Long-term follow-up of deep brain stimulation of peduncolopontine nucleus in progressive supranuclear palsy: Report of three cases

Background: Progressive supranuclear palsy (PSP) is a neurodegenerative disease due to mitochondrial dysfunction. The PSP syndrome presents generally with gait disorder, Parkinsonism, ophthalmoparesis and cognitive alteration. Few reports exist on deep brain stimulation (DBS) in patients with atypical Parkinsonism. The aim of our study was to evaluate further the potential role of DBS in PSP. Case Description: We report three patients with PSP with long-term follow up undergoing DBS. Two patients had right peripedunculopontine nucleus (PPN) stimulation and one patient had simultaneous right PPN and bilateral globus pallidus internus DBS. DBS of the PPN alone or combined with globus pallidus internus (GPi ) determined an improvement in gait and a reduction in falls sustained over time. Combined target stimulation (GPi-PPN) was correlated with better clinical outcome than single target (PPN) DBS for PSP. Conclusions: Although few data on DBS for PSP exist, reported clinical results are encouraging. DBS might be considered as an alternative therapeutic option for patients with PSP presenting with relevant gait imbalance and frequent falls, who fail to respond to pharmacological treatment. Larger cohorts with longer follow-ups are needed to evaluate more exhaustively the efficacy of DBS in PSP.