title: Slowly progressive L-DOPA nonresponsive pure akinesia due to nigropallidal degeneration: a clinicopathological case study.
abstract: We report an autopsy case of a 51-year old man who showed slowly progressive pure akinesia: freezing phenomenon and festination during 21 years of illness 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 portion of the internal globus pallidus (GPi), without neurofibrillary tangles, neuropil threads, and glial fibrillary tangles. So this case was clearly distinguished with progressive nuclear palsy and pallidonigroluisian atrophy. It was first reported to describe that L-DOPA nonresponsive pure akinesia can arise from nigopallidal atrophy.
authors.affiliation: Third Department of Internal Medicine, Hiroshima University School of Medicine, Japan. k-sada@msg.biglobe.ne.jp
authors.forename: S
authors.initials: S
authors.lastname: Katayama
authors.forename: C
authors.initials: C
authors.lastname: Watanabe
authors.forename: T
authors.initials: T
authors.lastname: Khoriyama
authors.forename: M
authors.initials: M
authors.lastname: Oka
authors.forename: J J
authors.initials: JJ
authors.lastname: Mao
authors.forename: Y
authors.initials: Y
authors.lastname: Yamamura
authors.forename: E
authors.initials: E
authors.lastname: Tahara
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D018450:Disease Progression; D015259:Dopamine Agents / Q000627:therapeutic use*; D005917:Globus Pallidus / Q000473:pathology*; D006801:Humans; D007980:Levodopa / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000188:drug therapy*; D019636:Neurodegenerative Diseases / Q000188:drug therapy*; D013378:Substantia Nigra / Q000473:pathology*; D017211:Treatment Failure
chemical_list: D015259:Dopamine Agents; D007980:Levodopa