Volume 184 - Issue 7
Establishment of an Australian motor neurone disease registry
Authors: Matthew C Kiernan, Paul Talman, Robert D Henderson, Rodney Harris and on behalf of the Australian Motor Neurone Disease Registry Steering Committee
Med J Aust 2006; 184 (7): 367-368. || doi: 10.5694/j.1326-5377.2006.tb00279.x
Published online: 3 April 2006
Published online: 3 April 2006
To the Editor: Motor neurone disease (MND) is a relentlessly progressive neurodegenerative disease with a median survival of 1–3 years. It results in the death of nearly 400 Australians per year.1 The management of MND remains problematic, tending to be offered in a heterogeneous and ad-hoc fashion across Australia. This heterogeneity arises in part from a lack of understanding of the aetiology of the disease and its progression in different patients,2 the absence of established guidelines for standard care,3 and a lack of concentrated experience among medical practitioners, nursing and allied health care workers in treating patients with MND.
With new diagnostic techniques, treatments and interventions for MND undergoing trials, there is a clear need for more baseline information on MND management and outcomes and a method for monitoring any changes on a population basis.4 To facilitate this, the Australian Motor Neurone Disease Registry (AMNDR) has been developed. The Registry is governed by a steering committee comprising specialist physicians from each state and territory around Australia, neuroscientists, an epidemiologist and patient representatives.
AMNDR was launched in Sydney in June 2004 to coincide with Motor Neurone Disease Global Awareness Day and, to November 2005, had enrolled 351 patients from 67 study locations, with 88% of registrations coming from 10 major sites. As of September 2005, 90 patients had undergone at least one further follow-up assessment. A copy of the registration, assessment and completion case report forms can be viewed on the AMNDR website (http://ww.amndr.org.au).
From the patient information collected in the registration data, three distinct clinical phenotypes have emerged (Box). Patients with the “global” MND phenotype (combined upper and lower motor neurone signs in at least two regions) tended to have the shortest survival time.
AMNDR was designed to obtain information that will increase the understanding of MND and its progression in different patients. Through the process of registry establishment, those involved in care, management and scientific research related to MND have been galvanised into a more effective and cooperative working unit. Ownership of the database has been retained by all who have contributed to data collection, and the information has been increasingly used to promote research into the causes and treatment of MND, locally and internationally.5 Through participation, it is expected that treating doctors will be able to evaluate their current management and associated patient outcomes relative to other centres around Australia.
Demographic data for 351 patients with motor neurone disease enrolled in the Australian Motor Neurone Disease Registry to November 2005, by phenotype
Acknowledgements
AMNDR is supported by an unrestricted research grant from sanofi–aventis. The contributions of Stephen McKechnie and Penny Williams (sanofi–aventis) in the initial development of AMNDR and ongoing studies, respectively, is gratefully acknowledged.
References
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- Talman P, Mathers S, Mostert E, Forbes A. Evaluation of clinical patterns and rate of progression of motor neuron disease. Amyotroph Lateral Scler Other Motor Neuron Disord 2002; 3: 93. 0_CHDEEJED
- Talman P, Rowe D, Kiernan MC. Australian Motor Neurone Disease Registry (AMNDR). J Neurol Sci 2005; 238 Suppl 1: S214. 0_i1092084