Mortality in Friedreich ataxia

AY Tsou, EK Paulsen, SJ Lagedrost, SL Perlman… - Journal of the …, 2011 - Elsevier
Background Although cardiac dysfunction is widely accepted as the most common cause of
mortality in Friedreich ataxia (FRDA), no studies have evaluated this since the advent of
specific clinical and genetic diagnostic criteria. Methods We performed a retrospective study
of FRDA patients to determine cause of death followed by a case–control analysis
comparing characteristics of deceased patients with living, age-and sex-matched FRDA
controls. Results Causes of death were cardiac dysfunction (59%), probable cardiac …