Epidemiological features of amyotrophic lateral sclerosis in a large clinic-based African American population

Kazamel, Mohamed; Cutter, Gary; Claussen, Gwendolyn C.; Alsharabati, Mohammad; Oh, Shin J.; Liang, Lu; King, Peter H.

doi:10.3109/21678421.2013.770030

Cited by 25 publications

(16 citation statements)

References 14 publications

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“…To validate this finding, we tested a large cohort of ALS and control muscle samples by qRT-PCR (Tables 1, 2). The ALS cohort ( n = 27) reflected demographics that matched our overall ALS population, including a mean age of 61 years at diagnosis, and a slight male predominance 23. Medical Research Council (MRC) grades were available for all muscles and ranged from 0 to 5.…”

Section: Resultsmentioning

confidence: 99%

Smads as muscle biomarkers in amyotrophic lateral sclerosis

Cui

Kim

et al. 2014

Ann Clin Transl Neurol

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ObjectiveTo identify molecular signatures in muscle from patients with amyotrophic lateral sclerosis (ALS) that could provide insight into the disease process and serve as biomarkers.MethodsRNA sequencing was performed on ALS and control muscle samples to identify Smad family members as potential markers of disease. Validation studies were performed in a cohort of 27 ALS patients and 33 controls. The markers were assessed in the G93A superoxide dismutase (SOD)1 mouse at different stages of disease and in a model of sciatic nerve injury.ResultsSmad8, and to a lesser extent Smad1 and 5, mRNAs were significantly elevated in human ALS muscle samples. The markers displayed a remarkably similar pattern in the G93A SOD1 mouse model of ALS with increases detected at preclinical stages. Expression at the RNA and protein levels as well as protein activation (phosphorylation) significantly increased with disease progression in the mouse. The markers were also elevated to a lesser degree in gastrocnemius muscle following sciatic nerve injury, but then reverted to baseline during the muscle reinnervation phase.InterpretationThese data indicate that Smad1, 5, 8 mRNA and protein levels, as well as Smad phosphorylation, are elevated in ALS muscle and could potentially serve as markers of disease progression or regression.

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Section: Resultsmentioning

confidence: 99%

Smads as muscle biomarkers in amyotrophic lateral sclerosis

Cui

Kim

et al. 2014

Ann Clin Transl Neurol

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“…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. Interestingly, age at disease onset was also significantly lower in African–Americans than in subjects of European ancestry (55 vs 61 years, p=0.011) in a hospital-based cohort of patients with ALS evaluated in the USA 17. Systematic inclusion of the youngest African patients with ALS cannot be discarded.…”

Section: Discussionmentioning

confidence: 96%

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

Luna

Diagana

Aissa

et al. 2018

J Neurol Neurosurg Psychiatry

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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.

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“…There are limited survival data for persons with ALS in the United States [7, 8, 11, 13] , particularly for cases reported by all neurologists in a defined geographic area who used the El Escorial criteria [14] to determine case eligibility. This report contains the first description of the survival of a state-wide cohort of ALS patients in the United States.…”

Section: Discussionmentioning

confidence: 99%

“…Older age at diagnosis has been found consistently to be a strong predictor of shorter survival [7–9, 11–13] . In this cohort, the median survival time among those aged less than 55 years at diagnosis was more than three times longer than those aged 75 years or older.…”

Section: Discussionmentioning

confidence: 99%

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Effects of Demographic Factors on Survival Time after a Diagnosis of Amyotrophic Lateral Sclerosis

et al. 2015

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Background: The Agency for Toxic Substances and Disease Registry established surveillance projects to determine the incidence, prevalence, and demographic characteristics of persons with Amyotrophic Lateral Sclerosis (ALS) in defined geographic areas. There is a need to characterize and account for the survival and prognostic factors among a population-based cohort of ALS cases in the United States. Methods: A cohort of incident cases diagnosed from 2009-2011 in New Jersey was followed until death or December 31, 2013, whichever happened first. Survival was assessed using Kaplan-Meier curves and Cox proportional hazards regression was used to identify prognostic factors. Results: Sixty-four percent of incident cases died between 2009 and 2013, 93.7% specifically from ALS. Among the 456 cases studied in the survival analysis, the median survival from diagnosis was 21 months; 46% of cases survived longer than two years from diagnosis. Older age predicted shorter survival. While there is some indication of differences because of sex, race, and ethnicity, these differences were not statistically significant when accounting for age. Conclusions: New Jersey mortality data were queried to determine the vital status of a cohort of incident ALS cases and used to investigate relationships between demographic factors and survival. Results are consistent with other population-based studies. Older age was a strong predictor of shorter survival time. Additional follow-up time is needed to characterize longer-term survival.

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Epidemiological features of amyotrophic lateral sclerosis in a large clinic-based African American population

Cited by 25 publications

References 14 publications

Smads as muscle biomarkers in amyotrophic lateral sclerosis

Smads as muscle biomarkers in amyotrophic lateral sclerosis

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

Effects of Demographic Factors on Survival Time after a Diagnosis of Amyotrophic Lateral Sclerosis

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