Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1992 Dec;55(12):1106–1115. doi: 10.1136/jnnp.55.12.1106

Adult onset motor neuron disease: worldwide mortality, incidence and distribution since 1950.

A M Chancellor 1, C P Warlow 1
PMCID: PMC1015320  PMID: 1479386

Abstract

This review examines the commonly held premise that, apart from the Western Pacific forms, motor neuron disease (MND), has a uniform worldwide distribution in space and time; the methodological problems in studies of MND incidence; and directions for future epidemiological research. MND is more common in men at all ages. Age-specific incidence rises steeply into the seventh decade but the incidence in the very elderly is uncertain. A rise in mortality from MND over recent decades has been demonstrated wherever this has been examined and may be real rather than due to improved case ascertainment. Comparison of incidence studies in different places is complicated by non-standardised methods of case ascertainment and diagnosis but there appear to be differences between well studied populations. In developed countries in the northern hemisphere there is a weak positive correlation between standardised, age-specific incidence and distance from the equator. There is now strong evidence for an environmental factor as the cause of the Western Pacific forms of MND. A number of clusters of sporadic MND have been reported from developed countries, but no single agent identified as responsible.

Full text

PDF
1106

Images in this article

1115Image
on p.1115

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Abarbanel J. M., Herishanu Y., Frisher S. Motor neuron disease in textile factory workers. Isr J Med Sci. 1985 Nov;21(11):924–925. [PubMed] [Google Scholar]
  2. Adams C. R., Ziegler D. K., Lin J. T. Mercury intoxication simulating amyotrophic lateral sclerosis. JAMA. 1983 Aug 5;250(5):642–643. [PubMed] [Google Scholar]
  3. Argov Z., Navon R. Clinical and genetic variations in the syndrome of adult GM2 gangliosidosis resulting from hexosaminidase A deficiency. Ann Neurol. 1984 Jul;16(1):14–20. doi: 10.1002/ana.410160105. [DOI] [PubMed] [Google Scholar]
  4. Armon C., Daube J. R., O'Brien P. C., Kurland L. T., Mulder D. W. When is an apparent excess of neurologic cases epidemiologically significant? Neurology. 1991 Nov;41(11):1713–1718. doi: 10.1212/wnl.41.11.1713. [DOI] [PubMed] [Google Scholar]
  5. BASTRON J. A., LAMBERT E. H., MULDER D. W. Hyperinsulin neuropathy. Neurology. 1956 Sep;6(9):627–635. doi: 10.1212/wnl.6.9.627. [DOI] [PubMed] [Google Scholar]
  6. Bale G. S. Amyotrophic lateral sclerosis in Iowa. J Chronic Dis. 1975 Jun;28(5-6):305–310. doi: 10.1016/0021-9681(75)90011-9. [DOI] [PubMed] [Google Scholar]
  7. Bobowick A. R., Brody J. A. Epidemiology of motor-neuron diseases. N Engl J Med. 1973 May 17;288(20):1047–1055. doi: 10.1056/NEJM197305172882005. [DOI] [PubMed] [Google Scholar]
  8. Boothby J. A., DeJesus P. V., Rowland L. P. Reversible forms of motor neuron disease. Lead "neuritis". Arch Neurol. 1974 Jul;31(1):18–23. doi: 10.1001/archneur.1974.00490370044005. [DOI] [PubMed] [Google Scholar]
  9. Brody J. A., Edgar A. H., Gillespie M. M. Amyotrophic lateral sclerosis. No increase among US construction workers in Guam. JAMA. 1978 Aug 11;240(6):551–560. doi: 10.1001/jama.240.6.551. [DOI] [PubMed] [Google Scholar]
  10. Brody J. A., Hirano A., Scott R. M. Recent neuropathologic observations in amyotrophic lateral sclerosis and parkinsonism-dementia of Guam. Neurology. 1971 May;21(5):528–536. doi: 10.1212/wnl.21.5.528. [DOI] [PubMed] [Google Scholar]
  11. Brody J. A., Stanhope J. M., Kurland L. T. Patterns of Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia on Guam. Contemp Neurol Ser. 1975;12:45–70. [PubMed] [Google Scholar]
  12. Brownell B., Oppenheimer D. R., Hughes J. T. The central nervous system in motor neurone disease. J Neurol Neurosurg Psychiatry. 1970 Jun;33(3):338–357. doi: 10.1136/jnnp.33.3.338. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Buchanan D. S., Malamud N. Motor neuron disease with renal cell carcinoma and postoperative neurologic remission. A clinicopathologic report. Neurology. 1973 Aug;23(8):891–894. doi: 10.1212/wnl.23.8.891. [DOI] [PubMed] [Google Scholar]
  14. Buckley J., Warlow C., Smith P., Hilton-Jones D., Irvine S., Tew J. R. Motor neuron disease in England and Wales, 1959-1979. J Neurol Neurosurg Psychiatry. 1983 Mar;46(3):197–205. doi: 10.1136/jnnp.46.3.197. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Campbell A. M., Williams E. R., Pearce J. Late motor neuron degeneration following poliomyelitis. Neurology. 1969 Nov;19(11):1101–1106. doi: 10.1212/wnl.19.11.1101. [DOI] [PubMed] [Google Scholar]
  16. 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. doi: 10.1111/j.1600-0404.1970.tb05817.x. [DOI] [PubMed] [Google Scholar]
  17. Chad D., Mitsumoto H., Adelman L. S., Bradley W. G., Munsat T. L., Zieper I. Conjugal motor neuron disease. Neurology. 1982 Mar;32(3):306–307. doi: 10.1212/wnl.32.3.306. [DOI] [PubMed] [Google Scholar]
  18. Chazot F., Vallat J. M., Hugon J., Lubeau M., Dumas M. Amyotrophic lateral sclerosis in Limousin (Limoges area, France). Neuroepidemiology. 1986;5(1):39–46. doi: 10.1159/000110811. [DOI] [PubMed] [Google Scholar]
  19. Christensen P. B., Højer-Pedersen E., Jensen N. B. Survival of patients with amyotrophic lateral sclerosis in 2 Danish counties. Neurology. 1990 Apr;40(4):600–604. doi: 10.1212/wnl.40.4.600. [DOI] [PubMed] [Google Scholar]
  20. Dalakas M. C., Elder G., Hallett M., Ravits J., Baker M., Papadopoulos N., Albrecht P., Sever J. A long-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms. N Engl J Med. 1986 Apr 10;314(15):959–963. doi: 10.1056/NEJM198604103141505. [DOI] [PubMed] [Google Scholar]
  21. Dalakas M. C., Pezeshkpour G. H. Neuromuscular diseases associated with human immunodeficiency virus infection. Ann Neurol. 1988;23 (Suppl):S38–S48. doi: 10.1002/ana.410230713. [DOI] [PubMed] [Google Scholar]
  22. Datta A., Dasadhikary C., Ghosh S. Syphilitic amyotrophy. J Indian Med Assoc. 1966 Sep 16;47(6):287–290. [PubMed] [Google Scholar]
  23. De Domenico P., Malara C. E., Marabello L., Puglisi R. M., Meneghini F., Serra S., Gallitto G., Musolino R. Amyotrophic lateral sclerosis: an epidemiological study in the Province of Messina, Italy, 1976-1985. Neuroepidemiology. 1988;7(3):152–158. doi: 10.1159/000110149. [DOI] [PubMed] [Google Scholar]
  24. Dorsen M., Ehni G. Cervical spondylotic radiculopathy producing motor manifestations mimicking primary muscular atrophy. Neurosurgery. 1979 Oct;5(4):427–431. doi: 10.1227/00006123-197910000-00003. [DOI] [PubMed] [Google Scholar]
  25. Duncan M. W., Steele J. C., Kopin I. J., Markey S. P. 2-Amino-3-(methylamino)-propanoic acid (BMAA) in cycad flour: an unlikely cause of amyotrophic lateral sclerosis and parkinsonism-dementia of Guam. Neurology. 1990 May;40(5):767–772. doi: 10.1212/wnl.40.5.767. [DOI] [PubMed] [Google Scholar]
  26. Durrleman S., Alperovitch A. Increasing trend of ALS in France and elsewhere: are the changes real? Neurology. 1989 Jun;39(6):768–773. doi: 10.1212/wnl.39.6.768. [DOI] [PubMed] [Google Scholar]
  27. Eisen A. A., Hudson A. J. Amyotrophic lateral sclerosis: concepts in pathogenesis and etiology. Can J Neurol Sci. 1987 Nov;14(4):649–652. [PubMed] [Google Scholar]
  28. Eldridge R., Ryan E., Rosario J., Brody J. A. Amyotrophic lateral sclerosis and parkinsonism dementia in a migrant population from Guam. Neurology. 1969 Nov;19(11):1029–1037. doi: 10.1212/wnl.19.11.1029. [DOI] [PubMed] [Google Scholar]
  29. Fisher M., Mateer J. E., Ullrich I., Gutrecht J. A. Pyramidal tract deficits and polyneuropathy in hyperthyroidism, Combination clinically mimicking amyotrophic lateral sclerosis. Am J Med. 1985 Jun;78(6 Pt 1):1041–1044. doi: 10.1016/0002-9343(85)90231-1. [DOI] [PubMed] [Google Scholar]
  30. Flaten T. P. Rising mortality from motoneuron disease. Lancet. 1989 May 6;1(8645):1018–1019. doi: 10.1016/s0140-6736(89)92661-5. [DOI] [PubMed] [Google Scholar]
  31. Fleet W. S., Watson R. T. From benign fasciculations and cramps to motor neuron disease. Neurology. 1986 Jul;36(7):997–998. doi: 10.1212/wnl.36.7.997. [DOI] [PubMed] [Google Scholar]
  32. Forsgren L., Almay B. G., Holmgren G., Wall S. Epidemiology of motor neuron disease in northern Sweden. Acta Neurol Scand. 1983 Jul;68(1):20–29. doi: 10.1111/j.1600-0404.1983.tb04810.x. [DOI] [PubMed] [Google Scholar]
  33. GOLDBERG I. D., KURLAND L. T. Mortality in 33 countries from diseases of the nervous system. World Neurol. 1962 Jun;3:444–465. [PubMed] [Google Scholar]
  34. Gajdusek D. C. Foci of motor neuron disease in high incidence in isolated populations of East Asia and the Western Pacific. Adv Neurol. 1982;36:363–393. [PubMed] [Google Scholar]
  35. Gajdusek D. C., Salazar A. M. Amyotrophic lateral sclerosis and parkinsonian syndromes in high incidence among the Auyu and Jakai people of West New Guinea. Neurology. 1982 Feb;32(2):107–126. doi: 10.1212/wnl.32.2.107. [DOI] [PubMed] [Google Scholar]
  36. Gajdusek D. C. 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. doi: 10.1002/9780470715406.ch5. [DOI] [PubMed] [Google Scholar]
  37. Gallagher J. P. ALS. South Med J. 1988 Mar;81(3):417–417. doi: 10.1097/00007611-198803000-00036. [DOI] [PubMed] [Google Scholar]
  38. Gallagher J. P., Talbert O. R. Motor neuron syndrome after electric shock. Acta Neurol Scand. 1991 Feb;83(2):79–82. doi: 10.1111/j.1600-0404.1991.tb04653.x. [DOI] [PubMed] [Google Scholar]
  39. Garruto R. M., Gajdusek C., Chen K. M. Amyotrophic lateral sclerosis among Chamorro migrants from Guam. Ann Neurol. 1980 Dec;8(6):612–619. doi: 10.1002/ana.410080612. [DOI] [PubMed] [Google Scholar]
  40. Garruto R. M., Gajdusek D. C., Chen K. M. Amyotrophic lateral sclerosis and parkinsonism-dementia among Filipino migrants to Guam. Ann Neurol. 1981 Oct;10(4):341–350. doi: 10.1002/ana.410100405. [DOI] [PubMed] [Google Scholar]
  41. Garruto R. M., Shankar S. K., Yanagihara R., Salazar A. M., Amyx H. L., Gajdusek D. C. Low-calcium, high-aluminum diet-induced motor neuron pathology in cynomolgus monkeys. Acta Neuropathol. 1989;78(2):210–219. doi: 10.1007/BF00688211. [DOI] [PubMed] [Google Scholar]
  42. Garruto R. M., Swyt C., Yanagihara R., Fiori C. E., Gajdusek D. C. Intraneuronal co-localization of silicon with calcium and aluminum in amyotrophic lateral sclerosis and parkinsonism with dementia of Guam. N Engl J Med. 1986 Sep 11;315(11):711–712. doi: 10.1056/NEJM198609113151115. [DOI] [PubMed] [Google Scholar]
  43. Garruto R. M., Yanagihara R., Gajdusek D. C. Disappearance of high-incidence amyotrophic lateral sclerosis and parkinsonism-dementia on Guam. Neurology. 1985 Feb;35(2):193–198. doi: 10.1212/wnl.35.2.193. [DOI] [PubMed] [Google Scholar]
  44. Gastaut J. L., Michel B., Figarella-Branger D., Somma-Mauvais H. Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis. Arch Neurol. 1988 May;45(5):509–513. doi: 10.1001/archneur.1988.00520290037011. [DOI] [PubMed] [Google Scholar]
  45. Ghatak N. R., Hirano A., Zimmerman H. M. [Rheumatoid arthritis with arteritis and neuropathy masking amyotrophic lateral sclerosis]. Rinsho Shinkeigaku. 1972 Aug;12(8):401–410. [PubMed] [Google Scholar]
  46. Giagheddu M., Puggioni G., Masala C., Biancu F., Pirari G., Piras M. R., Rachele M. G. Epidemiologic study of amyotrophic lateral sclerosis in Sardinia, Italy. Acta Neurol Scand. 1983 Dec;68(6):394–404. doi: 10.1111/j.1600-0404.1983.tb04849.x. [DOI] [PubMed] [Google Scholar]
  47. Gibbs C. J., Jr, Gajdusek D. C. Amyotrophic lateral sclerosis, Parkinson's disease, and the amyotrophic lateral sclerosis-Parkinsonism-dementia complex on Guam: a review and summary of attempts to demonstrate infection as the aetiology. J Clin Pathol Suppl (R Coll Pathol) 1972;6:132–140. [PMC free article] [PubMed] [Google Scholar]
  48. Granieri E., Carreras M., Tola R., Paolino E., Tralli G., Eleopra R., Serra G. Motor neuron disease in the province of Ferrara, Italy, in 1964-1982. Neurology. 1988 Oct;38(10):1604–1608. doi: 10.1212/wnl.38.10.1604. [DOI] [PubMed] [Google Scholar]
  49. Granieri E., Rosati G., Tola R., Pinna L., Paolino E., D'Agostini G. Amyotrophic lateral sclerosis frequency in Italy. Incidence and prevalence in the province of Ferrara. Acta Neurol (Napoli) 1981 Aug;3(4):549–557. [PubMed] [Google Scholar]
  50. Gunnarsson L. G., Lindberg G., Söderfelt B., Axelson O. The mortality of motor neuron disease in Sweden. Arch Neurol. 1990 Jan;47(1):42–46. doi: 10.1001/archneur.1990.00530010050018. [DOI] [PubMed] [Google Scholar]
  51. Hawkes C. H., Fox A. J. Motor neurone disease in leather workers. Lancet. 1981 Feb 28;1(8218):507–507. doi: 10.1016/s0140-6736(81)91900-0. [DOI] [PubMed] [Google Scholar]
  52. Hochberg F. H., Bryan J. A., 2nd, Whelan M. A. Letter: Clustering of amyotrophic lateral sclerosis. Lancet. 1974 Jan 5;1(7845):34–34. doi: 10.1016/s0140-6736(74)93037-2. [DOI] [PubMed] [Google Scholar]
  53. Hoffman P. M., Brody J. A. The reliability of death certificate reporting for amyotrophic lateral sclerosis. J Chronic Dis. 1971 Jun;24(1):5–8. doi: 10.1016/0021-9681(71)90053-1. [DOI] [PubMed] [Google Scholar]
  54. Hoffman P. M., Festoff B. W., Giron L. T., Jr, Hollenbeck L. C., Garruto R. M., Ruscetti F. W. Isolation of LAV/HTLV-III from a patient with amyotrophic lateral sclerosis. N Engl J Med. 1985 Aug 1;313(5):324–325. doi: 10.1056/NEJM198508013130511. [DOI] [PubMed] [Google Scholar]
  55. Hoffman P. M., Robbins D. S., Gibbs C. J., Jr, Gajdusek D. C., Garruto R. M., Terasaki O. I. Histocompatibility antigens in amyotrophic lateral sclerosis and parkinsonism-dementia on Guam. Lancet. 1977 Oct 1;2(8040):717–717. doi: 10.1016/s0140-6736(77)90525-6. [DOI] [PubMed] [Google Scholar]
  56. Holloway S. M., Emery A. E. The epidemiology of motor neuron disease in Scotland. Muscle Nerve. 1982 Feb;5(2):131–133. doi: 10.1002/mus.880050209. [DOI] [PubMed] [Google Scholar]
  57. Holloway S. M., Mitchell J. D. Motor neurone disease in the Lothian Region of Scotland 1961-81. J Epidemiol Community Health. 1986 Dec;40(4):344–350. doi: 10.1136/jech.40.4.344. [DOI] [PMC free article] [PubMed] [Google Scholar]
  58. Hudson A. J. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain. 1981 Jun;104(2):217–247. doi: 10.1093/brain/104.2.217. [DOI] [PubMed] [Google Scholar]
  59. Hudson A. J., Davenport A., Hader W. J. The incidence of amyotrophic lateral sclerosis in southwestern Ontario, Canada. Neurology. 1986 Nov;36(11):1524–1528. doi: 10.1212/wnl.36.11.1524. [DOI] [PubMed] [Google Scholar]
  60. Hyser C. L., Kissel J. T., Mendell J. R. Three cases of amyotrophic lateral sclerosis in a common occupational environment. J Neurol. 1987 Aug;234(6):443–444. doi: 10.1007/BF00314096. [DOI] [PubMed] [Google Scholar]
  61. Højer-Pedersen E., Christensen P. B., Jensen N. B. Incidence and prevalence of motor neuron disease in two Danish counties. Neuroepidemiology. 1989;8(3):151–159. doi: 10.1159/000110177. [DOI] [PubMed] [Google Scholar]
  62. Jokelainen M. Amyotrophic lateral sclerosis in Finland. I: An epidemiologic study. Acta Neurol Scand. 1977 Sep;56(3):185–193. doi: 10.1111/j.1600-0404.1977.tb01424.x. [DOI] [PubMed] [Google Scholar]
  63. Jokelainen M. The epidemiology of amyotrophic lateral sclerosis in Finland. A study based on the death certificates of 421 patients. J Neurol Sci. 1976 Sep;29(1):55–63. doi: 10.1016/0022-510x(76)90080-0. [DOI] [PubMed] [Google Scholar]
  64. Jokelainen M., Wikström J., Palo J. Effect of birthplace on the development of amyotrophic lateral sclerosis and multiple sclerosis. A study among Finnish war evacuees. Acta Neurol Scand. 1979;60(5):283–288. doi: 10.1111/j.1600-0404.1979.tb02983.x. [DOI] [PubMed] [Google Scholar]
  65. Juergens S. M., Kurland L. T., Okazaki H., Mulder D. W. ALS in Rochester, Minnesota, 1925-1977. Neurology. 1980 May;30(5):463–470. doi: 10.1212/wnl.30.5.463. [DOI] [PubMed] [Google Scholar]
  66. Juvarra G., Bettoni L., Bortone E., Garavelli A., Montanari E., Rocca M. Amyotrophic lateral sclerosis in the province of Parma, Italy: a clinical and epidemiological study in the period 1960-1980. Ital J Neurol Sci. 1983 Dec;4(4):473–478. doi: 10.1007/BF02125629. [DOI] [PubMed] [Google Scholar]
  67. KURLAND L. T. 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] [Google Scholar]
  68. Kahana E., Alter M., Feldman S. Amyotrophic lateral sclerosis: a population study. J Neurol. 1976 Jun 14;212(3):205–213. doi: 10.1007/BF00314523. [DOI] [PubMed] [Google Scholar]
  69. Kahana E., Zilber N. Changes in the incidence of amyotrophic lateral sclerosis in Israel. Arch Neurol. 1984 Feb;41(2):157–160. doi: 10.1001/archneur.1984.04050140055023. [DOI] [PubMed] [Google Scholar]
  70. Kahn P. Cysticercosis of the central nervous system with amyotrophic lateral sclerosis: case report and review of the literature. J Neurol Neurosurg Psychiatry. 1972 Feb;35(1):81–87. doi: 10.1136/jnnp.35.1.81. [DOI] [PMC free article] [PubMed] [Google Scholar]
  71. Kennedy P. G. On the possible role of viruses in the aetiology of motor neurone disease: a review. J R Soc Med. 1990 Dec;83(12):784–787. doi: 10.1177/014107689008301211. [DOI] [PMC free article] [PubMed] [Google Scholar]
  72. Kilness A. W., Hichberg F. H. Amyotrophic lateral sclerosis in a high selenium environment. JAMA. 1977 Jun 27;237(26):2843–2844. [PubMed] [Google Scholar]
  73. Kondo K., Tsubaki T. Changing mortality patterns of motor neuron disease in Japan. J Neurol Sci. 1977 Jul;32(3):411–424. doi: 10.1016/0022-510x(77)90023-5. [DOI] [PubMed] [Google Scholar]
  74. Korosue K., Shibasaki H., Kuroiwa Y., Machi T., Sawada K., Kitamura K., Ikeda J. Cyst of the conus medullaris manifesting amyotrophic lateral sclerosis syndrome. Folia Psychiatr Neurol Jpn. 1981;35(4):507–510. doi: 10.1111/j.1440-1819.1981.tb00248.x. [DOI] [PubMed] [Google Scholar]
  75. Krarup C., Stewart J. D., Sumner A. J., Pestronk A., Lipton S. A. A syndrome of asymmetric limb weakness with motor conduction block. Neurology. 1990 Jan;40(1):118–127. doi: 10.1212/wnl.40.1.118. [DOI] [PubMed] [Google Scholar]
  76. Kurland L. T. Geographic isolates: their role in neuroepidemiology. Adv Neurol. 1978;19:69–82. [PubMed] [Google Scholar]
  77. Kurland L. T., Molgaard C. A. Guamanian ALS: hereditary or acquired? Adv Neurol. 1982;36:165–171. [PubMed] [Google Scholar]
  78. Kuroda Y., Sugihara H. Autopsy report of HTLV-I-associated myelopathy presenting with ALS-like manifestations. J Neurol Sci. 1991 Dec;106(2):199–205. doi: 10.1016/0022-510x(91)90258-9. [DOI] [PubMed] [Google Scholar]
  79. Leone M., Chandra V., Schoenberg B. S. Motor neuron disease in the United States, 1971 and 1973-1978: patterns of mortality and associated conditions at the time of death. Neurology. 1987 Aug;37(8):1339–1343. doi: 10.1212/wnl.37.8.1339. [DOI] [PubMed] [Google Scholar]
  80. Leone M., Chio A., Mortara P., Rosso M. G., Schiffer D. Motor neuron disease in the Province of Turin, Italy, 1971-1980. Acta Neurol Scand. 1983 Nov;68(5):316–327. doi: 10.1111/j.1600-0404.1983.tb04839.x. [DOI] [PubMed] [Google Scholar]
  81. Leys D., Petit H. Clinical signs of amyotrophic lateral sclerosis developing after polyradiculoneuropathy associated with amitriptyline. BMJ. 1990 Mar 3;300(6724):614–614. doi: 10.1136/bmj.300.6724.614-b. [DOI] [PMC free article] [PubMed] [Google Scholar]
  82. Li T. M., 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. doi: 10.1136/jnnp.53.12.1043. [DOI] [PMC free article] [PubMed] [Google Scholar]
  83. Li T. M., Swash M., Alberman E., Day S. J. Diagnosis of motor neuron disease by neurologists: a study in three countries. J Neurol Neurosurg Psychiatry. 1991 Nov;54(11):980–983. doi: 10.1136/jnnp.54.11.980. [DOI] [PMC free article] [PubMed] [Google Scholar]
  84. Lilienfeld D. E., Chan E., Ehland J., Godbold J., Landrigan P. J., Marsh G., Perl D. P. Rising mortality from motoneuron disease in the USA, 1962-84. Lancet. 1989 Apr 1;1(8640):710–713. doi: 10.1016/s0140-6736(89)92218-6. [DOI] [PubMed] [Google Scholar]
  85. López-Vega J. M., Calleja J., Combarros O., Polo J. M., Berciano J. Motor neuron disease in Cantabria. Acta Neurol Scand. 1988 Jan;77(1):1–5. doi: 10.1111/j.1600-0404.1988.tb06965.x. [DOI] [PubMed] [Google Scholar]
  86. Marco R., Rivera-Reyes L. Motor neuron disease in Puerto Rico: a study of 76 cases. Bol Asoc Med P R. 1983 Sep;75(9):400–402. [PubMed] [Google Scholar]
  87. Martyn C. N., Barker D. J., Osmond C. Motoneuron disease and past poliomyelitis in England and Wales. Lancet. 1988 Jun 11;1(8598):1319–1322. doi: 10.1016/s0140-6736(88)92129-0. [DOI] [PubMed] [Google Scholar]
  88. Martyn C. N. Poliovirus and motor neuron disease. J Neurol. 1990 Oct;237(6):336–338. doi: 10.1007/BF00315655. [DOI] [PubMed] [Google Scholar]
  89. Matsumoto N., Worth R. M., Kurland L. T., Okazaki H. Epidemiologic study of amyotrophic lateral sclerosis in Hawaii. Identification of high incidence among Filipino men. Neurology. 1972 Sep;22(9):934–940. doi: 10.1212/wnl.22.9.934. [DOI] [PubMed] [Google Scholar]
  90. Melmed C., Krieger C. A cluster of amyotrophic lateral sclerosis. Arch Neurol. 1982 Sep;39(9):595–596. doi: 10.1001/archneur.1982.00510210065018. [DOI] [PubMed] [Google Scholar]
  91. Mitchell D. M., Olczak S. A. Remission of a syndrome indistinguishable from motor neurone disease after resection of bronchial carcinoma. Br Med J. 1979 Jul 21;2(6183):176–177. doi: 10.1136/bmj.2.6183.176-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
  92. Mitchell J. D., Gibson H. N., Gatrell A. Amyotrophic lateral sclerosis in Lancashire and South Cumbria, England, 1976-1986. A geographical study. Arch Neurol. 1990 Aug;47(8):875–880. doi: 10.1001/archneur.1990.00530080057011. [DOI] [PubMed] [Google Scholar]
  93. Mitsumoto H., Sliman R. J., Schafer I. A., Sternick C. S., Kaufman B., Wilbourn A., Horwitz S. J. Motor neuron disease and adult hexosaminidase A deficiency in two families: evidence for multisystem degeneration. Ann Neurol. 1985 Apr;17(4):378–385. doi: 10.1002/ana.410170413. [DOI] [PubMed] [Google Scholar]
  94. Moser H. W., Naidu S., Kumar A. J., Rosenbaum A. E. The adrenoleukodystrophies. Crit Rev Neurobiol. 1987;3(1):29–88. [PubMed] [Google Scholar]
  95. Mulder D. W., Howard F. M., Jr Patient resistance and prognosis in amyotrophic lateral sclerosis. Mayo Clin Proc. 1976 Sep;51(9):537–541. [PubMed] [Google Scholar]
  96. Mulrow C. D. The medical review article: state of the science. Ann Intern Med. 1987 Mar;106(3):485–488. doi: 10.7326/0003-4819-106-3-485. [DOI] [PubMed] [Google Scholar]
  97. Murray T. J., Cameron J., Heffernan L. P., MacDonald H. N., King D. B., Bedwell S. R., Scott M., Patil J., Shears A., Malik H. G. Amyotrophic lateral sclerosis in Nova Scotia. Adv Exp Med Biol. 1987;209:345–349. doi: 10.1007/978-1-4684-5302-7_53. [DOI] [PubMed] [Google Scholar]
  98. Murros K., Fogelholm R. Amyotrophic lateral sclerosis in Middle-Finland: an epidemiological study. Acta Neurol Scand. 1983 Jan;67(1):41–47. doi: 10.1111/j.1600-0404.1983.tb04543.x. [DOI] [PubMed] [Google Scholar]
  99. Norris F. H., Denys E. H., Sang K., Mukai E. [The natural history of ALS in a specified population, with comments on risk factors, prognosis and symptomatic treatments]. Rinsho Shinkeigaku. 1989 Dec;29(12):1485–1492. [PubMed] [Google Scholar]
  100. O'Malley F., Dean G., Elian M. Multiple sclerosis and motor neurone disease: survival and how certified after death. J Epidemiol Community Health. 1987 Mar;41(1):14–17. doi: 10.1136/jech.41.1.14. [DOI] [PMC free article] [PubMed] [Google Scholar]
  101. Olivares L., Estéban E. S., Alter M. Mexican "resistance" to amyotrophic lateral sclerosis. Arch Neurol. 1972 Nov;27(5):397–402. doi: 10.1001/archneur.1972.00490170029005. [DOI] [PubMed] [Google Scholar]
  102. Olney R. K., Aminoff M. J., So Y. T. Clinical and electrodiagnostic features of X-linked recessive bulbospinal neuronopathy. Neurology. 1991 Jun;41(6):823–828. doi: 10.1212/wnl.41.6.823. [DOI] [PubMed] [Google Scholar]
  103. Oryema J., Ashby P., Spiegel S. Monomelic atrophy. Can J Neurol Sci. 1990 May;17(2):124–130. doi: 10.1017/s0317167100030316. [DOI] [PubMed] [Google Scholar]
  104. Pall H. S., Williams A. C., Waring R., Elias E. Motoneurone disease as manifestation of pesticide toxicity. Lancet. 1987 Sep 19;2(8560):685–685. doi: 10.1016/s0140-6736(87)92468-8. [DOI] [PubMed] [Google Scholar]
  105. Paolino E., Granieri E., Tola M. R., Rosati G. Conjugal amyotrophic lateral sclerosis. Ann Neurol. 1983 Dec;14(6):699–699. doi: 10.1002/ana.410140627. [DOI] [PubMed] [Google Scholar]
  106. Patten B. M., Engel W. K. Phosphate and parathyroid disorders associated with the syndrome of amyotrophic lateral sclerosis. Adv Neurol. 1982;36:181–200. [PubMed] [Google Scholar]
  107. Pestronk A., Cornblath D. R., Ilyas A. A., Baba H., Quarles R. H., Griffin J. W., Alderson K., Adams R. N. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol. 1988 Jul;24(1):73–78. doi: 10.1002/ana.410240113. [DOI] [PubMed] [Google Scholar]
  108. Preston D. C. Paraneoplastic motor neuron disease. Neurology. 1990 Oct;40(10):1638–1638. doi: 10.1212/wnl.40.10.1638. [DOI] [PubMed] [Google Scholar]
  109. Qizilbash N., Bates D. Incidence of motor neurone disease in the northern region. J Epidemiol Community Health. 1987 Mar;41(1):18–20. doi: 10.1136/jech.41.1.18. [DOI] [PMC free article] [PubMed] [Google Scholar]
  110. Radhakrishnan K., Ashok P. P., Sridharan R., Mousa M. E. Descriptive epidemiology of motor neuron disease in Benghazi, Libya. Neuroepidemiology. 1986;5(1):47–54. doi: 10.1159/000110812. [DOI] [PubMed] [Google Scholar]
  111. Rafalowska J., Wasowicz B. Syringomyelia simulating amyotrophic lateral sclerosis. Pol Med J. 1968;7(5):1214–1218. [PubMed] [Google Scholar]
  112. Reed D. M., Brody J. A. Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam, 1945-1972. I. Descriptive epidemiology. Am J Epidemiol. 1975 Apr;101(4):287–301. doi: 10.1093/oxfordjournals.aje.a112097. [DOI] [PubMed] [Google Scholar]
  113. Reed D. M., Torres J. M., Brody J. A. Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam, 1945-1972. II. Familial and genetic studies. Am J Epidemiol. 1975 Apr;101(4):302–310. doi: 10.1093/oxfordjournals.aje.a112098. [DOI] [PubMed] [Google Scholar]
  114. Reed D., Labarthe D., Chen K. M., Stallones R. A cohort study of amyotrophic lateral sclerosis and parkinsonism-dementia on Guam and Rota. Am J Epidemiol. 1987 Jan;125(1):92–100. doi: 10.1093/oxfordjournals.aje.a114515. [DOI] [PubMed] [Google Scholar]
  115. Riggs J. E. Longitudinal Gompertzian analysis of amyotrophic lateral sclerosis mortality in the U.S., 1977-1986: evidence for an inherently susceptible population subset. Mech Ageing Dev. 1990 Sep;55(3):207–220. doi: 10.1016/0047-6374(90)90149-a. [DOI] [PubMed] [Google Scholar]
  116. Rodgers-Johnson P., Garruto R. M., Yanagihara R., Chen K. M., Gajdusek D. C., Gibbs C. J., Jr Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam: a 30-year evaluation of clinical and neuropathologic trends. Neurology. 1986 Jan;36(1):7–13. doi: 10.1212/wnl.36.1.7. [DOI] [PubMed] [Google Scholar]
  117. Rosati G., Govoni E., Aiello I., Granieri E., Tola R., Bragaglia M. M. Hyperthyroidism as a precipitating factor of amyotrophic lateral sclerosis: a case report. Acta Neurol (Napoli) 1980 Jun;2(3):166–172. [PubMed] [Google Scholar]
  118. Rosati G., Pinna L., Granieri E., Aiello I., Tola R., Agnetti V., Pirisi A., de Bastiani P. Studies on epidemiological, clinical, and etiological aspects of ALS disease in Sardinia, Southern Italy. Acta Neurol Scand. 1977 Mar;55(3):231–244. doi: 10.1111/j.1600-0404.1977.tb05642.x. [DOI] [PubMed] [Google Scholar]
  119. Sadowsky C. H., Sachs E., Jr, Ochoa J. Postradiation motor neuron syndrome. Arch Neurol. 1976 Nov;33(11):786–787. doi: 10.1001/archneur.1976.00500110054010. [DOI] [PubMed] [Google Scholar]
  120. Salemi G., Fierro B., Arcara A., Cassata M., Castiglione M. G., Savettieri G. Amyotrophic lateral sclerosis in Palermo, Italy: an epidemiological study. Ital J Neurol Sci. 1989 Oct;10(5):505–509. doi: 10.1007/BF02333946. [DOI] [PubMed] [Google Scholar]
  121. Sanders M. Clustering of amyotrophic lateral sclerosis. JAMA. 1980 Aug 1;244(5):435–435. [PubMed] [Google Scholar]
  122. Scarpa M., Colombo A., Panzetti P., Sorgato P. Epidemiology of amyotrophic lateral sclerosis in the province of Modena, Italy. Influence of environmental exposure to lead. Acta Neurol Scand. 1988 Jun;77(6):456–460. doi: 10.1111/j.1600-0404.1988.tb05940.x. [DOI] [PubMed] [Google Scholar]
  123. Sienko D. G., Davis J. P., Taylor J. A., Brooks B. R. 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. doi: 10.1001/archneur.1990.00530010046017. [DOI] [PubMed] [Google Scholar]
  124. Sonies B. C., Dalakas M. C. Dysphagia in patients with the post-polio syndrome. N Engl J Med. 1991 Apr 25;324(17):1162–1167. doi: 10.1056/NEJM199104253241703. [DOI] [PubMed] [Google Scholar]
  125. Spencer P. S., Nunn P. B., Hugon J., Ludolph A. C., Ross S. M., Roy D. N., Robertson R. C. Guam amyotrophic lateral sclerosis-parkinsonism-dementia linked to a plant excitant neurotoxin. Science. 1987 Jul 31;237(4814):517–522. doi: 10.1126/science.3603037. [DOI] [PubMed] [Google Scholar]
  126. Spencer P. S., Nunn P. B., Hugon J., Ludolph A., Roy D. N. Motorneurone disease on Guam: possible role of a food neurotoxin. Lancet. 1986 Apr 26;1(8487):965–965. doi: 10.1016/s0140-6736(86)91059-7. [DOI] [PubMed] [Google Scholar]
  127. Stanhope J. M., Brody J. A., Morris C. E. Epidemiologic features of amyotropic lateral sclerosis and parkinsonism-dementia in Guam, Mariana Islands. Int J Epidemiol. 1972 Autumn;1(3):199–210. doi: 10.1093/ije/1.3.199. [DOI] [PubMed] [Google Scholar]
  128. Tandan R., Bradley W. G. Amyotrophic lateral sclerosis: Part 2. Etiopathogenesis. Ann Neurol. 1985 Oct;18(4):419–431. doi: 10.1002/ana.410180402. [DOI] [PubMed] [Google Scholar]
  129. Teitelbaum J. S., Zatorre R. J., Carpenter S., Gendron D., Evans A. C., Gjedde A., Cashman N. R. Neurologic sequelae of domoic acid intoxication due to the ingestion of contaminated mussels. N Engl J Med. 1990 Jun 21;322(25):1781–1787. doi: 10.1056/NEJM199006213222505. [DOI] [PubMed] [Google Scholar]
  130. 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. 1992 Jul;55(7):536–541. doi: 10.1136/jnnp.55.7.536. [DOI] [PMC free article] [PubMed] [Google Scholar]
  131. Tyler H. R. Nonfamilial amyotrophy with dementia or multisystem degeneration and other neurological disorders. Adv Neurol. 1982;36:173–180. [PubMed] [Google Scholar]
  132. Vemireddi N. K., Crevoiserat C. A., Jr Amyotrophic lateral sclerosis in cancer. J Miss State Med Assoc. 1978 Nov;19(11):201–204. [PubMed] [Google Scholar]
  133. Williams D. B., Windebank A. J. Motor neuron disease (amyotrophic lateral sclerosis). Mayo Clin Proc. 1991 Jan;66(1):54–82. doi: 10.1016/s0025-6196(12)61175-6. [DOI] [PubMed] [Google Scholar]
  134. World Federation of Neurology Research Committee Research Group on Neuromuscular Diseases. J Neurol Sci. 1988 Sep;86(2-3):333–360. [PubMed] [Google Scholar]
  135. Yanagihara R. T., Garruto R. M., Gajdusek D. C. Epidemiological surveillance of amyotrophic lateral sclerosis and parkinsonism-dementia in the Commonwealth of the Northern Mariana Islands. Ann Neurol. 1983 Jan;13(1):79–86. doi: 10.1002/ana.410130117. [DOI] [PubMed] [Google Scholar]
  136. Yanagihara R., Garruto R. M., Gajdusek D. C., Tomita A., Uchikawa T., Konagaya Y., Chen K. M., Sobue I., Plato C. C., Gibbs C. J., Jr Calcium and vitamin D metabolism in Guamanian Chamorros with amyotrophic lateral sclerosis and parkinsonism-dementia. Ann Neurol. 1984 Jan;15(1):42–48. doi: 10.1002/ana.410150108. [DOI] [PubMed] [Google Scholar]
  137. Yoshida S., Mulder D. W., Kurland L. T., Chu C. P., Okazaki H. Follow-up study on amyotrophic lateral sclerosis in Rochester, Minn., 1925 through 1984. Neuroepidemiology. 1986;5(2):61–70. doi: 10.1159/000110815. [DOI] [PubMed] [Google Scholar]
  138. Younger D. S., Chou S., Hays A. P., Lange D. J., Emerson R., Brin M., Thompson H., Jr, Rowland L. P. Primary lateral sclerosis. A clinical diagnosis reemerges. Arch Neurol. 1988 Dec;45(12):1304–1307. doi: 10.1001/archneur.1988.00520360022005. [DOI] [PubMed] [Google Scholar]
  139. Younger D. S., Rowland L. P., Latov N., Hays A. P., Lange D. J., Sherman W., Inghirami G., Pesce M. A., Knowles D. M., Powers J. Lymphoma, motor neuron diseases, and amyotrophic lateral sclerosis. Ann Neurol. 1991 Jan;29(1):78–86. doi: 10.1002/ana.410290114. [DOI] [PubMed] [Google Scholar]
  140. Younger D. S., Rowland L. P., Latov N., Sherman W., Pesce M., Lange D. J., Trojaborg W., Miller J. R., Lovelace R. E., Hays A. P. Motor neuron disease and amyotrophic lateral sclerosis: relation of high CSF protein content to paraproteinemia and clinical syndromes. Neurology. 1990 Apr;40(4):595–599. doi: 10.1212/wnl.40.4.595. [DOI] [PubMed] [Google Scholar]
  141. Zack M. M., Levitt L. P., Schoenberg B. Motor neuron disease in Lehigh county, Pennsylvania: an epidemiologic study. J Chronic Dis. 1977 Dec;30(12):813–818. doi: 10.1016/0021-9681(77)90009-1. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES