A comparative study of South African and Portuguese amyotrophic lateral sclerosis cohorts.

OBJECTIVE

Data on the epidemiological and clinical aspects of ALS originate from a few world regions, and very little is known about ALS in low and middle-income countries, in particular Sub-Saharan Africa. This brief report attempts to provide preliminary perspectives on the clinical features and management of ALS in Sub-Saharan Africa by comparing two cohorts from South Africa (SA) and Portugal.

METHODS

The study was performed at ALS clinics at Tygerberg Hospital, Cape Town, South Africa, and Centro Hospitalar Universitário Lisboa-Norte, Portugal. We included all patients diagnosed over a four-year period, and collected demographic and clinical data at diagnosis, longitudinal data on disease progression and management over 12 months, and mortality rates at 12 and 24 months.

RESULTS

SA patients were younger and had a higher rate of spinal-onset disease than their Portuguese counterparts. During the 12-month follow-up, NIV was introduced in half of Portuguese patients, but only a quarter of SA patients. Parenteral nutrition was introduced in less than 10% of patients in both groups. No SA patients used riluzole, while 100% of Portuguese patients did. Mortality rates were significantly higher in the SA cohort at both 12 months (35% vs 16%; p < .0001) and 24 months (63% vs 39%; p < .0001).

CONCLUSIONS

Although SA patients were younger and more likely to have spinal-onset disease, mortality was higher in this cohort. There was a significant difference in utilisation of NIV and riluzole between the two cohorts, both of which may influence survival.