Progressive supranuclear palsy-progressive non-fluent aphasia syndrome

Orpha code: 240112OMIM code:

Definition

An atypical variant of progressive supranuclear palsy (PSP), a rare late-onset neurodegenerative disease, characterized by an initial presentation of an isolated speech and language disorder (apraxia of speech, agrammatism, and phonemic errors) years before developing other motor features of PSP. Neuropathological characteristics includes tau pathology and neuronal loss in specific brain areas, especially in the temporal cortex and superior frontal gyrus.

Disease data
Classification

Clinical subtype

Synonyms
PSP-AOS
Postępujące porażenie nadjądrowe - apraksja mowy
PSP-AOS
PSP-PNFA
PSP-PNFA
Progressive supranuclear palsy-apraxia of speech syndrome
ORPHA code
240112
OMIM code
-
ICD10 code
G23.1
ICD11 code
-

No additional description.

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