National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Progressive non-fluent aphasia


Other Names:
Non-fluent primary progressive aphasia; Non-fluent variant PPA
Categories:
This disease is grouped under:
The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

Orpha Number: 100070

Definition
Progressive non-fluent aphasia (PNFA) is a form of frontotemporal dementia (FTD; see this term), characterized by agrammatism, laborious speech, alexia, and agraphia, frequently accompanied by apraxia of speech (AOS). Language comprehension is relatively preserved.

Visit the Orphanet disease page for more resources.
Last updated: 6/1/2014

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Dysphasia 0002357
Frontotemporal cerebral atrophy 0006892
Frontotemporal dementia 0002145
Grammar-specific speech disorder 0006977
Memory impairment
Forgetfulness
Memory loss
Memory problems
Poor memory
[ more ] 		0002354
Spoken Word Recognition Deficit 0030391
Temporal cortical atrophy 0007112
Thickened nuchal skin fold
Thickened skin folds of neck
Thickened skin over the neck
[ more ] 		0000474
30%-79% of people have these symptoms
Abnormal brain FDG positron emission tomography 0012658
Abnormality of the cerebral white matter 0002500
Alexia
Text blindness
Word blindness
[ more ] 		0010523
Anxiety
Excessive, persistent worry and fear
0000739
Apraxia 0002186
Depressivity
Depression
0000716
EEG with continuous slow activity 0011204
5%-29% of people have these symptoms
Abnormal lower motor neuron morphology 0002366
Astrocytosis 0002446
Dysgraphia 0010526
Motor aphasia
Loss of expressive speech
0002427
Mutism
Inability to speak
Muteness
[ more ] 		0002300
Parkinsonism 0001300
Perseveration 0030223
Personality changes
Personality change
0000751
Restlessness 0000711
Senile plaques 0100256
Percent of people who have these symptoms is not available through HPO
Abdominal aortic aneurysm 0005112
Autosomal dominant inheritance 0000006
Autosomal recessive inheritance 0000007
Multifactorial inheritance 0001426
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Last updated: 7/1/2020

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Progressive non-fluent aphasia. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Progressive non-fluent aphasia. Click on the link to view a sample search on this topic.

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