Rising amyotrophic lateral sclerosis mortality in France 1968?1990: Increased life expectancy and inter-disease competition as an explanation

Neilson, Stuart; Robinson, Ian; Alpérovitch, Annick

doi:10.1007/bf00900964

Cited by 50 publications

(38 citation statements)

References 37 publications

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“…3, 4). Trends in an increasing age at disease onset have also been reported in other countries (11)(12)(13)(14)(15)(16). There are several possible causes of the increase in age at disease onset, one of which is the rising life expectancy observed in the general population.…”

Section: Discussionmentioning

confidence: 74%

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

et al. 2013

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Objective To assess longitudinal changes in the clinical features of patients with amyotrophic lateral sclerosis (ALS), we performed a retrospective hospital-based study covering 35 years. Methods We investigated 287 patients (154 men and 133 women) with sporadic ALS hospitalized at the Department of Neurology at Gunma University Hospital (Japan) between 1978 and 2012. All patients fulfilled the diagnostic criteria for definitive, probable or laboratory-supported probable ALS according to the revised El Escorial criteria. Results Two hundred patients (69.7%) exhibited limb onset and 87 patients (30.3%) exhibited bulbar onset of the disease. The percentage of patients who showed bulbar onset of the disease increased steadily over the 35 years from 14.2% to 38.3% (2008-12) (p<0.01, r=0.470). The mean age at onset was 62.1 ± 11.7 years, and the age at onset increased significantly over time from 51.7 years (1978-82) to 64.9 years (2008-12) (p<0.001, r=0.294). In addition, the percentage of patients whose age at onset was 70 years or more increased from 0% (1978-82) to 38.2% . The percentage of ALS patients with dementia increased from 0% (1978-82) to 20.2% (2008-12). Conclusion Our findings demonstrate that, among patients with sporadic ALS, the age at disease onset, the proportion of patients with disease onset at 70 years of age or higher, the proportion of patients with dementia and the proportion of patients with bulbar onset ALS have increased significantly over the past 35 years. The longitudinal changes observed in the clinical features of ALS may reflect the increasing age at disease onset.

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Section: Discussionmentioning

confidence: 74%

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

et al. 2013

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“…That is, people are dying from ALS because they are not dying from other diseases 24 . As therapeutic progress is slow, the social burden due to the disease is bound to increase.…”

Section: Discussionmentioning

confidence: 99%

Amyotrophic lateral sclerosis in Brazil: 1998 national survey

Dietrich-neto¹,

Callegaro

Dias‐Tosta

et al. 2000

Arq. Neuro-Psiquiatr.

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-Objectives: To assess the epidemiologic characteristics of amyotrophic lateral sclerosis (ALS) in Brazil in 1998. Method: Structured Clinical Report Forms (CRFs) sent to 2,505 Brazilian neurologists from January to September 1998 to be filled with demographic and clinical data regarding any ALS patient seen at any time during that year. Results: Five hundred and forty CRFs were returned by 168 neurologists. Data on 443 patients meeting the criteria of probable or definite ALS according to El Escorial definition were analysed: 63 probable (14.2%) and 380 definite (85.8%). Two hundred and fifty-nine (58.5%) of the patients were male, mean age of onset was 52. Spinal onset occurred in 306 patients (69%); bulbar onset in 82 (18.5%), and both in 52 (11.7%). Twenty-six (5.9%) had a family history of ALS. Two hundred and fifty-nine (58.6%) were seen by private practitioners, and 178 (40.2%) at a hospital clinic. Age-ajusted incidence shows a peak incidence at the 65-74 years old range. Conclusions: The disease's characteristics are similar to those described in international studies, except for age of onset (Brazilian patients are younger). This difference is not confirmed when figures are age-adjusted.KEY WORDS: amyotrophic lateral sclerosis, ALS, epidemiology, prevalence, incidence. Esclerose lateral amiotrófica no Brasil: registro nacional, 1998RESUMO -Objetivos: Avaliar as características epidemiológicas da esclerose lateral amiotrófica (ELA) no Brasil durante o ano de 1998. Método: Formulários estruturados enviados a 2.505 neurologistas brasileiros de janeiro a setembro de 1998 para serem preenchidos com dados demográficos e clínicos de todos os pacientes com ELA atendidos no ano de 1998. Resultados: Quinhentos e quarenta formulários retornaram, enviados por 168 neurologistas. Dados sobre 443 pacientes que se enquadravam nos critérios de ELA provável ou definida de acordo com El Escorial foram analisados: 63 provável (14,2%) e 380 definida (85,8%). Duzentos e cinquenta (58,5%) eram do sexo masculino. A idade média de aparecimento dos primeiros sintomas foi de 52 anos. O início em membros ocorreu em 306 pacientes (69%), bulbar em 82 (18,5%) e generallizada em 52 (11,7%). Vinte e seis (5,9%) tinham história familiar. Duzentos e cinquenta e nove (58,6%) foram atendidos em clínicas ou consultórios, e 178 (40,2%) em ambulatório de hospital. Ajustando para a distribuição da população brasileira segundo faixa etária, a maior incidência ocorre entre 65 e 74 anos de idade. Conclusões: As características epidemiológicas da doença são semelhantes àquelas descritas em estudos internacionais, exceto para idade dos primeiros sintomas (pacientes brasileiros são mais jovens). Esta diferença desaparece quando os valores são ajustados para idade. PALAVRAS-CHAVE: esclerose lateral amiotrófica, ELA, epidemiologia, prevalência, incidência.Amyotrophic lateral sclerosis (ALS) is an invariably fatal neurodegenerative disease encompassed in the spectrum of neuromuscular disorders (NMD) and characterized by progressive

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“…An increase in both incidence and mortality during the last decades has been reported in several surveys [9][10][11][12][13][14][15][16]. This finding has been related to a growing population at risk for the disease because of ageing of the population [1,17], but a true increase in ALS incidence over time cannot be excluded. A previous epidemiological investigation of 230 Neuroepidemiology 2003;22:229-234 Govoni/Granieri/Capone/Manconi/Casetta ALS in the area of Ferrara, northern Italy, carried out by our research group [18] found an incidence rate for the years 1964-1982 which fell into the above-mentioned range of reported rates [1][2][3][4][5][6][7][8].…”

Section: Introductionmentioning

confidence: 88%

“…This could be due to changes in lifestyle and behaviour among women who could be more exposed to some factors contributing to the occurrence of the disease than they had been in the past [25]. Otherwise, if the increase in ALS incidence is related to population ageing [1,17], a larger increase in women could be due to a more prominent ageing in the subpopulation of women than the subpopulation of men giving an artifactual increase in incidence in women as compared with men [26]. The official statistics seem to support this explanation.…”

Section: Discussionmentioning

confidence: 99%

Incidence of Amyotrophic Lateral Sclerosis in the Local Health District of Ferrara, Italy, 1964–1998

Govoni¹,

Granieri²,

Capone³

et al. 2003

Neuroepidemiology

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One of the epidemiologic characteristics of amyotrophic lateral sclerosis (ALS) still under discussion is whether the incidence of ALS is increasing over time. We performed a new investigation expanding our previous study of ALS in the local health district (LHD) of Ferrara, northern Italy, to determine whether there have been any changes in the incidence of ALS in the years 1964–1998. We used a complete enumeration approach by reviewing all possible sources of case collection available in the study area. We selected all patients with definite and probable ALS according to the World Federation of Neurology criteria. The mean annual crude incidence rate for 1964–1998 was 1.63 per 100,000 population (95% CI 1.31–2.00). An increase in incidence from 1.07 to 2.19 per 100,000 population was observed during the study period. It was greater in women and in individuals of 70 years old and over. Substantial population ageing occurred in the LHD of Ferrara during the study period and it was more prominent in women. This increase in incidence seems to be explained mainly by the ageing of the population. Moreover, greater precision in diagnosis of ALS in elderly women, rather than better case ascertainment of diagnosed patients, may have contributed to the increase. The role of environmental factors cannot be excluded, but based on the present findings, it seems to be of little importance.

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Rising amyotrophic lateral sclerosis mortality in France 1968?1990: Increased life expectancy and inter-disease competition as an explanation

Cited by 50 publications

References 37 publications

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

Changes in the Clinical Features of Amyotrophic Lateral Sclerosis in Rural Japan

Amyotrophic lateral sclerosis in Brazil: 1998 national survey

Incidence of Amyotrophic Lateral Sclerosis in the Local Health District of Ferrara, Italy, 1964–1998

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