Treatment of early onset multiple sclerosis with subcutaneous interferon beta-1a.

abstract：OBJECTIVE:To describe the phenotypic spectrum and genetics of periventricular nodular heterotopia (PNH) caused by FLN1 mutations in four men. BACKGROUND:X-linked PNH caused by FLN1 mutations (MIM #300049) implies prenatal or early postnatal lethality in boys and 50% recurrence risk in daughters of affected women. MET...

journal_title：Neurology

authors： Guerrini R,Mei D,Sisodiya S,Sicca F,Harding B,Takahashi Y,Dorn T,Yoshida A,Campistol J,Krämer G,Moro F,Dobyns WB,Parrini E

更新日期：2004-07-13 00:00:00

abstract：PURPOSE:To understand the role of the angiopoietin-like 6 gene (ANGPTL6) in intracranial aneurysms (IAs), we investigated its role in a large cohort of familial IAs. METHODS:Individuals with family history of IA were recruited to the Genetic and Observational Subarachnoid Haemorrhage (GOSH) study. The ANGPTL6 gene was...

journal_title：Neurology

authors： Hostettler IC,O'Callaghan B,Bugiardini E,O'Connor E,Vandrovcova J,Davagnanam I,Alg V,Bonner S,Walsh D,Bulters D,Kitchen N,Brown MM,Grieve J,Werring DJ,Houlden H,Genetics and Observational Subarachnoid Haemorrhage (GOSH) Stu

更新日期：2020-10-26 00:00:00

abstract：OBJECTIVE:We endeavored to develop an unruptured intracranial aneurysm (UIA) treatment score (UIATS) model that includes and quantifies key factors involved in clinical decision-making in the management of UIAs and to assess agreement for this model among specialists in UIA management and research. METHODS:An internat...

journal_title：Neurology

authors： Etminan N,Brown RD Jr,Beseoglu K,Juvela S,Raymond J,Morita A,Torner JC,Derdeyn CP,Raabe A,Mocco J,Korja M,Abdulazim A,Amin-Hanjani S,Al-Shahi Salman R,Barrow DL,Bederson J,Bonafe A,Dumont AS,Fiorella DJ,Gruber A,H

更新日期：2015-09-08 00:00:00

abstract：:A wide variety of movement disorders are associated with alcohol abuse. Some idiopathic movement disorders are markedly improved by small amounts of alcohol and this response occasionally may lead to alcoholism. Alcohol abuse alone or combined with hepatic encephalopathy can cause various types of tremor, asterixis, a...

journal_title：Neurology

authors： Neiman J,Lang AE,Fornazzari L,Carlen PL

更新日期：1990-05-01 00:00:00

abstract：BACKGROUND:Several lines of evidence suggest that inflammatory mechanisms contribute to AD. OBJECTIVE:To examine whether several markers of inflammation are associated with cognitive decline in African-American and white well-functioning elders. METHODS:The authors studied 3,031 African-American and white men and wom...

journal_title：Neurology

authors： Yaffe K,Lindquist K,Penninx BW,Simonsick EM,Pahor M,Kritchevsky S,Launer L,Kuller L,Rubin S,Harris T

更新日期：2003-07-08 00:00:00

abstract：:An emerging issue in stroke prevention and stroke treatment is that therapies shown to be effective in clinical trials are not being used or are being used suboptimally. One of many explanations relates to cost, ranging from concerns about inappropriate use of societal resources to more immediate concerns of organizat...

journal_title：Neurology

authors： Matchar DB

更新日期：1998-09-01 00:00:00

abstract：OBJECTIVE:To investigate whether the presence of cerebral microbleeds, which present as focal lesions on imaging, is associated with a diffuse loss of white matter microstructural integrity in the brain. METHODS:In the prospective, population-based Rotterdam Scan Study, a total of 4,493 participants underwent brain MR...

journal_title：Neurology

authors： Akoudad S,de Groot M,Koudstaal PJ,van der Lugt A,Niessen WJ,Hofman A,Ikram MA,Vernooij MW

更新日期：2013-11-26 00:00:00

abstract：:We studied two patients with Meige syndrome who developed alpha-methyldopa-induced parkinsonism. Opposite responses of parkinsonian and dystonic symptoms to antiparkinson drugs in some cases suggest a functionally reciprocal relationship between these disorders. ...

journal_title：Neurology

authors： Sechi GP,Demontis G,Rosati G

更新日期：1985-11-01 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：OBJECTIVE:To test the hypotheses predicted in a hypothetical model of Alzheimer disease (AD) biomarkers that rates of β-amyloid (Aβ) accumulation on PET imaging are not related to hippocampal neurodegeneration whereas rates of neurodegenerative brain atrophy depend on the presence of both amyloid and neurodegeneration ...

journal_title：Neurology

authors： Jack CR Jr,Wiste HJ,Knopman DS,Vemuri P,Mielke MM,Weigand SD,Senjem ML,Gunter JL,Lowe V,Gregg BE,Pankratz VS,Petersen RC

更新日期：2014-05-06 00:00:00

abstract：:Kuru plaques are the pathologic hallmark in Gerstmann-Sträussler syndrome (GSS). To demonstrate that prion protein (PrP) is a component of kuru plaque cores, we fractionated and sequenced kuru plaque core derived peptides, following digestion with Achromobacter lyticus protease I. We identified 3 PrP-derived peptides ...

journal_title：Neurology

authors： Kitamoto T,Yamaguchi K,Doh-ura K,Tateishi J

更新日期：1991-02-01 00:00:00

abstract：:Cisternography and ventriculography were performed in five normal anesthetized dogs to study the dynamics of tracer dispersal. Indium 111-diethylenetriaminepentaacetic acid was injected into lateral ventricles, cisterna magna, thoracic subarachnoid space, and lumbar sac. The patterns of dispersal from each injection s...

journal_title：Neurology

authors： Schossberger PF,Touya JJ

更新日期：1976-03-01 00:00:00

abstract：:Tolcapone is a potent, reversible inhibitor of catechol O-methyltransferase (COMT) intended for use as an adjunct to levodopa therapy for Parkinson's disease (PD). Findings from the first pharmacokinetics/pharmacodynamics and tolerability studies of tolcapone in volunteers are reviewed. Following linear and dose-propo...

journal_title：Neurology

authors： Jorga KM

更新日期：1998-05-01 00:00:00

abstract：OBJECTIVE:To determine the mechanism of ictal downbeat nystagmus in Ménière disease (MD), we compared the head impulse gain of the vestibulo-ocular reflex (VOR) for each semicircular canal between patients with (n = 7) and without (n = 70) downbeat nystagmus during attacks of MD. METHODS:We retrospectively analyzed th...

journal_title：Neurology

authors： Lee SU,Kim HJ,Choi JY,Kim JS

更新日期：2020-10-27 00:00:00

abstract：OBJECTIVES:We evaluated the frequency and predictive value of ocular fundus abnormalities among patients who presented to the emergency department (ED) with focal neurologic deficits to determine the utility of these findings in the evaluation of patients with suspected TIA and stroke. METHODS:In this cross-sectional ...

journal_title：Neurology

authors： Vuong LN,Thulasi P,Biousse V,Garza P,Wright DW,Newman NJ,Bruce BB

更新日期：2015-07-21 00:00:00

abstract：:We studied the effects of intravenous immunoglobulin (IVIg) in 12 patients with motor neuron syndromes associated with high titers of anti-GM1 antibodies. Five of the patients had conduction blocks. The study design was a double-blind, placebo-controlled, crossover trial with IVIg (0.4 g/kg body weight per day injecte...

journal_title：Neurology

authors： Azulay JP,Blin O,Pouget J,Boucraut J,Billé-Turc F,Carles G,Serratrice G

更新日期：1994-03-01 00:00:00

abstract：OBJECTIVE:To elucidate the phenotype, genotype, and MRI findings of Korean patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and mutation carriers. METHODS:The authors studied 40 members of nine unrelated Korean CADASIL families. After genetic analysis o...

journal_title：Neurology

authors： Kim Y,Choi EJ,Choi CG,Kim G,Choi JH,Yoo HW,Kim JS

更新日期：2006-05-23 00:00:00

abstract：:To determine the frequency of subjective sleep problems in spinocerebellar ataxia type 6 (SCA6), the authors surveyed 25 patients and 25 age-matched controls with the Epworth Sleepiness Scale (ESS) and Pittsburgh Sleep Quality Index (PSQI). The ESS was higher in patients with SCA6 (9.12 +/- 5.80; CI: 2.28) than in con...

journal_title：Neurology

authors： Howell MJ,Mahowald MW,Gomez CM

更新日期：2006-05-09 00:00:00

abstract：:Recurrent episodes of oscillopsia, rotational vertigo, and postural imbalance were elicited and modulated by changing the horizontal head positions of a patient with an arachnoid cyst in the right cerebellopontine angle that distorted the vestibulocochlear nerve. Oculomotor analysis revealed two different types of att...

journal_title：Neurology

authors： Arbusow V,Strupp M,Dieterich M,Jäger L,Hischa A,Schulz P,Brandt T

更新日期：1998-11-01 00:00:00

abstract：:We evaluated ventilation during cycle exercise in four men lacking myophosphorylase. In submaximal exercise of similar relative intensity, ventilation was higher relative to oxygen uptake in McArdle patients than in normal men. The exercise ventilatory response returned to normal after glucose infusion, by fasting to ...

journal_title：Neurology

authors： Haller RG,Lewis SF

更新日期：1986-05-01 00:00:00

abstract：OBJECTIVES:To assess the therapeutic efficacy, required dose, and tolerability of pregabalin in patients with idiopathic restless legs syndrome (RLS). METHODS:This was a double-blind, placebo-controlled trial with polysomnographic control, providing Class II evidence. Ninety-eight patients underwent a 2-week single-bl...

journal_title：Neurology

authors： Garcia-Borreguero D,Larrosa O,Williams AM,Albares J,Pascual M,Palacios JC,Fernandez C

更新日期：2010-06-08 00:00:00

abstract：:Although people with migraine are aware of their headaches they are often not aware that they have migraine. This can lead to inappropriate or ineffective use of treatments, delays in seeking appropriate care, and miscommunication during clinical encounters. This article assesses self-recognition of migraine and ident...

journal_title：Neurology

authors： Lipton RB,Stewart WF,Liberman JN

更新日期：2002-05-14 00:00:00

abstract：OBJECTIVE:To examine the relationships between age at onset and duration of seizure disorder with severity of hippocampal sclerosis (HS) and cognitive functioning in patients with HS and unilateral temporal lobe epilepsy. METHODS:Twenty-six subjects had left temporal lobe seizure onset; 20 had right temporal onset. Me...

journal_title：Neurology

authors： Fuerst D,Shah J,Kupsky WJ,Johnson R,Shah A,Hayman-Abello B,Ergh T,Poore Q,Canady A,Watson C

更新日期：2001-07-24 00:00:00

abstract：:A 60-year-old man presented with progressive large fiber sensory loss in the right first three fingers and, to a lesser extent, in both fourth and fifth fingers. Electrophysiologic studies were characteristic of chronic sensory demyelinating polyneuropathy, a variant of chronic inflammatory demyelinating polyneuropath...

journal_title：Neurology

authors： Thonnard JL,Detrembleur C,Van den Bergh PY

更新日期：1997-07-01 00:00:00

abstract：OBJECTIVE:To analyze the associations between cardiovascular fitness at age 18 years and future risk of epilepsy. METHODS:Population-based cohort study of Swedish male conscripts (n = 1,173,079) born in 1950-1987, who were followed for up to 40 years. Data on cardiovascular fitness were collected during conscription e...

journal_title：Neurology

authors： Nyberg J,Aberg MA,Torén K,Nilsson M,Ben-Menachem E,Kuhn HG

更新日期：2013-09-17 00:00:00

abstract：OBJECTIVE:The aim of this study was to review 4 patients with encephalomyeloradiculoneuropathy (EMRN) and assess for autoantibodies against neutral glycolipids. METHODS:We studied the progression of clinical, radiologic, neurophysiologic, and CSF findings, as well as anti-neutral glycolipid antibodies in sera. RESULT...

journal_title：Neurology

authors： Shima S,Kawamura N,Ishikawa T,Masuda H,Iwahara C,Niimi Y,Ueda A,Iwabuchi K,Mutoh T

更新日期：2014-01-14 00:00:00

abstract：:Although referrals for narcolepsy are increasing, physicians continue to confront obstacles in diagnosing and treating this condition. Patients with narcolepsy also face challenges at work, at school, and at home. One of the more disturbing problems is the lack of public awareness of the disorder and its potential the...

journal_title：Neurology

authors： Douglas NJ

更新日期：1998-02-01 00:00:00

abstract：OBJECTIVES:The authors report the results of a prospective, placebo-controlled, randomized study to evaluate the effectiveness of 3,4-diaminopyridine (DAP) in patients with Lambert-Eaton myasthenic syndrome (LEMS) and to determine the acute and long-term side effects of DAP. METHODS:Twenty-six patients with LEMS compl...

journal_title：Neurology

authors： Sanders DB,Massey JM,Sanders LL,Edwards LJ

更新日期：2000-02-08 00:00:00

abstract：OBJECTIVE:We aimed to assess sensitivity and specificity of the updated criteria for behavioral variant frontotemporal dementia (bvFTD) based on a large autopsy-confirmed cohort of patients with dementia. METHODS:Two hundred thirty-nine consecutive pathologically confirmed dementia patients, clinically assessed in a s...

journal_title：Neurology

authors： Harris JM,Gall C,Thompson JC,Richardson AM,Neary D,du Plessis D,Pal P,Mann DM,Snowden JS,Jones M

更新日期：2013-05-14 00:00:00

abstract：BACKGROUND:Elevated serum retinol, CSF retinol, and serum retinol binding protein (RBP) levels have been found in some patients with idiopathic intracranial hypertension (IIH), but serum and CSF retinol levels have not been studied in matched serum and CSF samples in patients with IIH. OBJECTIVE:To determine whether s...

journal_title：Neurology

authors： Tabassi A,Salmasi AH,Jalali M

更新日期：2005-06-14 00:00:00