Abstract：
:The aim of this work was to determine whether survival changed during 2002-2009 at a French amyotrophic lateral sclerosis (ALS) center. We included all patients with ALS who were seen consecutively at the center from January 2002-May 2009. Participants were followed from date of first visit through death, date of censoring, or December 31, 2009, whichever occurred first. Cox proportional hazard models computed hazard ratios (HR; 95% confidence interval CI) of death, and flexible modeling of continuous predictors (splines) assessed trends in survival. We analyzed a total of 2,037 ALS patients, of whom 1,471 died before the end of follow-up. Median survival was 2.83 years from onset and 1.65 years from first visit. Compared to patients first seen before 2004, the HR of death was 0.97 (95% CI = 0.85-1.11, p = 0.6721) for patients first seen in 2004-2005, 0.96 (95% CI = 0.83-1.10, p = 0.5125) for 2006-2007, and 0.56 (95% CI = 0.46-0.69, p < 0.0001) after 2007, while adjusting for other survival predictors. Spline analysis confirmed that survival remained stable during 2002-2006, then markedly improved. The proportion of patients receiving non-invasive ventilation (NIV) increased from 16 (2004) to 51% (2008). At this large ALS center, survival improved after 2006. Because more aggressive use of NIV was the principal therapeutic adaptation, our data suggest that better survival resulted from improved respiratory care.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Gordon PH,Salachas F,Bruneteau G,Pradat PF,Lacomblez L,Gonzalez-Bermejo J,Morelot-Panzini C,Similowski T,Elbaz A,Meininger Vdoi
10.1007/s00415-011-6403-4subject
Has Abstractpub_date
2012-09-01 00:00:00pages
1788-92issue
9eissn
0340-5354issn
1432-1459journal_volume
259pub_type
杂志文章abstract：OBJECTIVE:To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6. METHODS:Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched health...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-008-0907-6
更新日期：2008-08-01 00:00:00
abstract：BACKGROUND:Studies provide inconclusive results on the question whether loss of mental wellbeing is actually associated with decline in physical function in amyotrophic lateral sclerosis (ALS). The purpose of this study was to determine predictors of mental wellbeing in ALS. METHODS:In total, n = 330 ALS patients were...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-10027-x
更新日期：2020-12-01 00:00:00
abstract：:Despite the fact that almost one-third of patients suffer from visual deficits following brain damage; neuro-visual rehabilitation to compensate for visual field deficits is relatively neglected in the clinical setting. This is in contrast to physio and speech therapies, which are the bread and butter of rehabilitativ...
journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-016-8291-0
更新日期：2017-06-01 00:00:00
abstract：:The influence of portal-systemic shunting on maximal motor nerve conduction velocity (MCV) was analyzed in rats with portacaval shunts. At 3 and 8 weeks after the shunting there was a marked but transient fall in MCV. This was not prevented by colectomy. At 16 weeks the MCV had almost normalized, despite sustained por...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313181
更新日期：1980-01-01 00:00:00
abstract：:Horizontal eye-movement responses of four patients with Alzheimer-type dementia were recorded using the infrared oculographic technique. Abnormally frequent saccadic intrusions occurred during the fixation and slow, smooth pursuit tasks in all four patients. Saccadic intrusions have previously been reported in the lit...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313742
更新日期：1983-01-01 00:00:00
abstract：:Patients in a minimally conscious state (MCS) show restricted signs of awareness but are unable to communicate. We assessed cerebral glucose metabolism in MCS patients and tested the hypothesis that this entity can be subcategorized into MCS- (i.e., patients only showing nonreflex behavior such as visual pursuit, loca...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-011-6303-7
更新日期：2012-06-01 00:00:00
abstract：BACKGROUND:Perivascular spaces can become detectable on magnetic resonance imaging (MRI) upon enlargement, referred to as enlarged perivascular spaces (EPVS) or Virchow-Robin spaces. EPVS have been linked to small vessel disease. Some studies have also indicated an association of EPVS to neuroinflammation and/or neurod...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-09971-5
更新日期：2020-11-01 00:00:00
abstract：BACKGROUND:Glatiramer acetate (GA) therapy following brief, low-dose induction with mitoxantrone was safe and more effective than GA alone in suppressing inflammatory disease activity, as determined by a significant reduction in gadolinium (Gd)- enhancing MRI lesions, in a 15- month, randomized, single-blind study of r...
journal_title：Journal of neurology
pub_type： 杂志文章,随机对照试验
doi：10.1007/s00415-008-0911-x
更新日期：2008-10-01 00:00:00
abstract：BACKGROUNDS:Wake-Up Stroke (WUS) patients are generally excluded from thrombolytic therapy (rTPA) due to the unknown time of stroke onset. This study aimed to investigate the effects of rTPA in WUS patients during every day clinical scenarios, by measuring ischemic lesion volume and functional outcomes compared to non-...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-019-09603-7
更新日期：2020-03-01 00:00:00
abstract：:Five patients are described who developed unilateral narrowing of the palpebral fissure that was aggravated by smiling. They had ischaemic lesions involving mainly the contralateral lenticulocapsular area. This rare but distinct symptom may be an action induced focal eyelid dystonia, and could be described as'smile-wi...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150170179
更新日期：2001-05-01 00:00:00
abstract：:An ultrastructural study of biopsied muscles was performed in seven patients with rimmed vacuolar distal myopathy, which was characterized by prominent rimmed vacuoles in the muscle fibers. The earliest changes noted were focal proliferation of the Golgi's apparatus and mitochondrial degeneration with myofibrillar los...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00314132
更新日期：1987-04-01 00:00:00
abstract：:PNPLA6 mutations, known to be associated with the development of motor neuron phenotypes, have recently been identified in families with Boucher-Neuhäuser syndrome. Boucher-Neuhäuser is a rare autosomal recessive syndrome characterized by the co-occurrence of cerebellar ataxia, hypogonadotropic hypogonadism, and chori...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-014-7516-3
更新日期：2014-12-01 00:00:00
abstract：:Neuromyelitis optica (NMO) is an uncommon, demyelinating disease that causes long-term disability in adults. Though much has recently been learned about its pathogenesis, there are still only a few studies regarding the epidemiology of NMO. The aim of the study was to describe the epidemiology of NMO among adults in t...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-013-6926-y
更新日期：2013-08-01 00:00:00
abstract：OBJECTIVE:Widespread metabolic changes are seen in neurodegenerative disease and could be used as biomarkers for diagnosis and disease monitoring. They may also reveal disease mechanisms that could be a target for therapy. In this study we looked for blood-based biomarkers in syndromes associated with frontotemporal lo...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-09824-1
更新日期：2020-08-01 00:00:00
abstract：OBJECTIVE:The aim of this study was to investigate the relationship between type II diabetes mellitus (T2DM) and ALS incidence using the National Health Insurance Research Database and Serious Disabling Disease database of Taiwan. METHODS:This was a population-based cohort study. The index date was the date of the fir...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-019-09405-x
更新日期：2019-09-01 00:00:00
abstract：:We have studied the relationship between the apolipoprotein E gene (APOE) and the development of myoclonus, tremors, rigidity and seizures in 168 patients with probable early-onset Alzheimer's disease (AD). There was a statistically significantly lower risk of tremor for carriers of the epsilon 4 allele of APOE. This ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00900501
更新日期：1996-06-01 00:00:00
abstract：:This article describes the clinical features of anterior semicircular canal benign paroxysmal positional vertigo (AC-BPPV) and a new therapeutic maneuver for its management. Our study was a retrospective review of cases from an ambulatory tertiary referral center. Thirteen patients afflicted with positional paroxysmal...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-009-5208-1
更新日期：2009-11-01 00:00:00
abstract：OBJECTIVE:Gluten neuropathy (GN) is the term used to describe peripheral neuropathy that occurs in patients with gluten sensitivity (GS) or coeliac disease (CD) in the absence of other risk factors. We aimed to describe the neurophysiological progression rate of GN across time and look into the potential role of geneti...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-020-10137-6
更新日期：2021-01-01 00:00:00
abstract：:Serial magnetic resonance imaging (MRI) detects substantial subclinical disease activity in multiple sclerosis (MS) and is presently included in most treatment trials as an objective outcome measure. Our current knowledge of the role of MRI in MS treatment trials is derived from very limited patient studies, and the a...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150050053
更新日期：1997-02-01 00:00:00
abstract：:During a period of 42 months, we studied the prevalence of epilepsy in a specific health district, composing by four towns with 98,405 inhabitants older than 10 years. This has been accomplished by a two-phase cross-sectional study. The prevalence rate observed was 4.12/1000 inhabitants for all types of epilepsy. No s...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150170091
更新日期：2001-09-01 00:00:00
abstract：:There is still uncertainty regarding risk factors for cancer occurrence in patients with myasthenia gravis (MG). The objective of this study is to determine the prevalence of extrathymic neoplasms in patients with MG and the factors associated with tumor occurrence. The archives of four tertiary MG centers were consul...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-009-5091-9
更新日期：2009-08-01 00:00:00
abstract：:Autonomic dysfunction is frequently observed in patients with multiple sclerosis (MS), but clinical studies disagree on the frequency and type of abnormalities in autonomic function tests. Orthostatic dizziness (OD) has been reported in up to 49% of patients, but the pathophysiological mechanisms are poorly understood...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150050407
更新日期：1999-07-01 00:00:00
abstract：:A comparative study of clinical and morphological findings in three fatal cases of acute necrotizing hemorrhagic encephalomyelitis (ANHE) and hyperacute experimental allergic encephalomyelitis (HEAE) in rhesus monkeys is reported. In all cases ANHE was characterized clinically by definite prodromal respiratory infecti...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313109
更新日期：1979-08-01 00:00:00
abstract：OBJECTIVE:To analyse clinical and therapeutic aspects of epilepsy secondary to ulegyria in adults. PATIENTS:Out of 1,020 consecutive patients studied at a tertiary care epilepsy centre, eight cases of ulegyria were identified. All patients had comprehensive clinical evaluation, neuropsychological testing, interictal E...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-005-0829-5
更新日期：2005-10-01 00:00:00
abstract：:Acid maltase deficiency is described in non-identical adult twins. The onset of the disease can be traced into late infancy; the clinical picture is one of severe muscular dystrophy; respiratory insuficiency was the cause of death in one case. The autopsy showed the central nervous system, heart and liver to be spared...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313272
更新日期：1976-08-06 00:00:00
abstract：:Levamisole seems to regulate cell-mediated immunity by restoring T-cell function. Since a deficiency of T lymphocytes has been described by various authors in multiple sclerosis patients. Of the 85 patients involved in the trial, evaluation of functional and neurological scores was possible in 54 (32 with placebo and ...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章
doi：10.1007/BF00313411
更新日期：1982-01-01 00:00:00
abstract：OBJECTIVE:To evaluate the role of MR morphometry in the characterization of cerebral microangiopathy (CMA) in relation to clinical and neuropsychological impairment. SUBJECTS AND METHODS:3D MR images of 27 patients and 27 age-matched controls were morphometrically analysed for regional thickness. The normalized values...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-005-0671-9
更新日期：2005-04-01 00:00:00
abstract：:A spontaneous dissecting aneurysm of the intracranial portion of the dominant right vertebral artery presented as massive subarachnoid hemorrhage, excruciating headache, and respiratory arrest in a 57-year-old white man with a history of systemic hypertension. He died on the 3rd day. Postmortem examination revealed a ...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00313594
更新日期：1983-01-01 00:00:00
abstract：:To determine the epidemiological, clinical, and genetic characteristics of congenital hypomyelinating leukodystrophies, including Pelizaeus-Merzbacher disease (PMD), we conducted a nationwide epidemiological survey in Japan. A two-step survey targeting all medical institutions specializing in pediatric neurology and c...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-014-7263-5
更新日期：2014-04-01 00:00:00
abstract：BACKGROUND:Progression of parkinsonian motor impairment is usually rapid and relentless in multiple system atrophy (MSA). However, it may also be subject to considerable variation. Prospective natural history studies using validated rating scales are required to accurately determine the progression of parkinsonism in M...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-005-0617-2
更新日期：2005-01-01 00:00:00
