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Journal of Neurology

, Volume 266, Issue 4, pp 817–825 | Cite as

Changing epidemiology of motor neurone disease in Scotland

  • Danielle J. Leighton
  • Judith Newton
  • Laura J. Stephenson
  • Shuna Colville
  • Richard Davenport
  • George Gorrie
  • Ian Morrison
  • Robert Swingler
  • Siddharthan Chandran
  • Suvankar PalEmail author
  • on behalf of the CARE-MND Consortium
Original Communication

Abstract

Objectives

Scotland benefits from an integrated national healthcare team for motor neurone disease (MND) and a tradition of rich clinical data capture using the Scottish MND Register (launched in 1989; one of the first national registers). The Scottish register was re-launched in 2015 as Clinical Audit Research and Evaluation of MND (CARE-MND), an electronic platform for prospective, population-based research. We aimed to determine if incidence of MND is changing over time.

Methods

Capture–recapture methods determined the incidence of MND in 2015–2016. Incidence rates for 2015–2016 and 1989–1998 were direct age and sex standardised to allow time-period comparison. Phenotypic characteristics and socioeconomic status of the cohort are described.

Results

Coverage of the CARE-MND platform was 99%. Crude incidence in the 2015–2017 period was 3.83/100,000 person-years (95% CI 3.53–4.14). Direct age-standardised incidence in 2015 was 3.42/100,000 (95% CI 2.99–3.91); in 2016, it was 2.89/100,000 (95% CI 2.50–3.34). The 1989–1998 direct standardised annual incidence estimate was 2.32/100,000 (95% CI 2.26–2.37). 2015–2016 standardised incidence was 66.9% higher than Northern European estimates. Socioeconomic status was not associated with MND.

Conclusions

Our data show a changing landscape of MND in Scotland, with a rise in incidence by 36.0% over a 25-year period. This is likely attributable to ascertainment in the context of improved neurological services in Scotland. Our data suggest that CARE-MND is a reliable national resource and findings can be extrapolated to the other Northern European populations.

Keywords

Epidemiology Motor neurone disease Amyotrophic lateral sclerosis 

Notes

Acknowledgements

We thank all the people with motor neurone disease (MND) who participated in this study. The work was supported by the CARE-MND Consortium: Andrew Bethell, Gillian Craig, Laura Cunningham, Callum Duncan, Carole Ferguson, Moira Flett, Dianne Fraser, Gillian Hall, Janice Hatrick, Helen Lennox, Laura Marshall, Dympna McAleer, Alison McEleney, Kitty Millar, Ann Silver, Susan Stewart, Dorothy Storey, Gill Stott, Carol Thornton, and Carolyn Webber. We are grateful to the CARE-MND electronic platform team: David Buchanan, Harry Gordon, Giulia Melchiorre, and Laura Sherlock. We thank our funders and supporters at MND Scotland and the Euan MacDonald Centre for MND Research. DL receives doctoral funding from the Chief Scientist Office for Scotland, MND Scotland, and the MND Association (grant CAF/MND/15/01).

Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflicts of interest.

Ethical standard statement

The authors confirm that this article complies with ethical standards.

Supplementary material

415_2019_9190_MOESM1_ESM.docx (16 kb)
Supplementary material 1 (DOCX 16 KB)

References

  1. 1.
    Al-Chalabi A, Hardiman O (2013) The epidemiology of ALS: a conspiracy of genes, environment and time. Nat Rev Neurol 9(11):617–628CrossRefGoogle Scholar
  2. 2.
    Al-Chalabi A, Calvo A, Chio A, Colville S, Ellis CM, Hardiman O et al (2014) Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study. Lancet Neurol 13(11):1108–1113CrossRefGoogle Scholar
  3. 3.
    Marin B, Logroscino G, Boumédiene F, Labrunie A, Couratier P, Babron MC et al (2016) Clinical and demographic factors and outcome of amyotrophic lateral sclerosis in relation to population ancestral origin. Eur J Epidemiol 31:229–245 (Springer Netherlands)CrossRefGoogle Scholar
  4. 4.
    Marin B, Boumédiene F, Logroscino G, Couratier P, Babron M-C, Leutenegger AL et al (2016) Variation in worldwide incidence of amyotrophic lateral sclerosis: a meta-analysis. Int J Epidemiol 46(1):57–74Google Scholar
  5. 5.
    Holloway SM, Emery AEH (1982) The epidemiology of motor neuron disease in Scotland. Muscle Nerve 5(2):131–133CrossRefGoogle Scholar
  6. 6.
    Chancellor AM, Slattery JM, Fraser H, Swingler RJ, Holloway SM, Warlow CP (1993) The prognosis of adult-onset motor neuron disease: a prospective study based on the Scottish Motor Neuron Disease Register. J Neurol 240(6):339–346CrossRefGoogle Scholar
  7. 7.
    Forbes RB, Colville S, Parratt J, Swingler RJ (2007) The incidence of motor nueron disease in Scotland. J Neurol 254(7):866–869CrossRefGoogle Scholar
  8. 8.
    Hardiman O, Al-Chalabi A, Brayne C, Beghi E, Van Den Berg LH, Chio A et al (2017) The changing picture of amyotrophic lateral sclerosis: lessons from European registers. J Neurol Neurosurg Psychiatry 88:557–563 (BMJ Publishing Group)CrossRefGoogle Scholar
  9. 9.
    Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1(5):293–299CrossRefGoogle Scholar
  10. 10.
    National Records of Scotland (2017) Statistics and Data: Mid-Year Population Estimates [Internet]. National Records of Scotland. National Records of Scotland. https://www.nrscotland.gov.uk/statistics-and-data/statistics/statistics-by-theme/population/population-estimates/mid-year-population-estimates. Accessed 28 Feb 2018
  11. 11.
    United States Census Bureau (2010) 2010 Census Data Products: United States [Internet]. United States Census Bureau. 2010. https://www.census.gov/population/www/cen2010/glance/. Accessed 28 Feb 2018
  12. 12.
    R Core Team (2017) R: a language and environment for statistical computing. R Foundation for Statistical Computing, VienneGoogle Scholar
  13. 13.
    Chiò A, Mora G, Moglia C, Manera U, Canosa A, Cammarosano S et al (2017) Secular trends of amyotrophic lateral sclerosis: the Piemonte and Valle d’Aosta register. JAMA Neurol 74(9):1097–1104CrossRefGoogle Scholar
  14. 14.
    Donaghy C, Clarke J, Patterson C, Kee F, Hardiman O, Patterson V (2010) The epidemiology of motor neuron disease in Northern Ireland using capture-recapture methodology. Amyotroph Lateral Scler 11(4):374–378CrossRefGoogle Scholar
  15. 15.
    Rosenbohm A, Peter RS, Erhardt S, Lulé D, Rothenbacher D, Ludolph AC et al (2017) Epidemiology of amyotrophic lateral sclerosis in Southern Germany. J Neurol 264(4):749–757CrossRefGoogle Scholar
  16. 16.
    Horrocks S, Wilkinson T, Schnier C, Ly A, Woodfield R, Rannikmäe K et al (2017) Accuracy of routinely-collected healthcare data for identifying motor neurone disease cases: a systematic review. Le W, editor. PLoS One 12(2):e0172639CrossRefGoogle Scholar
  17. 17.
    Doyle P, Brown A, Beral V, Reeves G, Green J (2012) Incidence of and risk factors for motor neurone disease in UK women: a prospective study. BMC Neurol 12:25CrossRefGoogle Scholar
  18. 18.
    Maxwell R, Wells C, Verne J (2010) Under-reporting of progressive supranuclear palsy. Lancet (London, England) 376(9758):2072CrossRefGoogle Scholar
  19. 19.
    Scialò C, Novi G, Bandettini di Poggio M, Canosa A, Sormani MP, Mandich P et al (2016) Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: an update of LIGALS register. Amyotroph Lateral Scler Front Degener 17(7–8):535–542CrossRefGoogle Scholar
  20. 20.
    Demetriou CA, Hadjivasiliou PM, Kleopa KA, Christou YP, Leonidou E, Kyriakides T et al (2017) Epidemiology of amyotrophic lateral sclerosis in the Republic of Cyprus: A 25-Year Retrospective Study. Neuroepidemiology 48(1–2):79–85CrossRefGoogle Scholar
  21. 21.
    Joensen P (2012 Jul) Incidence of amyotrophic lateral sclerosis in the Faroe Islands. Acta Neurol Scand 126(1):62–66CrossRefGoogle Scholar
  22. 22.
    Georgoulopoulou E, Vinceti M, Bonvicini F, Sola P, Goldoni CA, Girolamo G, De et al (2011 Nov) Changing incidence and subtypes of ALS in Modena, Italy: a 10-years prospective study. Amyotroph Lateral Scler 12(6):451–457CrossRefGoogle Scholar
  23. 23.
    Huisman MHB, de Jong SW, van Doormaal PTC, Weinreich SS, Schelhaas HJ, van der Kooi AJ et al (2011) Population based epidemiology of amyotrophic lateral sclerosis using capture-recapture methodology. J Neurol Neurosurg Psychiatry 82(10):1165–1170CrossRefGoogle Scholar
  24. 24.
    Arthur KC, Calvo A, Price TR, Geiger JT, Chiò A, Traynor BJ (2016) Projected increase in amyotrophic lateral sclerosis from 2015 to 2040. Nat Commun 7:12408CrossRefGoogle Scholar
  25. 25.
    Marin B, Fontana A, Arcuti S, Copetti M, Boumédiene F, Couratier P et al (2018 Jul) Age-specific ALS incidence: a dose–response meta-analysis. Eur J Epidemiol 33(7):621–634CrossRefGoogle Scholar
  26. 26.
    Information Services Division (ISD) S (2017) Annual Trends in Consultant-led Outpatient Activity [Internet]. ISD Scotland. http://www.isdscotland.org/Health-Topics/Hospital-Care/Outpatient-Activity/. Accessed 16 May 2018
  27. 27.
    Davenport RJ, Swingler RJ, Chancellor AM, Warlow CP (1996) Avoiding false positive diagnoses of motor neuron disease: lessons from the Scottish Motor Neuron Disease Register. J Neurol Neurosurg Psychiatry 60(2):147–151CrossRefGoogle Scholar
  28. 28.
    Abrahams S, Newton J, Niven E, Foley J, Bak TH (2014) Screening for cognition and behaviour changes in ALS. Amyotroph Lateral Scler Frontotemporal Degener 15(1–2):9–14CrossRefGoogle Scholar
  29. 29.
    Beghi E, Logroscino G, Chiò A, Hardiman O, Mitchell D, Swingler R et al (2006) The epidemiology of ALS and the role of population-based registries. Biochim Biophys Acta 1762:1150–1157CrossRefGoogle Scholar
  30. 30.
    Forbes RB, Colville S, Swingler RJ, Scottish ALS/MND, Register (2004) The epidemiology of amyotrophic lateral sclerosis (ALS/MND) in people aged 80 or over. Age Ageing 33(2):131–134CrossRefGoogle Scholar
  31. 31.
    Rooney JPK, Tobin K, Crampsie A, Vajda A, Heverin M, Mclaughlin R et al (2015) Social deprivation and population density are not associated with small area risk of amyotrophic lateral sclerosis. Environ Res 142:141–147CrossRefGoogle Scholar
  32. 32.
    Roberts AL, Johnson NJ, Chen JT, Cudkowicz ME, Weisskopf MG (2016) Race/ethnicity, socioeconomic status, and ALS mortality in the United States. Neurology 87(22):2300–2308CrossRefGoogle Scholar
  33. 33.
    Information Services Division (ISD) S (2017) Scottish Heart Disease Statistics [Internet]. http://www.isdscotland.org/Health-Topics/Heart-Disease/Publications/2017-02-21/2017-02-21-Heart-Disease-Report.pdf. Accessed 25 Jun 2018
  34. 34.
    Rohrer JD, Isaacs AM, Mizlienska S, Mead S, Lashley T, Wray S et al (2015) C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis. Lancet Neurol 14(3):291–301CrossRefGoogle Scholar
  35. 35.
    Renton AE, Majounie E, Waite A, Simón-Sánchez J, Rollinson S, Gibbs JR et al (2011) A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 72(2):257–268CrossRefGoogle Scholar
  36. 36.
    Black HA, Leighton DJ, Cleary EM, Rose E, Stephenson L, Colville S et al (2017) Genetic epidemiology of motor neuron disease-associated variants in the Scottish population. Neurobiol Aging 51:178.e11–178.e20CrossRefGoogle Scholar
  37. 37.
    Cleary EM, Pal S, Azam T, Moore DJ, Swingler R, Gorrie G et al (2016) Improved PCR based methods for detecting C9orf72 hexanucleotide repeat expansions. Mol Cell Probes 30(4):218–224CrossRefGoogle Scholar
  38. 38.
    Hayward C, Swingler RJ, Simpson SA, Brock DJ (1996) A specific superoxide dismutase mutation is on the same genetic background in sporadic and familial cases of amyotrophic lateral sclerosis. Am J Hum Genet 59(5):1165–1167Google Scholar
  39. 39.
    Lohmueller KE, Indap AR, Schmidt S, Boyko AR, Hernandez RD, Hubisz MJ et al (2008) Proportionally more deleterious genetic variation in European than in African populations. Nature 451:994CrossRefGoogle Scholar
  40. 40.
    Falconer DS (1965) The inheritance of liability to certain diseases, estimated from the incidence among relatives. Ann Hum Genet 29(1):51–76CrossRefGoogle Scholar
  41. 41.
    Hern JEC, Dundee RK, Davidson D, Forster A, Roberts R, Swingler RJ et al (1992) The Scottish motor neuron disease register: a prospective study of adult onset motor neuron disease in Scotland. Methodology, demography and clinical features of incident cases in 1989. J Neurol Neurosurg Psychiatry 55:536–541CrossRefGoogle Scholar
  42. 42.
    Brooks BR (1994) El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and th. J Neurol Sci 124 Suppl:96–107CrossRefGoogle Scholar
  43. 43.
    Tobin K, Gilthorpe MS, Rooney J, Heverin M, Vajda A, Staines A et al (2016) Age-period-cohort analysis of trends in amyotrophic lateral sclerosis incidence. J Neurol 263(10):1919–1926CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Danielle J. Leighton
    • 1
    • 2
    • 3
  • Judith Newton
    • 1
    • 2
    • 3
  • Laura J. Stephenson
    • 2
  • Shuna Colville
    • 1
    • 2
    • 3
  • Richard Davenport
    • 3
    • 4
  • George Gorrie
    • 2
    • 5
  • Ian Morrison
    • 6
  • Robert Swingler
    • 1
    • 2
  • Siddharthan Chandran
    • 1
    • 2
    • 3
    • 4
  • Suvankar Pal
    • 1
    • 2
    • 3
    • 4
    Email author
  • on behalf of the CARE-MND Consortium
  1. 1.Centre for Clinical Brain SciencesUniversity of EdinburghEdinburghUK
  2. 2.Euan MacDonald Centre for Motor Neurone Disease ResearchUniversity of EdinburghEdinburghUK
  3. 3.Anne Rowling Regenerative Neurology ClinicUniversity of EdinburghEdinburghUK
  4. 4.Department of Clinical NeurosciencesNHS LothianEdinburghUK
  5. 5.Institute of NeurosciencesNHS Greater Glasgow and ClydeGlasgowUK
  6. 6.Department of NeurologyNHS TaysideDundeeUK

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