Atypical neuronal ceroid-lipofuscinosis.

abstract：OBJECTIVE:To determine the rate of relapse occurrence during pregnancy and postpartum. METHODS:In a cross-sectional study using the national multiple sclerosis (MS) registry, pregnant women with relapsing MS were identified. Data on demographics, clinical characteristics, and disease-modifying therapies (DMTs), includ...

journal_title：Neurology

authors： Alroughani R,Alowayesh MS,Ahmed SF,Behbehani R,Al-Hashel J

更新日期：2018-03-06 00:00:00

abstract：:We investigated the association of PD with a recently reported I93M mutation of the ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) gene, and with a new and common polymorphic variant S18Y of the same gene. We did not identify the I93M mutation in any of 139 unrelated PD cases or 113 controls. However, S18Y polymorph...

journal_title：Neurology

authors： Maraganore DM,Farrer MJ,Hardy JA,Lincoln SJ,McDonnell SK,Rocca WA

更新日期：1999-11-10 00:00:00

abstract：BACKGROUND:The epsilon 4 allele of the APOE gene (APOE) is more frequent in patients with AD than in the general population, but studies are inconclusive as to whether it affects rate of progression or survival. Because survival in AD is generally longer in women than in men, the authors investigated whether APOE affec...

journal_title：Neurology

authors： Dal Forno G,Carson KA,Brookmeyer R,Troncoso J,Kawas CH,Brandt J

更新日期：2002-04-09 00:00:00

abstract：OBJECTIVE:To evaluate the usefulness of ocular motor information in the early diagnosis of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). METHODS:Seven PSP patients, six CBD patients, and three atypical CBD patients were followed longitudinally with repeated electrooculographic (EOG) recordi...

journal_title：Neurology

authors： Rivaud-Péchoux S,Vidailhet M,Gallouedec G,Litvan I,Gaymard B,Pierrot-Deseilligny C

更新日期：2000-03-14 00:00:00

abstract：OBJECTIVE:To examine the relationship of migraine and other severe headaches with panic disorder. METHODS:Representative samples of persons with migraine, non-migrainous severe headaches, and controls with no history of severe headaches, identified by a telephone survey, were interviewed in person, using a standardize...

journal_title：Neurology

authors： Breslau N,Schultz LR,Stewart WF,Lipton R,Welch KM

更新日期：2001-02-13 00:00:00

abstract：:We reviewed 44 cases of ischemia and infarction of the spinal cord at two university hospitals. Three patients experienced transient ischemic attacks. Etiologies of completed strokes were diverse and included rupture and surgical repair of aortic aneurysms, aortic dissection, aortic rupture and thrombosis, global isch...

journal_title：Neurology

authors： Cheshire WP,Santos CC,Massey EW,Howard JF Jr

更新日期：1996-08-01 00:00:00

abstract：:The forearm segment of the ulnar nerve is difficult to assess if the nerve has been injured severely at the wrist. A new, simple nerve conduction technique of recording from the sensory dorsal branch of the nerve localizes the lesion and enables easier evaluation of that segment than do other techniques. ...

journal_title：Neurology

authors： Jabre JF

更新日期：1980-08-01 00:00:00

abstract：:We report the cases of 5 patients from 2 sibships with the "adult" or chronic form of GM2 gangliosidosis and 2 patients from another sibship with the juvenile form. We demonstrated hexosaminidase A deficiency in all cases but in 1 sibship the enzymatic profile was identical to that in Tay-Sachs disease, whereas in the...

journal_title：Neurology

authors： Specola N,Vanier MT,Goutières F,Mikol J,Aicardi J

更新日期：1990-01-01 00:00:00

abstract：OBJECTIVE:To evaluate the efficacy and safety of eptinezumab, a humanized anti-calcitonin gene-related peptide monoclonal antibody, in the preventive treatment of chronic migraine (CM). METHODS:The Prevention of Migraine via Intravenous ALD403 Safety and Efficacy-2 (PROMISE-2) study was a phase 3, multicenter, randomi...

journal_title：Neurology

authors： Lipton RB,Goadsby PJ,Smith J,Schaeffler BA,Biondi DM,Hirman J,Pederson S,Allan B,Cady R

更新日期：2020-03-31 00:00:00

abstract：BACKGROUND:PD was associated with increased mortality before levodopa therapy became available. There have been conflicting reports of PD mortality in the modern era. OBJECTIVE:To assess current mortality rates in a large unselected population receiving treatment for parkinsonism (PKM) followed for up to 6 years. MET...

journal_title：Neurology

authors： Guttman M,Slaughter PM,Theriault ME,DeBoer DP,Naylor CD

更新日期：2001-12-26 00:00:00

abstract：OBJECTIVES:To estimate patient-acceptable symptom state (PASS) cut points for myasthenia gravis (MG) health scales. METHODS:We conducted an electronic survey that included the Myasthenia Gravis Impairment Index (MGII), EuroQol 5-Dimension (EQ5D), and a simple PASS question. PASS-anchored thresholds were estimated for ...

journal_title：Neurology

authors： Mendoza M,Tran C,Bril V,Katzberg HD,Barnett C

更新日期：2020-09-22 00:00:00

abstract：OBJECTIVE:To validate the ability of a specifically developed cognitive risk score to identify patients at risk of poststroke neurocognitive disorders (NCDs) who are eligible for a comprehensive cognitive assessment. METHODS:After assessing 404 patients (infarct 91.3%) in the Groupe de Réflexion pour l'Evaluation Cogn...

journal_title：Neurology

authors： Godefroy O,Yaïche H,Taillia H,Bompaire F,Nédélec-Ciceri C,Bonnin C,Varvat J,Vincent-Grangette F,Diouf M,Mas JL,Canaple S,Lamy C,Arnoux A,Leclercq C,Tasseel-Ponche S,Roussel M,Barbay M,GRECogVASC Study Group.

更新日期：2018-11-20 00:00:00

abstract：:We report a patient with unusual MRI abnormalities that had the physical characteristics of ferromagnetic artifact. We believe that the MRI artifacts were due to microscopic embolic metal fragments, most likely from a mechanical heart valve prosthesis. Potential sources of metal emboli should be considered in patients...

journal_title：Neurology

authors： Wingerchuk DM,Krecke KN,Fulgham JR

更新日期：1997-11-01 00:00:00

abstract：:A seismic shift in our understanding of the ability to diagnose Alzheimer disease (AD) is occurring. For the last several decades, AD has been a clinical-pathologic diagnosis, and this conceptualization of the disease has served the field well. Typically, the clinician would identify a syndrome such as mild cognitive ...

journal_title：Neurology

authors： Petersen RC

更新日期：2018-08-28 00:00:00

abstract：OBJECTIVE:To assess the evidence demonstrating efficacy, tolerability, and safety of seven new antiepileptic drugs (AEDs) (gabapentin, lamotrigine, topiramate, tiagabine, oxcarbazepine, levetiracetam, and zonisamide) in the treatment of children and adults with refractory partial and generalized epilepsies. METHODS:A ...

journal_title：Neurology

authors： French JA,Kanner AM,Bautista J,Abou-Khalil B,Browne T,Harden CL,Theodore WH,Bazil C,Stern J,Schachter SC,Bergen D,Hirtz D,Montouris GD,Nespeca M,Gidal B,Marks WJ Jr,Turk WR,Fischer JH,Bourgeois B,Wilner A,Faught R

更新日期：2004-04-27 00:00:00

abstract：:Adult-onset dystonia-parkinsonism is a syndrome in search of a pathology. We therefore reviewed the literature on dystonic manifestations in autopsy-proven cases of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and idiopathic Parkinson's disease (PD). Only 6 of 140 autopsy reports of MSA remarke...

journal_title：Neurology

authors： Rivest J,Quinn N,Marsden CD

更新日期：1990-10-01 00:00:00

abstract：:The urinary excretion of some important phenylethylamines, catecholamines, their metabolites, amphetamine, and methamphetamine were measured in parkinsonian patients on Sinemet (L-dopa plus carbidopa, a peripheral dopadecarboxylase inhibitor) and depressed patients after chronic (-) deprenyl treatment. Deprenyl was ef...

journal_title：Neurology

authors： Karoum F,Chuang LW,Eisler T,Calne DB,Liebowitz MR,Quitkin FM,Klein DF,Wyatt RJ

更新日期：1982-05-01 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：OBJECTIVE:To evaluate the presence of prodromal markers of Parkinson disease (PD) in patients with longstanding idiopathic REM sleep behavior disorder (IRBD), a small subgroup of individuals with IRBD with long-term follow-up thought not to be at risk of developing PD. METHODS:Demographic, clinical, and neuroimaging m...

journal_title：Neurology

authors： Iranzo A,Stefani A,Serradell M,Martí MJ,Lomeña F,Mahlknecht P,Stockner H,Gaig C,Fernández-Arcos A,Poewe W,Tolosa E,Högl B,Santamaria J,SINBAR (Sleep Innsbruck Barcelona) group.

更新日期：2017-07-18 00:00:00

abstract：OBJECTIVE:In the work-up of patients presenting with a clinically isolated syndrome (CIS), 3T MRI might offer a higher lesion detection than 1.5T, but it remains unclear whether this affects the fulfilment of the diagnostic criteria for multiple sclerosis (MS). METHODS:We recruited 66 patients with CIS within 6 months...

journal_title：Neurology

authors： Hagens MHJ,Burggraaff J,Kilsdonk ID,de Vos ML,Cawley N,Sbardella E,Andelova M,Amann M,Lieb JM,Pantano P,Lissenberg-Witte BI,Killestein J,Oreja-Guevara C,Ciccarelli O,Gasperini C,Lukas C,Wattjes MP,Barkhof F,MAGNIMS St

更新日期：2018-07-17 00:00:00

abstract：OBJECTIVE:We assessed whether clinical and imaging features of subjects with apraxia of speech (AOS) more severe than aphasia (dominant AOS) are more similar to agrammatic primary progressive aphasia (agPPA) or to primary progressive AOS (PPAOS). METHODS:Sixty-seven subjects (PPAOS = 18, dominant AOS = 10, agPPA = 9, ...

journal_title：Neurology

authors： Josephs KA,Duffy JR,Strand EA,Machulda MM,Senjem ML,Lowe VJ,Jack CR Jr,Whitwell JL

更新日期：2013-07-23 00:00:00

abstract：OBJECTIVE:To study the difference in risk for dementing diseases between men and women. BACKGROUND:Previous studies suggest women have a higher risk for dementia than men. However, these studies include small sample sizes, particularly in the older age groups, when the incidence of dementia is highest. METHODS:Pooled...

journal_title：Neurology

authors： Andersen K,Launer LJ,Dewey ME,Letenneur L,Ott A,Copeland JR,Dartigues JF,Kragh-Sorensen P,Baldereschi M,Brayne C,Lobo A,Martinez-Lage JM,Stijnen T,Hofman A

更新日期：1999-12-10 00:00:00

abstract：BACKGROUND:Muscle channelopathies such as paramyotonia, hyperkalemic periodic paralysis, and potassium-aggravated myotonia are caused by gain-of-function Na+ channel mutations. METHODS:Methods: Implementation of a three-dimensional radial 23Na magnetic resonance (MR) sequence with ultra-short echo times allowed the au...

journal_title：Neurology

authors： Weber MA,Nielles-Vallespin S,Essig M,Jurkat-Rott K,Kauczor HU,Lehmann-Horn F

更新日期：2006-10-10 00:00:00

abstract：BACKGROUND:Because of the relatively poor results of frontal lobe epilepsy (FLE) surgery, identification of prognostic factors for surgical outcome is of great importance. METHODS:To identify predictive factors for FLE surgery, we analyzed the data of 61 patients (mean age at surgery 19.2) who had undergone presurgica...

journal_title：Neurology

authors： Janszky J,Jokeit H,Schulz R,Hoppe M,Ebner A

更新日期：2000-04-11 00:00:00

abstract：OBJECTIVES:To investigate the relationship between markers of vitamin B(12) status and brain volume loss per year over a 5-year period in an elderly population. METHODS:A prospective study of 107 community-dwelling volunteers aged 61 to 87 years without cognitive impairment at enrollment. Volunteers were assessed year...

journal_title：Neurology

authors： Vogiatzoglou A,Refsum H,Johnston C,Smith SM,Bradley KM,de Jager C,Budge MM,Smith AD

更新日期：2008-09-09 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 received an overdose of intravascular contrast medium and experienced protracted seizures associated with radiographic evidence of prolonged retention of the contrast agent in the cerebral cortex, basal ganglia, and thalami. This is only the second report and the first survivor of a similar neurologic syndro...

journal_title：Neurology

authors： May EF,Ling GS,Geyer CA,Jabbari B

更新日期：1993-04-01 00:00:00

abstract：OBJECTIVE:To evaluate the influence of the single nucleotide polymorphism rs1080985 in the cytochrome P450 2D6 (CYP2D6) gene on the efficacy of donepezil in patients with mild to moderate Alzheimer disease (AD). METHODS:This was a multicenter, prospective cohort study of 127 white patients with AD according to the Nat...

journal_title：Neurology

authors： Pilotto A,Franceschi M,D'Onofrio G,Bizzarro A,Mangialasche F,Cascavilla L,Paris F,Matera MG,Pilotto A,Daniele A,Mecocci P,Masullo C,Dallapiccola B,Seripa D

更新日期：2009-09-08 00:00:00

abstract：BACKGROUND:Clinical trials involving patients with glioblastoma (GBM) distinguish cohorts who are treated with enzyme-inducing anticonvulsants (EIAC). Such anticonvulsants induce hepatic P450 microsomal enzymes, which accelerate the metabolism of certain chemotherapy and molecular targeted agents. However, the resultan...

journal_title：Neurology

authors： Jaeckle KA,Ballman K,Furth A,Buckner JC

更新日期：2009-10-13 00:00:00

abstract：OBJECTIVE:To describe the diagnosis and management of a 49-year-old woman with multiple sclerosis (MS) developing a progressive hemiparesis and expanding MRI lesion suspicious of progressive multifocal leukoencephalopathy (PML) 19 months after starting natalizumab. RESULTS:Polyomavirus JC (JCV)-specific qPCR in CSF wa...

journal_title：Neurology

authors： Kuhle J,Gosert R,Bühler R,Derfuss T,Sutter R,Yaldizli O,Radue EW,Ryschkewitsch C,Major EO,Kappos L,Frank S,Hirsch HH

更新日期：2011-12-06 00:00:00