HUNTINGTONS-DISEASE - PATHOGENESIS, DIAGNOSIS AND TREATMENT

This review of the clinical features of Huntington's disease incorpora tes recent developments in pathophysiology, preclinical diagnosis and treatment. Although the mechanism initiating and guiding the cell dest ruction in this illness is currently unknown, the excitatory neurotoxi n and the energy metabolism models may provide a valuable direction fo r future research. Similarly, although the precise relation between th e neuroanatomical damage in Huntington's disease and the functional di sability is not clear, applications of recently developed neural conne ction models have implicated a number of important brain-behavior asso ciations. Preclinical diagnostic procedures have evolved through succe ssive iterations that have each contributed to increased reliability. New functional brain imaging techniques are sure to add to this promis ing domain in the future. Preclinical diagnosis has been stimulated by the recent isolation of the Huntington's gene which has also rekindle d awareness of the importance of informed genetic counselling and the inherent ethical dilemmas in genetic testing. Treatment approaches to Huntington's disease have have been confined to palliative care with s econdary symptom management and psychotherapeutic support. Experimenta l therapeutic strategies for the illness itself have had a rather disa ppointing record to date. Further developments in NMDA antagonism and neural cell grafting may provide some hope for the future.