Slowly progressive L-DOPA nonresponsive pure akinesia due to nigropallidaldegeneration: a clinicopathological case study

We report an autopsy case of a 51-year old man who showed slowly progressiv e pure akinesia: freezing phenomenon and festination during 21 years of ill ness without tremor, rigidity, upward gaze palsy, bradykinesia and dementia , which were not responded to L-DOPA clinically. Neuropathological findings revealed the circumscribed regions in the substantia nigra and middle port ion of the internal globus pallidus (GPi), without neurofibrillary tangles, neuropil threads, and glial fibrillary tangles. So this case was clearly d istinguished with progressive nuclear palsy and pallidonigroluisian atrophy . It was first reported to describe that L-DOPA nonresponsive pure akinesia can arise from nigopallidal atrophy. (C) 1998 Published by Elsevier Scienc e B.V. All rights reserved.