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Deep brain stimulation for monogenic dystonia
Bhooma R Aravamuthan1, Jeff L Waugh, Scellig S Stone
1aDepartment of Neurology, Boston Children's Hospital bHarvard Medical School cDepartment of Neurology, Massachusetts General Hospital dDepartment of Neurosurgery, Boston Children's Hospital, Boston, Massachusetts, USA.
Insights
Deep brain stimulation (DBS) offers a promising treatment for medically refractory dystonia in children, particularly for specific monogenic forms. Early intervention with DBS may lead to better outcomes for eligible pediatric patients.
Area of Science:
- Neurology
- Pediatric Medicine
- Neurosurgery
Background:
- Deep brain stimulation (DBS) is an established treatment for adult dystonia.
- Its application in pediatric dystonia is a recent development.
- Monogenic dystonias are a primary focus for DBS efficacy in children.
Purpose of the Study:
- To review the clinical recognition of childhood-onset monogenic dystonias.
- To assess the relative efficacy of DBS for key monogenic dystonias in children.
- To highlight DBS as a management option for pediatric dystonia.
Main Methods:
- Review of current literature on DBS for pediatric dystonia.
- Clinical recognition criteria for monogenic dystonias.
- Analysis of DBS efficacy based on dystonia type and duration.
Main Results:
- DBS is increasingly used in children with dystonia.
- Shorter duration of dystonia correlates with greater benefit from DBS.
- Specific monogenic dystonias show higher response rates to DBS.
Conclusions:
- DBS should be considered early for pediatric patients with medically refractory dystonia.
- Differentiating monogenic dystonias is crucial for prognostication and treatment decisions.
- Early DBS consideration is vital for monogenic dystonias with high response rates.
Purpose Of Review:
Deep brain stimulation (DBS) has recently emerged as an important management option in children with medically refractory dystonia. DBS is most commonly used, best studied, and thought to be most efficacious for a select group of childhood or adolescent onset monogenic dystonias (designated with a standard 'DYT' prefix). We review how to clinically recognize these types of dystonia and the relative efficacy of DBS for key monogenic dystonias.
Recent Findings:
Though used for dystonia in adults for several years, DBS has only lately been used in children. Recent evidence shows that patients with shorter duration of dystonia often experience greater benefit following DBS. This suggests that early recognition of the appropriate dystonic phenotypes and consideration of DBS in these patients may improve the management of dystonia.
Summary:
DBS should be considered early in patients who have medically refractory dystonia, especially for the monogenic dystonias that have a high response rate to DBS. It is important to differentiate between these monogenic dystonias and dystonias of other causes to properly prognosticate for these patients and to determine whether DBS is an appropriate management option.
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