Advancements in surgical treatments for Huntington disease: From pallidotomy to experimental therapies.

Huntington disease (HD) is an autosomal dominant neurodegenerative disorder characterized by choreic movements, behavioral changes, and cognitive impairment. The pathogenesis of this process is a consequence of mutant protein toxicity in striatal and cortical neurons. Thus far, neurosurgical management of HD has largely been limited to symptomatic relief of motor symptoms using ablative and stimulation techniques. These interventions, however, do not modify the progressive course of the disease. More recently, disease-modifying experimental therapeutic strategies have emerged targeting intrastriatal infusion of neurotrophic factors, cell transplantation, HTT gene silencing, and delivery of intrabodies. Herein we review therapies requiring neurosurgical intervention, including those targeting symptom management and more recent disease-modifying agents, with a focus on safety, efficacy, and surgical considerations.

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Crown Copyright © 2024. Published by Elsevier Inc. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Shervin Rahimpour reports a relationship with Abbott that includes: consulting or advisory and speaking and lecture fees. Shervin Rahimpour reports a relationship with Boston Scientific Corporation that includes: consulting or advisory and speaking and lecture fees. Andres M. Lozano reports a relationship with Functional Neuromodulation that includes: board membership. Andres M. Lozano reports a relationship with Medtronic Inc that includes: consulting or advisory. Andres M. Lozano reports a relationship with Abbott that includes: consulting or advisory. Andres M. Lozano reports a relationship with Boston Scientific Corporation that includes: consulting or advisory. Andres M. Lozano reports a relationship with Insightec that includes: consulting or advisory. Andres M. Lozano reports a relationship with Focused Ultrasound Foundation that includes: consulting or advisory. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.