Prognosis and epidemiology of amyotrophic lateral sclerosis

Traxinger, Kim; Kelly, Crystal; Johnson, Brent A.; Lyles, Robert H.; Glass, Jonathan D.

doi:10.1212/cpj.0b013e3182a1b8ab

Cited by 119 publications

(89 citation statements)

References 18 publications

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“…In fact, we did not find an association between prediagnosis BMI and ALS survival; only BMI at diagnosis and BMI change between age 40 years and diagnosis were associated with ALS survival. Other studies support the hypothesis that BMI change immediately before ALS diagnosis is both a common feature of ALS and a prognostic factor, whereas evidence that BMI at ALS diagnosis is a prognostic factor is inconsistent, possibly due to small sample sizes in some studies (12,(25)(26)(27).…”

Section: Discussionmentioning

confidence: 98%

Body Mass Index and Amyotrophic Lateral Sclerosis: A Study of US Military Veterans

Mariosa¹,

Beard²,

Umbach³

et al. 2017

American Journal of Epidemiology

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Amyotrophic lateral sclerosis (ALS) may be associated with low body mass index (BMI) at the time of diagnosis. However, the role of premorbid BMI in the development of ALS and survival after diagnosis remains unclear. In 2005-2010, we interviewed 467 patients with ALS from the US National Registry of Veterans with ALS and 975 frequency-matched veteran controls. In this sample, we evaluated the association of BMI and BMI change at different ages with ALS risk using unconditional logistic models and with survival after ALS diagnosis using Cox proportional hazards models. After adjustment for confounders, compared with a moderate increase in BMI between ages 25 and 40 years, stable or decreasing BMI was positively associated with ALS risk (odds ratio (OR) = 1.61, 95% confidence interval (CI): 1.20, 2.16). A 1-unit increase in BMI at age 40 years (OR = 0.95, 95% CI: 0.91, 0.98) but not at age 25 years (OR = 0.99, 95% CI: 0.95, 1.03) was inversely associated with ALS. These associations were similar for bulbar and spinal ALS but stronger for those with a delay of less than 1 year between symptom onset and diagnosis. We found no association between prediagnosis BMI and survival. A decreasing BMI from early to middle age and a low BMI in middle age may be positively associated with ALS risk.

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Section: Discussionmentioning

confidence: 98%

Body Mass Index and Amyotrophic Lateral Sclerosis: A Study of US Military Veterans

Mariosa¹,

Beard²,

Umbach³

et al. 2017

American Journal of Epidemiology

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“…In comparison, other countries report a median survival from onset to death ranging from 20 to 48 months, with 10-20% of patients surviving more than 10 years from diagnosis [1]. Median survival times measured from ALS symptom onset are approximately 1 year longer than from ALS diagnosis [17,20,21,22,23,24]. Older age (≥40 years) of onset and bulbar (compared to limb) onset are associated with poorer prognosis [1,24].…”

Section: Discussionmentioning

confidence: 99%

Epidemiology of Amyotrophic Lateral Sclerosis: A Population-Based Study in Israel

et al. 2016

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Background: Globally, the annual incidence and prevalence of amyotrophic lateral sclerosis (ALS) are estimated at 1.9 and 4.5 per 100,000 population, respectively. This study is aimed at describing the epidemiology of ALS in Israel in a real-world setting. Methods: A retrospective study was performed using the databases of Maccabi Healthcare Services (MHS), a 2-million-member health maintenance organization in Israel. The study included all MHS adults diagnosed with ALS between 1997 and 2013. In 2013, characteristics of ALS patients were compared to those of age-sex-matched patients without ALS. Survival after ALS diagnosis was assessed until death and until tracheostomy or death (follow-up through 2014). Results: In 2013 (n = 158), the prevalence of ALS was 8.1 per 100,000 population in MHS. In 1997-2013, a total of 375 ALS patients were diagnosed, corresponding to an average annual incidence of 1.8 per 100,000 population in MHS. The median survival from diagnosis to death was 3.5 years (95% CI 2.9-4.1), with approximately 28% surviving at least 10 years. Median tracheostomy-free survival was 2.5 years (95% CI 2.1-2.9). Conclusions: Results suggest that there is a relatively high prevalence of ALS in Israel. Further research is needed to investigate factors that may contribute to the survival of patients with ALS in Israel.

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“…The majority of ALS cases are sporadic and are phenotypically indistinguishable from the 5-10% of cases that are classified as familial [1]. ALS is universally fatal, typically within 3-5 years of symptom onset [2]. …”

Section: Introductionmentioning

confidence: 99%

Epidemiology of Amyotrophic Lateral Sclerosis in the Republic of Cyprus: A 25-Year Retrospective Study

Demetriou

Hadjivasiliou

Kleopa³

et al. 2017

Neuroepidemiology

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Introduction: Amyotrophic lateral sclerosis (ALS) is a rare, rapidly progressive neurodegenerative disease. Despite wide variability in the incidence and prevalence of ALS, there is evidence of positive temporal trends and an increase in incidence with age. The aim of this study was to conduct a detailed epidemiological investigation of ALS in Cyprus. Methods: All registered Cypriot ALS patients in the Republic of Cyprus from January 1985 until December 2014 were included. Socio-demographic information was extracted from patient files. Results: The study identified 179 ALS patients, of whom 7 had a positive family history. The mean age at onset was 58.6 years and a slight male predominance was observed. Average annual crude incidence was 1.26 cases/100,000 person-years and at the beginning of 2015, prevalence of ALS was 7.9 cases/100,000 population. Both incidence and prevalence displayed an increasing trend, even after age-standardization of incidence rates. Conclusions: Incidence, prevalence and main socio-demographic characteristics of ALS in Cyprus were similar to those of other European countries, without any geographic clustering of the disease. Additionally, an increased incidence through the years was confirmed. However, observations such as a higher male prevalence and a younger mean age of onset compared to published literature require further investigation.

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Prognosis and epidemiology of amyotrophic lateral sclerosis

Cited by 119 publications

References 18 publications

Body Mass Index and Amyotrophic Lateral Sclerosis: A Study of US Military Veterans

Body Mass Index and Amyotrophic Lateral Sclerosis: A Study of US Military Veterans

Epidemiology of Amyotrophic Lateral Sclerosis: A Population-Based Study in Israel

Epidemiology of Amyotrophic Lateral Sclerosis in the Republic of Cyprus: A 25-Year Retrospective Study

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