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Deep brain stimulation for chorea-acanthocytosis: a systematic review.
Yang Wu1, Yang-Yang Xu1, Yuan Gao1
1Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guoxue Alley, Chengdu, Sichuan Province, China.
Neurosurgical Review
|January 12, 2022
Summary
Deep brain stimulation (DBS) offers a promising treatment for chorea-acanthocytosis (ChAc), showing significant symptom improvement. While generally safe, some adverse events were noted, warranting further investigation.
Area of Science:
- Neurology
- Neurosurgery
- Movement Disorders
Background:
- Chorea-acanthocytosis (ChAc) is a rare neurodegenerative disorder.
- Deep brain stimulation (DBS) is a potential treatment, but its efficacy and safety are not well-established due to ChAc's low prevalence.
Purpose of the Study:
- To systematically review and analyze the existing literature on the safety and efficacy of DBS in patients with ChAc.
- To consolidate data from case reports to provide a comprehensive overview of DBS outcomes for ChAc.
Main Methods:
- Systematic literature review of PubMed and EMBASE databases.
- Inclusion of English-language studies reporting on DBS efficacy or safety in ChAc patients.
- Pooling of data from 20 studies involving 34 ChAc patients.
Main Results:
- The majority of patients (29/34) received globus pallidus interna (GPi)-DBS.
- DBS demonstrated significant improvement in chorea (88.5%) and dystonia (76.9%), but not dysarthria (85.7% no response).
- The Unified Huntington's Disease Rating Scale-Motor Score improved by a median of 46.7%, with a mean score decrease from 43.2 to 22.3.
Conclusions:
- Deep brain stimulation (DBS) appears to be a safe and effective treatment option for managing chorea-acanthocytosis (ChAc) symptoms, particularly chorea and dystonia.
- Globus pallidus interna (GPi)-DBS is the most commonly applied method, suggesting its potential as a preferred target for ChAc treatment.
- Further research is warranted to optimize DBS parameters and fully elucidate its long-term impact on ChAc management.
