Deep brain stimulation versus pallidotomy for status dystonicus: a single-center case series

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OBJECTIVE

First-line pharmacological therapies have shown limited efficacy in status dystonicus (SD), while surgery is increasingly reported as remediable in refractory cases. In this context, there is no evidence regarding which neurosurgical approach is the safest and most effective. The aim of this study was to assess the clinical outcomes and surgery-related complications of globus pallidus internus deep brain stimulation (GPi DBS) and pallidotomy for the treatment of drug-resistant SD.

METHODS

The authors reviewed the records of patients with drug-resistant SD who had undergone GPi DBS or pallidotomy at their institution between 2003 and 2017. The severity of the dystonia was evaluated using the Barry-Albright Dystonia (BAD) Scale. Surgical procedures were performed bilaterally in all cases.

RESULTS

Fourteen patients were eligible for inclusion in the study. After surgery, the mean follow-up was 40.6 ± 30 months. DBS ended the dystonic storm in 87.5% of cases (7/8), while pallidotomy had a success rate of 83.3% (5/6). No significant differences were observed between the two techniques in terms of failure rates (risk difference DBS vs pallidotomy −0.03, 95% CI −0.36 to 0.30), SD mean resolution time (DBS 34.8 ± 19 days, pallidotomy 21.8 ± 20.2 days, p > 0.05), or BAD scores at each postoperative follow-up (p > 0.05). The long-term hardware complication rate after DBS was 37.5%, whereas no surgery-related complications were noted following pallidotomy.

CONCLUSIONS

The study data suggest that DBS and pallidotomy are equally safe and effective therapies for drug-resistant SD. The choice between the two techniques should be tailored on a case-by-case basis, depending on factors such as the etiology and evolution pattern of the underlying dystonia and the clinical conditions at the moment of SD onset. Given the limitation of the low statistical power of this study, further multicentric investigations are needed to confirm its findings.

ABBREVIATIONS AC-PC = anterior commissure–posterior commissure; BAD = Barry-Albright Dystonia; DBS = deep brain stimulation; GPi = globus pallidus internus; ITB = intrathecal baclofen; MNI = Montreal Neurological Institute; SD = status dystonicus; SP = staged pallidotomy.
Article Information

Contributor Notes

Correspondence Vincenzo Levi: Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. vincenzo.levi@istituto-besta.it.INCLUDE WHEN CITING Published online December 20, 2019; DOI: 10.3171/2019.10.JNS191691.

V.L. and G.Z. share first authorship, and N.N. and A.F. share senior authorship.

Disclosures Dr. Levi is a medical advisor at Newronika S.R.L. (neurostimulator manufacturer), but this activity is not relevant to the paper.
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