Anesthetic Approach to Huntington’s Chorea

Abstract

To the editor,
Huntington’s Chorea (HC) is a rare autosomal dominant inherited disease which affects the caudate nucleus and basal ganglia and is
characterised with progressive cellular degeneration [1]. A gene mutation on the short arm of the 4th chromosome is responsible for
this disease. In HC, an increase in huntingtin production, a mutant protein resulting from the expansion of the CAG repeat in the IT15
gene, results in cell loss and atrophy, especially in the GABAergic striatal mid-spinal output neurons of the caudate, putamen and
cortex [1,2]. The prevalence is around 4-6 per 100.000 and it is usually seen between 30-50 years of age. It is characterised by a
triad of personality change, dementia and choreiform movements.Personality change starts ten years before choreiform movements
with depression usually being the first symptom. While motor symptoms, dysphagia and dysarthria are the leading symptoms,
intellectual losses are also frequently observed [3].