The Effectiveness of Deep Brain Stimulation in Dystonia: A Patient-Centered Approach

Abstract

Background: To systematically evaluate the effectiveness of deep brain stimulation of the globus pallidus internus (GPi-DBS) in dystonia on pre-operatively set functional priorities in daily living. Methods: Fifteen pediatric and adult dystonia patients (8 male; median age 32y, range 8–65) receiving GPi-DBS were recruited. All patients underwent a multidisciplinary evaluation before and 1-year post DBS implantation. The Canadian Occupational Performance Measure (COPM) first identified and then measured changes in functional priorities. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to evaluate dystonia severity. Results: Priorities in daily functioning substantially varied between patients but showed significant improvements on performance and satisfaction after DBS. Clinically significant COPM-score improvements were present in 7/8 motor responders, but also in 4/7 motor non-responders. Discussion: The use of a patient-oriented approach to measure GPi-DBS effectiveness in dystonia provides an unique insight in patients’ priorities and demonstrates that tangible improvements can be achieved irrespective of motor response. Highlights Functional priorities in life of dystonia patients and their caregivers vary greatly The effect of DBS on functional priorities did not correlate with motor outcome Half of the motor ‘non-responder’ patients reported important changes in their priorities The effect of DBS in dystonia should not be measured by motor outcome alone

Starting a DBS service for children: It’s not the latitude but the attitude - Establishment of the paediatric DBS centre in Northern Finland

“…Despite very severe movement disorders, we still found benefits of pDBS, many of which were noticeable in the daily care of the patients. This observation is in line with other studies that pDBS effects should not be measured by motor scores alone (Eggink et al 2020).…”

Elective and Emergency Deep Brain Stimulation in Refractory Pediatric Monogenetic Movement Disorders Presenting with Dystonia: Current Practice Illustrated by Two Cases

“…For example, individual perception of performance and satisfaction can be measured with the Canadian Occupational Performance Measure scale, which has proven to be efficacious in measuring functional outcomes and improvements after DBS in both primary and secondary pediatric dystonia. 31 32 The BFMDRS is often employed to quantify pre- and postoperative dystonia severity in the literature, although it is not specific to pediatric dystonia. 30 Other outcome scales, like the Barry-Albright Dystonia Scale, the Movement Disorder-Childhood Rating Scale or the Dyskinesia Impairment Scale, are mainly designed to evaluate pediatric acquired dystonia presenting with concomitant movement disorders.…”

Dystonia Management: What to Expect From the Future? The Perspectives of Patients and Clinicians Within DystoniaNet Europe

“…A different approach could be to aim directly for functional improvement in daily life, as defined by the patients themselves ( 114 , 115 ). One previous study examined the effectiveness of GPi DBS in dystonia patients on preoperatively set of functional priorities in daily living ( 116 ), measured with the Canadian Occupational Performance Measure (COPM). Priorities varied between patients but showed a significant improvement in performance and satisfaction after DBS in all.…”

“…Importantly, improvement was reported both by the motor responders and by several patients classified as non-responders based on the motor outcome. Such an approach focuses on important improvements for the individual patient that would not have been objectified with a general motor or non-motor symptom rating scale only and could be applied also to other treatment such as BoNT, psychotherapy or PT, or even to potential new treatments being investigated in clinical trials ( 116 , 117 ). A similar effect was also shown in other studies, were patients' perceptions in changes in life after DBS were studied with thematic interviews, instead of motor rating scales ( 118 , 119 ).…”