Outcome of deep brain stimulation in slowly progressive multiple system atrophy: A clinico-pathological series and review of the literature

• STN DBS efficacy is short-lasting in MSA.
• STN DBS should not be recommended for MSA patients.
• Better diagnosis tools are mandatory not to misdiagnose slowly progressive MSA.

ObjectivesTo highlight the risk of clinical worsening after deep brain stimulation in histologically proven multiple system atrophy (MSA) patients presenting slow and relatively benign disease progression mimicking Parkinson's disease (PD). In such cases but also in more typical MSA patients, the results of deep brain stimulation have been mostly reported as case reports and small patient series.MethodsThe present study describes the outcome of the largest series of histologically proven MSA patients who underwent deep brain stimulation (DBS) of the subthalamic nucleus because they were considered as having PD at the time of surgery.ResultsThree patients showed significant improvement of motor signs after surgery while two did not. Clinical improvement was short-lasting and rapidly followed by the occurrence of disabling manifestations of MSA that counteracted DBS benefits.ConclusionsTogether with previous reports, our study demonstrates that DBS should not be recommended for MSA patients. It also underlines that detecting subtle red flags is crucial to avoid DBS surgery in this population.