Objective: Globus Pallidus internus (GPi) deep brain stimulation (DBS) is established in treatment of dystonia. We evaluated safety and efficacy of bilateral GPi stimulation in a patient with severe, mobile, generalized dystonia due to Wilson’s disease (WD).

Background: Copper accumulation and toxicity in WD affects multiple organs, e.g. the central nervous system, with subsequent risk of developing secondary dystonia. Medical treatment of secondary dystonia and tremor in WD can be challenging. In single cases, GPi stimulation was shown to be effective reducing mobile, secondary dystonia in Wilson’s disease patients.

Method: A 49 year old patient with an 18 year history of secondary dystonia first affecting the neck, subsequently changing to a generalized choreo- and ballistic like dystonic movement disorder. Medical treatment with botulinumtoxin, anticholinergics, penicillamine, trientine and zinc in adequate dosages did not sufficiently enough reduce symptoms. After exclusion of contraindications the patient underwent bilateral deep brain stimulation of the GPi. The primary endpoint was the change of dystonia which was evaluated using the Burke-Fahn-Marsden scale – movement scale (BFMDRS-M) pre and 6 month post surgery. Secondary endpoints comprised the Abnormal Involuntary Movement Scale (AIMS) and the Blepharospasm Disability Indix (BSDI).

Results: Dystonic symptoms were significantly reduced by 12.5 points (32%) on the BFMDRS (pre-opepative: 39.5 points, 6 month post GPi DBS: 27 points). Furthermore, involuntary movements decreased by 9 points (31%) on the AIMS (pre-op. 29, post-op.: 20) and blepharospasm by 3 points (33%) on the BSDI (pre-op. 9, post-op. 6). The patient died due to a sudden cardiac death 11 month postoperative. This severe adverse event was evaluated to be unrelated to the GPi stimulation and the surgery.

Conclusion: GPi DBS efficiently reduced mobile generalized dystonia in a Wilson’s disease patient. The GPi DBS surgery and stimulation were feasibly and safe. The fatal serious adverse event of a sudden cardiac death was evaluated to be unrelated to the operation and the stimulation but to be possibly related to the underlying disease. *This case report was presented at the “Deutscher Kongress für Parkinson und Bewegungsstörungen” in Düsseldorf, Germany (March 2019).

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MDS Abstracts - https://www.mdsabstracts.org/abstract/internal-pallidum-stimulation-reduces-mobile-generalized-dystonia-in-wilsons-disease/