Orphanet Rare Disease Ontology

Last uploaded: December 4, 2024
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Id http://www.orpha.net/ORDO/Orphanet_95432
http://www.orpha.net/ORDO/Orphanet_95432
Preferred Name

Primary progressive aphasia
Definitions
Primary progressive aphasia (PPA) is a neurodegenerative disorder, characterized by a primary dissolution of language, with relative sparing of other mental faculties for at least the first 2 years of illness. PPA is recognized as the language variant in the frontotemporal dementia (FTD; see this term) spectrum of disorders. PPA can be classified into 3 subtypes based on specific speech and language features: semantic dementia (SD), progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (lv-PPA) (see these terms).
Synonyms
Mesulam syndrome
PPA
Type http://www.w3.org/2002/07/owl#Class

All Properties

definition_citation

Orphanet
definition

Primary progressive aphasia (PPA) is a neurodegenerative disorder, characterized by a primary dissolution of language, with relative sparing of other mental faculties for at least the first 2 years of illness. PPA is recognized as the language variant in the frontotemporal dementia (FTD; see this term) spectrum of disorders. PPA can be classified into 3 subtypes based on specific speech and language features: semantic dementia (SD), progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (lv-PPA) (see these terms).
label
Primary progressive aphasia
prefLabel
Primary progressive aphasia
hasDbXref
ICD-11:6D83
MeSH:D018888
MedDRA:10081268
UMLS:C0282513
notation
ORPHA:95432
Clinical group
alternative_term
Mesulam syndrome
PPA
present_in
Worldwide AND has_point_prevalence_average_value : 7.0 AND has_point_prevalence_range : 1-9 / 100 000
prefixIRI
ORDO:Orphanet_95432
expertlink
subClassOf
type
has_inheritance
Not applicable
Multigenic/multifactorial
has_age_of_onset
Adult
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