Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study.

OBJECTIVE: We describe and compare the sociodemographic and clinical features, treatments, and prognoses and survival times of patients with amyotrophic lateral sclerosis (ALS) in Africa. METHODOLOGY: We conducted a multicentre, hospital-based cohort study in Africa. Patients with ALS diagnosed in the neurology departments of participating hospitals from 2005 to 2017 were included. Subgroup analysis was performed by subcontinent. Survival analyses were conducted using the Cox proportional hazards model. RESULTS: Nine centres from eight African countries participated. A total of 185 patients with ALS were included: 114 from Northern Africa, 41 from Western Africa and 30 from Southern Africa. A male predominance (male to female ratio 2.9) was evident. The median age at onset was 53.0 years (IQR 44.5-64.0 years). The onset was bulbar in 22.7%. Only 47 patients (26.3%) received riluzole, mainly in Northern and Western Africa. The median survival from the time of diagnosis was 14.0 months (95% CI 10.7 to 17.2 months). The median survival was longer in Northern Africa (19.0 months, 95% CI 10.8 to 27.2 months) than in Western (4.0 months, 95% CI 0.8 to 7.1 months) and Southern (11.0 months, 95% CI 5.6 to 16.4 months) Africa (Breslow test, p<0.0001). Both subcontinental location and riluzole treatment independently affected survival. CONCLUSION: More African patients with ALS were male and younger and exhibited a lower proportion of bulbar onset compared with patients with ALS from Western nations. Survival was consistent with that in Western registers but far shorter than what would be expected for young patients with ALS. The research improves our understanding of the disease in Africa.

Predictive factors for gastrostomy at time of diagnosis and impact on survival in patients with amyotrophic lateral sclerosis.

BACKGROUND: Gastrostomy is recommended in patients with Amyotrophic Lateral Sclerosis (ALS) in the presence of weight loss over 10% as compared to usual weight, repeated aspirations or meal time duration longer than 45 min. Currently, the impact of gastrostomy on survival of ALS patients is not clear. AIMS: i) to describe diagnosis factors associated with the indication for gastrostomy ii) to evaluate survival of ALS patients with gastrostomy indication according to their acceptance of feeding tube placement. METHODS: Patients with ALS were included and followed in the ALS referral centre of Limoges's teaching hospital between 2006 and 2017. Neurological, nutritional and respiratory status was assessed prospectively from diagnosis to death. Statistical analysis was performed using Mann-Whitney test, Chi2 tests, Cox model and multivariate logistic regression. RESULTS: Two hundred and eighty-five patients were included. Among the 182 for whom gastrostomy was indicated, 63.7% accepted the placement. The median time was 7.3 months [IQR: 3.2-15.0] and 2.7 months [IQR: 0.9-5.8] respectively from diagnosis to indication and from indication to placement. Weight loss >5% significantly increased the risk of death by 17% (p < 0.0001). At time of diagnosis, bulbar onset, a loss of one point in the body mass index or on the bulbar functional scale were all positively associated with indication for gastrostomy (aOR = 10.0 [95%CI: 1.96-25.0]; p = 0.002, aOR = 1.17 [95%CI: 1.02-1.36]; p = 0.025 and aOR = 1.19 [95%CI: 1.06-1.32]; p = 0.002, respectively). However, gastrostomy placement did not have any impact on survival (aHR = 1.25 [95%CI: 0.88-1.79]; p = 0.22). CONCLUSION: Both neurological and nutritional criteria were associated with an indication for gastrostomy at diagnosis. Gastrostomy placement had no impact on survival. The study of earlier gastrostomy placement might be of interest in further prospective studies.