A Clustering of Conjugal Amyotrophic Lateral Sclerosis in Southeastern France

Corcia, Philippe; Jafari-Schluep, Helene-Farnase; Lardillier, Dominique; Mazyad, Hassan; Giraud, Pieric; Clavelou, Pierre; Pouget, Jean; Camu, William

doi:10.1001/archneur.60.4.553

Cited by 41 publications

(22 citation statements)

References 25 publications

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“…A cluster discovered in France [65] where 9 couples developed ALS between 1975 and 1992, implied a possible role of shared environmental exposure to exogenous factors or infections. Two significant population clusters were found in Finland with over a third of ALS cases, but only 24% of Finland's population, demonstrating a relation between the clusters and the place of birth (more than to the place of death).…”

Section: Discussionmentioning

confidence: 99%

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Geographic distribution of amyotrophic lateral sclerosis through motor neuron disease mortality data

Uccelli

Binazzi²,

Altavista

et al. 2007

Eur J Epidemiol

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Amyotrophic lateral sclerosis (ALS) is a rare and devastating neurological disorder of the adult age with a prognosis of about 2-3 years from the onset of the disease. No clear cause has been identified but it seems to be a multifactorial disease with genetic and environmental components involved. Increments of mortality rates were observed since 1980 both in Italy and in many other countries. The objective of the present study is to describe the distribution of ALS mortality in Italy in the period 1980-2001 detecting single municipalities or clusters with high mortality levels for motor neuron disease (MND). ALS represents the main part (85%) of the MND group which is globally identified by the IX ICD (International Classification of Diseases and Causes of Death) 335.2 code. Death numbers and standardized mortality ratios (SMR) for MND were calculated for all Italian municipalities through the ENEA mortality database system (data source: National Institute of Statistics-ISTAT), using national mortality rates as reference. Subsequently, in order to detect municipal clusters, spatial analysis was performed. Out of the 8,099 Italian municipalities, 132 where characterized by SMR values higher than expected. Moreover 16 clusters with significant high relative risk values (RR) were identified, 12 out of them including only a single municipality. Only 22 of the municipalities with high SMR were included in the clusters. In conclusion, the two different epidemiological methodologies demonstrated to be widely complementary in detecting the geographical distribution of the disease in terms of risk for populations. A first selection of the priority areas where analytical studies should be carried on, in order to identify risk factors associated to ALS, is tentatively suggested.

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Section: Discussionmentioning

confidence: 99%

“…ALS seems affecting men more frequently than women (ratio 1.4:1.0) [1,6] and the age class with higher risk is [65][66][67][68][69][70][71][72][73][74]. Prognosis is about 2-3 years from the beginning of the disease, though some 12-15 years survival cases have been observed, mainly in the older age classes [7,8,9,10,11].…”

Section: Introductionmentioning

confidence: 99%

Geographic distribution of amyotrophic lateral sclerosis through motor neuron disease mortality data

Uccelli

Binazzi²,

Altavista

et al. 2007

Eur J Epidemiol

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“…However, this was an isolated observation and it does not permit a definite conclusion. The largest series on conjugal ALS 24 observed that the long period of conjugal life is more compatible with environmental factors, but concluded that the possibility coincidence cannot be rejected.…”

Section: Resultsmentioning

confidence: 99%

“…conjugal ALS was noted in 10 couples on Guam, where ALS was 50 to 100 times more common 15 . Since then there were other reports outside the Western Pacific [16][17][18][19][20][21][22][23][24][25] . except in Guam, where an environmental factor was considered, none of the previous reports pointed out a clear explanation for appearance of these clusters.…”

Section: Resultsmentioning

confidence: 99%

Conjugal amyotrophic lateral sclerosis in Brazil

Godeiro-Júnior

Oliveira

Felı́cio

et al. 2009

Arq. Neuro-Psiquiatr.

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-The origin of amyotrophic lateral sclerosis (ALS) remains unknown, although it seems to be multifactorial. The role of environmental factors has been frequently investigated and suspicion of its influence can be obtained when clusters of a rare disease are described. Objective: To describe conjugal cases of ALS in Brazil. Method: We describe 2 couples in which both spouses were affected by ALS. Both couples had lived in southeast Brazil and were married for at least 20 years. Results: There was a great variability in clinical presentation of ALS in our patients. In both couples the interval between disease onsets was short. No precise environmental factors could be identified at the origin of these conjugal cases. Conclusion: The occurrence of ALS in couples living in the same area may be epidemiologically important, but we cannot exclude that cases may be due to a chance association.Key WordS: amyotrophic lateral sclerosis, conjugal, environmental factors, etiology.esclerose lateral amiotrófica em casais no Brasil resumo -A origem da esclerose lateral amiotrófica (eLA) permanece desconhecida. o papel de fatores ambientais tem sido freqüentemente investigado e a suspeição de sua influência pode ser obtida quando são descritas salvas de casos de uma doença rara. Objetivo: descrever casos de eLA em casais no Brasil. Método: Apresentamos dois casais em que ambos os cônjuges forma acometidos pela eLA. Ambos os casais residiram na região sudeste do Brasil e estiveram casados por pelo menos 20 anos. Resultados: Houve grande variabilidade na apresentação clínica da eLA em nossos pacientes. em ambos os casais, o intervalo de início da doença foi curto. Nenhum fator ambiental foi identificado na etiologia destes casos conjugais. Conclusão: A ocorrência de eLA em casais que habitam a mesma região pode ser epidemiologicamente importante, mas não podemos excluir que estes casos tenham sido mera associação.PALAvrAS-cHAve: esclerose lateral amiotrófica, casais, fatores ambientais, etiologia.

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“…3,4 Most of these appear to have dropped by the wayside in recent years. Nevertheless, reports of clear-cut clusters of patients with ALS in nonendemic areas of the world should return our attention to environmental causes of ALS 5,6 (E. W. Stommel, 2007, personal communication). We may now have some clues to at least one of the environmental factors that can cause ALS.…”

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confidence: 99%