Abstract:
BACKGROUND AND PURPOSE:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral small vessel disease that may lead to disability and whose phenotype modulators are still unknown. METHODS:In the MIcrovascular LEukoencephalopathy Study (MILES), we assessed the influence of vascular risk factors and the effect of different cognitive domains (memory, psychomotor speed and executive functions) performances on functional abilities in CADASIL in comparison with age-related leukoencephalopathy (ARL). RESULTS:We evaluated 51 CADASIL patients (mean age 50.3 ± 13.8 years, 47.1% males) and 68 ARL patients (70.6 ± 7.4 years, 58.8% males). Considering vascular risk factors, after adjustment for age, CADASIL patients had higher mean BMI values than ARL patients. Stroke history frequency was similar in the two groups. After adjustment for age, more CADASIL patients were disabled (impaired on ≥ 2 items of the Instrumental Activities of Daily Living scale) in comparison with ARL patients, and CADASIL patients had worse functional performances evaluated with the Disability Assessment for Dementia (DAD) scale. In CADASIL patients, hypertension was related to both DAD score and disability. The cognitive profile of CADASIL and ARL patients was similar, but on a stepwise linear regression analysis functional performances were mainly associated with the memory index (β = -0.418, P < 0.003) in CADASIL patients and the executive function index (β = -0.321, P = 0.028) in ARL. CONCLUSIONS:This study suggests that hypertension may contribute to functional impairment in CADASIL and that memory impairment has a large influence on functional decline in contrast with that observed in a sample of subjects with ARL.
journal_name
Eur J Neuroljournal_title
European journal of neurologyauthors
Ciolli L,Pescini F,Salvadori E,Del Bene A,Pracucci G,Poggesi A,Nannucci S,Valenti R,Basile AM,Squarzanti F,Bianchi S,Dotti MT,Adriano E,Balestrino M,Federico A,Gandolfo C,Inzitari D,Pantoni Ldoi
10.1111/ene.12241subject
Has Abstractpub_date
2014-01-01 00:00:00pages
65-71issue
1eissn
1351-5101issn
1468-1331journal_volume
21pub_type
杂志文章,多中心研究abstract::We describe a patient who, three years after the onset of an olivopontocerebellar atrophy, developed a right cerebral tumour. The cerebellar symptomatology also included, as in other cerebellar patients previously described, a peripheral dysgraphia. Because this deficit of writing is generally reported in patients wit...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2000.00071.x
更新日期:2000-05-01 00:00:00
abstract::Progression of atherosclerosis at extracranial carotid and intracranial arteries in patients with ischemic heart disease (IHD) is not well defined. We carried out a 5-year longitudinal study with magnetic resonance angiography (MRA) of patients with IHD to assess the incidence of progression of atherosclerosis at extr...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2003.00620.x
更新日期:2003-09-01 00:00:00
abstract:BACKGROUND AND PURPOSE: Multiple sclerosis (MS) has been associated with increased mortality rates. However, influence of lifestyle parameters remains unknown, and inconsistencies exist regarding findings for causes of death. METHODS: We conducted a population-based cohort study using the General Practice Research D...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2012.03668.x
更新日期:2012-07-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Screening batteries to narrow down a target-at-risk population are essential for trials testing neuroprotective compounds aiming to delay or prevent onset of Parkinson's disease (PD). METHODS:The PRIPS study focuses on early detection of incident PD in 1847 at baseline PD-free subjects, and asse...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2012.03798.x
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Spastic pes equinovarus is a frequent pathological posture of the lower extremity. Botulinum toxin (BoNT/A) has been successfully applied to treat lower limb spasticity. However, the best time to initiate treatment remains unclear. A beneficial effect of an early treatment has been suggested in p...
journal_title:European journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1111/ene.12381
更新日期:2014-08-01 00:00:00
abstract:BACKGROUND AND PURPOSE:People with multiple sclerosis (MS) have to face important decisions with regard to their medical treatment. The aim of this study was to evaluate whether a targeted cognitive training reduces framing effects and thus improves medical judgments. METHODS:This was a randomized, double-blind, cross...
journal_title:European journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1111/ene.13778
更新日期:2019-01-01 00:00:00
abstract::Upper limb spasticity affecting elbow, wrist, and finger flexors can be safely and effectively reduced with injections of botulinum toxin type-A (BoNT-A). It has been best studied in adults in the context of post-stroke spasticity. The clinical benefits include reduction in pain and deformity, improvement in washing a...
journal_title:European journal of neurology
pub_type: 共识发展会议,杂志文章,实务指引
doi:10.1111/j.1468-1331.2010.03129.x
更新日期:2010-08-01 00:00:00
abstract::Multiple sclerosis (MS) is a T-cell-mediated autoimmune demyelinating disease of the central nervous system, associated with an altered cytokine network. We previously assayed peripheral blood T-lymphocyte binding for two prototypic cytokines, tumour necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6), and foun...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2000.00139.x
更新日期:2000-11-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Hereditary sensory and autonomic neuropathies (HSANs) are a group of clinically and genetically heterogeneous neurological disorders characterized by sensory dysfunctions. Here, 21 affected Chinese families are reported, including 19 with congenital insensitivity to pain with anhidrosis (CIPA; na...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14234
更新日期:2020-08-01 00:00:00
abstract::We here present the case of a patient with a sleep disturbance attributed to the exploding head syndrome, recently redefined as 'episodic cranial sensory shock'. The patient, who suffered with concomitant migraine, was treated for headache prevention with daily single-pulse transcranial magnetic stimulation. Following...
journal_title:European journal of neurology
pub_type:
doi:10.1111/ene.14747
更新日期:2021-01-18 00:00:00
abstract::We describe a case of cardiac myxoma whose clinical presentation mimicked that of polyarteritis nodosa. The serum levels of MPO-ANCA and IL-6 were elevated on laboratory investigation and normalized after the removal of the tumor. We suggest that a 'true' vasculitic mechanism contributes to the pathogenesis of pseudov...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2005.01042.x
更新日期:2005-08-01 00:00:00
abstract:BACKGROUND:The purpose of the study is to investigate the epidemiology and medical expenses of Wilson's disease in Taiwan. METHODS:Cases of Wilson's disease were identified from the National Health Insurance Research Database with corresponding International Classification of Diseases, Ninth Revision (ICD-9) code 275....
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2009.02946.x
更新日期:2010-06-01 00:00:00
abstract:BACKGROUND: Myasthenia gravis (MG) is an autoimmune disorder mediated by antibodies against the acethylcholine receptor (AchR) of the neuromuscular junction in the majority of patients. METHODS: Here, we examined IgG antibodies against the type 1 nuclear antigen of Epstein-Barr virus (EBNA-1) in the sera of 158 patien...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2011.03636.x
更新日期:2012-06-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Falling is considered an important public health problem among older people. A recent cross-sectional study suggested that cognitive frailty (CF) is associated with falls. We aimed to explore whether CF is a risk factor for falls in a population-based longitudinal study. METHODS:Using data from ...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14572
更新日期:2021-02-01 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
pub_type: 杂志文章
doi:10.1111/ene.13649
更新日期:2018-07-01 00:00:00
abstract::Epilepsies are amongst the most frequent chronic neurological conditions. Patients suffer from spontaneously recurring seizures because of sudden extensive electrical gray matter discharges. Although the current drug therapy allows many patients at least some degree of a satisfying course of the disease, a substantial...
journal_title:European journal of neurology
pub_type: 杂志文章,评审
doi:10.1111/j.1468-1331.2007.01706.x
更新日期:2007-10-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Whether the neuromelanin-positive substantia nigra pars compacta area (NM-SNc) on neuromelanin magnetic resonance imaging (NM-MRI) and the specific binding ratio (SBR) on 123 I-N-v-fluoropropyl-2b-carbomethoxy3b-(4-iodophenyl)nortropane single photon emission computed tomography (DaT-SPECT) can b...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14009
更新日期:2019-11-01 00:00:00
abstract:BACKGROUND AND PURPOSE:The incidence, underlying physiopathology, features and association with lesion topography of visual hallucinations in acute stroke have scarcely been investigated. METHODS:Patients with a diagnosis of acute stroke (ischaemic or haemorrhagic) in any vascular territory, admitted within 24 h after...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.13278
更新日期:2017-05-01 00:00:00
abstract:BACKGROUND AND PURPOSE:In patients with spinal muscular atrophy (SMA), functional disease scores are frequently used to evaluate the course of the disease and the efficacy of treatment. The aim of the present study was to propose minimal clinically important difference (MCID) values for motor scores in order to estimat...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14472
更新日期:2020-12-01 00:00:00
abstract::Complex painful reflex syndrome is sometimes described in the chronic phase of stroke. Acute autonomic dysfunction (AAD), which is occasionally present in cases of acute stroke, has not been studied prospectively. The aim of the study was to investigate AAD on the hemibody contralateral to the lesion in the acute phas...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2006.01488.x
更新日期:2006-11-01 00:00:00
abstract:OBJECTIVE:Observational study to evaluate long-term effects of deep brain stimulation (DBS) of the globus pallidus internus (GPi) and the ventral intermediate thalamic nucleus (VIM) on patients with medically refractory myoclonus dystonia (MD). BACKGROUND:More recently, pallidal as well as thalamic DBS have been appli...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14737
更新日期:2021-01-16 00:00:00
abstract:BACKGROUND:Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological and haematological features. Objectives. To prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein (paraproteinaemic de...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2006.01467.x
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Our aim was to identify early imaging surrogate markers of clinical progression in patients after the first demyelinating event suggestive of multiple sclerosis treated with weekly intramuscular interferon β-1a. In a prospective observational study, the predictive role of baseline and 6-month cha...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.12716
更新日期:2015-07-01 00:00:00
abstract::Four hundred and sixty records of patients with primary torsion dystonia (296 women and 164 men) were evaluated. The mean age at disease onset was 48.3 +/- 17.7 years; 13 patients carried the DYT1 CAG deletion. The distribution of age at onset was represented by a bi-modal curve, with a nadir at 21 year separating ear...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2006.01387.x
更新日期:2006-10-01 00:00:00
abstract:BACKGROUND AND PURPOSE:In amyotrophic lateral sclerosis, studies using threshold-tracking transcranial magnetic stimulation (TMS) have identified corticomotoneuronal dysfunction as a key pathogenic mechanism. Some patients, however, display no motor response at maximal TMS intensities, termed here an 'inexcitable' moto...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14515
更新日期:2021-01-01 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
pub_type: 杂志文章
doi:10.1111/ene.13887
更新日期:2019-05-01 00:00:00
abstract:OBJECTIVE:To investigate changes in serum complements and their regulators in the pathogenesis of myasthenia gravis (MG). METHODS:Forty-four patients with acetylcholine receptor antibody-positive MG, as well as 20 patients with non-inflammatory neurological disorders were enrolled. Serum complements (C3, C4 and solubl...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14500
更新日期:2021-01-01 00:00:00
abstract::The diagnosis of mitochondrial encephalomyopathies is complex and a system for classification of the diagnosis as definite, probable, and possible has been proposed. The objective of this study was to explore the spectrum of epileptic disorders associated with probable and definite mitochondrial disease in children us...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1351-5101.2003.00724.x
更新日期:2004-02-01 00:00:00
abstract::The authors report results of a retrospective pilot study showing a strong association between patients with stroke/TIA and the presence of circulating IgG and IgA antibodies to Chlamydia pneumoniae. These results support the hypothesis that chronic active or persistent infection may play a role in the mechanism of th...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2003.00605.x
更新日期:2003-07-01 00:00:00
abstract::We performed a 2-year population-based study on status epilepticus (SE) in adults in the rural area of Lugo di Romagna, northern Italy, to verify whether an area of low-level urbanization has a lower risk of occurrence of SE (as recently suggested), different clinical features and short-term prognosis than areas of hi...
journal_title:European journal of neurology
pub_type: 信件
doi:10.1111/j.1468-1331.2005.01073.x
更新日期:2005-11-01 00:00:00