Compactotomy in Huntington's chorea

Advances in neuroradiological and neurosurgical techniques have lead to a g rowing interest in functional neurosurgical interventions for medically int ractable movement disorders. The majority of these procedures are performed in patients with hypokinetic movement disorders, especially Parkinson's di sease. However, relatively few interventions were done in hyperkinetic diso rders such as Huntington's disease (HD), mainly owing to the lack of an ade quate target nucleus. We have recently described the case of a reversible c horea in a genetically confirmed HD patient. We subsequently identified a m arked bilateral degeneration of the substantia nigra as the probable reason for choreatic cessation. We therefore suggest that primary striatal atroph y causing hyperkinesia and secondary substantia nigra atrophy favouring hyp okinesia were balanced in this patient, thus resulting in a close-to-physio logic GABAergic basal ganglia output. We postulate that deep brain stimulat ion of the substantia nigra pars compacta may ameliorate hyperkinesia in ch oreatic, movement disorders, thus representing the first effective therapy in Huntington's chorea. Several lines of evidence in recent neurophysiologi cal research support our hypothesis and are discussed below. (C) 2001 Harco urt Publishers Ltd.