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J Neurol Neurosurg Psychiatry. Jul 1992; 55(7): 536–541.
PMCID: PMC489161

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.


The Scottish Motor Neuron Disease Register (SMNDR) is a prospective, collaborative, population based study of motor neuron disease (MND) in Scotland. The register started in January 1989 with the aim of studying the clinical and epidemiological features of MND by prospectively identifying incident patients. It is based on a system of registration by recruitment from multiple sources, followed by the collection of complete clinical data and follow up, mainly through general practitioners. In this report the register's methodology and the demography and incidence data for the first year of study are presented. One hundred and fourteen newly diagnosed patients were identified in 1989 giving a crude incidence for Scotland of 2.24/100,000/year. Standardised incidence ratios showed a non-significant trend towards lower rates in north eastern regions and island areas.

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  • Munsat TL, Skerry L, Korf B, Pober B, Schapira Y, Gascon GG, al-Rajeh SM, Dubowitz V, Davies K, Brzustowicz LM, et al. Phenotypic heterogeneity of spinal muscular atrophy mapping to chromosome 5q11.2-13.3 (SMA 5q). Neurology. 1990 Dec;40(12):1831–1836. [PubMed]
  • Mulder DW, Kurland LT, Offord KP, Beard CM. Familial adult motor neuron disease: amyotrophic lateral sclerosis. Neurology. 1986 Apr;36(4):511–517. [PubMed]
  • Siddique T, Figlewicz DA, Pericak-Vance MA, Haines JL, Rouleau G, Jeffers AJ, Sapp P, Hung WY, Bebout J, McKenna-Yasek D, et al. Linkage of a gene causing familial amyotrophic lateral sclerosis to chromosome 21 and evidence of genetic-locus heterogeneity. N Engl J Med. 1991 May 16;324(20):1381–1384. [PubMed]
  • Tandan R, Bradley WG. Amyotrophic lateral sclerosis: Part 2. Etiopathogenesis. Ann Neurol. 1985 Oct;18(4):419–431. [PubMed]
  • Williams DB, Windebank AJ. Motor neuron disease (amyotrophic lateral sclerosis). Mayo Clin Proc. 1991 Jan;66(1):54–82. [PubMed]
  • Younger DS, Chou S, Hays AP, Lange DJ, Emerson R, Brin M, Thompson H, Jr, Rowland LP. Primary lateral sclerosis. A clinical diagnosis reemerges. Arch Neurol. 1988 Dec;45(12):1304–1307. [PubMed]
  • Stanhope JM, Brody JA, Morris CE. Epidemiologic features of amyotropic lateral sclerosis and parkinsonism-dementia in Guam, Mariana Islands. Int J Epidemiol. 1972 Autumn;1(3):199–210. [PubMed]
  • Steele JC, Guzman T. Observations about amyotrophic lateral sclerosis and the parkinsonism-dementia complex of Guam with regard to epidemiology and etiology. Can J Neurol Sci. 1987 Aug;14(3 Suppl):358–362. [PubMed]
  • Pestronk A, Cornblath DR, Ilyas AA, Baba H, Quarles RH, Griffin JW, Alderson K, Adams RN. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol. 1988 Jul;24(1):73–78. [PubMed]
  • Teräväinen H, Calne DB. Developments in understanding the physiology and pharmacology of parkinsonism. Acta Neurol Scand. 1979 Jul;60(1):1–11. [PubMed]
  • Salemi G, Fierro B, Arcara A, Cassata M, Castiglione MG, Savettieri G. Amyotrophic lateral sclerosis in Palermo, Italy: an epidemiological study. Ital J Neurol Sci. 1989 Oct;10(5):505–509. [PubMed]
  • López-Vega JM, Calleja J, Combarros O, Polo JM, Berciano J. Motor neuron disease in Cantabria. Acta Neurol Scand. 1988 Jan;77(1):1–5. [PubMed]
  • Li TM, Alberman E, Swash M. Clinical features and associations of 560 cases of motor neuron disease. J Neurol Neurosurg Psychiatry. 1990 Dec;53(12):1043–1045. [PMC free article] [PubMed]
  • Juergens SM, Kurland LT, Okazaki H, Mulder DW. ALS in Rochester, Minnesota, 1925-1977. Neurology. 1980 May;30(5):463–470. [PubMed]
  • Yoshida S, Mulder DW, Kurland LT, Chu CP, Okazaki H. Follow-up study on amyotrophic lateral sclerosis in Rochester, Minn., 1925 through 1984. Neuroepidemiology. 1986;5(2):61–70. [PubMed]
  • Murros K, Fogelholm R. Amyotrophic lateral sclerosis in Middle-Finland: an epidemiological study. Acta Neurol Scand. 1983 Jan;67(1):41–47. [PubMed]
  • Holloway SM, Emery AE. The epidemiology of motor neuron disease in Scotland. Muscle Nerve. 1982 Feb;5(2):131–133. [PubMed]
  • Holloway SM, Mitchell JD. Motor neurone disease in the Lothian Region of Scotland 1961-81. J Epidemiol Community Health. 1986 Dec;40(4):344–350. [PMC free article] [PubMed]
  • Hoffman PM, Brody JA. The reliability of death certificate reporting for amyotrophic lateral sclerosis. J Chronic Dis. 1971 Jun;24(1):5–8. [PubMed]
  • Buckley J, Warlow C, Smith P, Hilton-Jones D, Irvine S, Tew JR. Motor neuron disease in England and Wales, 1959-1979. J Neurol Neurosurg Psychiatry. 1983 Mar;46(3):197–205. [PMC free article] [PubMed]
  • Gajdusek DC. Urgent opportunistic observations: the study of changing, transient and disappearing phenomena of medical interest in disrupted primitive human communities. Ciba Found Symp. 1977;(49):69–94. [PubMed]
  • Kurland LT. Geographic isolates: their role in neuroepidemiology. Adv Neurol. 1978;19:69–82. [PubMed]
  • Bharucha NE, Schoenberg BS, Raven RH, Pickle LW, Byar DP, Mason TJ. Geographic distribution of motor neuron disease and correlation with possible etiologic factors. Neurology. 1983 Jul;33(7):911–915. [PubMed]
  • Kurland LT. Amyotrophic lateral sclerosis and Parkinson's disease complex on Guam linked to an environmental neurotoxin. Trends Neurosci. 1988 Feb;11(2):51–54. [PubMed]
  • KURLAND LT. Epidemiologic investigations of amyotrophic lateral sclerosis. III. A genetic interpretation of incidence and geographic distribution. Proc Staff Meet Mayo Clin. 1957 Aug 21;32(17):449–462. [PubMed]
  • Bobowick AR, Brody JA. Epidemiology of motor-neuron diseases. N Engl J Med. 1973 May 17;288(20):1047–1055. [PubMed]
  • Olivares L, Estéban ES, Alter M. Mexican "resistance" to amyotrophic lateral sclerosis. Arch Neurol. 1972 Nov;27(5):397–402. [PubMed]
  • Cendrowski W, Wender M, Owsianowski M. Analyse epidemiologique de la sclerose laterale amyotrophique sur le territoire de la grande-Pologne. Acta Neurol Scand. 1970;46(4):609–617. [PubMed]
  • Mitchell JD, Gibson HN, Gatrell A. Amyotrophic lateral sclerosis in Lancashire and South Cumbria, England, 1976-1986. A geographical study. Arch Neurol. 1990 Aug;47(8):875–880. [PubMed]
  • Sienko DG, Davis JP, Taylor JA, Brooks BR. Amyotrophic lateral sclerosis. A case-control study following detection of a cluster in a small Wisconsin community. Arch Neurol. 1990 Jan;47(1):38–41. [PubMed]
  • Paolino E, Granieri E, Tola MR, Rosati G. Conjugal amyotrophic lateral sclerosis. Ann Neurol. 1983 Dec;14(6):699–699. [PubMed]
  • Chad D, Mitsumoto H, Adelman LS, Bradley WG, Munsat TL, Zieper I. Conjugal motor neuron disease. Neurology. 1982 Mar;32(3):306–307. [PubMed]
  • Hochberg FH, Bryan JA, 2nd, Whelan MA. Letter: Clustering of amyotrophic lateral sclerosis. Lancet. 1974 Jan 5;1(7845):34–34. [PubMed]

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