Beyond the Burke–Fahn–Marsden Dystonia Rating Scale: Deep brain stimulation in childhood secondary dystonia

Gimeno, Hortensia; Tustin, Kylee; Selway, Richard; Lin, Jean-Pierre

doi:10.1016/j.ejpn.2011.12.014

Cited by 104 publications

(110 citation statements)

References 48 publications

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“…25 Regarding functional domains, a recent report has advocated for a shift away from impairment-focused measures, such as the BFMDRS, toward functional, multidimensional assessment tools that incorporate outcome measures of body function and structure, as well as activity and participation, to demonstrate the extent to which DBS improves performance of functional tasks, enables participation in activities, and improves the quality of life of children and families. 10 Using various nonmotor functionally based scales, Gimeno et al found that 6 children originally designated as "non-responders" according to BFMDRS scoring demonstrated significant benefits in pain and comfort, school attendance, seating tolerance, access to assistive technology, and caretaker burden. 10 We advocate for a multidimensional rating system that evaluates both motor and nonmotor changes.…”

Section: Dystonia Rating Scalesmentioning

confidence: 99%

“…10 Using various nonmotor functionally based scales, Gimeno et al found that 6 children originally designated as "non-responders" according to BFMDRS scoring demonstrated significant benefits in pain and comfort, school attendance, seating tolerance, access to assistive technology, and caretaker burden. 10 We advocate for a multidimensional rating system that evaluates both motor and nonmotor changes.…”

Section: Dystonia Rating Scalesmentioning

confidence: 99%

“…In these instances, the particular body region was assigned the previous month's score. Although the BFMDRS-M may be limited in evaluating secondary dystonia, 3,5,10,22,24,27 scores were included to allow for comparison with other published literature.…”

Section: Data Collectionmentioning

confidence: 99%

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Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Keen¹,

Przekop

Olaya

et al. 2014

PED

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Object Deep brain stimulation (DBS) for dystonic cerebral palsy (CP) has rarely been reported, and its efficacy, though modest when compared with that for primary dystonia, remains unclear, especially in the pediatric population. The authors present a small series of children with dystonic CP who underwent bilateral pallidal DBS, to evaluate the treatment's efficacy and safety in the pediatric dystonic CP population. Methods The authors conducted a retrospective review of patients (under the age of 18 years) with dystonic CP who had undergone DBS of the bilateral globus pallidus internus between 2010 and 2012. Two of the authors independently assessed outcomes using the Barry-Albright Dystonia Scale (BADS) and the Burke-Fahn-Marsden Dystonia Rating Scale–movement (BFMDRS-M). Results Five children were diagnosed with dystonic CP due to insults occurring before the age of 1 year. Mean age at surgery was 11 years (range 8–17 years), and the mean follow-up was 26.6 months (range 2–42 months). The mean target position was 20.6 mm lateral to the midcommissural point. The mean preoperative and postoperative BADS scores were 23.8 ± 4.9 (range 18.5–29.0) and 20.0 ± 5.5 (range 14.5–28.0), respectively, with a mean overall percent improvement of 16.0% (p = 0.14). The mean preoperative and postoperative BFMDRS-M scores were 73.3 ± 26.6 (range 38.5–102.0) and 52.4 ± 21.5 (range 34.0–80.0), respectively, with a mean overall percent improvement of 28.5% (p = 0.10). Those stimulated at least 23 months (4 patients) improved 18.3% (p = 0.14) on the BADS and 30.5% (p = 0.07) on the BFMDRS-M. The percentage improvement per body region yielded conflicting results between rating scales; however, BFMDRS-M scores for speech showed some of the greatest improvements. Two patients required hardware removal (1 complete system, 1 unilateral electrode) within 4 months after implantation because of infections that resolved with antibiotics. Conclusions All postoperative dystonia rating scale scores improved with pallidal stimulation, and the greatest improvements occurred in those stimulated the longest. The results were modest but comparable to findings in other similar series. Deep brain stimulation remains a viable treatment option for childhood dystonic CP, although young children may have an increased risk of infection. Of particular note, improvements in the BFMDRS-M subscores for speech were comparable to those for other muscle groups, a finding not previously reported.

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Section: Dystonia Rating Scalesmentioning

confidence: 99%

Section: Dystonia Rating Scalesmentioning

confidence: 99%

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Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Keen¹,

Przekop

Olaya

et al. 2014

PED

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“…15 Specifically, Gimeno et al suggest using tools such as the Canadian Occupational Performance Measure (COPM) and goal attainment scaling (GAS) to identify concerns and functional changes which are important to the child and family and measure the extent to which families feel both performance and satisfaction have changed in these areas. 15 These tools assess important concerns such as pain, comfort, ability to attend school, sitting tolerance, daily care, and burden to caregiver. 15 In another study, this group has also looked at improvements in upper limb function in dystonic children after DBS.…”

mentioning

confidence: 99%

“…15 These tools assess important concerns such as pain, comfort, ability to attend school, sitting tolerance, daily care, and burden to caregiver. 15 In another study, this group has also looked at improvements in upper limb function in dystonic children after DBS. Their data can be used to guide children and their families in terms of goals and time frame for clinical improvements after DBS.…”

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confidence: 99%

Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

Olaya

Christian

Ferman

et al. 2013

FOC

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Background Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. It can be classified as primary or secondary. There is no cure for dystonia and the goal of treatment is to provide a better quality of life for the patient. Surgical intervention is considered for patients in whom an adequate trial of medical treatment has failed. Deep brain stimulation (DBS), specifically of the globus pallidus interna (GPi), has been shown to be extremely effective in primary generalized dystonia. There is much less evidence for the use of DBS in patients with secondary dystonia. However, given the large number of patients with secondary dystonia, the significant burden on the patients and their families, and the potential for DBS to improve their functional status and comfort level, it is important to continue to investigate the use of DBS in the realm of secondary dystonia. Object The objective of this study is to review a series of cases involving patients with secondary dystonia who have been treated with pallidal DBS. Methods A retrospective review of 9 patients with secondary dystonia who received treatment with DBS between February 2011 and February 2013 was performed. Preoperative and postoperative videos were scored using the Barry-Albright Dystonia Scale (BADS) and Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) by a neurologist specializing in movement disorders. In addition, the patients' families completed a subjective questionnaire to assess the perceived benefit of DBS. Results The average age at DBS unit implantation was 15.1 years (range 6–20 years). The average time to follow-up for the BADS evaluation from battery implantation was 3.8 months (median 3 months). The average time to follow-up for the subjective benefit evaluation was 10.6 months (median 9.5 months). The mean BADS scores improved by 9% from 26.5 to 24 (p = 0.04), and the mean BFMDRS scores improved by 9.3% (p = 0.055). Of note, even in patients with minimal functional improvement, there seemed to be decreased contractures and spasms leading to improved comfort. There were no complications such as infections or hematoma in this case series. In the subjective benefit evaluation, 3 patients' families reported “good” benefit, 4 reported “minimal” benefit, and 1 reported no benefit. Conclusions These early results of GPi stimulation in a series of 9 patients suggest that DBS is useful in the treatment of secondary generalized dystonia in children and young adults. Objective improvements in BADS and BFMDRS scores are demonstrated in some patients with generalized secondary dystonia but not in others. Larger follow-up studies of DBS for secondary dystonia, focusing on patient age, history, etiology, and patterns of dystonia, are needed to learn which patients will respond best to DBS.

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The Burke‐Fahn‐Marsden Dystonia Rating Scale is Age‐Dependent in Healthy Children

Kuiper

Vrijenhoek

Brandsma

et al. 2016

Movement Disord Clin Pract

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View Supplementary Video 1 Background The Burke‐Fahn‐Marsden Dystonia Rating Scale is a universally applied instrument for the quantitative assessment of dystonia in both children and adults. However, immature movements by healthy young children may also show “dystonic characteristics” as a consequence of physiologically incomplete brain maturation. This could implicate that Burke‐Fahn‐Marsden scale scores are confounded by pediatric age. Objective In healthy young children, we aimed to determine whether physiologically immature movements and postures can induce an age‐related effect on Burke‐Fahn‐Marsden movement and disability scale scores. Methods Nine assessors specializied in movement disorders (3 adult neurologists, 3 pediatric neurologists, and 3 MD/PhD students) independently scored the Burke‐Fahn‐Marsden movement scale in 52 healthy children (4–16 years of age; 2 boys and 2 girls per year of age). Independent of that, parents scored their children's functional motor development according to the Burke‐Fahn‐Marsden disability scale in another 52 healthy children (4–16 years of age; 2 boys and 2 girls per year of age). By regression analysis, we determined the association between Burke‐Fahn‐Marsden movement and disability scales outcomes and pediatric age. Results In healthy children, assessment of physiologically immature motor performances by the Burke‐Fahn‐Marsden movement and disability scales showed an association between the outcomes of both scales and age (until 16 years and 12 years of age, β = −0.72 and β = −0.60, for Burke‐Fahn‐Marsden movement and disability scale, respectively [both P < 0.001]). Conclusions The Burke‐Fahn‐Marsden movement and disability scales are influenced by the age of the child. For accurate interpretation of longitudinal Burke‐Fahn‐Marsden Dystonia Rating Scale scores in young dystonic children, consideration of pediatric age‐relatedness appears advisory.

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Beyond the Burke–Fahn–Marsden Dystonia Rating Scale: Deep brain stimulation in childhood secondary dystonia

Cited by 104 publications

References 48 publications

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy

Deep brain stimulation in children and young adults with secondary dystonia: the Children's Hospital Los Angeles experience

The Burke‐Fahn‐Marsden Dystonia Rating Scale is Age‐Dependent in Healthy Children

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