{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T01:08:00Z","timestamp":1792804080773,"version":"4.5.0"},"reference-count":31,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2013,3,1]],"date-time":"2013-03-01T00:00:00Z","timestamp":1362096000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2013,6]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>The aim of this study was to examine the impact of dystonia aetiology and duration, contracture, and age at deep brain stimulation (<jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content>) surgery on outcome in a cohort of children with medically refractory, disabling primary, secondary\u2010static, or secondary\u2010progressive dystonias, including neurodegeneration with brain iron accumulation (<jats:styled-content style=\"fixed-case\">NBIA<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>Dystonia severity was assessed using the <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>urke\u2013<jats:styled-content style=\"fixed-case\">F<\/jats:styled-content>ahn\u2013<jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>arsden <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>ystonia Rating Scale (<jats:styled-content style=\"fixed-case\">BFMDRS<\/jats:styled-content>) motor score at baseline and 6 and 12\u00a0months postoperatively in a cohort of 70 consecutive children undergoing <jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content> between June 2005 and July 2011.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Two children (3%) received unilateral <jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content> for hemidystonia and were excluded and five (7%) developed infections requiring part\u2010<jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content> removal within 6\u00a0months, leaving 63 children (90%) undergoing bilateral <jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content> for follow\u2010up (34 males, 29 females; mean age at surgery for the whole group 10y 4mo, <jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> 4y 2mo, range 1\u201314y). Seventeen children were classified with primary dystonia: mean age 12\u00a0years 11\u00a0months, <jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> 4\u00a0years 6\u00a0months range 4\u00a0years 6\u00a0months to 17\u00a0years 3\u00a0months; 28 as having secondary\u2010static dystonia: mean age 10\u00a0years 2\u00a0months, <jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> 4\u00a0years 9\u00a0months (range 3y 3mo\u201320y); five as having secondary\u2010progressive dystonia: mean age 8\u00a0years 11\u00a0months, <jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> 3\u00a0years 9\u00a0months (range 5y 5mo\u201313y 1mo); and 13 as having <jats:styled-content style=\"fixed-case\">NBIA<\/jats:styled-content> dystonia: mean age 10\u00a0years 2\u00a0months, <jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> 3\u00a0years 11\u00a0months (range 1\u201314y). Children with primary dystonias demonstrated greater improvements in <jats:styled-content style=\"fixed-case\">BFMDRS<\/jats:styled-content> motor score than those in the other aetiological categories (<jats:styled-content style=\"fixed-case\">K<\/jats:styled-content>ruskal\u2013<jats:styled-content style=\"fixed-case\">W<\/jats:styled-content>allis test, <jats:italic>p<\/jats:italic>&lt;0.001), which correlated negatively with dystonia duration and more strongly still against the ratio of dystonia duration normalized to age at surgery (<jats:styled-content style=\"fixed-case\">DD<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">AS<\/jats:styled-content> ratio) at 1\u00a0year (Spearman's rank correlation coefficient 0.4752 and \u22120.599 respectively). A similar significant negative correlation was found in the secondary\u2010static dystonia group between outcome at 1\u00a0year and <jats:styled-content style=\"fixed-case\">DD<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">AS<\/jats:styled-content> ratio (\u22120.461). Poorer outcome in secondary dystonia coincided with the absence of a period of normal motor development in comparison with the primary dystonia group. A significant improvement in <jats:styled-content style=\"fixed-case\">BFMDRS<\/jats:styled-content> motor score was seen in the <jats:styled-content style=\"fixed-case\">NBIA<\/jats:styled-content> group at 6, but not 12\u00a0months (<jats:styled-content style=\"fixed-case\">W<\/jats:styled-content>ilcoxon signed rank test <jats:italic>p<\/jats:italic>=0.028, <jats:italic>p<\/jats:italic>=0.85 respectively). No reduction in efficacy was seen in children with a musculoskeletal deformity at the time of surgery.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Response to pallidal <jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content> in the treatment of dystonia declines with the proportion of life lived with dystonia in primary and secondary dystonia. Other intrinsic factors reduce the median magnitude of reduction in secondary dystonia after <jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content>. <jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content> should be offered early, preferably within 5\u00a0years of onset, to maximize benefits and reduce the childhood experience of dystonia, including musculoskeletal deformity. Other multidimensional assessments are required to understand how <jats:styled-content style=\"fixed-case\">DBS<\/jats:styled-content> improves the lives of children with dystonia.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.12117","type":"journal-article","created":{"date-parts":[[2013,3,2]],"date-time":"2013-03-02T05:21:20Z","timestamp":1362201680000},"page":"567-574","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":167,"title":["Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia"],"prefix":"10.1111","volume":"55","author":[{"given":"Daniel E","family":"Lumsden","sequence":"first","affiliation":[{"name":"Complex Motor Disorders Service Evelina Children's Hospital Guy's and St Thomas' NHS Foundation Trust London 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