AGE-OF-ONSET, SEX, AND CARDIOMYOPATHY AS PREDICTORS OF DISABILITY ANDSURVIVAL IN FRIEDREICHS-DISEASE - A RETROSPECTIVE STUDY ON 119 PATIENTS

We performed a retrospective study on a series of 119 of our patients who have Friedreich's disease to assess the predictive value of age at onset, gender, and left ventricular hypertrophy in regard to disease progression. Outcome variables were survival, time to loss of independ ent gait, and time to confinement in a wheelchair. Diabetes was consid ered to be an outcome variable when defining time to diabetes and an e xplanatory variable when testing its effect on survival. Eleven patien ts died. The median estimated survival from onset was 36 years, and th e median time to loss of independent gait was 8 years and to confineme nt in a wheelchair was 15 years from onset. Nineteen patients develope d diabetes after a median time of 16 years. The presence of left ventr icular hypertrophy or diabetes significantly reduced survival based on univariate analysis. Onset at the age of less than or equal to 20 yea rs and the presence of left ventricular hypertrophy predicted a faster rate of progression of the disease.