REDUCED LIFE EXPECTANCY IN 40 CASES OF EARLY-ONSET CEREBELLAR-ATAXIA WITH RETAINED TENDON REFLEXES - A POPULATION-BASED STUDY

A survival analysis of 40 cases of early onset cerebellar ataxia (EOCA ) with retained tendon reflexes was performed. They represent all case s of EOCA diagnosed between 1945 and 1990 among residents of a defined area of Northwestern Italy, followed up to December 31, 1990. The sur vival rates were respectively 92%, 87% and 77% at 10, 20 and 30-years, worse than expected in a disease which is usually considered benign. The relative death rate was 4 times higher than expected for the gener al population. Prognosis was significantly worse for males than for fe males, whereas the age of onset and the calendar year of onset did not affect survival.