[Association between site of onset and ventilation dysfunction in patients with amyotrophic lateral sclerosis].

OBJECTIVE: To study the association between site of onset and ventilation dysfunction in Chinese patients with amyotrophic lateral sclerosis.

METHODS: The clinical data of 344 patients with sporadic amyotrophic lateral sclerosis (SALS) in Department of Neurology of Peking University Third Hospital from January 2009 to September 2013 were retrospectively reviewed. The differences of Forced Vital Capacity (FVC) between groups with distinct site of onset were analyzed.

RESULTS: There were 55, 200 and 89 patients in the group with bulbar, upper limb and lower limb onset, respectively. The values of FVC were 84% ± 14%, 83% ± 17% and 88% ± 16%, and the cases of FVC < 80% were 24 (43.64%), 81 (40.50%) and 25 (28.09%) in each group. The mean value of FVC was significantly lower and the frequency of FVC< 80% statistically higher in the group with upper limb onset in comparison with the group with lower limb onset. The mean value of FVC was lower and the frequency of FVC< 80% higher in the group with bulbar onset than in the group with lower limb onset. However, the differences were not significant between the two groups.

CONCLUSIONS: Compared with SALS with upper limb onset, ventilation function disorder may occur less and later in patients with lower limb onset, who may also require noninvasive positive pressure ventilation (NIPPV) less and later.