Prednisone/prednisolone and deflazacort regimens in the CINRG Duchenne Natural History Study.

abstract：OBJECTIVE:Clinical trials of fetal neural tissue transplantation for Huntington disease (HD) have been conducted with variable clinical results. However, no long-term analysis of graft survival and integration has been published. Here, we report the pathologic findings in two patients with HD who died 74 and 79 months ...

journal_title：Neurology

authors： Keene CD,Sonnen JA,Swanson PD,Kopyov O,Leverenz JB,Bird TD,Montine TJ

更新日期：2007-06-12 00:00:00

abstract：:A 71-year-old man developed severe limb, bulbar, and respiratory weakness over 18 months. A muscle biopsy showed only a moderate degree of type 2 atrophy, but immunocytochemistry showed absence of chondroitin sulfate C glycosaminoglycan in the endomysium. Prednisone produced a marked increase in strength. Diffuse loss...

journal_title：Neurology

authors： al-Lozi MT,Hemelt VB,Pestronk A

更新日期：1998-02-01 00:00:00

abstract：BACKGROUND:The topodiagnostic value and specificity of nystagmus in patients with mesencephalic lesions and its relation to tonic torsional deficits and vertical saccade deficits is controversial and anecdotal. METHODS:The authors examined 11 patients with vascular MRI-identified mesencephalic lesions and clinical evi...

journal_title：Neurology

authors： Helmchen C,Rambold H,Kempermann U,Büttner-Ennever JA,Büttner U

更新日期：2002-12-24 00:00:00

abstract：:Two successive, 6-month, randomized, double-blind, controlled trials of prednisone showed that 0.75 mg/kg/d was the optimal dose to improve strength in boys with Duchenne muscular dystrophy (DMD). We attempted to maintain 93 boys on that dose for an additional 2 years. During the 3 years of observation, the decline in...

journal_title：Neurology

authors： Fenichel GM,Florence JM,Pestronk A,Mendell JR,Moxley RT 3rd,Griggs RC,Brooke MH,Miller JP,Robison J,King W

更新日期：1991-12-01 00:00:00

abstract：BACKGROUND:Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with complicated pathogenesis that poses challenges with respect to diagnosis and monitoring of disease progression. OBJECTIVES:To identify a biomarker panel that elucidates ALS disease pathogenesis, distinguishes patients with A...

journal_title：Neurology

authors： Mitchell RM,Freeman WM,Randazzo WT,Stephens HE,Beard JL,Simmons Z,Connor JR

更新日期：2009-01-06 00:00:00

abstract：OBJECTIVE:To examine the association between Miller Fisher syndrome (MFS) and antecedent Haemophilus influenzae infection. BACKGROUND:Little is known about agents in prior respiratory tract infection of MFS, whereas antecedent upper respiratory symptoms are frequent. H. influenzae is a major pathogen that can cause hu...

journal_title：Neurology

authors： Koga M,Yuki N,Tai T,Hirata K

更新日期：2001-08-28 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：:In an attempt to determine the opinions and practices of neurologists regarding the use of antithrombotic drugs in the treatment of acute ischemic stroke, we conducted a survey of 349 randomly selected neurologists from throughout the United States. Responses were received from 247 (71%), and 219 identified themselves...

journal_title：Neurology

authors： Marsh EE 3rd,Adams HP Jr,Biller J,Wasek P,Banwart K,Mitchell V,Woolson R

更新日期：1989-12-01 00:00:00

abstract：BACKGROUND:The relationship between age-related memory decline and hippocampal anatomic changes is a matter of debate. OBJECTIVES:To investigate the relationship between age-related memory decline and MRI hippocampal anatomic changes in a cohort of healthy individuals. METHODS:In this cross-sectional study, 76 health...

journal_title：Neurology

authors： Carlesimo GA,Cherubini A,Caltagirone C,Spalletta G

更新日期：2010-01-19 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：OBJECTIVE:To assess the determinants of amyotrophic lateral sclerosis (ALS) phenotypes in a population-based cohort. METHODS:The study population included 2,839 patients with ALS diagnosed in Piemonte, Italy (1995-2015). Patients were classified according to motor (classic, bulbar, flail arm, flail leg, predominantly ...

journal_title：Neurology

authors： Chiò A,Moglia C,Canosa A,Manera U,D'Ovidio F,Vasta R,Grassano M,Brunetti M,Barberis M,Corrado L,D'Alfonso S,Iazzolino B,Peotta L,Sarnelli MF,Solara V,Zucchetti JP,De Marchi F,Mazzini L,Mora G,Calvo A

更新日期：2020-02-25 00:00:00

abstract：:Patients with Parkinson's disease (PD) commonly complain of impaired visual function and difficulty reading, despite normal visual acuity. Although previous studies have evaluated contrast sensitivity, color vision, visuospatial processing, visual hallucinations, and ocular movements, none has systematically evaluated...

journal_title：Neurology

authors： Biousse V,Skibell BC,Watts RL,Loupe DN,Drews-Botsch C,Newman NJ

更新日期：2004-01-27 00:00:00

abstract：:Age at 1st risk for seizures may predict anomia following dominant anterior temporal lobectomy. We assessed confrontation naming before and 2 to 3 weeks after surgery in 45 right-handed patients grouped by side of focus and presence or absence of early (less than or equal to 5 years) risk factors. After left lobectomy...

journal_title：Neurology

authors： Stafiniak P,Saykin AJ,Sperling MR,Kester DB,Robinson LJ,O'Connor MJ,Gur RC

更新日期：1990-10-01 00:00:00

abstract：OBJECTIVE:To determine the diagnostic value of high-resolution ultrasound (HRUS) for detection of chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis-Sumner syndrome (LSS), and multifocal motor neuropathy (MMN). METHODS:Between January 2013 and January 2015, we enrolled 75 consecutive treatment-naive patie...

journal_title：Neurology

authors： Goedee HS,van der Pol WL,van Asseldonk JH,Franssen H,Notermans NC,Vrancken AJ,van Es MA,Nikolakopoulos S,Visser LH,van den Berg LH

更新日期：2017-01-10 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：OBJECTIVE:To investigate whether the correlation of age and sex with the risk of recurrence and death seen in patients with previous ischemic stroke is also evident in patients with embolic stroke of undetermined source (ESUS). METHODS:We pooled datasets of 11 stroke registries from Europe and America. ESUS was define...

journal_title：Neurology

authors： Ntaios G,Lip GYH,Vemmos K,Koroboki E,Manios E,Vemmou A,Rodríguez-Campello A,Cuadrado-Godia E,Roquer J,Arnao V,Caso V,Paciaroni M,Diez-Tejedor E,Fuentes B,Pérez Lucas J,Arauz A,Ameriso SF,Pertierra L,Gómez-Schneider M

更新日期：2017-08-08 00:00:00

abstract：OBJECTIVE:To investigate possible correlations between tumor location and genetic alterations in a series of oligodendrogliomas. METHODS:A series of 158 consecutive oligodendrogliomas were retrospectively reviewed. In each case, the radiologic picture and the chromosome 1p (chr 1p) status of the tumor detected by the ...

journal_title：Neurology

authors： Laigle-Donadey F,Martin-Duverneuil N,Lejeune J,Crinière E,Capelle L,Duffau H,Cornu P,Broët P,Kujas M,Mokhtari K,Carpentier A,Sanson M,Hoang-Xuan K,Thillet J,Delattre JY

更新日期：2004-12-28 00:00:00

abstract：OBJECTIVE:To examine the ability of diffusion-weighted MRI (DWI) to detect ongoing cerebral ischemia in patients with vascular dementia (VaD). BACKGROUND:VaD due to small-vessel disease results from the cumulative impact of recurrent cerebral ischemia. Cerebral ischemia may produce clinical manifestations, producing t...

journal_title：Neurology

authors： Choi SH,Na DL,Chung CS,Lee KH,Na DG,Adair JC

更新日期：2000-01-11 00:00:00

abstract：OBJECTIVE:To describe the detection frequency and clinical associations of immunoglobulin G (IgG) targeting dipeptidyl-peptidase-like protein-6 (DPPX), a regulatory subunit of neuronal Kv4.2 potassium channels. METHODS:Specimens from 20 patients evaluated on a service basis by tissue-based immunofluorescence yielded a...

journal_title：Neurology

authors： Tobin WO,Lennon VA,Komorowski L,Probst C,Clardy SL,Aksamit AJ,Appendino JP,Lucchinetti CF,Matsumoto JY,Pittock SJ,Sandroni P,Tippmann-Peikert M,Wirrell EC,McKeon A

更新日期：2014-11-11 00:00:00

abstract：:[Methyl-3H]epsilon-N-trimethyl-L-lysine was administered to patients with primary systemic carnitine deficiency and to controls. In both groups, labeled carnitine appeared in blood and urine within 2 hours, and the specific radioactivity of urinary carnitine peaked between 2 and 6 hours. The specific radioactivity of ...

journal_title：Neurology

authors： Rebouche CJ,Engel AG

更新日期：1981-07-01 00:00:00

abstract：:A 53-year-old man presented with a painful, demyelinating sensorimotor peripheral neuropathy with lymphomatous infiltration on sural nerve biopsy, but no evidence of systemic lymphoma. The neuropathy responded to cytotoxic therapy. Seven years later he developed generalized lymphadenopathy due to B cell lymphoplasmacy...

journal_title：Neurology

authors： Ince PG,Shaw PJ,Fawcett PR,Bates D

更新日期：1987-07-01 00:00:00

abstract：:We assessed the ability to recognize epileptiform abnormalities on a video review of ambulatory cassette EEGs (A/EEG) by comparing two independent interpretations of an A/EEG to those derived from a routine recording and intensive monitoring by cable telemetry (C/EEG) of the same 40 patients. Both A/EEG readings concu...

journal_title：Neurology

authors： Ebersole JS,Leroy RF

更新日期：1983-07-01 00:00:00

abstract：:Cross-sectional studies have not adequately resolved the question of whether subjects infected with HIV-1 may suffer cognitive decline during the early, asymptomatic stages of the infection. We studied longitudinally 238 asymptomatic healthy HIV-1-infected homosexual/bisexual men (CDC groups 2 and 3) and 170 uninfecte...

journal_title：Neurology

authors： Selnes OA,Miller E,McArthur J,Gordon B,Muñoz A,Sheridan K,Fox R,Saah AJ

更新日期：1990-02-01 00:00:00

abstract：BACKGROUND:Two recent studies suggest that the risk of ALS is increased among Gulf War veterans. It is not known whether military service outside of the Gulf War is associated with increased risk of ALS. METHODS:The authors prospectively assessed the relation between service in the military and ALS mortality among par...

journal_title：Neurology

authors： Weisskopf MG,O'Reilly EJ,McCullough ML,Calle EE,Thun MJ,Cudkowicz M,Ascherio A

更新日期：2005-01-11 00:00:00

abstract：:We studied 10 patients with transient global amnesia (TGA). Routine electroencephalograms (EEGs) were normal in six; two showed mild temporal lobe abnormalities, one had bitemporal delta activity with rare sharp waves, and one showed bioccipital rhythmic theta activity. Nasopharyngeal (NP) recordings were obtained in ...

journal_title：Neurology

authors： Rowan AJ,Protass LM

更新日期：1979-06-01 00:00:00

abstract：:We investigated levodopa intravenous infusion rates, plasma concentrations, and clinical responses in 23 parkinsonian patients with a fluctuating response to the drug. There was a linear relationship between rate of levodopa infusion and plasma concentration when carbidopa was coadministered. Minimum-therapeutically e...

journal_title：Neurology

authors： Nutt JG,Woodward WR

更新日期：1986-06-01 00:00:00

abstract：:A 60-year-old patient suffered a lethal hemispheric infarction 3 days after angiographically documented occlusion of the ipsilateral cervical internal carotid artery and while receiving anticoagulant therapy. Pathologic evidence is consistent with embolism from the distal "tail" of a propagated carotid thrombus as the...

journal_title：Neurology

authors： Finklestein S,Kleinman GM,Cuneo R,Baringer JR

更新日期：1980-01-01 00:00:00

abstract：OBJECTIVE:To describe the clinical features of cardiac injury associated with neurogenic pulmonary edema (NPE) in patients with acute subarachnoid hemorrhage (SAH). BACKGROUND:NPE is generally viewed as a form of noncardiogenic pulmonary edema related to massive sympathetic discharge. METHODS:Case series. RESULTS:We...

journal_title：Neurology

authors： Mayer SA,Fink ME,Homma S,Sherman D,LiMandri G,Lennihan L,Solomon RA,Klebanoff LM,Beckford A,Raps EC

更新日期：1994-05-01 00:00:00

abstract：:Many studies have demonstrated that dipyridamole is not purely an antiplatelet agent but also exerts its antithrombotic properties through the vessel wall. Using a laboratory model in which bovine endothelial cells are seeded to form a subendothelial matrix (SEM), investigators have shown that dipyridamole enhances th...

journal_title：Neurology

authors： Eisert WG

更新日期：2001-01-01 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