The Swedish motor neuron disease quality registry

Abstract

Objective: We set up the Swedish Motor Neuron Disease (MND) Quality Registry to assure early diagnosis and high-quality health care for all MND patients (mainly amyotrophic lateral sclerosis, ALS), and to create a research base by prospectively following the entire MND population in Sweden. Methods: Since 2015, the MND Quality Registry continuously collects information about a wide range of clinical measures, biological samples, and quality of life outcomes from all MND patients recruited at the time of MND diagnosis in Sweden and followed at each clinic visit approximately every 12 weeks. The Registry includes an Internet based patient own reporting portal that involves patients in the registration of their current symptoms and health status. Results: As of 20th January 2017, the MND Quality Registry included 99% of the MND patients of the Stockholm area (N = 194), consisting mostly of ALS patients (N = 153, 78.9%), followed by patients labeled as MND due to a neurophysiology finding but not fulfilling the criteria for ALS (N = 20, 10.3%), primary lateral sclerosis (N = 13, 6.7%), and progressive spinal muscular atrophy patients (N = 8, 4.1%). A higher proportion of these patients were women (N = 100, 52%), and women and men had a similar age at symptoms onset (59 years). Conclusions: Main strengths of the MND Quality Registry are its clinical, quantitative, qualitative, and prospective nature, providing the researchers potential means of identifying appropriate candidates for clinical trials and other research projects, as well as assuring to the patients an effective and adequate time spent on-site with the healthcare professionals.

Keywords:

• Amyotrophic lateral sclerosis
• motor neuron disease
• national registry
• prospective cohort
• epidemiology

Acknowledgements

We are indebted to the patients and their families for participation in these studies. We are also grateful to the clinicians whom provided patient material, Dr Bo Höjeberg, Dr Jan Mathé, Dr Pia Dijkgraaf, and Dr Martin Lindberger. We are also grateful to the clinicians whom provided input for the registry variables, Dr Ingela Nygren, Dr Johan Jacobsson, Dr Susanne Tivienius, Dr Hans Rosen, Dr Olof Danielsson, and Dr Gert Staaf. The authors would like to thank Karolina Palmbäck, Parvin Safari, Eva-Carin Jacobsson, and Anna Nilzén for registration services and more.

Declaration of interest

The authors report no conflicts of interest.

Additional information

Funding

This study was supported by grants from Ulla-Carin Lindquist foundation, Bjorklund foundation, Neuro Sweden foundation, the Swedish Association of Local Authorities and Regions (Sveriges Kommuner och Landsting), the Board of Social Welfare (Socialstyrelsen), the Swedish Research Council (grant No. 2015-03170), and the Karolinska Institutet (Senior Researcher Award and Strategic Research Area in Epidemiology).