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Published on: May 31, 2016
Gross motor function outcomes following deep brain stimulation for childhood-onset dystonia: A descriptive report
Kylee Tustin1, Markus C Elze2, Daniel E Lumsden1
1Complex Motor Disorder Service, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Floor 2 Beckett House, Lambeth Palace Road, London, SE1 7EU, United Kingdom.
Insights
Deep brain stimulation (DBS) improved gross motor function in children with inherited dystonias, but responses varied significantly in other dystonia types. Further research is needed to identify predictors of DBS treatment success in childhood-onset dystonia.
Area of Science:
- Neurology
- Pediatric Movement Disorders
- Neurosurgery
Background:
- Dystonic movement disorders significantly impair gross motor function in children.
- Deep brain stimulation (DBS) is a therapeutic option for severe dystonia.
- Understanding DBS efficacy across different dystonia etiologies is crucial.
Purpose of the Study:
- To evaluate the impact of DBS on gross motor function in pediatric patients with dystonic movement disorders.
- To analyze DBS outcomes based on the underlying cause of dystonia (inherited, acquired, idiopathic).
Main Methods:
- Prospective audit of children with dystonia who underwent DBS between 2007 and 2015.
- Assessment of gross motor function using the Gross Motor Function Measure (GMFM-88) and the Barry-Falmouth Movement Disorder Rating Scale (BFM-M).
- Analysis of outcomes across etiological subgroups, considering factors like proportion of life with dystonia (PLD) and baseline motor capacity.
Main Results:
- Sixty children (median age 10y 11mo) were analyzed.
- Inherited monogenetic dystonias showed significant GMFM-88 improvements at one year (median 6.9%, p=0.021).
- Acquired, heredodegenerative, and idiopathic dystonias exhibited variable and often non-significant gross motor responses to DBS.
Conclusions:
- DBS demonstrates consistent gross motor improvements in inherited monogenetic dystonias.
- Responses in acquired, heredodegenerative, and idiopathic dystonias are highly variable and often disappointing.
- Further research, including single-case experimental designs, is necessary to determine DBS efficacy and identify predictive factors for treatment response in childhood-onset dystonias.
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.
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