An autopsy was performed on a 48-year-old woman with clinical features of parkinsonism-plus syndrome with dominating akinesia. Neuropathological examination revealed striato-nigral degeneration (SND) and neurofibrillary tangles (NFT) characterizing progressive supranuclear palsy. Such an unusual combination of pathological findings may constitute a distinct clinico-pathological entity, with akinesia as the main clinical symptom, and with a pathological substrate of SND and NFT. We suggest that such cases may establish a separate variant within multisystem atrophies syndromes.