Epidemiological characteristics of motor neuron disease in Chinese patients

Cui, Fang; Liu, Ming-Sheng; Chen, Y.; Huang, Xusheng; Chen, Pu; Lu, Jiahong; Zhou, Dong; Zhang, C.; Yan, Chuanzhu; Li, C.; Ding, Xiaojun; Liu, Y.; Li, X.; Jiang, Yanfeng; Zhang, J.; Shang, Huifang; Yao, Xiaoli; Ding, Yan; Niu, Qi; Wang, L.

doi:10.1111/ane.12240

Cited by 24 publications

(19 citation statements)

References 26 publications

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“…The average survival time is only 3-5 years in the Chinese Han population [15] . However, in clinical experience, we find some patients die within 1 or 2 years after diagnosis and some could live >5 years after diagnosis, except patients with bulbar-onset and advanced age [5,6,16] , which are indicators of poor prognosis for sALS patients.…”

Section: Discussionmentioning

confidence: 99%

“…Some studies had reported that weight loss is a serious issue in ALS [20,21] , though some researchers put forward that weight loss cannot always be attributed to dysphagia because of high calorie nutritional supplements and percutaneous endoscopic gastrostomy (PEG) [22] . In Chinese ALS patients, only 27.1% used PEG and 29.1% used nasal feed [15] . So bulbar-involved was an indicator of quick progression in limb-onset sALS.…”

Section: Discussionmentioning

confidence: 99%

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Spread Direction and Prognostic Factors in Limb-Onset Sporadic Amyotrophic Lateral Sclerosis

Jin

Jia

et al. 2016

Eur Neurol

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Objective: To investigate the spread direction and prognostic factors in limb-onset sporadic amyotrophic lateral sclerosis (sALS). Methods: Medical records of 128 patients with sALS were reviewed. Variables studied were age at symptom onset, gender, region and lateralization of onset, onset to diagnosis interval (ODI), progression direction, bulbar-involved, time from onset to bulbar-involved, ALSFRS-r, upper motor neuron (UMN) signs and progression rate. Results: First, the horizontal and vertical directions are major spreading directions in limb-onset sALS. Second, in crossed and interposed groups, while ODI is shorter, the progression rate is faster and UMN signs are more pronounced (p < 0.05). Third, ALSFRS-r, UMN signs and progression rate have significant differences between with-bulbar-involved and without-bulbar-involved (p < 0.05). The progression rate is related to the time from onset symptoms to bulbar-involved (correlation coefficient -0.535, p = 0.00). Fourth, in multivariate regression analysis, progression rate could respectively increase by about 1.14 and 3.89 times with shorter ODI and bulbar-involved in limb-onset sALS patients. Conclusions: The horizontal and vertical directions are the major spread directions in limb-onset sALS. Except for ALSFRS-r and ODI, bulbar-involved is an adverse factor for ALS progression, and progression rate is related to the time from onset symptoms to bulbar-involved.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Spread Direction and Prognostic Factors in Limb-Onset Sporadic Amyotrophic Lateral Sclerosis

Jin

Jia

et al. 2016

Eur Neurol

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“…The frequency is lower in South Korea and Taiwan, respectively 12 71. In China, 56% of patients with ALS (n=441) presenting to urban tertiary centres were on either gastrostomy or nasogastric feeding 75. This was in sharp contrast to a different study of patients with ALS from the less-developed South-West China where only 15 of 1116 (0.01%) patients were reported to have percutaneous gastrostomy 14…”

Section: Disease Progression and Survival In Asiamentioning

confidence: 95%

Amyotrophic lateral sclerosis and motor neuron syndromes in Asia

Shahrizaila

Sobue

Kuwabara

et al. 2016

J Neurol Neurosurg Psychiatry

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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. Furthermore, Asia represents a diverse population in terms of ethnic, social and cultural backgrounds. In this review, an overview is presented that covers what is currently known of ALS in Asia from basic epidemiology and genetic influences, through to disease characteristics including atypical phenotypes which manifest a predilection for Asians. With the recent establishment of the Pan-Asian Consortium for Treatment and Research in ALS to facilitate collaborations between clinicians and researchers across the region, it is anticipated that Asia and the Pacific will contribute to unravelling the uncertainties in ALS.

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“…In order to address these questions, quantitative assessment of cerebellar neurons in different forms of ALS is needed. At end stage, two main forms of ALS can be identified, which should affect cerebellar circuits differently-classical ALS with both upper and lower motor neuron degeneration, which should affect both cerebro-and spinocerebellar circuits, and cases with only lower motor neuron degeneration (progressive muscular atrophy; PMA), 14 which should have only spinocerebellar involvement. The present study quantifies Purkinje and granule cells in these cerebellar functional zones with three aims, to: (1) determine the effect of repeat expansions in the C9ORF72 and ATXN2 genes on cerebellar neurons; (2) determine whether the cerebellar degeneration identified in sporadic ALS using in vivo imaging indicates underlying neuronal degeneration; and (3) elucidate the effect of motor cortical degeneration on cerebellar neurons by contrasting pathological ALS subtypes.…”

mentioning

confidence: 99%

Cerebellar neuronal loss in amyotrophic lateral sclerosis cases with ATXN2 intermediate repeat expansions

Tan

Kril

McGinley

et al. 2016

Annals of Neurology

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The present study has established a selective loss of Purkinje cells in the cerebellar vermis of ALS cases with intermediate repeat expansions in the ATXN2 gene, suggesting a divergent pathogenic mechanism independent of upper and lower motor neuron degeneration in ALS. We discuss these findings in the context of large repeat expansions in ATXN2 and spinocerebellar ataxia type 2, providing evidence that intermediate repeats in ATXN2 cause significant, albeit less substantial, spinocerebellar damage compared with longer repeats in ATXN2.

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Epidemiological characteristics of motor neuron disease in Chinese patients

Cited by 24 publications

References 26 publications

Spread Direction and Prognostic Factors in Limb-Onset Sporadic Amyotrophic Lateral Sclerosis

Spread Direction and Prognostic Factors in Limb-Onset Sporadic Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis and motor neuron syndromes in Asia

Cerebellar neuronal loss in amyotrophic lateral sclerosis cases with ATXN2 intermediate repeat expansions

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