Patterns of mortality from hereditary ataxias in the United States, 1971 and 1973-1978

Mortality rates for deaths 'due to' and 'with' hereditary ataxias are presented for the first time. Age-adjusted mortality rates were higher for whites than for nonwhites of either sex, and for men in both racial groups. Age-specific mortality rates for whites showed a peak for 'hereditary spinal ataxia' in the 20-29 age group. For the other types of ataxias they were low until age 40 and increased thereafter. Heart diseases were a frequent cause of death in people dying 'with' hereditary ataxias. The median survival (from birth) for hereditary spinal ataxia in whites was 35 years.