Objective: The Australian Motor Neurone Disease Registry was established in 2004. It is a cohort study of patients presenting with MND collecting data relating to clinical phenotypes, time to major interventions, health service utilization and survival.
Methods: Patients with a diagnosis of MND were invited to participate in the registry from major centres and clinicians throughout Australia. Case report forms were submitted at registration, from ongoing assessments and at completion to the central data registry for data entry or directly entered from via the web.
Results: 827 patients were registered as of December 2009 with a gender ration of 1.5:1 male to female. The mean age of onset was 60 (males) and 61 years (females). Mean survival for all patients from symptom onset was 34 months for males and 37 months for females. Clinical phenotypes determined by region of onset and clinical signs demonstrated similar survival and gender distribution as has been demonstrated. Patients designated as Global_Bulbar onset had the shortest survival while Flail_arm and Flail Leg had the longest survival times.
Conclusions: The national MND registry is proving to be a robust tool for analysis of survival and the identification of clinical phenotypes for MND, critical for stratification of patients in future treatment trials. This database provides mechanism to compare survival times, therapeutic interventions and health service utilization at a national level. This transcontinental registry forms part of a growing worldwide trend to obtain epidemiological and natural history information for MND, and provides a hub for collaborative approaches.