Objective: To assess long term benefit of multiple source steering of GPi-DBS in a patient with secondary dystonia due to kernicterus related perinatal encephalopathy.

Background: GPi-DBS proved to be effective in patients with primary generalized disabling dystonia. There is comparatively less experience about its effects on secondary dystonia, particularly in the context of to cerebral palsy secondary to kernicterus.

Methods: This case report is in accordance with Declaration of Helsinki. Patient is a 16 years old female with non-progressive perinatal encephalopathy or cerebral palsy, secondary to kernicterus and severely disabling generalised mobile dystonia. She had marked dysarthria and dysphagia. She never walked by herself, needing a wheelchair and had to have all her 4 limbs bounded due to the violence of her dystonic movements. She was dependant for all activities of daily living. Bilateral GPi-DBS implant was performed, employing an eight contacts lead per hemisphere and a rechargeable pulse generator.

Results: Score on the Burke-Fahn-Marsden Scale Movement (BFMDRS-M) was 110 and 27 on Disability (BFMDRS-D), pre-DBS. Improvement was readily observed 15 days post-DBS. She improved by 38 % and 18 % 3 months post-DBS and by 45% and 27% 6 months post-DBS. 24 months post-DBS, BFMDRS-M score had improved by 59% and the BFMDRS-D score by 44%. Accordingly, Health-related quality of life improved significantly. Best monopolar stimulation was used. Variable combinations of multipolar fractionalized current stimulation of adjacent contacts were tried in 8 sessions with 1 week intervals, with no further improvement. No adverse events related to the therapy occurred.

Conclusions: In our case, we had a long term 50% improvement in motor and disability scales, similarly to what had been reported for patients with primary generalized dystonia in controlled studies. This result is encouraging, particularly considering the relatively scarce data on the outcome for this dystonia subtype in the literature. This DBS system showed to be safe and clinically effective in the long term. Current steering showed no extra benefit in this case. No hardware problems or recharge issues were reported up to date.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/patient-with-secondary-dystonia-treated-with-internal-globus-pallidus-deep-brain-stimulation-gpi-dbs-2-years-results-a-case-report/