Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia

• DOI: 10.1111/dmcn.12117
• Publication Date: 2013-03-02T05:21:20Z
• Authors: Daniel E Lumsden; Margaret Kaminska; Hortensia Gimeno; Kylee Tustin; Lesley Baker; Sarah Perides; Keyoumars Ashkan; Richard Selway; Jean‐Pierre Lin
• Keywords: Pallidotomy

The aim of this study was to examine the impact dystonia aetiology and duration, contracture, age at deep brain stimulation (DBS) surgery on outcome in a cohort children with medically refractory, disabling primary, secondary-static, or secondary-progressive dystonias, including neurodegeneration iron accumulation (NBIA).Dystonia severity assessed using Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor score baseline 6 12 months postoperatively 70 consecutive undergoing DBS between June 2005 July 2011.Two (3%) received unilateral for hemidystonia were excluded five (7%) developed infections requiring part-DBS removal within months, leaving 63 (90%) bilateral follow-up (34 males, 29 females; mean whole group 10y 4mo, SD 4y 2mo, range 1-14y). Seventeen classified primary dystonia: years 11 4 17 3 months; 28 as having secondary-static 10 2 9 (range 3y 3mo-20y); 8 5y 5mo-13y 1mo); 13 NBIA Children dystonias demonstrated greater improvements BFMDRS than those other aetiological categories (Kruskal-Wallis test, p<0.001), which correlated negatively duration more strongly still against ratio normalized (DD/AS ratio) 1 year (Spearman's rank correlation coefficient 0.4752 -0.599 respectively). A similar significant negative found DD/AS (-0.461). Poorer secondary coincided absence period normal development comparison group. improvement seen 6, but not (Wilcoxon signed test p=0.028, p=0.85 No reduction efficacy musculoskeletal deformity time surgery.Response pallidal treatment declines proportion life lived dystonia. Other intrinsic factors reduce median magnitude after DBS. should be offered early, preferably 5 onset, maximize benefits childhood experience dystonia, deformity. multidimensional assessments are required understand how improves lives