Deep brain stimulation and stereotactic-assisted brain graft injection targeting fronto-striatal circuits for Huntington's disease: an update. (2nd September 2022)

• Record Type: Journal Article
• Authors: Kinfe, Thomas; Del Vecchio, Alessandro; Nüssel, Martin; Zhao, Yining; Stadlbauer, Andreas; Buchfelder, Michael
• Is Part Of: Expert review of neurotherapeutics. Volume 22:Number 9(2022)
• Page Start: 781
• Page End: 788
• Publication Date: 2022-09-02
• Subjects: Huntington´s disease -- fronto-striatal circuits -- chorea -- parkinsonism -- dystonia -- globus pallidus externus/internus -- deep-brain stimulation -- striatum -- brain grafts -- unified Huntington´s disease rating scale (UHDRS) -- chorea -- safety -- efficacy -- quality of life; Neuropharmacology -- Periodicals; 615.7805
• DOI: 10.1080/14737175.2022.2091988
• Languages: English
• ISSNs: 1473-7175

Abstract

ABSTRACT: Introduction: Huntington's Disease as progressive neurological disorders associated with motor, behavioral, and cognitive impairment poses a therapeutic challenge in case of limited responsiveness to established therapeutics. Pallidal deep brain stimulation and neurorestorative strategies (brain grafts) scoping to modulate fronto-striatal circuits have gained increased recognition for the treatment of refractory Huntington's disease (HD). Areas covered: A review (2000–2022) was performed in PubMed, Embase, and Cochrane Library covering clinical trials conceptualized to determine the efficacy and safety of invasive, stereotactic-guided deep-brain stimulation and intracranial brain-graft injection targeting the globus pallidus and adjunct structures (striatum). Expert opinion: Stereotactic brain-grafting strategies were performed in few HD patients with inconsistent findings and mild-to-moderate clinical responsiveness with a recently published large, randomized-controlled trial (NCT 00190450) yielding negative results. We identified 19 in-human DBS trials (uncontrolled) targeting the globus pallidus internus/externus along with randomized-controlled trial pending report (NCT 02535884). We did not detect any significant changes in the UHDRS total score after restorative injections, while in contrast, the use of deep-brain stimulation resulted in a significant reduction of chorea. GPi-DBS should be considered in cases where selective chorea is present. However, both invasive therapies remain experimental and are not ready for the implementation in clinical use.