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Start free with EleplanProgressive non-fluent aphasia
ORPHA:100070Disease
Also called Agramatic variant of PPA · Agramatic variant of primary progressive aphasia · Non-fluent variant PPA
What it is
Progressive non-fluent aphasia (PNFA) is a form of frontotemporal dementia (FTD), characterized by agrammatism, laborious speech, alexia, and agraphia, frequently accompanied by apraxia of speech (AOS). Language comprehension is relatively preserved.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial, Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
9Common30–79%
7Sometimes5–29%
12- Abnormality of extrapyramidal motor function
- Abnormal lower motor neuron morphology
- Astrocytosis
- Atypical behavior
- Dysgraphia
- Motor aphasia
- Mutism
- Parkinsonism
and 4 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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