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Atypical progressive supranuclear palsy syndrome

Disease definition

A form of 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: -

  A summary on this disease is available in Deutsch (2010) Italiano (2010) Português (2010) Español (2021) Français (2021) Nederlands (2021)

Detailed information

Guidelines

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.