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Shikun Zhan, Fafa Sun, Yixin Pan, Wei Liu, Peng Huang, Chunyan Cao, Jing Zhang, Dianyou Li and Bomin Sun

OBJECTIVE

Subthalamic nucleus deep brain stimulation has been shown to be effective in reducing symptoms of primary Meige syndrome. However, assessments of its efficacy and safety have been limited to several case reports and small studies.

METHODS

The authors performed a retrospective study to assess the efficacy and safety of bilateral subthalamic nucleus stimulation in 15 patients with primary Meige syndrome who responded poorly to medical treatments or botulinum toxin injections. Using the movement and disability subscores of the Burke-Fahn-Marsden Dystonia Rating Scale, the authors evaluated the severity of patients’ dystonia and related before surgery and at final follow-up during neurostimulation. The movement scale was assessed based on preoperative and postoperative video documentation by an independent rater who was unaware of each patient’s neurostimulation status. Quality of life was assessed with the Medical Outcomes Study 36-Item Short-Form General Health Survey.

RESULTS

The dystonia movement subscores in 14 consecutive patients improved from 19.3 ± 7.6 (mean ± standard deviation) before surgery to 5.5 ± 4.5 at final follow-up (28.5 ± 16.5 months), with a mean improvement of 74% (p < 0.05). The disability subscore improved from 15.6 ± 4.9 before surgery to 6.1 ± 3.5 at final follow-up (p < 0.05). In addition, the postoperative SF-36 scores increased markedly over those at baseline. The authors also found that bilateral stimulation of the subthalamic nucleus immediately improved patient symptoms after stimulation and required lower stimulation parameters than those needed for pallidal deep brain stimulation for primary Meige syndrome. Four adverse events occurred in 3 patients; all of these events resolved without permanent sequelae.

CONCLUSIONS

These findings provide further evidence to support the long-term efficacy and safety of subthalamic nucleus stimulation as an alternative treatment for patients with medically intractable Meige syndrome.

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Chencheng Zhang, Zhengdao Deng, Yixin Pan, Jing Zhang, Kristina Zeljic, Haiyan Jin, Odin van der Stelt, Hengfen Gong, Shikun Zhan, Dianyou Li and Bomin Sun

OBJECTIVE

A current challenge is finding an effective and safe treatment for severely disabled patients with Tourette’s syndrome (TS) and comorbid psychiatric disorders, in whom conventional treatments have failed. The authors aimed to evaluate the utility of globus pallidus internus deep brain stimulation (GPi-DBS) combined with bilateral anterior capsulotomy in treating these clinically challenging patients.

METHODS

The authors conducted a retrospective review of the clinical history and outcomes of 10 severely disabled patients with treatment-refractory TS and a psychiatric comorbidity, who had undergone GPi-DBS combined with bilateral anterior capsulotomy in their hospital. At the time of surgery, patients presented mainly with obsessive-compulsive disorder and affective disorders. Clinical outcome assessments of tic and psychiatric symptoms, as well as of general adaptive functioning and quality of life, were performed at the time of surgery and at 6, 12, and between 24 and 96 months postsurgery.

RESULTS

After surgery, all patients showed significant progressive improvements in tic and psychiatric symptoms, along with improvements in general adaptive functioning and quality of life. Tic alleviation reached 64% at 12 months and 77% at the last follow-up on the Yale Global Tic Severity Scale. At the final follow-up, patients had functionally recovered and displayed no or only mild tic and psychiatric symptoms. All patients tolerated treatment reasonably well, with no serious side effects.

CONCLUSIONS

GPi-DBS combined with bilateral anterior capsulotomy seems to offer major clinical benefits to severely disabled patients with otherwise treatment-refractory TS and psychiatric comorbidities.

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Yijie Lai, Peng Huang, Chencheng Zhang, Liangyun Hu, ME, Zhengdao Deng, Dianyou Li, Bomin Sun, Wei Liu and Shikun Zhan

OBJECTIVE

Selective peripheral denervation (SPD) is a widely accepted surgery for medically refractory cervical dystonia (CD), but when SPD has failed, the available approaches are limited. The authors investigated the results from a cohort of CD patients treated with unilateral pallidotomy after unsatisfactory SPD.

METHODS

The authors retrospectively analyzed patients with primary CD who underwent unilateral pallidotomy after SPD between April 2007 and August 2019. The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was used to evaluate symptom severity before surgery, 7 days postsurgery, 3 months postsurgery, and at the last follow-up. TWSTRS subscores for disability and pain and the 24-item Craniocervical Dystonia Questionnaire (CDQ-24) were used to assess quality of life.

RESULTS

At a mean final follow-up of 5 years, TWSTRS severity subscores and total scores were significantly improved (n = 12, mean improvement 57.3% and 62.3%, respectively, p = 0.0022 and p = 0.0022), and 8 of 12 patients (66.7%) were characterized as responders (improvement ≥ 25%). Patients with rotation symptoms before pallidotomy showed greater improvement in TWSTRS severity subscores than those who did not (p = 0.049). The most common adverse event was mild upper-limb weakness (n = 3). Patients’ quality of life was also improved.

CONCLUSIONS

Unilateral pallidotomy seems to offer an effective and safe option for patients with CD who have otherwise experienced limited benefits from SPD.