What causes Logopenic PPA?
Primary progressive aphasia is caused by a shrinking (atrophy) of certain sections (lobes) of the brain responsible for speech and language. In this case, the frontal, temporal or parietal lobes, primarily on the left side of the brain, are affected.
Is Logopenic PPA genetic?
Logopenic PPA can be sporadic, familial or hereditary. The majority of cases are not hereditary.
What is logopenic variant?
The logopenic variant has emerged as a distinct clinical subtype of primary progressive aphasia with a unique cognitive-linguistic, anatomical and, most likely, neuropathological profile. Research to date has clarified the clinical phenotype, suggesting a prominent impairment of phonological memory.
What does the word logopenic mean?
Disease definition. Logopenic progressive aphasia (lv-PPA) is a form of primary progressive aphasia (PPA; see this term), characterized by impaired single-word retrieval and naming and impaired repetition with spared single-word comprehension and object knowledge.
How fast does PPA progress?
Although it is often said that the course of the illness progresses over approximately 7–10 years from diagnosis to death, recent studies suggest that some forms of PPA may be slowly progressive for 12 or more years (Hodges et al. 2010), with reports of up to 20 years depending on how early a diagnosis is made.
What are the final stages of PPA?
All forms of PPA
- heavily reduced or unintelligible speech.
- difficulty understanding other people (both with spoken and written information)
- increased difficulty making complex decisions (around finances and money, for example)
- difficulty with judgment, planning and concentration, affecting activities such as driving.
When is PPA used?
“Aphasia” is a general term used to refer to deficits in language functions. PPA is caused by degeneration in the parts of the brain that are responsible for speech and language. PPA begins very gradually and initially is experienced as difficulty thinking of common words while speaking or writing.
How long does someone live with primary progressive aphasia?
People who have the disease typically live about 3-12 years after they are originally diagnosed. In some people, difficulty with language remains the primary symptom, while others may develop additional problems including cognitive or behavioral changes or difficulty coordinating movements.
Is PPA inherited?
About 40-50% of people with primary progressive aphasia (PPA) have other family members who are also affected by the disease. When PPA is caused by a genetic change ( mutations or pathogenic variants) in the GRN gene , it is inherited in an autosomal dominant manner.
What are final stages of PPA?
All forms of PPA heavily reduced or unintelligible speech. difficulty understanding other people (both with spoken and written information) increased difficulty making complex decisions (around finances and money, for example) difficulty with judgment, planning and concentration, affecting activities such as driving.
How long can people live with PPA?
People who have the disease typically live about 3-12 years after they are originally diagnosed.
What causes death in PPA patients?
Although PPA itself is a life-shortening condition, people with PPA will often be affected by another illness, such as pneumonia. This is because PPA affects how the body copes with infection and with other physical problems. Pneumonia is the cause of death in up to two thirds of people with a dementia.
