls. In post hoc analysis, 11 patients with lacunar stroke having neither hypertension nor diabetes mellitus (non-HDM group) had a higher frequency of right-to-left shunt (RLS) (82%) than patients with risk factors...

journal_title：Neurology

authors： Ueno Y,Kimura K,Iguchi Y,Shibazaki K,Inoue T,Urabe T

更新日期：2007-02-13 00:00:00

abstract：:We report 9 patients with an isolated infarct of the anterior part of the rostral cerebellum, ie, the territory of the lateral branch of the superior cerebellar artery. Clinicoanatomic correlations are based on CT, MRI, or both in 8 patients and on pathologic data in the ninth. The main clinical features were ipsilate...

journal_title：Neurology

authors： Amarenco P,Roullet E,Goujon C,Chéron F,Hauw JJ,Bousser MG

更新日期：1991-02-01 00:00:00

abstract：OBJECTIVE:To report 2 patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who presented acutely with Miller Fisher syndrome and polyneuritis cranialis, respectively. METHODS:Patient data were obtained from medical records from the University Hospital "Príncipe de Asturias," Alcalá de He...

journal_title：Neurology

authors： Gutiérrez-Ortiz C,Méndez-Guerrero A,Rodrigo-Rey S,San Pedro-Murillo E,Bermejo-Guerrero L,Gordo-Mañas R,de Aragón-Gómez F,Benito-León J

更新日期：2020-08-04 00:00:00

abstract：:Recent years have seen major changes in our understanding of Parkinson disease (PD), challenging conventional wisdom, much of which was established during the Decade of the Brain. In this article, we highlight important changes in our understanding of PD in six general categories: definition, etiology, pathology, path...

journal_title：Neurology

authors： Marras C,Lang A

更新日期：2008-05-20 00:00:00

abstract：BACKGROUND:The annual relapse rate has been commonly used as a primary efficacy endpoint in phase III multiple sclerosis (MS) clinical trials. The aim of this study was to determine the relative contribution of different possible prognostic factors available at baseline to the on-study relapse rate in MS. METHODS:A to...

journal_title：Neurology

authors： Held U,Heigenhauser L,Shang C,Kappos L,Polman C,Sylvia Lawry Centre for MS Research.

更新日期：2005-12-13 00:00:00

abstract：:We evaluated the effects of isoniazid on tremor in 13 patients with MS. Patients were evaluated before treatment, after 1 month of therapy (1,000 mg daily), and 1 month after the last dose. Ten patients improved on at least one of three methods of evaluation. Transient side effects were common, possibly because there ...

journal_title：Neurology

authors： Duquette P,Pleines J,du Souich P

更新日期：1985-12-01 00:00:00

abstract：OBJECTIVE:To describe and evaluate the incidence and risk factors of severe neurologic events (SNE) in pediatric recipients of allogeneic or autologous hematopoietic stem cell transplantation (HSCT) for hematologic or nonhematologic diseases. METHODS:Retrospective analysis of 272 consecutive children admitted to the G...

journal_title：Neurology

authors： Faraci M,Lanino E,Dini G,Fondelli MP,Morreale G,Dallorso S,Manzitti C,Calevo MG,Gaggero R,Castagnola E,Haupt R

更新日期：2002-12-24 00:00:00

abstract：OBJECTIVE:To evaluate attention deficit in Alzheimer's disease (AD) and its relationship to attention deficits associated with aging and with medications altering alertness. METHODS:Ten patients with probable AD, 10 healthy old controls, and 15 young controls performed a covert orienting of spatial attention task. You...

journal_title：Neurology

authors： Oken BS,Kishiyama SS,Kaye JA,Howieson DB

更新日期：1994-04-01 00:00:00