Objectives: To investigate the effectiveness of riluzole in a long-term follow-up of cohort with sporadic amyotrophiclateral sclerosis (ALS) in a real-world study. Methods: Patients with ALS between 2007 and 2013 were followed up every 3 months. Survival and tracheotomy were predefined as primary outcome measures. The cumulative defined daily dose (cDDD) of riluzole was estimated. The patients in the riluzole group were classified...查看详情
While the past 2 decades have witnessed an increasing understanding of amyotrophic lateral sclerosis (ALS) arising from East Asia, particularly Japan, South Korea, Taiwan and China, knowledge of ALS throughout the whole of Asia remains limited. Asia represents >50% of the world population, making it host to the largest patient cohort of ALS...查看详情
OBJECTIVE:The existing screening batteries assessing multiple neuropsychological functions are not specific to amyotrophic lateral sclerosis (ALS) patients and are limited to their physical dysfunctions, whereas category cognitive tests are too time-consuming to assess all the domains. The Edinburgh Cognitive and Behavioural ALSScreen (ECAS) was recently developed as a fast and easy cognitive screening tool specifically ...查看详情
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of obscure etiology. Multiple genetic studies have been conducted to advance our understanding of the disease, employing a variety of techniques such as linkage mapping in families, to genome-wide association studies and sequencing based approaches such as whole exome sequencing and whole genome sequencing and a few epigenetic analyses...查看详情
The purpose of this study was to assess the frequency of optineurin (OPTN) mutation in amyotrophic lateral sclerosis (ALS) patients from mainland China, as well as to characterize the relationship between OPTN mutation and clinical phenotypes. We examined the coding region of OPTN in 511 unrelated Chinese sporadic ALS (SALS) subjects and 204 healthy controls using a PCR direct sequencing strategy. Nine OPTN variants ...查看详情
OBJECTIVES:To describe the natural history and clinical features of sporadic amyotrophic lateral sclerosis (ALS) in Chinese patients, and to report data on the prognostic factors for survival.
METHODS:All patients referred to our ALS centre between 2003 and 2012 were followed up every 3 months. Survival and tracheotomy ...查看详情
Background – The epidemiology, diagnosis, and treatment of motor neuron disease (MND) in Chinese patients are ill known. Methods –A registered study of 461 MND patients was conducted across 10 facilities in 7 Chinese cities from February 2009 to March 2010. Results – Patients were classified as amyotrophic lateral sclerosis (ALS) (84.4%), progressive bulbar palsy (PBP) (4.1%), progressive muscular atrophy (PMA) (10.4%), or primary ...查看详情
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder with unclear etiology. Recently, intermediate CAG repeatexpansions in ATXN2, the gene responsible for spinocerebellar ataxia type 2 (SCA2), have been identified as a possible genetic risk factor for ALS. In this study, we analyzed the ATXN2 CAG repeat length in Chinese patients with ALS to evaluate the relationship between the genotype and phenotype. We studied ...查看详情