A recent review highlights the importance of population-based registers of amyotrophic lateral sclerosis (ALS) in Europe, which for more than 20 years have helped scientists understand the disease’s incidence, prevalence, characterization and genetics, as well as the outcome assessment of clinical trials.
Population-based ALS registers capture all cases in a given area regardless of age, health or socioeconomic status. They also provide useful information about disease incidence, prevalence, spatial distribution, differences in clinical characterization, outcome and risk analysis.
Ireland and Italy were the first European countries to launch such registers, in the 1990s; Britain and the Netherlands followed soon after. In each case, country-specific registers have provided valuable knowledge on epidemiology, disease progression, health service planning, quality of life assessment, and mapping of patients’ journeys.
According to the authors, an analysis of ALS across European registers yields an average ALS incidence rate of 2.6 person-years per 100,000 inhabitants, and prevalence rates of 7 to 9 per 100,000 inhabitants, with a mean life expectancy of 30 months from the first symptoms.
Data from these registers can also help identify any hidden bias that skews incidence estimates or survival rates, making the ALS diagnosis more inclusive, since knowing about the different manifestations of ALS helps doctors diagnose their patients more accurately.
“Population-based registers can inform health services. The availability of precise incident, prevalent and clinical trajectory data can permit detailed service planning and can enable projection of future societal needs,” researchers wrote. “Registers also permit high-level comparative studies of different interventions within individual geographic regions using outcomes such as hospitalizations and survival.”
In addition, such registers can improve clinical trials, as collecting data about outcomes after exposure or intervention in ALS patients provides real-world information on the longer-term effect of a specific treatment.
“Prospective population-based disease registers are invaluable in patient-oriented research of rare diseases,” researchers wrote. “As exemplified by the success of European ALS registers, population-based databases can identify and address biases that are intrinsic to other types of data. Notwithstanding their limitations, registers can provide unique and often unexpected insights into disease epidemiology and pathobiology and can inform the types of healthcare that are of greatest benefit to patients.”
Finally, they stress that funding agencies, healthcare providers and institutional review bodies understand the value of these registers and protect them, so they can continue to provide important information that affects the lives of ALS patients.