Progressive supranuclear palsy-pure akinesia with gait freezing syndrome
Synonyms: PSP-PAGF | PSP-pure akinesia with gait freezing
An atypical variant of progressive supranuclear palsy (PSP) a rare late-onset neurodegenerative disease characterized by progressive freezing of gait speech and writing early in the disease course. Later axial rigidity and facial immobility can occur and supranuclear downgaze paresis may emerge after a decade. Neuropathological characteristics include tau pathology and neuronal loss in specific brain areas especially in the globus pallidus subthalamic nucleus and substantia nigra. The tau pathology is less widespread compared to the other PSP sub-types.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version October 2023.
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