Quadruple deep brain stimulation in Huntington’s disease, targeting pallidum and subthalamic nucleus: case report and review of the literature
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- Gruber, D., Kuhn, A.A., Schoenecker, T. et al. J Neural Transm (2014) 121: 1303. 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.
KeywordsHuntington disease Deep brain stimulation Globus pallidus Subthalamic nucleus Chorea
Part I: Presurgical clip with the patient (HD) in resting and sitting position; gait and postural stability are separately assessed. Part II-IV: One–3 years postsurgery following GPi–STN-DBS in resting and sitting position; gait and postural stability are separately assessed. Note that bradykinesia increased in comparison to preoperative state. Part V: 4 years postsurgery a) GPi–STN-DBS OFF in resting, sitting and lying position, HD patient is unable to stand; b) GPi–STN-DBS ON in resting and sitting position; gait and postural stability are separately assessed. Note that bradykinesia increased in comparison to preoperative state. (M4V 16915 kb)