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Atypical progressive supranuclear palsy syndrome

Disease definition

A form or progressive supranuclear palsy syndrome (PSP), a rare late-onset neurodegenerative disease, characterized by an underlying PSP-tau pathology, that does not conform to the classic presentation of PSP. The clinical phenotype is variable and comprises PSP with predominant Parkinsonism (PSP-P), PSP with progressive gait freezing (PSP-PGF), PSP with predominant corticobasal syndrome (PSP-CBS), PSP with predominant speech/language disorder (PSP-SL), PSP with predominant frontal presentation (PSP-F), PSP with predominant ocular motor dysfunction (PSP-OM), and PSP with predominant postural instability (PSP-PI).

ORPHA:99750

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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