An individual patient analysis of the efficacy of using GPi-DBS to treat Huntington's disease.

OBJECTIVE

The efficacy of globus pallidus internus-deep brain stimulation (GPi-DBS) for the treatment of Huntington's disease (HD) has not been validated in large-scale studies. We conducted an individual patient analysis to pool outcomes of all of the published HD-GPi-DBS studies.

METHODS

PubMed, Embase and the Cochrane Library were searched for relevant articles. The Unified Huntington's Disease Rating Scale (UHDRS)-motor and UHDRS-chorea improvements were analyzed during different follow-up periods. Secondary outcomes, including UHDRS-motor subitem scores and functional assessment results, were also analyzed. Correlation and regression analyses were conducted to find improvement predictors. This study was registered in PROSPERO (CRD42018105995).

RESULTS

Eighteen studies including 39 patients with 124 visits were analyzed. GPi-DBS significantly improved the UHDRS-motor score in <3 months (p = 0.001), 3-9 months (p < 0.001), and 9-12 months (p < 0.001), but did not continue in later follow-ups. UHDRS-chorea was significantly improved even in the >30-month follow-up (p = 0.003). Functional assessment was not improved 12 months postoperatively (p = 0.196). The Westphal variant of HD (W-HD) gained no motor benefits 6 months postoperatively (p = 0.178). The Westphal variant was the only risk factor for DBS efficacy (p = 0.044). The rate of stimulation-related adverse events was 87.2%.

CONCLUSIONS

GPi-DBS has a stable effect on chorea symptoms in HD patients. Chorea-dominant patients may be the best candidates for surgery, while attention should be paid to postoperative stimulation-related complications. Given that GPi-DBS has limited effects on other motor symptoms, W-HD patients are not surgical candidates.