SINGLE CASE STUDY. Kluver-Bucy Syndrome in Huntingtonʼs Chorea

Abstract

In this paper the first case of Kluver-Bucy syndrome (KBS) in Huntington's chorea is reported. The patient, a 46-year-old man with advanced Huntington's disease, displayed prosopagnosia, oral tendencies, emotional changes, hypersexual behavior, and hyperphagia associated with severe dementia. Haloperidol in moderate doses controlled both the KBS and the chorea, suggesting a possible role for the dopaminergic system in the pathogenesis of KBS in Huntington's disease. The presence of profound dementia in our patient supports the previous assertion that human cases of KBS are invariably associated with severe cognitive dysfunction. Since KBS was established as an entity, a great deal of attention has been directed to its neuroanatomical basis. However, due to the multidetermined nature of human behavior, the role of physiological, psychological, and environmental factors should also be taken into consideration with regard to the pathogenesis of this syndrome.