Results from the first French ALS register: the Limousin ALS register.
Résumé
Background: In France, until recently, there was no register of ALS cases. Hence description of the disease in France was only hospital based and then exposed to bias.Objective: To assess the incidence of ALS in the Limousin region of France using multiple sources of cases ascertainment, and to describe the sociodemographical and clinical profile of new ALS cases.Methods: A register has been settled in the Limousin region of France, 743,000 inhabitants (1). Multiple sources of information were used to ensure complete case ascertainment of individuals diagnosed with ALS according to Airlie House criteria, within the Limousin since 2000. Multiple sources of case were (i) Limousin ALS expert center and other ALS centers of France; (ii) private hospitals and clinics; (iii) health insurance structures (reimbursement of Rilutek® and long duration affection notification); and (iv) private neurologists. Data were collected in a secured database. Crude incidence per 100,000 inhabitants was assessed using estimates of population from the Insee (2). Direct standardized incidence based on 2010 US population was also calculated (3). Ninety-five percent confidence intervals (95% CI) were calculated assuming a poisson distribution.Results: Between 1 January 2000 and 31 December 2011 (12 years), 279 patients were diagnosed as having possible, probable, probable laboratory supported, or definite ALS. The median number of sources by patient was 2 (Interquartile range: 2–3). The average annual crude incidence rate was 3.18 per 100,000 person-years (95% CI 2.81–3.56). Standardized incidence on US population was 2.18 (95% CI 1.92–2.45). Mean age at diagnosis was 69.1 ± 11.2 years and the male/female sex-ratio was 1.45. First symptoms were mostly spinal (65.5%) and 6.45% of cases had familial ALS.Discussion and conclusion: We report here the results of the first ALS register settled in France. The epidemiological profile of ALS incidence in Limousin, France is consistent with those published by other European registers (2). The register will be the base of analytic and geoepidemioloical studies (clustering). We are now extending the register in other region of France: Languedoc Roussillon and some departments of Rhône Alpes.Acknowledgments: Sources of cases: French National coordination of ALS centers; Health insurance structures: Régime Général, Mutuelle Sociale Agricole, Régime Social des Indépendants, Caisse Nationale Militaire de Sécurité Sociale; Private Neurologists; Hospital and Clinics.