Longer CAG repeat length is associated with shorter survival after disease onset in Huntington disease.

ABSTRACT: It is well known that the length of the CAG trinucleotide expansion of the huntingtin gene is associated with many aspects of Huntington disease progression. These include age of clinical onset and rate of initial progression of disease severity. The relationship between CAG length and survival in Huntington disease is less studied. To address this, we obtained the complete Registry HD database from the European Huntington Disease Network and reanalyzed the time from reported age of disease onset until death. We conducted semiparametric proportional hazards modeling of 8,422 participants who had experienced onset of clinical Huntington disease, either retrospectively or prospectively. Of these, 826 had a recorded age of death. To avoid biased model estimates, retrospective onset ages were

SUBMITTER: Langbehn DR 

PROVIDER: S-EPMC8764269 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature