Compactotomy in Huntington's chorea

• Published in: Medical hypotheses 2001-10, Vol.57 (4), p.491-496
• Main Authors: Bonelli, R.M., Kenner, L., Gruber, A., Reisecker, F., Költringer, P.
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Humans; Huntington Disease - physiopathology; Huntington Disease - surgery; Medical sciences; Neurology; Substantia Nigra - physiopathology; Substantia Nigra - surgery
• ISSN: 0306-9877; 1532-2777
• DOI: 10.1054/mehy.2001.1372

Advances in neuroradiological and neurosurgical techniques have lead to a growing interest in functional neurosurgical interventions for medically intractable movement disorders. The majority of these procedures are performed in patients with hypokinetic movement disorders, especially Parkinson's disease. However, relatively few interventions were done in hyperkinetic disorders such as Huntington's disease (HD), mainly owing to the lack of an adequate target nucleus. We have recently described the case of a reversible chorea in a genetically confirmed HD patient. We subsequently identified a marked bilateral degeneration of the substantia nigra as the probable reason for choreatic cessation. We therefore suggest that primary striatal atrophy causing hyperkinesia and secondary substantia nigra atrophy favouring hypokinesia were balanced in this patient, thus resulting in a close-to-physiologic GABAergic basal ganglia output. We postulate that deep brain stimulation of the substantia nigra pars compacta may ameliorate hyperkinesia in choreatic movement disorders, thus representing the first effective therapy in Huntington's chorea. Several lines of evidence in recent neurophysiological research support our hypothesis and are discussed below.