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Progressive Non-Fluent Aphasia
Alias:
Agramatic Variant of Primary Progressive Aphasia
|
Primary Progressive Nonfluent Aphasia
|
Progressive Nonfluent Aphasia
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Agramatic Variant of Ppa
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Non-Fluent Variant Ppa
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Primary Progressive Non Fluent Aphasia
|
Nonfluent Agrammatic Ppa
Basic Information
Medical Symptom
Gene & Mutation
Related Drugs
Disease Model
References Literature
Progressive non-fluent aphasia (PNFA) is a form of frontotemporal dementia (FTD) characterized by agrammatism, laborious speech, alexia, and agraphia, often accompanied by apraxia of speech (AOS). Language comprehension remains relatively preserved. PNFA is one of three clinical syndromes associated with frontotemporal lobar degeneration, with an insidious onset of language deficits over time. The disorder primarily affects the left hemisphere, leading to expressive language deficits and potential disruptions in comprehending complex language.
Related ID:
Basic Information
Inheritance | Age of Onset | Prevalence | Related Genes | Related Mouse Models | Reference | MALACARDS |
|---|---|---|---|---|---|---|
Ot Other | Adult | 1-9/100000 Point prevalence: 1-9/100000 (Europe) | 50 | 723 | 0 |
Medical Symptom
Phenotype Information Associated with the Current Disease:
Categorization: Anatomical classification of the disease manifestations.
HPO Frequency/Orphanet Frequency: Indicates the probability of the manifestation occurring in the current disease, allowing sorting by probability.
HPO Source Accession: Links to HPO for detailed manifestation information.
Data Source: HPO, Orphanet
Gene & Mutation
Genes and Mutations Associated with the Current Disease:
Function: Primary biological roles of the genes.
Score: Indicates the strength of the association between the disease and the gene, with higher scores reflecting stronger associations.
Count: Number of mutations associated with the disease-gene pair. The number in parentheses represents the total data points linked to the same ClinVar ID. Clicking the number reveals mutation details.
Data Source: Clinvar
Related Drugs
Drugs Related to the Current Gene, Displaying CAS Number, Status and Phase.
Data Source: Clinical Trials
Related Mouse Models
Mouse Models Related to the Current Gene. Click on the model name to view detailed information.
Data Source: MGI, Cyagen
References Literature
Most Relevant Literature for the Current Gene, Filterable by Year, Article Type, and Sortable by Impact Factor.
Data Source: UniProt, PubMed