Orphanet: Atypical progressive supranuclear palsy syndrome

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Atypical progressive supranuclear palsy syndrome

Disease definition

A group of clinical syndromes associated with underlying PSP-tau pathology, that do not conform to the classic presentation of PSP (Richardson syndrome), a rare late-onset neurodegenerative disease. The group comprises PSP-Parkinsonism (PSP-P), PSP-Pure akinesia with gait freezing (PSP-PAGF), PSP-corticobasal syndrome (PSP-CBS) and PSP-progressive non fluent aphasia (PSP-PNFA).


Classification level: Subtype of disorder
  • Synonym(s):
    • Atypical PSP syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: -
  • Age of onset: -
  • ICD-10: G23.1
  • OMIM: 260540
  • UMLS: C1850077
  • MeSH: C537240
  • GARD: 4507
  • MedDRA: -
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