Objective: To analyze long-term results of pallidal (GPi) deep brain stimulation (DBS) in patients with Huntington’s disease (HD).

Background: In HD, DBS is suggested to treat debilitating pharmacoresistant motor symptoms. Although world experience is limited to small patient samples, GPi stimulation usually shows reduction in chorea severity. Its effect on dystonia, bradykinesia, gait, balance, speech, and functional state is contradictory [1-7].

Method: 4 HD patients with disabling motor symptoms were included in the study and underwent bilateral DBS GPi. In 3 patients, chorea was predominant (age of onset 34.7±3.8 years). The fourth patient suffered severe generalized dystonia (foremost truncal and cervical), which manifested as writer’s cramp at the age of 9 without chorea signs. Number of trinucleotide CAG repeats in HTT gene ranged from 45 to 47. Age at surgery was 37.5±11.4 years. Patients were assessed using Unified Huntington’s disease scale (UHDRS) with separate analysis for chorea, dystonia, bradykinesia, gait, and balance. Burke-Fahn-Marsden scale was used for dystonia. Neuropsychological and psychiatric examination were performed. Postoperative follow-up comprised 3.8±2.1 (1 to 6 years).

Results: Following DBS GPi, decrease in severity of motor symptoms according to UHDRS was observed. 3 patients demonstrated significant alleviation of chorea in short and long-term follow-up. The forth patient had prominent stable dystonia reduction up to 6 years after surgery, in absence of chorea manifestation. Most common stimulation-induced side effects were gait and speech impairment (3 patients), which could be partially ameliorated by reprogramming (lower frequency, shorter pulse duration, or bipolar mode). All patients suffered mental problems, including apathy, depression, eating disorders, and obsessive thoughts. In the long term, patient functional capacity decreased that was associated with progression of cognitive, postural, and psychiatric disorders. 75% of patients were dependent on constant caregiver support.

Conclusion: DBS GPi is effective for treatment disabling chorea and dystonia in Huntington’s disease. However, in long-term follow-up, patients retain disability caused by other motor and non-motor symptoms, which aggravate with disease progression. Additional gait and speech impairment on DBS GPi affects daily activity. Thus, possibility of DBS to improve functional state of HD patients remains debatable.

References: 1. Yin Z, Bai Y, Zhang H, Liu H, Hu W, Meng F, Yang A, Zhang J. An individual patient analysis of the efficacy of using GPi-DBS to treat Huntington’s disease. Brain Stimul. 2020;13(6):1722-1731. doi: 10.1016/j.brs.2020.09.025
2. Bonomo R, Elia AE, Bonomo G, Romito LM, Mariotti C, Devigili G, Cilia R, Giossi R, Eleopra R. Deep brain stimulation in Huntington’s disease: a literature review. Neurol Sci. 2021;42(11):4447-4457. doi: 10.1007/s10072-021-05527-1
3. Sanrey E, Macioce V, Gonzalez V, Cif L, Cyprien F, Chan Seng E, Coubes P, Poulen G. Does pallidal neuromodulation influence cognitive decline in Huntington’s disease? J Neurol. 2021;268(2):613-622. doi: 10.1007/s00415-020-10206-w
4. Kaczyńska J, Sitek EJ, Witkowski G, Rudzińska-Bar M, Janik P, Sławek J, Edwin EMG, Zielonka D. Is deep brain stimulation effective in Huntington’s Disease? – a systematic literature review. Neurol Neurochir Pol. 2022;56(4):299-307. doi: 10.5603/PJNNS.a2022.0050
5. Ferreira JJ, Rodrigues FB, Duarte GS, Mestre TA, Bachoud-Levi AC, Bentivoglio AR, Burgunder JM, Cardoso F, Claassen DO, Landwehrmeyer GB, Kulisevsky J, Nirenberg MJ, Rosser A, Roth J, Seppi K, Slawek J, Furr-Stimming E, Tabrizi SJ, Walker FO, Vandenberghe W, Costa J, Sampaio C. An MDS Evidence-Based Review on Treatments for Huntington’s Disease. Mov Disord. 2022;37(1):25-35. doi: 10.1002/mds.28855
6. Gonzalez-Baez Ardisana P, Solís-Mata JS, Carrillo-Ruiz JD. Neurosurgical therapy possibilities in treatment of Huntington disease: An update. Parkinsonism Relat Disord. 2024;125:107048. doi: 10.1016/j.parkreldis.2024.107048
7. Steinhardt J, Zittel S, Tadic V, Tronnier V, Moll C, Bäumer T, Münchau A, Rasche D, Brüggemann N. GPi/GPe borderland- a potential sweet spot for deep brain stimulation for chorea in Huntington’s disease? Neurol Res Pract. 2024;6(1):28. doi: 10.1186/s42466-024-00316-5

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MDS Abstracts - https://www.mdsabstracts.org/abstract/long-term-results-of-pallidal-deep-brain-stimulation-in-patients-with-huntingtons-disease/