AAN 2014

Estimate Huntington Disease Prevalence in Latin America

Emilia Gatto, Virginia Laura Parisi, Ana Sanguinetti, Gabriel Persi and Jose Luis Etcheverry

Objective: To analyze the mean of CAG repeats in control individuals from different countries of Latin America as an indirect estimation of prevalence of Huntington disease (HD) in this populationBackground: Huntington´s disease (HD) is a devastating neurodegenerative disorder, with a prevalence of 3-10 per 100,000 people in individuals with European ancestry as well as 0.11-0.45 per 100,000 people in non-European descendants. Studies involving Central or South America are scarce and limited to specific “foci” (vg. Maracaibo in Venezuela; Cañete in Peru). It has been suggested that in populations with low prevalence rates of HD, CAG repeat lengths are smaller than in Western countries.Design/Methods: Using a highly sensitive search strategy (Databases: PubMed, LILACS and SciELO) we identified epidemiological or descriptive studies, involving HD in Latin America (classical studies from Maracaibo, Venezuela or Cañete Valley in Peru were excluded).Results: The mean CAG-repeat length in normal HD genes was 19.04 in unaffected individuals from Mexico, 18.3 in Cuba, 17.9 in African -Brazilian population, 17.7 in Brazilian-Caucasoids and 17.82 in Argentina.The comparison of these results lead us to hypothesize that probably Mexican and Cuban populations, with a high prevalence of mestizo population, could show a higher HD prevalence with respect to Brazil or Argentina, where the CAG length appears similar to that from Western European countries.Conclusions: This is the first reported study comparing CAG size of HD gene from different countries of Latin America, as an indirect estimation of its prevalence.

1. Neurology April 8, 2014 vol. 82 no. 10 Supplement P4.062