Long-term effect of bone marrow transplantation in adult-onset adrenoleukodystrophy.

abstract：BACKGROUND:The presence of the apolipoprotein E (ApoE) 4 genotype is associated with an earlier age of onset for Alzheimer's disease (AD) and several other neurodegenerative disorders. The objective of this study was to investigate the effect of ApoE genotypes on the clinical course of amyotrophic lateral sclerosis (AL...

journal_title：European journal of neurology

authors： Jawaid A,Poon M,Strutt AM,Rice LK,McDowell EJ,Salamone AR,Qureshi SU,Simpson E,Appel SH,York MK,Schulz PE

更新日期：2011-04-01 00:00:00

abstract：BACKGROUND AND PURPOSE:We previously reported that certain optical coherence tomography (OCT) measures were sensitive and reliable in identifying idiopathic intracranial hypertension (IIH). This prospective study aimed to define OCT measures that allow differentiation of IIH with and without papilledema, thereby helpin...

journal_title：European journal of neurology

authors： Huang-Link Y,Eleftheriou A,Yang G,Johansson JM,Apostolou A,Link H,Jin YP

更新日期：2019-05-01 00:00:00

abstract：:Normal subjects use an open-loop motor control strategy in handwriting, but they are able to switch to closed-loop motor control when the demands on accuracy increase. These closed-loop handwriting movements of normal subjects resemble the inefficient movements found in writing-impaired patients. The hypothesis that s...

journal_title：European journal of neurology

authors： Schenk T,Walther EU,Mai N

更新日期：2000-05-01 00:00:00

abstract：:We report a 69-year-old male patient whose motor aphasia started at the age of 61. The language disability remained isolated and progressed over a period of eight years without any additional cognitive deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed moderate cortical atrophy with frontal...

journal_title：European journal of neurology

authors： Nagy TG,Jelencsik I,Szirmai I

更新日期：1999-07-01 00:00:00

abstract：:Mirror movements (MM), involuntary movements of homologous muscles opposite to unilateral limb activity, decrease in the course of motor development, but may reappear in adults after brain damage. To better characterize this type of acquired MM, grip forces of 36 healthy subjects and 35 patients suffering from focal b...

journal_title：European journal of neurology

authors： Uttner I,Mai N,Esslinger O,Danek A

更新日期：2005-12-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Predicting the course of behavioural variant frontotemporal dementia (bvFTD) remains a major clinical challenge. This study aimed to identify factors that predict survival and clinical progression in bvFTD. METHODS:Consecutive patients with clinically probable bvFTD were prospectively followed u...

journal_title：European journal of neurology

authors： Agarwal S,Ahmed RM,D'Mello M,Foxe D,Kaizik C,Kiernan MC,Halliday GM,Piguet O,Hodges JR

更新日期：2019-05-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Cognitive impairment (CI) is found in about half of the multiple sclerosis (MS) population and is an important contributor to employment status and social functioning. CI is encountered in all disease stages and correlates only moderately with disease duration or Expanded Disability Status Scale ...

journal_title：European journal of neurology

authors： Van Schependom J,D'hooghe MB,Cleynhens K,D'hooge M,Haelewyck MC,De Keyser J,Nagels G

更新日期：2014-09-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Current guidelines on cerebral venous thrombosis (CVT) diagnosis and management were issued by the European Federation of Neurological Societies in 2010. We aimed to update the previous European Federation of Neurological Societies guidelines using a clearer and evidence-based methodology. METHO...

journal_title：European journal of neurology

authors： Ferro JM,Bousser MG,Canhão P,Coutinho JM,Crassard I,Dentali F,di Minno M,Maino A,Martinelli I,Masuhr F,Aguiar de Sousa D,Stam J,European Stroke Organization.

更新日期：2017-10-01 00:00:00

abstract：BACKGROUND AND PURPOSE:CACNA1S encodes Cav 1.1, a voltage sensor for muscle excitation-contraction coupling, which activates the ryanodine receptor 1 (RYR1) leading to calcium release from the sarcoplasmic reticulum. CACNA1S mutations cause hypokalemic periodic paralysis, malignant hyperthermia and congenital myopathy....

journal_title：European journal of neurology

authors： Anandan C,Cipriani MA,Laughlin RS,Niu Z,Milone M

更新日期：2018-02-01 00:00:00

abstract：:This study is aimed at assessing the prevalence and features of psychotic symptoms in a sample of 180 patients with Alzheimer's disease (AD). Sixty-four patients (35.5% of the sample) showed one or more psychotic symptoms. The presence of these symptoms correlated with the severity of the patients' cognitive impairmen...

journal_title：European journal of neurology

authors： Sala SD,Francescani A,Muggia S,Spinnler H

更新日期：1998-11-01 00:00:00

abstract：BACKGROUND AND PURPOSE:A Consensus document on palliative care and neurology has made recommendations on the care of people with chronic and progressive neurological disease. This study aimed to investigate whether these recommendations are understood by, acceptable to and used in practice by neurologists in Belgium. ...

journal_title：European journal of neurology

authors： Vanopdenbosch LJ,Maes E,Oliver DJ

更新日期：2017-07-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Interferon beta (IFNbeta) preparations have some effect on the progressive phase of multiple sclerosis (MS). This limited effect might be partially because of a certain number of IFNbeta non-responders. Myxovirus resistance protein A (MxA)--a marker of IFNbeta bioactivity--was correlated with the...

journal_title：European journal of neurology

authors： Millonig A,Dressel A,Bahner D,Bitsch A,Bogumil T,Elitok E,Kitze B,Tumani H,Weber F,Gneiss C,Deisenhammer F

更新日期：2008-08-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Critical illness polyneuropathy and myopathy (CIP/CIM) is being increasingly recognized as a significant clinical problem in critically ill children especially if they have spent long periods in the intensive care unit. So the aim was to determine the frequency of CIP/CIM amongst mechanically ven...

journal_title：European journal of neurology

authors： Thabet Mahmoud A,Tawfik MAM,Abd El Naby SA,Abo El Fotoh WMM,Saleh NY,Abd El Hady NMS

更新日期：2018-07-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Approximately 9000 people in the UK are affected by Huntington's disease (HD). People with HD require ongoing health and social care support. There is a knowledge gap about costs of health and social care use associated with HD in the UK. This paper estimates the economic cost in the UK. METHODS...

journal_title：European journal of neurology

authors： Jones C,Busse M,Quinn L,Dawes H,Drew C,Kelson M,Hood K,Rosser A,Edwards RT

更新日期：2016-10-01 00:00:00

abstract：:Although visual hallucinations (VH) are relatively frequent in Parkinson's disease (PD) patients, their neural substrates are only known from neuropathological and functional magnetic resonance studies. The aim of this study was to investigate possible structural brain changes on MRI in non-demented PD patients with V...

journal_title：European journal of neurology

authors： Ramírez-Ruiz B,Martí MJ,Tolosa E,Giménez M,Bargalló N,Valldeoriola F,Junqué C

更新日期：2007-07-01 00:00:00

abstract：BACKGROUND AND PURPOSE:The aim was to explore the risk of early stroke recurrence within 3 months after watershed infarction and to investigate whether early dual antiplatelet therapy is more effective in decreasing such risk. METHODS:Patients enrolled in the Clopidogrel in High-risk Patients with Acute Non-disabling ...

journal_title：European journal of neurology

authors： Pu Y,Liu X,Wang Y,Meng X,Jing J,Zou X,Pan Y,Wang A,Zhao X,Johnston SC,Wang Y,Atchaneeyasakul K,Liebeskind DS,Liu L,CHANCE Investigators.

更新日期：2020-05-01 00:00:00

abstract：BACKGROUND:Cognitive tests are known to be influenced by language, culture and education. In addition, there may be an impact of 'epoch' in cognition, because there is secular increase in scores of IQ tests in children. If we assume this is a long lasting process, then it should persist later in life. METHODS:To test ...

journal_title：European journal of neurology

authors： Morgado J,Rocha CS,Maruta C,Guerreiro M,Martins IP

更新日期：2010-05-01 00:00:00

abstract：BACKGROUND AND PURPOSE:The expanded repeat length in ATXN1 negatively correlates with age at onset (AAO) of spinocerebellar ataxia type 1 (SCA1) but can explain only part of it, indicating that other factors affect AAO. Some studies have explored the influence of non-causative CAG repeats on the AAO of SCA patients. Ho...

journal_title：European journal of neurology

authors： Wang P,Chen Z,Peng Y,Cao L,Li X,Wang C,Yang H,Peng H,Shi Y,Zhou X,Li T,Feng L,Wu C,Qiu R,Xia K,Tang B,Jiang H

更新日期：2019-08-01 00:00:00

abstract：:Astasia, inability to stand unsupported despite good strength, resembles the marked balance impairment of patients with vestibulocerebellar disease. We describe a patient with unilateral thalamic infarct that presented with astasia. A 76-year-old hypertensive woman was admitted to our hospital because of marked unstea...

journal_title：European journal of neurology

authors： Lee PH,Lee JH,Joo US

更新日期：2005-04-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Despite numerous studies, the role of patent foramen ovale (PFO) as a risk factor for stroke due to paradoxical embolism is still controversial. On the assumption that specific lesion patterns, in particular multiple acute ischaemic lesions on diffusion-weighted magnetic resonance imaging, indica...

journal_title：European journal of neurology

authors： Feurer R,Sadikovic S,Esposito L,Schwarze J,Bockelbrink A,Hemmer B,Sander D,Poppert H

更新日期：2009-10-01 00:00:00

abstract：:In this report, we present the clinical and pathological details of a kindred of four individuals with a novel missense mutation (V272A) of the presenilin 1 gene (PSEN1) that experienced a subcortical dementia. The age of onset of symptoms ranged 26-36-year old, with an age at death of 36-46 years. Initial symptom was...

journal_title：European journal of neurology

authors： Jimenez-Escrig A,Rabano A,Guerrero C,Simon J,Barquero MS,Güell I,Ginestal RC,Montero T,Orensanz L

更新日期：2004-10-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Parkinson's disease (PD) is a progressive neurodegenerative disorder with motor and non-motor symptoms, including cognitive deficits. Several magnetic resonance imaging approaches have been applied to investigate brain atrophy in PD. The aim of this study was to detect early structural cortical a...

journal_title：European journal of neurology

authors： Danti S,Toschi N,Diciotti S,Tessa C,Poletti M,Del Dotto P,Lucetti C

更新日期：2015-12-01 00:00:00

abstract：BACKGROUND AND PURPOSE:X-linked dystonia-parkinsonism (XDP) is an inherited neurodegenerative adult-onset movement disorder associated with striatal atrophy. As the dopaminergic system has not yet been systemically studied in this basal ganglia model disease, it is unclear whether nigrostriatal dysfunction contributes ...

journal_title：European journal of neurology

authors： Brüggemann N,Rosales RL,Waugh JL,Blood AJ,Domingo A,Heldmann M,Jamora RD,Münchau A,Münte TF,Lee LV,Buchmann I,Klein C

更新日期：2017-05-01 00:00:00

abstract：BACKGROUND AND PURPOSE:The aim was to study the prevalence of epilepsy in a hospital-based systemic lupus erythematosus (SLE) cohort and to investigate the relationship between epilepsy and other manifestations of neuropsychiatric SLE (NPSLE). METHODS:The study population consisted of 440 SLE patients recruited from 1...

journal_title：European journal of neurology

authors： Hopia L,Andersson M,Svenungsson E,Khademi M,Piehl F,Tomson T

更新日期：2020-02-01 00:00:00

abstract：BACKGROUND:X-linked Charcot-Marie-Tooth disease (CMTX), caused by mutations in the gene encoding connexin32, is the second most common form of inherited demyelinating neuropathy, next to CMT 1A, and accounts for 10-20% of all hereditary demyelinating neuropathies. AIMS OF THE STUDY:To describe clinical and electrophys...

journal_title：European journal of neurology

authors： Mazzeo A,Di Leo R,Toscano A,Muglia M,Patitucci A,Messina C,Vita G

更新日期：2008-10-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Intracerebral hemorrhage (ICH) is a common and severe form of stroke but is scarcely studied in young adults. Our aim was to study risk factors, clinical presentation and early mortality of ICH in the young and compare these features with older patients. METHODS:All consecutive patients aged bet...

journal_title：European journal of neurology

authors： Koivunen RJ,Satopää J,Meretoja A,Strbian D,Haapaniemi E,Niemelä M,Tatlisumak T,Putaala J

更新日期：2015-01-01 00:00:00

abstract：:We examined the prevalence and correlates of apathy and irritability in a consecutive series of 101 patients with probable Alzheimer's disease (AD). Based on clinical criteria, 46 (46%) patients had apathy, and 13 (13%) patients had irritability. Apathy was significantly associated with more severe impairments in acti...

journal_title：European journal of neurology

authors： Starkstein SE,Migliorelli R,Manes F,Tesón A,Petracca G,Chemerinski E,Sabe L,Leiguarda R

更新日期：1995-12-01 00:00:00

abstract：BACKGROUND AND OBJECTIVES:The ataxias are a challenging group of neurological diseases due the aetiological heterogeneity and the complexity of the genetic subtypes. This guideline focuses on the heredodegenerative ataxias. The aim is to provide a peer-reviewed evidence-based guideline for clinical neurologists and oth...

journal_title：European journal of neurology

authors： van de Warrenburg BP,van Gaalen J,Boesch S,Burgunder JM,Dürr A,Giunti P,Klockgether T,Mariotti C,Pandolfo M,Riess O

更新日期：2014-04-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Risk-stratification models based on patient and disease characteristics are useful for aiding clinical decisions and for comparing the quality of care between different physicians or hospitals. In addition, prediction of mortality is beneficial for optimizing resource utilization. We evaluated th...

journal_title：European journal of neurology

authors： Peng SY,Chuang YC,Kang TW,Tseng KH

更新日期：2010-07-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Better understanding the incidence, predictors and mechanisms of early neurological deterioration (END) following intravenous thrombolysis (IVT) for acute stroke with mild symptoms and isolated internal carotid artery occlusion (iICAo) may inform therapeutic decisions. METHODS:From a multicenter...

journal_title：European journal of neurology

authors： Boulenoir N,Turc G,Henon H,Laksiri N,Mounier-Véhier F,Girard Buttaz I,Duong DL,Papassin J,Yger M,Triquenot A,Lyoubi A,Ter Schiphorst A,Denier C,Baron JC,Seners P,MINOR-STROKE Collaborators.

更新日期：2021-02-01 00:00:00