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Pallidal deep brain stimulation in individuals with dystonia due to cerebral palsy: expanded experience
Author(s) -
L BAILEY,
K ZEBRACKI,
K CHLAN,
L VOGEL
Publication year - 2015
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.19_12887
Subject(s) - deep brain stimulation , dystonia , cerebral palsy , physical medicine and rehabilitation , medicine , stimulation , neuroscience , psychology , parkinson's disease , disease
Background/Objectives: Pallidal deep brain stimulation (DBS) is a viable treatment for individuals with many forms of progressive dystonia. Cerebral palsy (CP), is the most common etiology of pediatric onset dystonia. Due to the pharmacologically refractory nature of dystonia, and the difficulty managing the mixed abnormalities of tone often encountered in cerebral palsy, the applicability of DBS in children, adolescents, and young adults with non-progressive dystonias has garnered increased attention in the last decade. We present our expanded experience using pallidal stimulation for management of motor impairment due to dystonia caused by cerebral palsy. Study Design: Prospective cohort study. Study Participants and Setting: All patients undergoing DBS at Cook Children’s Medical Center. Materials/Methods: Review of all patients undergoing DBS at Cook Children’s Medical Center between September 2007 and December 2014. Patients were assessed using BurkeFahn-Marsden Motor scores (BFM), Barry Albright Scales (BAS) and the BFM Disability Rating Scales (DRS). Patients were stratified by age at time of implant. Sixteen years of age was used as the dividing point between groups as a determinant of skeletal maturity. Results: Ninety-one patients underwent primary implant of DBS, including sixty-five with secondary dystonias. Thirty patients with cerebral palsy all underwent bilateral implantation of the globus pallidus internus. One patient was explanted due to early infection and therefore excluded from analysis. At baseline, the mean BFM score for the combined 29 patient cohort was 75.59. Patients older than 15 years (n = 11) had a greater degree of motor impairment than younger patients (BFM = 87.18 vs 68.50, p = 0.008). Twenty-one patients (mean age 16.09yrs, range 7–28) had follow-up assessments of tone after at least 6 months of treatment, including 11 in the younger age group. BFM scores decreased from 75.59 at baseline to 63.45 (19%, p = 0.010). A similar reduction of DRS (4%; p = 0.081) and BAS (6%; p = 0.095) scores was seen. BFM reduction was more apparent in younger patients (26% vs 11%). After 2 years of stimulation, improvements in motor function from baseline were maintained for the entire cohort (n = 13, BFM = 64.27; 17%; p = 0.034). Functional improvement was also maintained (DRS = 10%, p = 0.066; BAS = 11%, p = 0.052). Again, greater improvement was observed in younger patients (n = 8) in both motor (BFM = 21% vs 9%) and functional scores (DRS = 17% vs 2% and BAS = 16% v 1%). Conclusions/Significance: Pallidal deep brain stimulation offers an effective option for the amelioration of severe motor dysfunction in individuals with dystonia due to cerebral palsy. Improvement is often seen by 6 months, and maintained even after two or more years of stimulation. Children implanted prior to age 16 years experience greater reduction in motor impairment and improvement in functional ability than those implanted later in life.