Progressive nonfluent aphasia
Encyclopedia
Progressive nonfluent aphasia is one of three clinical syndromes associated with frontotemporal lobar degeneration
. The major symptom is progressive difficulties with the production of speech.
, phonemic breakdown (difficulties with sounds) and other problems. However, it is rare for patients to have just one of these problems and most people will present with more than one problem. Features include:
As the disease develops, speech quantity decreases and many patients will become mute
.
Cognitive domains other than language are rarely affected early on. However, as the disease progresses other domains can be affected. Problems with writing, reading and speech comprehension can occur as can behavioural features similar to frontotemporal dementia.
- PPA) included patients with both progressive non-fluent aphasia (PNFA) and semantic dementia (SD). The Neary criteria for PNFA and SD are therefore different from the Mesulam criteria for PPA. This is further confused by some doctors using the term primary progressive aphasia to mean patients with progressive non-fluent aphasia only.
areas is seen.
Comprehensive meta-analyses on MRI and FDG-PET studies identified alterations in the whole left frontotemporal network for phonological and syntactical processing as the most consistent finding. Based on these imaging methods, progressive nonfluent aphasia can be regionally dissociated from the other subtypes of frontotemporal lobar degeneration, frontotemporal dementia and semantic dementia.
Frontotemporal lobar degeneration
-Further reading:*Hodges, John R. The Frontotemporal Dementia Syndromes. Cambridge University Press. 2007 ISBN 978-0-521-85477-1-External links:****Mayo Clinic - **...
. The major symptom is progressive difficulties with the production of speech.
Clinical features
The main clinical features are progressive difficulties with speech production. There can be problems in different parts of the speech production system, hence patients can present with articulatory breakdownManner of articulation
In linguistics, manner of articulation describes how the tongue, lips, jaw, and other speech organs are involved in making a sound. Often the concept is only used for the production of consonants, even though the movement of the articulars will also greatly alter the resonant properties of the...
, phonemic breakdown (difficulties with sounds) and other problems. However, it is rare for patients to have just one of these problems and most people will present with more than one problem. Features include:
- Hesitant, effortful speech
- Speech 'apraxiaApraxiaApraxia is a disorder caused by damage to specific areas of the cerebrum. Apraxia is characterized by loss of the ability to execute or carry out learned purposeful movements, despite having the desire and the physical ability to perform the movements...
' - Stutter (including return of a childhood stutter)
- Anomia
- Phonemic paraphasiaParaphasiaParaphasia is a feature of aphasia in which one loses the ability of speaking correctly, substitutes one word for another, and changes words and sentences in an inappropriate way. It often develops after a stroke or brain injury. The patient's speech is fluent but is error-prone, e.g...
(sound errors in speech e.g. 'gat' for 'cat') - AgrammatismAgrammatismAgrammatism is a form of expressive aphasia that refers to the inability to speak in a grammatically correct fashion. People with agrammatism may have telegraphic speech, a unique speech pattern with simplified formation of sentences , akin to that found in telegraph messages...
(using the wrong tense or word order)
As the disease develops, speech quantity decreases and many patients will become mute
Muteness
Muteness or mutism is an inability to speak caused by a speech disorder. The term originates from the Latin word mutus, meaning "silent".-Causes:...
.
Cognitive domains other than language are rarely affected early on. However, as the disease progresses other domains can be affected. Problems with writing, reading and speech comprehension can occur as can behavioural features similar to frontotemporal dementia.
Classification
There is some confusion in the terminology used by different neurologists. Mesulam's original description in 1982 of progressive language problems caused by neurodegenerative disease (which he called primary progressive aphasiaPrimary progressive aphasia
Primary progressive aphasia is a type of dementia characterized most prominently by an insidious and progressive disorder of language and speech abilities...
- PPA) included patients with both progressive non-fluent aphasia (PNFA) and semantic dementia (SD). The Neary criteria for PNFA and SD are therefore different from the Mesulam criteria for PPA. This is further confused by some doctors using the term primary progressive aphasia to mean patients with progressive non-fluent aphasia only.
Imaging
Imaging studies have shown differing results which probably represents the heterogeneity of language problems than can occur in PNFA. However, classically atrophy of left perisylvianLanguage center
The term language center refers to the areas of the brain which serve a particular function for speech processing and production.- Current scientific consensus :...
areas is seen.
Comprehensive meta-analyses on MRI and FDG-PET studies identified alterations in the whole left frontotemporal network for phonological and syntactical processing as the most consistent finding. Based on these imaging methods, progressive nonfluent aphasia can be regionally dissociated from the other subtypes of frontotemporal lobar degeneration, frontotemporal dementia and semantic dementia.
See also
- Frontotemporal lobar degenerationFrontotemporal lobar degeneration-Further reading:*Hodges, John R. The Frontotemporal Dementia Syndromes. Cambridge University Press. 2007 ISBN 978-0-521-85477-1-External links:****Mayo Clinic - **...
- Frontotemporal dementiaFrontotemporal dementiaFrontotemporal dementia is a clinical syndrome caused by degeneration of the frontal lobe of the brain and may extend back to the temporal lobe...
- Semantic dementiaSemantic dementiaSemantic dementia is a progressive neurodegenerative disorder characterized by loss of semantic memory in both the verbal and non-verbal domains...
- Pick's diseasePick's diseasePick's disease, is a rare neurodegenerative disease that causes progressive destruction of nerve cells in the brain. Symptoms include loss of speech , and dementia. While some of the symptoms can initially be alleviated, the disease progresses and patients often die within two to ten years...
- Alzheimer's diseaseAlzheimer's diseaseAlzheimer's disease also known in medical literature as Alzheimer disease is the most common form of dementia. There is no cure for the disease, which worsens as it progresses, and eventually leads to death...
- Corticobasal degenerationCorticobasal degenerationCorticobasal degeneration or Corticobasal Ganglionic Degeneration is a rare progressive neurodegenerative disease involving the cerebral cortex and the basal ganglia. It is characterized by marked disorders in movement and cognitive dysfunction...
- Primary progressive aphasiaPrimary progressive aphasiaPrimary progressive aphasia is a type of dementia characterized most prominently by an insidious and progressive disorder of language and speech abilities...
External links
- FTD information and videos from the UCSF Memory and Aging Center
- FTD Info from the Mayo ClinicMayo ClinicMayo Clinic is a not-for-profit medical practice and medical research group specializing in treating difficult patients . Patients are referred to Mayo Clinic from across the U.S. and the world, and it is known for innovative and effective treatments. Mayo Clinic is known for being at the top of...
- The Association for Frontotemporal Dementias