Primary progressive aphasia
Encyclopedia
Primary progressive aphasia (PPA) is a type of dementia
characterized most prominently by an insidious and progressive disorder of language and speech abilities. It was first described as a distinct syndrome by Mesulam in 1982.
(FTLD) spectrum of disorders, although Mesulam in his reviews has acknowledged that one third of the cases have an underlying Alzheimer pathology. In the classical Mesulam criteria for PPA there are 2 variants: a non-fluent type (progressive non-fluent aphasia or PNFA) and a fluent aphasia. However, recent work has suggested that the underlying cognitive impairment in patients with progressive fluent aphasias is loss of semantic knowledge (semantic dementia
or SD). In the consensus criteria for FTLD described by Neary, et al. in 1989, PNFA and SD are the two classifications used.
The San Francisco group have recently suggested that there is a third variant of PPA (so-called logopaenic progressive aphasia). However, there are limited descriptions of this disorder in the medical literature, and early reports suggest that it is an atypical form of Alzheimer's disease rather than a disease falling into the frontotemporal lobar degeneration spectrum.
could be a risk factor for PPA in men. These results have yet to be replicated elsewhere.
PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration
, including PPA, are at slightly greater risk of developing PPA or another form of the condition.
off-label, an anti-TNF
treatment strategy also used for Alzheimer's, was recently reported. A video depicting the patient's improvement was published in conjunction with the print article. These findings have not been independently replicated, and remain controversial.
Dementia
Dementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...
characterized most prominently by an insidious and progressive disorder of language and speech abilities. It was first described as a distinct syndrome by Mesulam in 1982.
Diagnosis Criteria
The following diagnosis criteria were defined by Mesulam- Gradual impairment of word-processing, object naming, syntax and word-processing
- Premorbid language function is usually intact
- Alcalculia - inability to perform simple mathematical calculations
- Ideomotor ApraxiaIdeomotor apraxiaIdeomotor Apraxia, often IMA, is a neurological disorder characterized by the inability to correctly imitate hand gestures and voluntarily pantomime tool use, e.g. pretend to brush one's hair. The ability to spontaneously use tools, such as brushing one's hair in the morning without being...
- inability to perform pantinome movements
Classification
There is considerable controversy over the nosology (classification) of this disorder. Clinical and pathological overlap have led it to being considered as part of the 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 - **...
(FTLD) spectrum of disorders, although Mesulam in his reviews has acknowledged that one third of the cases have an underlying Alzheimer pathology. In the classical Mesulam criteria for PPA there are 2 variants: a non-fluent type (progressive non-fluent aphasia or PNFA) and a fluent aphasia. However, recent work has suggested that the underlying cognitive impairment in patients with progressive fluent aphasias is loss of semantic knowledge (semantic dementia
Semantic dementia
Semantic dementia is a progressive neurodegenerative disorder characterized by loss of semantic memory in both the verbal and non-verbal domains...
or SD). In the consensus criteria for FTLD described by Neary, et al. in 1989, PNFA and SD are the two classifications used.
The San Francisco group have recently suggested that there is a third variant of PPA (so-called logopaenic progressive aphasia). However, there are limited descriptions of this disorder in the medical literature, and early reports suggest that it is an atypical form of Alzheimer's disease rather than a disease falling into the frontotemporal lobar degeneration spectrum.
Risk
There are no known environmental risk factors for the progressive aphasias. However, one observational study has recently suggested that vasectomyVasectomy
Vasectomy is a surgical procedure for male sterilization and/or permanent birth control. During the procedure, the vasa deferentia of a man are severed, and then tied/sealed in a manner such to prevent sperm from entering into the seminal stream...
could be a risk factor for PPA in men. These results have yet to be replicated elsewhere.
PPA is not considered a hereditary disease. However, relatives of a person with any form of frontotemporal lobar degeneration
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 - **...
, including PPA, are at slightly greater risk of developing PPA or another form of the condition.
Treatment
There is no approved treatment. Rapid and sustained improvement in speech and dementia in a patient with primary progressive aphasia utilizing perispinal etanerceptEtanercept
Etanercept is a drug that treats autoimmune diseases by interfering with the tumor necrosis factor by acting as a TNF inhibitor. Pfizer describes in a SEC filing that the drug is used to treat rheumatoid, juvenile rheumatoid and psoriatic arthritis, plaque psoriasis and ankylosing spondylitis...
off-label, an anti-TNF
Tumor necrosis factor
Tumor necrosis factor is a cytokine involved in systemic inflammation and is a member of a group of cytokines that stimulate the acute phase reaction...
treatment strategy also used for Alzheimer's, was recently reported. A video depicting the patient's improvement was published in conjunction with the print article. These findings have not been independently replicated, and remain controversial.
External links
- FAQ on PPA from IMPPACT, the International PPA Connection
- PPA information from the UCSF Memory and Aging Center
- Contains the published video documenting rapid improvement in PPA following perispinal etanercept.
- http://www.brain.northwestern.edu/ppa/index.html from the Northwestern Cognitive Neurology and Alzheimer's Disease Center
- http://www.ftd-picks.org/ Association for Frontotemporal Dementia