Nearly ubiquitous tissue distribution of the scrapie agent precursor protein.

abstract：:In a series of 100 patients exhibiting clinical and molecular features of facioscapulohumeral muscular dystrophy (FSHMD), five patients had conduction defects or arrhythmia in the absence of cardiovascular risk factors--namely, intraventricular conduction delay and supraventricular arrhythmia induced by electrophysiol...

journal_title：Neurology

authors： Laforêt P,de Toma C,Eymard B,Becane HM,Jeanpierre M,Fardeau M,Duboc D

更新日期：1998-11-01 00:00:00

abstract：:We studied a patient with autopsy-proven Hallervorden-Spatz syndrome (HSS) and the previously unreported finding of high-density lesions in the basal ganglia on CT. The diagnosis of HSS should be considered in a patient with dystonia and basal ganglia mineralization on CT. ...

journal_title：Neurology

authors： Tennison MB,Bouldin TW,Whaley RA

更新日期：1988-01-01 00:00:00

abstract：OBJECTIVE:To investigate the efficacy and safety of aceneuramic acid extended-release (Ace-ER), a treatment intended to replace deficient sialic acid, in patients with GNE myopathy. METHODS:UX001-CL301 was a phase 3, double-blind, placebo-controlled, randomized, international study evaluating the efficacy and safety o...

journal_title：Neurology

authors： Lochmüller H,Behin A,Caraco Y,Lau H,Mirabella M,Tournev I,Tarnopolsky M,Pogoryelova O,Woods C,Lai A,Shah J,Koutsoukos T,Skrinar A,Mansbach H,Kakkis E,Mozaffar T

更新日期：2019-04-30 00:00:00

abstract：OBJECTIVES:To evaluate the frequency, clinical and etiologic features, and short- and long-term outcomes of early recurrent TIA. METHODS:This prospective observational cohort study enrolled all consecutive patients with TIA referred to our emergency department and diagnosed by a vascular neurologist. Expedited assessm...

journal_title：Neurology

authors： Foschi M,Pavolucci L,Rondelli F,Spinardi L,Favaretto E,Filippini M,Degli Esposti D,Strocchi E,Faggioli G,Cortelli P,Guarino M,Bologna TIA Study-Group.

更新日期：2020-09-22 00:00:00

abstract：OBJECTIVE:To investigate the pathogenesis of proximal diabetic neuropathy (PDN) with nerve and muscle biopsies. BACKGROUND:Recent evidence suggests that nerve ischemia secondary to immune-mediated vasculopathy rather than diabetic microangiopathy may be responsible for PDN. METHOD:- Fifteen patients with PDN and two ...

journal_title：Neurology

authors： Kelkar P,Masood M,Parry GJ

更新日期：2000-07-12 00:00:00

abstract：:Six patients with Parkinson's disease, six patients with essential or intention tremor, and nine controls were tested on a step-tracking task using a joystick control and oscilloscope display. Tremor subjects resembled controls in making an initial ballistic movement followed by (defective) corrections, and took longe...

journal_title：Neurology

authors： Flowers K

更新日期：1975-05-01 00:00:00

abstract：:We evaluated four formulas for estimating the intra-blood-brain barrier (IBBB) synthesis of IgG in a computer model of two types of BBB damage and in 1,629 consecutively received pairs of CSF and serum. These four formulas were the IgG synthesis rate (SR), IgG(loc) (ie, local), IgG index, and IgG extended index. Resul...

journal_title：Neurology

authors： Peter JB,Bowman RL

更新日期：1992-03-01 00:00:00

abstract：:When multiple sclerosis (MS) presents late in life (after the age of 50), it frequently gives diagnostic difficulty. In a large MS clinic population (N = 838), 9.4% of the patients had late onset of MS. Slow deterioration of motor function characterized the initial symptoms and subsequent course of this group of older...

journal_title：Neurology

authors： Noseworthy J,Paty D,Wonnacott T,Feasby T,Ebers G

更新日期：1983-12-01 00:00:00

abstract：OBJECTIVE:To examine the lifetime prevalence and the concordance risk of cluster headache (CH) in a twin sample representative of the Swedish general population. METHODS:The authors assessed CH as defined by the second edition of the International Classification of Headache Disorders in 31,750 registered twins born fr...

journal_title：Neurology

authors： Ekbom K,Svensson DA,Pedersen NL,Waldenlind E

更新日期：2006-09-12 00:00:00

abstract：BACKGROUND:Studies have suggested that mood status is associated with an increased risk of stroke, though mostly based on measures of depression defined by symptoms alone rather than diagnostic criteria representative of clinically important distress and impairment. We investigated this association based upon a large p...

journal_title：Neurology

authors： Surtees PG,Wainwright NW,Luben RN,Wareham NJ,Bingham SA,Khaw KT

更新日期：2008-03-04 00:00:00

abstract：OBJECTIVE:This study explored the association between long-term epilepsy surgery outcome and changes in depressive symptoms. METHODS:Adults were enrolled between 1996 and 2001 in a multicenter prospective study to evaluate outcomes of resective epilepsy surgery. The extent of depressive symptoms and depression case st...

journal_title：Neurology

authors： Hamid H,Liu H,Cong X,Devinsky O,Berg AT,Vickrey BG,Sperling MR,Shinnar S,Langfitt JT,Walczak TS,Barr WB,Dziura J,Bazil CW,Spencer SS

更新日期：2011-11-29 00:00:00

abstract：BACKGROUND:There is controversy over whether seizures, the most common manifestation of neonatal brain injury, may themselves damage the developing brain. OBJECTIVE:To determine if neonatal seizures are independently associated with brain injury in newborns with perinatal asphyxia. METHODS:Ninety term neonates were s...

journal_title：Neurology

authors： Miller SP,Weiss J,Barnwell A,Ferriero DM,Latal-Hajnal B,Ferrer-Rogers A,Newton N,Partridge JC,Glidden DV,Vigneron DB,Barkovich AJ

更新日期：2002-02-26 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:Several lines of evidence suggest that pathologic changes underlying Alzheimer disease (AD) begin years prior to the clinical expression of the disease, underscoring the need for studies of cognitively healthy adults to capture these early changes. The overall goal of the current study was to map the cortical...

journal_title：Neurology

authors： Rodrigue KM,Kennedy KM,Devous MD Sr,Rieck JR,Hebrank AC,Diaz-Arrastia R,Mathews D,Park DC

更新日期：2012-02-07 00:00:00

abstract：:Between 1982 and 1989, 197 patients with Parkinson's disease were treated with selegiline and levodopa for at least 0.3 years. Two groups of patients were included: a group of 65 patients (stage 2 or 3 on the Hoehn and Yahr Scale) who recently had been started on levodopa and were not experiencing response fluctuation...

journal_title：Neurology

authors： Lieberman A

更新日期：1992-04-01 00:00:00

abstract：:T lymphocytes may play a central role in MS. The search for more targeted immunosuppression than is currently available has led to recent clinical trials of novel therapeutics. We studied 29 patients in a double-blind placebo-controlled trial of the chimeric monoclonal anti-CD4 antibody, cM-T412 (Centocor, Leiden, Hol...

journal_title：Neurology

authors： Llewellyn-Smith N,Lai M,Miller DH,Rudge P,Thompson AJ,Cuzner ML

更新日期：1997-04-01 00:00:00

abstract：:MRI and CSF investigations revealed meningeal involvement in a 29-year-old patient with biopsy-confirmed Wegener's granulomatosis. The intracranial manifestation of Wegener's granulomatosis was supported by the detection of pathologic circulating antineutrophil cytoplasm (c-ANCA) in the CSF. We monitored disease activ...

journal_title：Neurology

authors： Spranger M,Schwab S,Meinck HM,Tischendorf M,Sis J,Breitbart A,Andrassy K

更新日期：1997-01-01 00:00:00

abstract：:The clinical and genetic findings are described for 16 patients from a large Italian family with a variant form of hereditary spastic paraplegia and congenital arachnoid cysts inherited as an autosomal dominant trait. A molecular study has revealed a novel missense mutation, T614I, in exon 17 of SPG4, which may play a...

journal_title：Neurology

authors： Orlacchio A,Gaudiello F,Totaro A,Floris R,St George-Hyslop PH,Bernardi G,Kawarai T

更新日期：2004-05-25 00:00:00

abstract：BACKGROUND:The prevalence of symptoms suggesting distal symmetric polyneuropathy (DSP) was reported to be higher among deployed veterans (DV) to the Persian Gulf in 1990-1991 than to control non-deployed veterans (NDV). The authors therefore compared the prevalence of DSP by direct examination of DV and their spouses t...

journal_title：Neurology

authors： Davis LE,Eisen SA,Murphy FM,Alpern R,Parks BJ,Blanchard M,Reda DJ,King MK,Mithen FA,Kang HK

更新日期：2004-09-28 00:00:00

abstract：BACKGROUND:To assess the temporal trend in multiple sclerosis (MS) onset during the last 50 years in Sardinia, Italy. METHODS:The authors used a cohort study to assess age at onset in 1,513 MS patients at the MS clinic in Cagliari, all born and living in Sardinia. They also assessed age at onset in 41 pairs of familia...

journal_title：Neurology

authors： Cocco E,Sardu C,Lai M,Spinicci G,Contu P,Marrosu MG

更新日期：2004-05-25 00:00:00

abstract：OBJECTIVE:To perform whole-brain morphometry in patients with frontal lobe epilepsy and evaluate the utility of group-level patterns for individualized diagnosis and prognosis. METHODS:We compared MRI-based cortical thickness and folding complexity between 2 frontal lobe epilepsy cohorts with histologically verified f...

journal_title：Neurology

authors： Hong SJ,Bernhardt BC,Schrader DS,Bernasconi N,Bernasconi A

更新日期：2016-02-16 00:00:00

abstract：:The authors recruited 19 nonambulant patients with Guillain-Barré syndrome into a pilot, double-blind, randomized, placebo-controlled safety trial of interferon beta 1a (IFN[beta]-1a) (Rebif). Participants received IFN[beta]-1a or placebo subcutaneously three times weekly, 22 microg for the first week and then 44 micr...

journal_title：Neurology

authors： Pritchard J,Gray IA,Idrissova ZR,Lecky BR,Sutton IJ,Swan AV,Willison HJ,Winer JB,Hughes RA

更新日期：2003-11-11 00:00:00

abstract：:Stenosis or occlusion of the major arteries of the head and neck may cause hemodynamic impairment of the distal cerebral circulation. Hemodynamic factors may play an important role in the pathogenesis of ischemic stroke for patients with cerebrovascular disease. Several neuroimaging methods are currently available for...

journal_title：Neurology

authors： Derdeyn CP,Grubb RL Jr,Powers WJ

更新日期：1999-07-22 00:00:00

abstract：:A patient with thymoma-associated neuromyotonia and voltage-gated potassium channel (Kv1.2 and Kv1.6) antibodies by immunoprecipitation and rat brain immunolabeling was treated successfully with immunoadsorption and cyclophosphamide. Curiously, glutamic acid decarboxylase antibodies, absent at onset, appeared later. S...

journal_title：Neurology

authors： Antozzi C,Frassoni C,Vincent A,Regondi MC,Andreetta F,Bernasconi P,Ciano C,Chang T,Cornelio F,Spreafico R,Mantegazza R

更新日期：2005-04-12 00:00:00

abstract：:The authors studied presymptomatic individuals with the Huntington disease (HD) mutation to determine whether cortical thinning was present. They found thinning that was regionally selective, semi-independent of striatal volume loss, and correlated with cognitive performance. Early, extensive cortical involvement occu...

journal_title：Neurology

authors： Rosas HD,Hevelone ND,Zaleta AK,Greve DN,Salat DH,Fischl B

更新日期：2005-09-13 00:00:00

abstract：OBJECTIVE:To compare the accuracy of Alberta Stroke Program Early Computed Tomography Score (ASPECTS) and CT perfusion to detect established infarction in acute anterior circulation stroke. METHODS:We performed an observational study in 59 acute anterior circulation ischemic stroke patients who underwent brain noncont...

journal_title：Neurology

authors： Demeestere J,Garcia-Esperon C,Garcia-Bermejo P,Ombelet F,McElduff P,Bivard A,Parsons M,Levi C

更新日期：2017-06-13 00:00:00

abstract：:We studied 15 dystonic patients with positron emission tomography (PET) and (18F)-2-fluoro-2-deoxy-D-glucose (FDG). The group comprised patients with focal (n = 5), multifocal (n = 1), and generalized (n = 4) dystonia as well as patients with hemidystonia (n = 5). The age at onset was during childhood in four, during ...

journal_title：Neurology

authors： Karbe H,Holthoff VA,Rudolf J,Herholz K,Heiss WD

更新日期：1992-08-01 00:00:00

abstract：OBJECTIVE:To study the effect of pregnancy on the frequency of neuromyelitis optica spectrum disorder (NMOSD) relapse and evaluate rates of pregnancy-related complications in an international multicenter setting. METHODS:We administered a standardized survey to 217 women with NMOSD from 7 medical centers and reviewed ...

journal_title：Neurology

authors： Klawiter EC,Bove R,Elsone L,Alvarez E,Borisow N,Cortez M,Mateen F,Mealy MA,Sorum J,Mutch K,Tobyne SM,Ruprecht K,Buckle G,Levy M,Wingerchuk D,Paul F,Cross AH,Jacobs A,Chitnis T,Weinshenker B

更新日期：2017-11-28 00:00:00

abstract：OBJECTIVE:To present the current terminology and natural history of neurofibromatosis 1 (NF1) cutaneous neurofibromas (cNF). METHODS:NF1 experts from various research and clinical backgrounds reviewed the terms currently in use for cNF as well as the clinical, histologic, and radiographic features of these tumors usin...

journal_title：Neurology

authors： Ortonne N,Wolkenstein P,Blakeley JO,Korf B,Plotkin SR,Riccardi VM,Miller DC,Huson S,Peltonen J,Rosenberg A,Carroll SL,Verma SK,Mautner V,Upadhyaya M,Stemmer-Rachamimov A

更新日期：2018-07-10 00:00:00

abstract：:Twenty-nine untreated patients diagnosed with probable AD and 15 control patients underwent two or more clinical and volumetric MRI assessments with intervals ranging from 5 months to 6 years. The change in global cerebral volume for an individual was calculated by a novel method of registration and subtraction of ser...

journal_title：Neurology

authors： Fox NC,Scahill RI,Crum WR,Rossor MN

更新日期：1999-05-12 00:00:00