Objective: To report clinical outcomes of two patients diagnosed with MSA-P who underwent bilateral deep brain stimulation (DBS).

Background: The role of DBS in patients with MSA remains controversial and in general, the outcomes are inferior to those seen in patients in idiopathic Parkinson’s disease (PD).

Methods: Two patients diagnosed with MSA-P underwent bilateral DBS targeting the globus pallidus, pars interna (GPi) because of motor fluctuations and disabling dyskinesias on levodopa therapy. The diagnosis of MSA-P was based on the Second Consensus Statement on the diagnosis of MSA. Both patients also underwent MIBG scan with delayed heart/mediastinum ratio study. Additional evaluations included UPDRS scores, part III (motor) and part IV (A: dyskinesias and B: clinical fluctuations) in off and on states preoperatively and part III and IV scores on therapy/on stimulation.

Results: The first patient is a 60 year old man who developed first symptoms at age of 50 years. He developed parkinsonism associated with autonomic dysfunction and early stridor. He responded to levodopa but he developed early fluctuations and very frequent disabling dyskinesias at the daily dose of 1600 mg of levodopa. Severe dyskinesias were dose limiting and lowering levodopa induced poorly tolerated off state. His presurgical off/on evaluation showed UPDRS III scores 59/39 and UPDRS IV score was 14 with disabling dyskinesias present 51-75% of time. His DBS improved his UPDRS III on medications/on stimulation to 30 points and his dyskinesias were absent. Patient had also severe dysphagia that did not change after his DBS and he refused PEG tube. Two years later he developed sepsis from aspiration and he died. His DBS benefits were sustained for two years. The second patient is a 66 year old woman who developed first symptoms at age 55 with parkinsonism, autonomic dysfunction and postural instability. She improved on 1000 mg of levodopa but experienced dyskinesias and left-sided dystonia in off state. Her UPDRS III off/on scores were 42/35 and her part IV score was 11. Her dyskinesias and dystonia improved after surgery with UPDRS IV scores 6 but she continued to have severe gait problems and multiple falls.

Conclusions: DBS with GPi targeting may improve levodopa-responsive motor fluctuations in MSA-P patients. The selection of these patients must also consider the progressive nature of levodopa-resistant symptoms in MSA-P. This work was previously presented at XXII Congress on PD and related disorders, November 12-16, 2017.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-role-of-deep-brain-stimulation-targeting-gpi-in-patients-with-multiple-system-atrophy/