Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis
Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis
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DOI：
10.1016/j.jns.2008.09.024
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发表时间：
2009-01-15
影响因子：
4.4
通讯作者：
Sobue, Gen
中科院分区：
文献类型：
--
作者：
Atsuta, Naoki;Watanabe, Hirohisa;Sobue, Gen
目的：描述散发性肌萎缩侧索硬化症（ALS）的大规模samples in Japanes.Methods的详细临床特征：我们评估了日本散发性ALS患者的临床特征，根据日本厚生劳动省的全国登记系统。我们描述了2003年至2006年期间3428例新登记病例，以分析首发症状和相关临床特征，描述了2005年单年登记的4202例ALS患者的横断面概况，气管切开正压通气（TPPV）2128例结果：发病年龄较大的ALS患者进展至TPPV期的速度明显快于发病年龄较小的ALS患者。长期TPPV患者亚群表现为眼肌麻痹，而发病年龄较大的患者其出现率低于发病年龄较小的患者。此外，发病年龄强烈影响初始症状的频率：构音障碍、吞咽困难、颈部无力和呼吸障碍在发病年龄较大的患者中更常见，而上肢或下肢无力在发病年龄较小的患者中更常见。此外，这些最初的症状仍然是最突出的后续阶段，表明最初的症状决定的主要临床特征，即使在先进的illumination.Conclusions：我们目前的研究表明，ALS的症状特征的强烈影响发病年龄的大规模的样本。(C)2008 Elsevier B.V.保留所有权利。
Purpose: To profile the detailed clinical features of sporadic amyotrophic lateral sclerosis (ALS) on large-scale samples in Japan.Methods: We assessed the clinical features of sporadic ALS patients in Japan, based on the nationwide registration system of the Ministry of Health, Labor and Welfare of Japan. We described 3428 new cases registered cases between 2003 and 2006 to analyze initial symptoms and related clinical features, 4202 cases registered in the single year of 2005 to describe the cross-sectional overview of the ALS patients, and a total of 2128 cases with tracheostomy positive pressure ventilation (TPPV) from all of the registration data from 2003 to 2006 to describe the features of ALS patients with TPPV.Results: The patients with an older age at onset progressed more rapidly to the TPPV stage than those with a Younger age at onset. The subpopulation of patients with long-standing TPPV showed ophthalmoplegia, while its appearance rate was less in the patients with an older age at onset than in those with a younger age at onset. Furthermore, age at onset strongly influenced the frequency of initial symptoms: dysarthria, dysphagia, neck weakness and respiratory disturbance were more frequent in patients with an older age at onset, while upper or lower limb weakness was observed more frequently in patients with a younger age at onset. In addition, those initial symptoms were still the most prominent at the follow-up stage, Suggesting that the initial symptoms determine the major clinical features even in advanced illness.Conclusions: Our present study demonstrated that symptomatic features of ALS are strongly influenced by the age at onset by the large scale of samples. (C) 2008 Elsevier B.V. All rights reserved.