Causes of death in Huntington disease as reported on death certificates

Huntington disease (HD) is a late onset autosomal dominant neurological disorder. Two hundred fifty-three death certificates of HD-affected individuals from four midwestern states were examined to determine the completeness of reporting HD on the death certificates. Overall, 66% of death certificates indicated HD as a primary or contributory cause of death. There was significantly better reporting on more recent death certificates, but even since 1979, 16% did not report HD. The implications to those researching HD family histories and to the accuracy of mortality rates are discussed.