Primary progressive aphasia
Synonyms: Mesulam syndrome · PPA
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
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) 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).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary progressive aphasia | Mesulam syndrome · PPA |
| fr | Aphasie primaire progressive | PPA · Syndrome de Mesulam |
| de | Aphasie, primäre progressive | Mesulam-Syndrom · PPA |
| es | Afasia progresiva primaria | APP · Síndrome de Mesulam |
| it | Afasia primitiva progressiva | PPA · Sindrome di Mesulam |
| nl | Primaire progressieve afasie | PPA · Syndroom van Mesulam |
| pt | Afasia primária progressiva | |
| pl | Pierwotna postępująca afazja | PPA · Zespół Mesulama |
| cs | Primární progresivní afázie | PPA · Mesulamův syndrom |
| uk | Первинна прогресуюча афазія | Синдром Мезулама · PPA |
| zh | 原发性进行性失语 | Mesulam综合征 |
| tr | Primer ilerleyici afazi | PPA · Mesulam sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | 6D83 | NTBT | index | validated |
| MONDO | 0019806 | E | validated | |
| MeSH | D018888 | E | validated | |
| UMLS | C0282513 | E | validated | |
| GARD | 8541 | E | validated | |
| MedDRA | 10081268 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98534Neurodegenerative disease with dementia
rare neurological diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 7 / 100 000 | Worldwide | Value and class | Not yet validated | 24108322[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/95432
- This record as JSON: /data/d/95432.json