Синдром прогресуючого надядерного паралічу-чистої акінезії із завмиранням ходи
ORPHA:240094· ICD-10 G23.1· Progressive supranuclear palsy-pure akinesia with gait freezing syndrome
Визначення(English summary)
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.
- Поширеність
- 1-9 / 100 000
- Успадкування
- Not applicable
- Вік початку
- Adult