Deficiency of syntrophin, dystroglycan, and merosin in a female infant with a congenital muscular dystrophy phenotype lacking cysteine-rich and C-terminal domains of dystrophin.

abstract：OBJECTIVE:To assess the effect of patient and drug characteristics on medication adherence in people with epilepsy (PWE) in a large cohort representative of the German population. METHODS:Information was obtained from the Disease Analyzer database that collects anonymous demographic and medical data from practice comp...

journal_title：Neurology

authors： Gollwitzer S,Kostev K,Hagge M,Lang J,Graf W,Hamer HM

更新日期：2016-08-02 00:00:00

abstract：:Outcome after carpal tunnel surgery was studied retrospectively in 32 patients with peripheral neuropathy and carpal tunnel syndrome. Nocturnal paresthesias were almost universally relieved, followed in order of responsiveness by pain, numbness, and weakness. Twenty-five of 28 patients said they would have the surgery...

journal_title：Neurology

authors： Morgenlander JC,Lynch JR,Sanders DB

更新日期：1997-10-01 00:00:00

abstract：:The malformation of inverse cerebellum and occipital encephalocele is situated morphologically between the Arnold-Chiari and Dandy-Walker malformations. In the three reported cases, hydrocephalus was not present, concomitant malformations of the lamina terminalis were present in two, and polymicrogyria was found in al...

journal_title：Neurology

authors： Smith MT,Huntington HW

更新日期：1977-03-01 00:00:00

abstract：:A 59-year-old woman with levodopa-responsive parkinsonism complicated by motor fluctuations and generalized levodopa dyskinesia underwent bilateral subthalamic deep brain stimulation (STN DBS) 7 years after symptom onset. DBS improved levodopa-responsive upper extremity bradykinesia but aggravated speech, swallowing, ...

journal_title：Neurology

authors： Tarsy D,Apetauerova D,Ryan P,Norregaard T

更新日期：2003-07-22 00:00:00

abstract：:We retrospectively studied the effects of changing antiepileptic drug levels on patterns of seizure discharge in 8 patients investigated with intracerebral electrodes during presurgical evaluation. We compared seizures recorded at high levels of medication to seizures recorded at low levels for changes in seizure dura...

journal_title：Neurology

authors： So N,Gotman J

更新日期：1990-03-01 00:00:00

abstract：:The term "Wernicke's area" is most often used as an anatomical label for the gyri forming the lower posterior left sylvian fissure. Although traditionally this region was held to support language comprehension, modern imaging and neuropsychological studies converge on the conclusion that this region plays a much large...

journal_title：Neurology

authors： Binder JR

更新日期：2015-12-15 00:00:00

abstract：:The administration of lecithin has failed to improve memory in aged or demented individuals. We studied the effect of lecithin on another cholinergic function, REM sleep latency. Intravenous doses of physostigmine shortened the latency to the first REM sleep period, but oral lecithin did not. In contrast to physostigm...

journal_title：Neurology

authors： Kapen S,Fleming PD,Drachman DA

更新日期：1986-08-01 00:00:00

abstract：OBJECTIVE:To study activin signaling and its blockade in sporadic inclusion body myositis (sIBM) through translational studies and a randomized controlled trial. METHODS:We measured transforming growth factor β signaling by SMAD2/3 phosphorylation in muscle biopsies of 50 patients with neuromuscular disease (17 with s...

journal_title：Neurology

authors： Amato AA,Sivakumar K,Goyal N,David WS,Salajegheh M,Praestgaard J,Lach-Trifilieff E,Trendelenburg AU,Laurent D,Glass DJ,Roubenoff R,Tseng BS,Greenberg SA

更新日期：2014-12-09 00:00:00

abstract：:A woman had a progressive neurologic syndrome beginning at age 3 and lasting for three decades. Clinical manifestations included severe mental deterioration, spastic paralysis, myoclonus, and tremors. A postmortem examination showed ubiquitous infiltration of neurons by lipofuscin and deposits of pigment in the globus...

journal_title：Neurology

authors： Jervis GA,Pullarkat RK

更新日期：1978-05-01 00:00:00

abstract：OBJECTIVES:To study the behavioral and neuroanatomic characteristics of perseverative behavior encountered on a target cancellation task in patients with neglect. METHODS:Motor perseverative behavior during line cancellation task was evaluated retrospectively in 60 patients with left hemispatial neglect from right hem...

journal_title：Neurology

authors： Na DL,Adair JC,Kang Y,Chung CS,Lee KH,Heilman KM

更新日期：1999-05-12 00:00:00

abstract：OBJECTIVE:Compare the cost and effectiveness of nonbenzodiazepine antiepileptic drugs (non-BZD AEDs) for treatment of BZD-resistant convulsive status epilepticus (SE). METHODS:Decision analysis model populated with effectiveness data from a systematic review and meta-analysis of the literature, and cost data from publ...

journal_title：Neurology

authors： Sánchez Fernández I,Gaínza-Lein M,Lamb N,Loddenkemper T

更新日期：2019-05-14 00:00:00

abstract：:Assay of anti-striated muscle antibodies (StrAb) is used as a diagnostic test for thymoma in individuals with myasthenia gravis. Unfortunately, false positive results are common, and their effect on test accuracy is magnified by the relatively low frequency of thymoma in myasthenics. We estimated the predictive values...

journal_title：Neurology

authors： Lanska DJ

更新日期：1991-04-01 00:00:00

abstract：OBJECTIVE:To determine whether longitudinal cognitive decline is associated with increased risk of sleep disturbance in older, nondemented, community-dwelling women. METHODS:We studied 2,474 women (mean age 68.9 years) who were part of a prospective study started in 1986; women with baseline or follow-up evidence of p...

journal_title：Neurology

authors： Yaffe K,Blackwell T,Barnes DE,Ancoli-Israel S,Stone KL,Study of Osteoporotic Fractures Group.

更新日期：2007-07-17 00:00:00

abstract：OBJECTIVE:Because age affects hormonal production differently in women compared with men, we sought to define sex and age interactions across a multiracial/ethnic population after intracerebral hemorrhage (ICH) to uncover evidence that loss of gonadal hormone production would result in loss of the known neuroprotective...

journal_title：Neurology

authors： James ML,Langefeld CD,Sekar P,Moomaw CJ,Elkind MSV,Worrall BB,Sheth KN,Martini SR,Osborne J,Woo D,ERICH Investigators.

更新日期：2017-09-05 00:00:00

abstract：:Seizures are not expected in progressive multifocal leukoencephalopathy (PML), a condition considered to be restricted to the white matter. Review of medical records of 89 patients with possible or proven PML showed an 18% prevalence of seizures. Seizures usually responded well to treatment and did not affect survival...

journal_title：Neurology

authors： Lima MA,Drislane FW,Koralnik IJ

更新日期：2006-01-24 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 examine associated demographic and clinical correlates and the development of excessive daytime sleepiness (EDS) over 8 years in a community-based cohort of patients with Parkinson disease (PD). METHODS:A total of 232 patients with PD were included in a population-based prevalence study in 1993. Patients ...

journal_title：Neurology

authors： Gjerstad MD,Alves G,Wentzel-Larsen T,Aarsland D,Larsen JP

更新日期：2006-09-12 00:00:00

abstract：:Until 1995, treatment of strokes consisted exclusively of efforts to prevent recurrence. Proof that tissue plasminogen activator is useful for acute management is changing the approach to stroke patients. The development process has been difficult and the current treatment recommendations have been controversial. Rece...

journal_title：Neurology

authors： Zivin JA

更新日期：1999-07-13 00:00:00

abstract：OBJECTIVE:To determine the prevalence of developmental venous anomaly in adult patients with diffuse glioma. METHODS:We performed a retrospective cohort study (2010-2016) of consecutive adult patients harboring a supratentorial diffuse glioma in 2 centers: Sainte-Anne Hospital (experimental and control sets) and Pitié...

journal_title：Neurology

authors： Roux A,Edjlali M,Porelli S,Tauziede-Espariat A,Zanello M,Dezamis E,Zah-Bi G,Sanson M,Puget S,Capelle L,Varlet P,Oppenheim C,Pallud J

更新日期：2019-01-01 00:00:00

abstract：BACKGROUND:Ependymoma is a rare type of glioma, representing 5% of all CNS malignancies. Radiotherapy (RT) is commonly administered, but there is no standard chemotherapy. At recurrence, ependymoma is notoriously refractory to therapy and the prognosis is poor. In recurrent glioblastoma, encouraging responses with beva...

journal_title：Neurology

authors： Green RM,Cloughesy TF,Stupp R,DeAngelis LM,Woyshner EA,Ney DE,Lassman AB

更新日期：2009-11-17 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：: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：:The aim of this randomized, double-blind, placebo-controlled cross-over study was to test whether valproic acid relieves painful polyneuropathy. Thirty-one patients completed two treatment phases of 4 weeks' duration with valproic acid (1,500 mg daily) and placebo. There was no significant difference between valproic ...

journal_title：Neurology

authors： Otto M,Bach FW,Jensen TS,Sindrup SH

更新日期：2004-01-27 00:00:00

abstract：OBJECTIVE:To analyze the spectrum and mechanisms of cutaneous adverse events (AEs) in patients with multiple sclerosis treated with daclizumab high-yield process (DAC-HYP). METHODS:A total of 31 participants in an institutional review board-approved open-label phase I study of DAC-HYP (NCT01143441) were prospectively ...

journal_title：Neurology

authors： Cortese I,Ohayon J,Fenton K,Lee CC,Raffeld M,Cowen EW,DiGiovanna JJ,Bielekova B

更新日期：2016-03-01 00:00:00

abstract：BACKGROUND:The best treatment for polyneuropathy associated with IgM monoclonal gammopathy (MGUS) is unknown. Oral cyclophosphamide combined with prednisone showed limited efficacy in a previous open label pilot study. We therefore performed a double-blind, randomized, placebo-controlled study of combined oral cyclopho...

journal_title：Neurology

authors： Niermeijer JM,Eurelings M,van der Linden MW,Lokhorst HM,Franssen H,Fischer K,Teunissen LL,van den Berg LH,Schobben F,Wokke JH,Notermans NC

更新日期：2007-07-03 00:00:00

abstract：:A 17-month-old boy developed herpes zoster ophthalmicus (HZO) and delayed contralateral hemiparesis following intrauterine varicella exposure. CT demonstrated multiple areas of hypodensity in the left basal ganglia, and angiography showed occlusion of left lenticulostriate arteries. As in most adults with HZO and dela...

journal_title：Neurology

authors： Leis AA,Butler IJ

更新日期：1987-09-01 00:00:00

abstract：OBJECTIVE:Frontotemporal lobar degeneration (FTLD) encompasses a variety of clinicopathologic entities. The antemortem prediction of the underlying pathologic lesions is reputed to be difficult. This study sought to characterize correlations between 1) the different clinical variants of primary progressive language and...

journal_title：Neurology

authors： Deramecourt V,Lebert F,Debachy B,Mackowiak-Cordoliani MA,Bombois S,Kerdraon O,Buée L,Maurage CA,Pasquier F

更新日期：2010-01-05 00:00:00

abstract：:Cytolytic T lymphocyte precursors (CTL-p) and natural killer precursors (NK-p) in the CSF of 15 MS patients and 11 patients with other neurologic disorders (OND) were quantitatively assessed, using a T-lymphocyte microculture system that allows clonal expansion of all human T cells. CSF CTL-p and NK-p frequencies were...

journal_title：Neurology

authors： Weber WE,Buurman WA,Vandermeeren MM,Medaer RH,Raus JC

更新日期：1987-03-01 00:00:00

abstract：:Dialysis encephalopathy is a progressive, fatal condition that occurs in patients receiving hemodialysis. It is characterized by abnormalities in speech, myoclonic jerks, and striking changes on the electroencephalogram. We observed dramatic reversal of the clinical symptoms and electroencephalographic abnormalities i...

journal_title：Neurology

authors： Nadel AM,Wilson WP

更新日期：1976-12-01 00:00:00

abstract：OBJECTIVE:To describe the phenotype and phenotype-genotype correlations in patients with amyotrophic lateral sclerosis (ALS) with TARDBP gene mutations. METHODS:French TARDBP+ patients with ALS (n = 28) were compared first to 3 cohorts: 737 sporadic ALS (SALS), 192 nonmutated familial ALS (FALS), and 58 SOD1 + FALS, a...

journal_title：Neurology

authors： Corcia P,Valdmanis P,Millecamps S,Lionnet C,Blasco H,Mouzat K,Daoud H,Belzil V,Morales R,Pageot N,Danel-Brunaud V,Vandenberghe N,Pradat PF,Couratier P,Salachas F,Lumbroso S,Rouleau GA,Meininger V,Camu W

更新日期：2012-05-08 00:00:00