Akinesia and axial rigidity without limb rigidity: an intermediate form between progressive supranuclear palsy and pure akinesia of Imai and Narabayashi

Abstract

We report five patients with atypical parkinsonism characterized by freezing phenomenon, akinesia and axial rigidity without limb rigidity or tremor. These patients were selected from 252 patients with parkinsonism who were referred to our clinic from 1986 to 1993. They have common clinical features consisting of freezing phenomena involving all four extremities, especially in the initiation of walking, and marked axial rigidity; otherwise, neither supranuclear ophthalmoparesis nor nuchal dystonia was noted. Their clinical features did not change over several years except in one patient who later developed typical manifestations of progressive supranuclear palsy (PSP). Levodopa was of no effect on their symptoms. After excluding other possibilities, it is our conclusion that they represent an atypical form of PSP lying between the pure akinesia of Imai and Narabayashi and the typical form of PSP.