We report on a patient suffering from epilepsy and severe personality
changes. Huntington disease was diagnosed by molecular-biological Inve
stigation. Clinical characteristics are discussed on the basis of mode
rn concepts of the genetic mechanism. Neuroradiological investigation
revealed marked cerebellar atrophy, while typical findings of Huntingt
on disease, such as caudate nucleus volume loss, were lacking. The cer
ebellar atrophy could be attributable either to long-term phenytoin-me
dication or to the pathological process itself.