Gross motor function outcomes following deep brain stimulation for childhood-onset dystonia: A descriptive report. (May 2019)

• Record Type: Journal Article
• Authors: Tustin, Kylee; Elze, Markus C.; Lumsden, Daniel E.; Gimeno, Hortensia; Kaminska, Margaret; Lin, Jean-Pierre
• Is Part Of: European journal of paediatric neurology. Volume 23:Number 3(2019:May)
• Page Start: 473
• Page End: 483
• Publication Date: 2019-05
• Subjects: Paediatric dystonia -- Deep brain stimulation -- DBS -- Gross motor function; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2019.02.005
• Languages: English
• ISSNs: 1090-3798

Abstract

Abstract: Aim: To examine the impact of deep brain stimulation (DBS) on gross motor function in children with dystonic movement disorders. Method: Prospective audit involving children implanted 2007–2015, followed for up to two years. Outcomes were evaluated across aetiological sub-groups (inherited, acquired, idiopathic) using the GMFM-88 and BFMDRS movement scale (BFM-M). The predictive value of proportion of life lived with dystonia (PLD) and baseline motor capacity were evaluated. Results: Data was available for 60 children (median surgery age 10y11mo). Inherited monogenetic dystonias demonstrated a median increase in GMFM-88 scores of 6.9% (p = 0.021) and 14.5% (p = 0.116) at one and two years. Heredodegenerative and idiopathic dystonias showed disparate responses, with non-significant changes seen in GMFM-88 and BFM-M scores, with the exception of improved one-year BFM-M scores in the idiopathic group [median change 5.5, p = 0.021 ]. Median GMFM-88 and BFM-M change scores were near zero for acquired dystonias, though improvement was noted in 9/18 CP cases with one-year GMFM-88 data. No significant relationship was found between PLD, or baseline GMFM-88, and GMFM-88 change following DBS. Conclusion: Gross motor response to DBS is similar in profile to literature reporting results using impairment-based dystonia rating scales. Relatively consistent improvements were seen in inherited monogenetic ("primary") dystonias, while highly variable, often disappointing, gross motor responses were found in acquired, heredodegenerative, and idiopathic dystonias. In view of such response variability, alternatives to mean group studies, such as single case experimental designs with multiple replications, are needed to determine the efficacy of DBS in childhood-onset dystonias. Ongoing research is needed to identify factors that predict treatment response. Highlights: Gross motor change after DBS is similar in profile to dystonia scale literature. More consistent improvements are seen in inherited monogenetic ("primary") movement disorders. Variable, often disappointing, responses were seen in other forms of dystonia. Alternatives to mean group studies are needed to evaluate DBS in childhood dystonias.