Deep Brain Stimulation in Spinocerebellar Ataxias: Review
Surabhi Garg1, Japleen Kaur2, Siddharth Agrawal3
1St. Johns medical college and hospital, 2student, Dayanand medical college and hospital, Ludhiana, 3New York medical college
Objective:

Review current literature on Deep Brain Stimulation (DBS) for Spinocerebellar Ataxias (SCA) including SCA types, DBS targets and outcomes

 


Background:
DBS has been employed for certain cases of SCAs globally but there is a lack of comprehensive data on DBS outcomes for different presentations.
Design/Methods:

 

We conducted a literature review on PubMed, Scopus, and Embase, using 'deep brain stimulation' and 'spinocerebellar ataxia' as search terms. English-language articles reporting DBS in genetically confirmed SCA cases were included. We performed descriptive statistical analysis using Microsoft Excel.


Results:

We included 16 studies reporting 23 patients with SCA. SCA3 was the most common subtype (n 10, 43.5%), followed by SCA2 (n=6, 26.1%). SCA6 and SCA17 each had 2 cases (8.7%), and SCA1, SCA27, and SCA31 each had 1 case (4.4%). The mean age at intervention was 42.1±13.9 years. Dystonia was the primary symptom targeted (n 11, 47.8%), with stimulation sites including bilateral Globus Pallidus internus (Gpi) in 7, unilateral Gpi in 1, ventral oralis posterior thalamic in 1, multi-target bilateral Gpi and subthalamic nucleus (STN) in 1, and bilateral Gpi and dentate nucleus in 1. Tremor was the next most common symptom targeted (9 cases) with Vim thalamus in 7 cases, bilateral STN in 1, and multi-target Vim and STN in 1. Ataxia was targeted in 2 cases with dentate nucleus stimulation. One patient with parkinsonism received bilateral STN stimulation. Outcomes showed dystonia improvement in 6 cases, worsening in 3, and no change in 1. Tremor responded positively in all cases. Ataxia improved in 2 cases while quality of life remained unchanged in 1. Motor scores improved for parkinsonism, but gait deteriorated.


Conclusions:
DBS for various manifestations of SCA can have mixed results and identification of proper targets is essential for best outcomes. It may be a promising therapeutic option and requires more studies to establish benefits.
10.1212/WNL.0000000000208349