Progressive supranuclear palsy-parkinsonism syndrome

Orpha code: 240085OMIM code: 260540

Definition

An atypical variant of progressive supranuclear palsy (PSP), a rare late-onset neurodegenerative disease, characterized by prominent early parkinsonism (tremor, limb bradykinesia, axial and limb rigidity) rather than falls and cognitive change. Over the years, patients ultimately develop clinical features characteristic of classical PSP. Neuropathological characteristics includes tau pathology and neuronal loss in specific brain areas, especially in the subthalamic nucleus and substantia nigra. The tau pathology is less severe than in classical PSP.

Disease data
Classification

Clinical subtype

Synonyms
PSP-p
PSP-p
PSP-parkinsonizm
PSP-parkinsonism
ORPHA code
240085
OMIM code
260540
ICD10 code
G23.1
ICD11 code
-

No additional description.

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