Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition 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. Disease data Classification Clinical subtype Synonyms PSP-PAGF PSP-czysta akinezja z zamrożeniem chodu PSP-PAGF PSP-pure akinesia with gait freezing ORPHA code 240094 OMIM code - ICD10 code G23.1 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl