Background/Aims: Trends in disease incidence and mortality can provide clues to disease etiology. Previously, we described a town in New Hampshire (N.H.), USA, with 25 times the expected incidence rate of amyotrophic lateral sclerosis (ALS). This study aimed to describe the incidence and mortality of ALS across the state to assess rates relative to other states and industrialized nations. Method: A retrospective review of records from regional ALS centers, clinics and ALS organizations was conducted to obtain demographics and diagnostic details for patients diagnosed with ALS or primary lateral sclerosis in N.H. from January 2004 to December 2007. Data on mortality from review of death certificates were obtained for a similar time frame. Results: We identified 113 N.H. residents diagnosed with ALS in 2004-2007, yielding an age-standardized incidence rate ranging from 1.3 to 2.2 per 100,000 of the population per year. During the same period, the standardized mortality rate per 100,000 varied from 2.6 to 3.5. ALS was more common among men (ratio 1.6:1), who were more likely than women to have an earlier age at onset (59 ± 14.2 vs. 65 ± 11.8 years, p = 0.01). Conclusion: While localized areas in N.H. with high ALS incidence rates have been reported previously, the overall incidence and mortality rates of ALS in N.H. are similar to those in other industrialized nations.

Keywords:
Amyotrophic lateral sclerosis, Motor neuron disease, Incidence
1.
Wolfson C, et al: Incidence and prevalence of amyotrophic lateral sclerosis in Canada: a systematic review of the literature. Neuroepidemiology 2009;33:79-88.
2.
Traynor BJ, et al: Incidence and prevalence of ALS in Ireland, 1995-1997: a population-based study. Neurology 1999;52:504-509.
3.
Logroscino G, et al: Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry 2010;81:385-390.
4.
Fang F, et al: Amyotrophic lateral sclerosis in Sweden, 1991-2005. Arch Neurol 2009;66:515-519.
5.
Boumediene F, et al: Contribution of geolocalisation to neuroepidemiological studies: incidence of ALS and environmental factors in Limousin, France. J Neurol Sci 2011;309:115-122.
6.
Chio A, et al: Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 2013;41:118-130.
7.
Chio A, et al: Epidemiology of ALS in Italy: a 10-year prospective population-based study. Neurology 2009;72:725-731.
8.
Noonan CW, et al: Temporal and geographic variation in United States motor neuron disease mortality, 1969-1998. Neurology 2005;64:1215-1221.
9.
Okamoto K, et al: Descriptive epidemiology of amyotrophic lateral sclerosis in Japan, 1995-2001. J Epidemiol 2005;15:20-23.
10.
Sejvar JJ, et al: Amyotrophic lateral sclerosis mortality in the United States, 1979-2001. Neuroepidemiology 2005;25:144-152.
11.
Worms PM: The epidemiology of motor neuron diseases: a review of recent studies. J Neurol Sci 2001;191:3-9.
12.
Neilson S, Gunnarsson LG, Robinson I: Rising mortality from motor neurone disease in Sweden 1961-1990: the relative role of increased population life expectancy and environmental factors. Acta Neurol Scand 1994;90:150-159.
13.
Fang F, et al: Workplace exposures and the risk of amyotrophic lateral sclerosis. Environ Health Perspect 2009;117:1387-1392.
14.
Kamel F, et al: Lead exposure as a risk factor for amyotrophic lateral sclerosis. Neurodegener Dis 2005;2:195-201.
15.
Johnson FO, Atchison WD: The role of environmental mercury, lead and pesticide exposure in development of amyotrophic lateral sclerosis. Neurotoxicology 2009;30:761-765.
16.
Rooney J: Further thoughts on mercury, epigenetics, genetics and amyotrophic lateral sclerosis. Neurodegener Dis 2011;8:523-524.
17.
Kamel F, et al: Pesticide exposure and amyotrophic lateral sclerosis. Neurotoxicology 2012;33:457-462.
18.
McGuire V, et al: Occupational exposures and amyotrophic lateral sclerosis. A population-based case-control study. Am J Epidemiol 1997;145:1076-1088.
19.
Banack SA, Caller TA, Stommel EW: The cyanobacteria derived toxin beta-N-methylamino-L-alanine and amyotrophic lateral sclerosis. Toxins (Basel) 2010;2:2837-2850.
20.
Schmidt S, et al: Association of ALS with head injury, cigarette smoking and APOE genotypes. J Neurol Sci 2010;291:22-29.
21.
Armon C: Occurrence of amyotrophic lateral sclerosis among Gulf War veterans. Neurology 2007;68:1083.
22.
Horner RD, et al: Amyotrophic lateral sclerosis among 1991 Gulf War veterans: evidence for a time-limited outbreak. Neuroepidemiology 2008;31:28-32.
23.
Marin B, et al: Can mortality data be used to estimate amyotrophic lateral sclerosis incidence? Neuroepidemiology 2011;36:29-38.
24.
Sorenson EJ, et al: Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology 2002;59:280-282.
25.
Nelson LM, et al: Population-based case-control study of amyotrophic lateral sclerosis in western Washington State. I. Cigarette smoking and alcohol consumption. Am J Epidemiol 2000;151:156-163.
26.
Torbick N, et al: Mapping amyotrophic lateral sclerosis lake risk factors across northern New England. Int J Health Geogr 2014;13:1.
27.
Caller TA, et al: Spatial clustering of amyotrophic lateral sclerosis and the potential role of BMAA. Amyotroph Lateral Scler 2012;13:25-32.
28.
Benatar M, et al: Preparing for a U.S. National ALS Registry: lessons from a pilot project in the State of Georgia. Amyotroph Lateral Scler 2011;12:130-135.
29.
Wittie M, et al: Utility of capture-recapture methodology to assess completeness of amyotrophic lateral sclerosis case ascertainment. Neuroepidemiology 2013;40:133-141.
30.
Pisa FE, et al: The accuracy of discharge diagnosis coding for amyotrophic lateral sclerosis in a large teaching hospital. Eur J Epidemiol 2009;24:635-640.
31.
Sutedja NA, et al: What we truly know about occupation as a risk factor for ALS: a critical and systematic review. Amyotroph Lateral Scler 2009;10:295-301.
32.
Huisman MH, et al: Population-based epidemiology of amyotrophic lateral sclerosis using capture-recapture methodology. J Neurol Neurosurg Psychiatry 2011;82:1165-1170.
33.
Logroscino G, et al: Incidence of amyotrophic lateral sclerosis in southern Italy: a population-based study. J Neurol Neurosurg Psychiatry 2005;76:1094-1098.
34.
Logroscino G, et al: Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry 2008;79:6-11.
35.
Factor-Litvak P, et al: Current pathways for epidemiological research in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2013;14(suppl 1):33-43.
36.
Swash M, Desai J, Misra VP: What is primary lateral sclerosis? J Neurol Sci 1999;170:5-10.
37.
Tomik B, Zur KA, Szczudlik A: Pure primary lateral sclerosis - case reports. Clin Neurol Neurosurg 2008;110:387-391.
38.
Le Forestier N, et al: Primary lateral sclerosis: further clarification. J Neurol Sci 2001;185:95-100.
39.
Rowland LP: Primary lateral sclerosis: disease, syndrome, both or neither? J Neurol Sci 1999;170:1-4.
40.
Paliwal VK, et al: ‘Wine-glass appearance' of pyramidal tracts in a patient with primary lateral sclerosis. Neurol India 2012;60:110-111.
41.
Flynn L, Stephen M, Floeter MK: Disease spread through contiguity and axonal tracts in primary lateral sclerosis. Muscle Nerve 2014;49:439-441.
42.
Korner S, et al: Onset and spreading patterns of upper and lower motor neuron symptoms in amyotrophic lateral sclerosis. Muscle Nerve 2011;43:636-642.
43.
Tan CF, et al: Primary lateral sclerosis: a rare upper-motor-predominant form of amyotrophic lateral sclerosis often accompanied by frontotemporal lobar degeneration with ubiquitinated neuronal inclusions? Report of an autopsy case and a review of the literature. Acta Neuropathol 2003;105:615-620.
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2015
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