Natural history and clinical features of sporadic XXXX in China.

@entity1 @entity4572 @entity17117 @entity4681 @entity746 @entity2057
OBJECTIVES: To describe the natural history and clinical features of sporadic @entity746 ( @entity746 ) in Chinese @entity1 , and to report data on the prognostic factors for survival. METHODS: All @entity1 referred to our @entity746 centre between 2003 and 2012 were followed up every 3   months. Survival and tracheotomy were predefined as primary outcome measures. Group differences were analysed using parametric and non-parametric tests as appropriate. Survival was analysed using the Kaplan-Meier method and Cox regression analysis. RESULTS: Of the 1624 @entity1 with @entity746 , 75.1% had limb-onset, 14.0% had bulbar-onset, 7.8% had @entity17117 ( @entity17117 ), 2.6% had progressive @entity2057 and 0.5% had primary @entity4681 . The male:female ratio was 1.7:1, and the mean age at onset was 49.8   years. The median diagnostic delay was 14   months, and the median survival time after symptom onset was 71   months. Male gender, older age at symptom onset, lower body mass index, shorter diagnostic delay, bulbar-onset @entity746 phenotype, higher Airlie House category at presentation, rural place of residence, use of traditional Chinese medicine and a history of contact with pesticides were associated with poorer survival, whereas female gender or an @entity17117 phenotype may have a better prognosis. CONCLUSIONS: The clinical characteristics and outcomes of Chinese @entity1 with @entity4572 were different compared with @entity1 from other countries. Compared with other studies, the age at onset of Chinese @entity1 was earlier, the percentage of bulbar-onset @entity746 was lower and the prognosis was better. This study substantially advances the understanding of the clinical features and epidemiology of this rare disease.
