Quadruple deep brain stimulation in Huntington’s disease, targeting pallidum and subthalamic nucleus: case report and review of the literature

• Published in: Journal of Neural Transmission Vol. 121; no. 10; pp. 1303 - 1312
• Main Authors: Gruber, D., Kuhn, A. A., Schoenecker, T., Kopp, U. A., Kivi, A., Huebl, J., Lobsien, E., Mueller, B., Schneider, G.-H., Kupsch, A.
• Format: Journal Article
• Language: English
• Published: Vienna Springer Vienna 01.10.2014; Springer Nature B.V
• Subjects: Adult; Deep Brain Stimulation - adverse effects; Deep Brain Stimulation - methods; Globus Pallidus - pathology; Globus Pallidus - physiopathology; Humans; Huntington Disease - pathology; Huntington Disease - physiopathology; Huntington Disease - psychology; Huntington Disease - therapy; Magnetic Resonance Imaging; Male; Medicine; Medicine & Public Health; Motor Activity - physiology; Neurology; Neurology and Preclinical Neurological Studies - Original Article; Neurosciences; Psychiatry; Subthalamic Nucleus - pathology; Subthalamic Nucleus - physiopathology; Treatment Outcome; Globus pallidus; Subthalamic nucleus; Deep brain stimulation; Chorea; Huntington disease
• ISSN: 0300-9564; 1435-1463
• DOI: 10.1007/s00702-014-1201-7

Deep brain stimulation (DBS) represents an established treatment option in a growing number of movement disorders. Recent case reports suggest beneficial effect of globus pallidus internus (GPi)-DBS in selected patients suffering from Huntington’s disease with marked disabling chorea. We present a 41-year-old man with genetically confirmed HD following quadruple GPi- and subthalamic nucleus (STN)-DBS. Motor function was assessed by Abnormal Involuntary Movement Scale (AIMS) and by Unified Huntington Disease Rating Scale (UHDRS) presurgery and postsurgery for up to 4 years. Furthermore, cognitive, neuropsychiatric state and quality of life (QoL) including life satisfaction (QLS) were annually evaluated. Chorea assessed by AIMS and UHDRS subscores improved by 52 and 55 %, 45 and 60 %, 35 and 45 % and 55–66 % at 1–4 years, respectively, compared to presurgical state following GPi–STN-DBS. During these time periods bradykinesia did not increase following separate STN- and combined GPi–STN-DBS compared to presurgical state. Mood, QoL and QLS were ameliorated. However, dysexecutive symptoms increased at 4 years postsurgery. The present case report suggests that bilateral GPi- and STN-DBS may represent a new treatment avenue in selected HD patients. Clinically, GPi-DBS attenuated chorea and was associated with a larger effect–adverse effect window compared to STN-DBS. However, GPi-DBS-induced bradykinesia may emerge as one main limitation of GPi-DBS in HD. Thus, quadruple GPi–STN-DBS may be indicated, if separate GPi-DBS does not result in sufficient control of motor symptoms. Future controlled studies need to confirm if the present anecdotal observation of additive beneficial effects of GPi- and STN-DBS in a HD patient with severe generalized chorea and relatively intact cognitive and affective functions indeed represents a new therapeutic option.