Early therapeutic success in Huntington's chorea: with a commentary on a localised community in the Highlands of Scotland

Abstract

Lack of insight often allows patients with Huntington's Chorea to escape from that mental anguish which might be expected if they could foresee their future. Life would be intolerable if they realised that a comparatively early death offered the only release from years of progressive chorea during which they would slowly develop a dementia and probably a psychosis. The suffering often falls instead to the families who are responsible for the care of these pathetic and distressing patients.

The hereditary nature of the disease intensifies the burden of anxiety for blood relations who must often wonder if they will develop the condition themselves or if they will pass it on to their children, which is worse.

Two responsibilities lie heavily on the medical advisors who find such a community in their midst: firstly, the accuracy of diagnosis necessary for the correct application of eugenics requires very painstaking scrutiny of family histories to establish the lines of inheritance which may be halted or on the other hand to remove a burden from the shoulders of the families which are proved to be untainted. Secondly, in treatment, endeavour must continue on a basis of prevention, cure and symptomatic relief. Any measure of success being a seed of hope in such a malady, where the prognosis is always regarded as hopeless and treatment of no avail.

The ensuing pages will demonstrate how these problems have been met in Ross -shire and will indicate the shape of the therapeutic seed which may flourish in the fertile future of medical advance and bloom into a real and permanent cure.

Huntington's Chorea has been defined by Russell Brain as a hereditary disorder characterized pathologically by degeneration of the ganglion cells of the fore brain and corpus striatum, and clinically by choreiform movements and progressive dementia, which usually begins in early middle life. The course is said to be progressive, terminating in death. No form of treatment is offered other than institutional care. I propose to show in this paper a marked improvement in such sufferers, sufficient in many instances to prevent the need for certification.