Progressive supranuclear palsy-pure akinesia with gait freezing syndrome

Orpha code: 240094OMIM code:

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
-

No additional description.

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