Introduction: The experience with deep-brain stimulation (DBS) in multiple-system atrophy (MSA) is sparse and generally disappointing. DBS is currently not recommended in MSA and its use is often related to a misdiagnosis.
Observation: We describe the outcome of bilateral DBS of the internal pallidum in a 46-year-old woman suffering from MSA that initially resembled Parkinson's disease with prominent levodopa-induced dyskinesias. DBS of the left internal pallidum was performed in 1998 after a ten-year clinical course and improved dyskinesias. Six months later, the right side was implanted. A few months after the second surgery, the patient progressively developed signs of cerebellar and dysautonomic impairment and MSA was diagnosed.
Conclusion: Our observation confirms the ineffectiveness of DBS of the internal pallidum in MSA and even suggests a harmful effect. DBS remains contra-indicated in atypical parkinsonism.