Exercise tolerance in carnitine palmitoyltransferase II deficiency with IV and oral glucose.

abstract：:A 48-year-old man presented with progressive spastic paraparesis and diffuse white matter involvement on neuroimaging that suggested a primary demyelinating disease. Brain biopsy 3 years after onset of symptoms demonstrated idiopathic granulomatous angiitis. In patients with MRI features of diffuse white matter diseas...

journal_title：Neurology

authors： Finelli PF,Onyiuke HC,Uphoff DF

更新日期：1997-12-01 00:00:00

abstract：OBJECTIVE:To determine the prevalence, incidence, and predictors of epilepsy among older adults in the Cardiovascular Health Study (CHS). METHODS:We analyzed data prospectively collected in CHS and merged with data from outpatient Medicare administrative claims. We identified cases with epilepsy using self-report, ant...

journal_title：Neurology

authors： Choi H,Pack A,Elkind MS,Longstreth WT Jr,Ton TG,Onchiri F

更新日期：2017-02-28 00:00:00

abstract：OBJECTIVE:To combine MRI-based cortical morphometry and diffusion white matter tractography to describe the anatomical correlates of repetition deficits in patients with primary progressive aphasia (PPA). METHODS:The traditional anatomical model of language identifies a network for word repetition that includes Wernic...

journal_title：Neurology

authors： Forkel SJ,Rogalski E,Drossinos Sancho N,D'Anna L,Luque Laguna P,Sridhar J,Dell'Acqua F,Weintraub S,Thompson C,Mesulam MM,Catani M

更新日期：2020-02-11 00:00:00

abstract：OBJECTIVES:Patients surviving retinopathy-positive cerebral malaria (CM) are at high risk for the development of epilepsy, developmental disabilities, and behavioral abnormalities. We aimed to establish whether retinopathy-negative CM is also a risk factor for these outcomes. METHODS:Between 2005 and 2007, survivors o...

journal_title：Neurology

authors： Postels DG,Taylor TE,Molyneux M,Mannor K,Kaplan PW,Seydel KB,Chimalizeni YF,Kawaza K,Birbeck GL

更新日期：2012-09-18 00:00:00

abstract：OBJECTIVE:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary arteriopathy caused by mutations of the Notch3 gene. The disease is characterized by T2-hyperintense lesions (subcortical white matter lesions), T1-hypointense lesions (lacunar lesions), and T2...

journal_title：Neurology

authors： Viswanathan A,Gschwendtner A,Guichard JP,Buffon F,Cumurciuc R,O'Sullivan M,Holtmannspötter M,Pachai C,Bousser MG,Dichgans M,Chabriat H

更新日期：2007-07-10 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：:Four patients with a clinical syndrome closely resembling amyotrophic lateral sclerosis recovered completely, without treatment, 5 to 12 months after onset. Electrodiagnostic tests revealed acute and chronic denervation, with normal motor and sensory nerve conduction studies. The CSF was normal, and tests for paraprot...

journal_title：Neurology

authors： Tucker T,Layzer RB,Miller RG,Chad D

更新日期：1991-10-01 00:00:00

abstract：BACKGROUND:Hypothalamic hamartomas (HHs), rare developmental abnormalities of the inferior hypothalamus, often cause refractory, symptomatic, mixed epilepsy, including gelastic seizures. We present 37 patients with HH who underwent transcortical transventricular endoscopic resection. METHODS:Between October 2003 and A...

journal_title：Neurology

authors： Ng YT,Rekate HL,Prenger EC,Wang NC,Chung SS,Feiz-Erfan I,Johnsonbaugh RE,Varland MR,Kerrigan JF

更新日期：2008-04-22 00:00:00

abstract：OBJECTIVE:To assess longitudinal follow-up of the incidence of multiple sclerosis (MS) through five decades and estimate the prevalence rate in Hordaland County, Norway, on January 1, 2003. METHODS:All patients with MS diagnosed from 1953 to 2003 were identified in the patient records of the Department of Neurology, H...

journal_title：Neurology

authors： Grytten N,Glad SB,Aarseth JH,Nyland H,Midgard R,Myhr KM

更新日期：2006-01-24 00:00:00

abstract：:Photoparoxysmal response (PPR) is sometimes incidentally encountered in EEGs performed for evaluation of nonepileptic symptoms. We conducted the first long-term study of a cohort of nonepileptic patients to determine their risk of having seizures subsequent to incidental recording of PPR. After 6 to 12 years (mean, 9 ...

journal_title：Neurology

authors： So EL,Ruggles KH,Ahmann PA,Olson KA

更新日期：1993-09-01 00:00:00

abstract：OBJECTIVE:To determine if progressive brain atrophy could be detected over 1- and 2-year intervals in relapsing MS, based on annual MR studies from the Multiple Sclerosis Collaborative Research Group (MSCRG) trial of interferon beta-1a (Avonex). METHODS:All subjects had mild to moderate disability, with baseline expan...

journal_title：Neurology

authors： Simon JH,Jacobs LD,Campion MK,Rudick RA,Cookfair DL,Herndon RM,Richert JR,Salazar AM,Fischer JS,Goodkin DE,Simonian N,Lajaunie M,Miller DE,Wende K,Martens-Davidson A,Kinkel RP,Munschauer FE 3rd,Brownscheidle CM

更新日期：1999-07-13 00:00:00

abstract：:Salla disease is a lysosomal storage disorder associated with increased urinary excretion of free sialic acid. The main clinical features in 34 patients were severe psychomotor retardation of early onset, ataxia, athetosis, rigidity, spasticity, and impaired speech. Growth retardation, thick calvarium, and exotropia w...

journal_title：Neurology

authors： Renlund M,Aula P,Raivio KO,Autio S,Sainio K,Rapola J,Koskela SL

更新日期：1983-01-01 00:00:00

abstract：OBJECTIVE:This study aimed to determine the frequency of CNS infection by dengue virus (DENV) in individuals with fatal outcomes. METHODS:Samples of 150 individuals suspect of an infection disease and with fatal outcomes were investigated for evidence of the presence of DENV. The sampling was made up of 150 CSF, 120 t...

journal_title：Neurology

authors： Araújo FM,Araújo MS,Nogueira RM,Brilhante RS,Oliveira DN,Rocha MF,Cordeiro RA,Araújo RM,Sidrim JJ

更新日期：2012-03-06 00:00:00

abstract：OBJECTIVE:To determine the diagnostic accuracy and clinical utility of electromagnetic source imaging (EMSI) in presurgical evaluation of patients with epilepsy. METHODS:We prospectively recorded magnetoencephalography (MEG) simultaneously with EEG and performed EMSI, comprising electric source imaging, magnetic sourc...

journal_title：Neurology

authors： Duez L,Tankisi H,Hansen PO,Sidenius P,Sabers A,Pinborg LH,Fabricius M,Rásonyi G,Rubboli G,Pedersen B,Leffers AM,Uldall P,Jespersen B,Brennum J,Henriksen OM,Fuglsang-Frederiksen A,Beniczky S

更新日期：2019-02-05 00:00:00

abstract：:Using a crossed-response task, monkeys with neglect induced by frontal lesions appear to have motor rather than sensory neglect. However, the crossed-response task may not reveal sensory neglect (inattention) if no perceptual discrimination is required. We therefore trained two monkeys in a perceptually complex crosse...

journal_title：Neurology

authors： Heilman KM,Valenstein E,Day A,Watson R

更新日期：1995-06-01 00:00:00

abstract：OBJECTIVE:Genetic variants ε2/ε4 within the APOE gene are established risk factors for lobar intracerebral hemorrhage (ICH). Published preliminary data suggest a potential role for APOE ε4 in risk of nonlobar ICH. We therefore investigated the role of APOE in recurrent nonlobar ICH, and sought to clarify whether effect...

journal_title：Neurology

authors： Raffeld MR,Biffi A,Battey TW,Ayres AM,Viswanathan A,Greenberg SM,Rosand J,Anderson CD

更新日期：2015-07-28 00:00:00

abstract：OBJECTIVE:To determine the incidence and clinical effects of antibodies that develop during treatment with natalizumab. METHODS:In two randomized, double-blind, placebo-controlled studies (natalizumab safety and efficacy in relapsing remitting multiple sclerosis [MS, AFFIRM] and safety and efficacy of natalizumab in c...

journal_title：Neurology

authors： Calabresi PA,Giovannoni G,Confavreux C,Galetta SL,Havrdova E,Hutchinson M,Kappos L,Miller DH,O'Connor PW,Phillips JT,Polman CH,Radue EW,Rudick RA,Stuart WH,Lublin FD,Wajgt A,Weinstock-Guttman B,Wynn DR,Lynn F,Panzar

更新日期：2007-10-02 00:00:00

abstract：:Mitochondrial and cytoplasmic malic isoenzymes were assayed fluorometrically in digitonin-fractionated fibroblasts from three patients with Friedreich's ataxia (FA). Normal activity was found, failing to verify an earlier report of reduced fibroblast mitochondrial malic enzyme activity in FA. The previously reported d...

journal_title：Neurology

authors： Fernandez RJ,Civantos F,Tress E,Maltese WA,De Vivo DC

更新日期：1986-06-01 00:00:00

abstract：OBJECTIVE:To evaluate the episodic migraine (EM)-obesity association and the influence of age, race, and sex on this relationship. METHODS:We examined the EM-obesity association and the influence of age, race, and sex in 3,862 adult participants of both black and white race interviewed in the National Comorbidity Surv...

journal_title：Neurology

authors： Peterlin BL,Rosso AL,Williams MA,Rosenberg JR,Haythornthwaite JA,Merikangas KR,Gottesman RF,Bond DS,He JP,Zonderman AB

更新日期：2013-10-08 00:00:00

abstract：:Spontaneous arm levitation (SAL) is well-recognized in cortical-basal ganglionic degeneration, but not in other neurodegenerative diseases. We report six cases of progressive supranuclear palsy (PSP) with SAL. Pathologic examination in four revealed the classic features of PSP with additional areas of focal cortical d...

journal_title：Neurology

authors： Barclay CL,Bergeron C,Lang AE

更新日期：1999-03-10 00:00:00

abstract：BACKGROUND:While stable xenon CT (Xe-CT) cerebral blood flow (CBF) is an accepted standard for quantitative assessment of cerebral hemodynamics, the accuracy of magnetic resonance perfusion-weighted imaging (PWI-MRI) is unclear. The Improved PWI Methodology in Acute Clinical Stroke Study compares PWI findings with Xe-C...

journal_title：Neurology

authors： Olivot JM,Mlynash M,Zaharchuk G,Straka M,Bammer R,Schwartz N,Lansberg MG,Moseley ME,Albers GW

更新日期：2009-03-31 00:00:00

abstract：OBJECTIVE:To determine the incidence of first seizures, epilepsy, and seizure mimics in a geographically defined area using the updated 2014 International League Against Epilepsy (ILAE) definition, which allows an epilepsy diagnosis after a single seizure when the risk of further seizures over the next 10 years is ≈60%...

journal_title：Neurology

authors： Maloney EM,Chaila E,O'Reilly ÉJ,Costello DJ

更新日期：2020-08-04 00:00:00

abstract：:Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types of the protease-resistant form of the prion protein (type 1 and type 2 PrP(Sc)). We investigated 14 cases of sporadic CJD and found that both type 1 and type 2 PrP(Sc) coexisted in 5 subjects. The disti...

journal_title：Neurology

authors： Puoti G,Giaccone G,Rossi G,Canciani B,Bugiani O,Tagliavini F

更新日期：1999-12-10 00:00:00

abstract：:We studied the cerebral effects of oxygen-derived free radicals generated from the xanthine oxidase/hypoxanthine/ADP-Fe3+ system. Xanthine oxidase/hypoxanthine/ADP-Fe3+ solution (0.1 ml) was infused into caudate putamen, and brain was frozen rapidly in situ. Brain water and sodium content increased concomitant with de...

journal_title：Neurology

authors： Chan PH,Schmidley JW,Fishman RA,Longar SM

更新日期：1984-03-01 00:00:00

abstract：BACKGROUND:The American Academy of Neurology (AAN) ALS Practice Parameter was published in April 1999. The ALS CARE Database has been collecting data on the management of patients with ALS in North America since 1996. OBJECTIVE:To compare the management of patients with ALS in North America as recorded in the ALS CARE...

journal_title：Neurology

authors： Bradley WG,Anderson F,Bromberg M,Gutmann L,Harati Y,Ross M,Miller RG,ALS CARE Study Group.

更新日期：2001-08-14 00:00:00

abstract：OBJECTIVE:To investigate the role of alcohol as a causal factor for intracerebral hemorrhage (ICH) and whether its effects might vary according to the pathogenic mechanisms underlying cerebral bleeding. METHODS:We performed a case-control analysis, comparing a cohort of consecutive white patients with ICH aged 55 year...

journal_title：Neurology

authors： Costa P,Grassi M,Iacoviello L,Zedde M,Marcheselli S,Silvestrelli G,DeLodovici ML,Sessa M,Zini A,Paciaroni M,Azzini C,Gamba M,Del Sette M,Toriello A,Gandolfo C,Bonifati DM,Tassi R,Cavallini A,Chiti A,Calabrò RS,Gri

更新日期：2018-07-17 00:00:00

abstract：:The effect of bilateral frontal leukotomy on motor function was evaluated by neurologic examination, tests of praxis, and appropriate neuropsychologic tests administered to five groups of subjects. Postleukotomy schizophrenic patients were subdivided into three degrees of recovery. Two nonleukotomized groups served as...

journal_title：Neurology

authors： Benson DF,Stuss DT

更新日期：1982-12-01 00:00:00

abstract：OBJECTIVE:To investigate the risk factors of neutralizing antibody (NAB)-induced complete secondary treatment failure (cSTF) during long-term botulinum neurotoxin (BoNT) treatment in various neurologic indications. METHODS:This monocenter retrospective cohort study analyzed the data of 471 patients started on BoNT the...

journal_title：Neurology

authors： Walter U,Mühlenhoff C,Benecke R,Dressler D,Mix E,Alt J,Wittstock M,Dudesek A,Storch A,Kamm C

更新日期：2020-05-19 00:00:00

abstract：:We compared CT and MRI findings among 78 Alzheimer's disease (AD), 66 vascular dementia (VaD), and 41 stroke without dementia (SWD) African-American patients to identify possible neuroimaging indicators of dementia. The patients with AD and VaD were generally older and less educated than those with SWD. VaD and SWD pa...

journal_title：Neurology

authors： Charletta D,Gorelick PB,Dollear TJ,Freels S,Harris Y

更新日期：1995-08-01 00:00:00

abstract：:Since cytomegalovirus (CMV) has been implicated in the pathogenesis of Rasmussen's syndrome, we treated four patients with ganciclovir, a potent anti-CMV drug. A 7-year-old girl with seizures escalating to 60/day over 3 months despite triple antiepileptic drug therapy became seizure-free 5 days after initiation of tre...

journal_title：Neurology

authors： McLachlan RS,Levin S,Blume WT

更新日期：1996-10-01 00:00:00