Abstract：
OBJECTIVE:In order to define the predictors of prolonged survival available at the time of first examination we performed a historical cohort study of amyotrophis sclerosis (ALS) patients referred to our ALS Clinic over the last 20 years. METHODS:In a group of 1034 patients with the diagnosis of definite or probable ALS the effects of individual prognostic factors on tracheostomy-free survival were assessed with the Kaplan-Meier life-table method. The prognostic value of each factor was estimated using univariate and multivariate Cox proportional hazard analyses. RESULTS:The median survival time was 3.45 years, (95%CI 3.27-3.74). Both the univariate and multivariate Cox models indicated that younger age, limb site of onset, longer diagnostic delay, lower Appel ALS score (AALSS) at first examination, lower AALSS-rate of change between first symptom and first exam (preslope), and higher baseline forced vital capacity (FVC) were associated with longer survival. In addition, four factors: age, diagnostic delay, baseline FVC and AALSS preslope have been identified as independent predictors of survival in our patient population. CONCLUSIONS:The identification of younger age, limb site of onset and longer diagnostic delay as predictors of prolonged survival in ALS clinic population supports the findings of several, earlier studies that were based on smaller groups of patients. More significantly, several additional variables assessed at the first examination predict longer survival: lower baseline AALSS, lower AALSS- preslope and higher baseline FVC. All of these parameters are of value in patient management and in clinical trial development.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Czaplinski A,Yen AA,Appel SHdoi
10.1007/s00415-006-0226-8subject
Has Abstractpub_date
2006-11-01 00:00:00pages
1428-36issue
11eissn
0340-5354issn
1432-1459journal_volume
253pub_type
杂志文章abstract：BACKGROUND AND OBJECTIVE:The number of neurological second opinions (SO) and tertiary referrals (TR) is increasing. The main purpose of this study was to assess whether a day-care admission made a meaningful contribution to standard neurological outpatient care, for a wide range of second opinions and tertiary referral...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s00415-008-0019-3
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abstract：:Uric acid (UA) is reduced in multiple sclerosis (MS), and possibly relates to MS outcomes, with lower UA levels in subjects experiencing a relapse or presenting higher disability scores. The present retrospective longitudinal study evaluated UA variations in MS, in relation to clinical relapses, disability progression...
journal_title：Journal of neurology
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doi：10.1007/s00415-015-7666-y
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abstract：:Myotonic dystrophy (DM) is an autosomal dominant neuromuscular disease with an estimated incidence of 1 in 8000 and is the most common form of muscular dystrophy affecting adults. An unstable, untranslated part of the myotonic dystrophy protein kinase gene on the long arm of chromosome 19, composed of CTG repeats, is ...
journal_title：Journal of neurology
pub_type： 临床试验,杂志文章,随机对照试验
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abstract：:Focal transcranial magnetic stimulation (TMS) of the motor cortex was used to study excitatory and inhibitory stimulation effects in 25 patients with writer's cramp and 25 healthy volunteers. We investigated excitatory and inhibitory corticospinally mediated motor effects in muscles contralateral to the stimulation si...
journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/s004150170269
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abstract：:Alexander disease (AD) is a rare genetic disorder of the central nervous system due to a dysfunction of astrocytes. The most common infantile form presents as a progressive leukodystrophy with macrocephalus. Recently, heterozygous de novo mutations in the gene encoding glial fibrillary acidic protein (GFAP) have been ...
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doi：10.1007/s00415-003-0995-2
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doi：10.1007/s00415-009-5156-9
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doi：10.1007/BF00314238
更新日期：1988-07-01 00:00:00
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doi：10.1007/s00415-003-0941-3
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journal_title：Journal of neurology
pub_type： 杂志文章,评审
doi：10.1007/s00415-003-1304-9
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doi：10.1007/s00415-011-6069-y
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journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00873519
更新日期：1994-08-01 00:00:00
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pub_type： 杂志文章
doi：10.1007/BF00873552
更新日期：1995-07-01 00:00:00
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journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00312944
更新日期：1977-09-12 00:00:00
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pub_type： 临床试验,杂志文章
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更新日期：1997-10-01 00:00:00
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doi：10.1007/s00415-011-5996-y
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journal_title：Journal of neurology
pub_type： 临床试验,杂志文章,随机对照试验
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abstract：:Cortical tubers are very common in tuberous sclerosis complex (TSC) and widely vary in size, appearance and location. The relationship between tuber features and clinical phenotype is unclear. The aim of the study is to propose a classification of tuber types along a spectrum of severity, using magnetic resonance imag...
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journal_title：Journal of neurology
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abstract：:Two cases of Addison's disease associated with myasthenia gravis are reported. This association has been described only rarely in the literature. In the first case, there were marked immunological abnormalities. It is most likely that the origin of the adrenocortical insufficiency in the second case is tuberculous. Th...
journal_title：Journal of neurology
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doi：10.1007/BF00313835
更新日期：1985-01-01 00:00:00
