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The aim of this article was to conduct a retrospective observational study on reported deaths due to Huntington's disease (HD) in Brazil in the past 25 years (from 1984 to 2008). Data were obtained from the Brazilian Mortality Information System (SIM/DATASUS), the official system of Brazilian Mortality Database. The data obtained included information regarding the gender of the deceased and the number of death notifications, which we stratified by demographic regions and states. HD mortality per 100,000 was calculated and plotted in a graph. Linear regression was calculated using ordinary least square technique. We observed that the mortality due to HD recorded by SIM/DATASUS from 1984 to 2008 had increased at much higher rates than the population in the same period. Also, some Brazilian regions still show very low rates of HD mortality compared to the national average of deaths due to HD. These findings suggest that HD mortality has been underestimated. Ignorance about the disease as well as the fact that death from HD can occur as a consequence of heart disease, pneumonia, or suicide can strongly contribute to the misguided notification of HD as the cause of death in the official reports.

Notification of Huntington’s disease as primary cause of death in Brazil from 1984 to 2008