Clinical and demographic factors and outcome of amyotrophic lateral sclerosis in relation to population ancestral origin

Marin, Benoît; Logroscino, Giancarlo; Boumédiène, Farid; Labrunie, Anaïs; Couratier, Philippe; Babron, Marie‐Claude; Leutenegger, Anne-Louise; Preux, Pierre‐Marie; Beghi, Ettore

doi:10.1007/s10654-015-0090-x

Cited by 94 publications

(70 citation statements)

References 124 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…We found that the median age at onset was 53.0 years, although eight cases (4.3%) experienced disease onset before 30 years of age (four before 25 years of age). The overall age of onset was far younger than those of occidental series, in which the age at onset ranged from 60 to 65 years 15. The age differences between African and Western patients with ALS reflect differences in population age structures, which in turn are associated with differences in life expectancy and birth rates 16.…”

Section: Discussionmentioning

confidence: 85%

“…Interestingly, Western studies published before 1990 consistently reported an SR around 2,22 which decreased to a modest male predominance (SR=1.3) in recent studies 15. This apparent trend of SR was explained by differences in study design (retrospective vs prospective in the most recent studies) and in case ascertainment (referral-based vs population-based studies in the most recent studies) 5.…”

Section: Discussionmentioning

confidence: 97%

“…Previous studies found that ALS differed in terms of clinical presentation by age, in that older patients exhibited more frequent bulbar onset 27 28. Interestingly, population-based studies from Europe also identified differences in the proportion of bulbar onset between Northern Europe (45.4%) and Western and Southern Europe (34.9% and 34.2%, respectively) 15. To date, such differences remain underexplored.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study

Luna

Diagana

Aissa

et al. 2018

J Neurol Neurosurg Psychiatry

Self Cite

View full text Add to dashboard Cite

ObjectiveWe describe and compare the sociodemographic and clinical features, treatments, and prognoses and survival times of patients with amyotrophic lateral sclerosis (ALS) in Africa.MethodologyWe conducted a multicentre, hospital-based cohort study in Africa. Patients with ALS diagnosed in the neurology departments of participating hospitals from 2005 to 2017 were included. Subgroup analysis was performed by subcontinent. Survival analyses were conducted using the Cox proportional hazards model.ResultsNine centres from eight African countries participated. A total of 185 patients with ALS were included: 114 from Northern Africa, 41 from Western Africa and 30 from Southern Africa. A male predominance (male to female ratio 2.9) was evident. The median age at onset was 53.0 years (IQR 44.5–64.0 years). The onset was bulbar in 22.7%. Only 47 patients (26.3%) received riluzole, mainly in Northern and Western Africa. The median survival from the time of diagnosis was 14.0 months (95% CI 10.7 to 17.2 months). The median survival was longer in Northern Africa (19.0 months, 95% CI 10.8 to 27.2 months) than in Western (4.0 months, 95% CI 0.8 to 7.1 months) and Southern (11.0 months, 95% CI 5.6 to 16.4 months) Africa (Breslow test, p<0.0001). Both subcontinental location and riluzole treatment independently affected survival.ConclusionMore African patients with ALS were male and younger and exhibited a lower proportion of bulbar onset compared with patients with ALS from Western nations. Survival was consistent with that in Western registers but far shorter than what would be expected for young patients with ALS. The research improves our understanding of the disease in Africa.

show abstract

Section: Discussionmentioning

confidence: 85%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study

Luna

Diagana

Aissa

et al. 2018

J Neurol Neurosurg Psychiatry

Self Cite

View full text Add to dashboard Cite

show abstract

“…The survivorship in published studies varied from around 25 months in Northern Europe to around 30 months in Western/Southern Europe. Survivorship was longer in North America [26] and much longer in Asia (particularly in Iran where it was 48 months). The bulbar onset was much more common in Northern Europe (45%) than in Southern Europe (30%).…”

Section: The Geographic Gradient and Ethnic Diversitymentioning

confidence: 99%

Amyotrophic Lateral Sclerosis Descriptive Epidemiology: The Origin of Geographic Difference

Logroscino

Piccininni

2019

Neuroepidemiology

Self Cite

View full text Add to dashboard Cite

Amyotrophic lateral sclerosis (ALS) epidemiology has rapidly developed in the last 30 years alongside the evolving changes in concepts in the field of clinical ALS and also due to the recent proposals of new classification system for motor neuron diseases. Many of these changes in the clinical scenario have been determined through the results of ALS population-based studies conducted in the last 20 years primarily in Europe. All the evidences converge to show that ALS risk is different across continents and ethnicities. In a European registry consortium named EURALS, ALS incident cases were drawn from a source population comprising almost 24 million people across Europe (ALS cases: 1028) and the estimated incidence was 2.2 per 100,000 person-years (py) for the general population. In contrast, other population-based studies have measured the lowest incidence in East Asia to be 0.89 per 100,000 py and in South Asia to be 0.79 per 100,000 py. A large part of Africa, Latin America and Asia does not have any population-based studies. The origin of geographic difference in ALS incidence is a matter of debate. Probably, this is partly due to genes (C9ORF72) and partly due to environmental risk factors. The rapid disappearance of ALS Foci in Guam, Kii, and West Guinea underline the importance of changes in lifestyle and environmental factors. The Global Burden of Disease, a project aiming to describe the burden of all diseases and injuries across all the countries of the world with a standardized protocol, has collected heterogeneous sources of data to estimate the burden of motor neuron diseases. The demographic changes related to increased expectation of life and the growth of the world population indicate that the load of motor neuron disease is rapidly moving toward 400 thousand prevalent cases. The burden is expected to shift toward Asia and Africa in the next decades for the rapid increase of expectation of life of countries with high demographic impact.

show abstract

“…As noted, there is now considerable evidence that the incidence of ALS varies significantly across countries,47 48 and the phenotype and outcome of the disease vary in relation to population ancestral origin 49. South American populations of mixed ancestral origin may have lower rates of ALS compared with those reported in Europe 47 50.…”

Section: Introductionmentioning

confidence: 99%

The changing picture of amyotrophic lateral sclerosis: lessons from European registers

Hardiman

Al‐Chalabi

Brayne

et al. 2017

J Neurol Neurosurg Psychiatry

Self Cite

View full text Add to dashboard Cite

Prospective population based Registers of amyotrophic lateral sclerosis (ALS) have operated in Europe for over two decades, and have provided important insights into our understanding of ALS.Here we review the benefits that population Registers have brought to the understanding of the incidence, prevalence, phenotype and genetics of ALS, and, outline the core operating principles that underlie these Registers and facilitate international collaboration. Going forward we offer lessons learned from our collective experience of operating ALS registers in Europe for over two decades.

show abstract

Clinical and demographic factors and outcome of amyotrophic lateral sclerosis in relation to population ancestral origin

Abstract: This review sets the scene for a collaborative study involving a wide international consortium to investigate, using a standard methodology, the link between ancestry, environment, and ALS phenotype.

Cited by 94 publications

References 124 publications

Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study

Clinical features and prognosis of amyotrophic lateral sclerosis in Africa: the TROPALS study

Amyotrophic Lateral Sclerosis Descriptive Epidemiology: The Origin of Geographic Difference

The changing picture of amyotrophic lateral sclerosis: lessons from European registers

Contact Info

Product

Resources

About