Background: Dementia is becoming a major public health problem in Latin America (LA), yet epidemiological information on dementia remains scarce in this region. This study analyzes data from epidemiological studies on the prevalence of dementia in LA and compares the prevalence of dementia and its causes across countries in LA and attempts to clarify differences from those of developed regions of the world.
ObjectivesThis study compares the clinical characteristics of patients with amyotrophic lateral sclerosis (ALS) within three clinic-based populations from Cuba, Uruguay and Ireland and determines the impact of known ALS-associated genetic variants on phenotypic manifestations within the Cuban population.MethodsDemographic and clinical information was collected on 115 Cuban, 220 Uruguayan and 1038 Irish patients with ALS attending national specialist clinics through 1996–2017. All Cuban patients and 676 Irish patients underwent next-generation DNA sequencing and were screened for the pathogenic C9orf72 repeat expansion.ResultsThe mean age of onset was younger in the Cuban (53.0 years, 95% CI 50.4 to 55.6) and Uruguayan (58.2 years, 95% CI 56.5 to 60.0) populations compared with the Irish population (61.6 years, 95% CI 60.9 to 62.4). No differences in survival between populations were observed. 1.7 % (95% CI 0.6 to 4.1) of Cubans with ALS carried the C9orf72 repeat expansion compared with 9.9% (95% CI 7.8 to 12.0) of Irish patients with ALS (p=0.004). Other known variants identified in the Cuban population included ANG (one patient), CHCHD10 (one patient) and DCTN1 (three patients).Conclusions and relevanceThis study is the first to describe the clinical characteristics of ALS in Cuban and Uruguayan populations and report differences between the Cuban and Irish genetic signature in terms of known ALS-associated genetic variants. These novel clinical and genetic data add to our understanding of ALS across different and understudied populations.
Objective: To determine the incidence and prevalence of amyotrophic lateral sclerosis (ALS) in the Republic of Uruguay.Methods: The study was performed in Uruguay (3,241,003 inhabitants) during a 2-year period (2002–2003). To ensure complete case ascertainment, multiple sources of information were used, including all the neurologists, other medical specialties, general physicians, neurophysiology laboratories, hospital medical records and death certificates. ALS diagnosis was based on El Escorial criteria. Although all patients with motor neuron disease were enrolled in the follow-up, only probable and definite cases are included in the study. Results: Between January 1st, 2002, and December 31st, 2003, 89 new patients were diagnosed with probable or definite ALS. The mean annual incidence rate was 1.37 per 100,000 persons. The incidence was higher for men (1.95) than for women (0.84) with a male to female ratio of 2:1. For both, the incidence increased throughout the years showing a peak in the 65–74 age group among men and the 55–64 age group among women. Mean age at onset of ALS disease was 58.7 years. The estimated mean annual incidence for ALS calculated by the capture recapture method was 1.42 (95% CI, 1.13–1.72). On December 31st, 2002, the crude prevalence was 1.9 per 100,000 inhabitants. Conclusions: ALS incidence is within a narrow range across countries despite the genetic, environmental and socioeconomic differences when similar prospective design, diagnosis criteria and data analyses are applied.
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