What are the three variants of PPA?
Three variants of PPA are now recognized: semantic variant, logopenic variant, and nonfluent/agrammatic variant. We discuss recent work characterizing the neurolinguistic, neuropsychological, imaging and pathologic profiles associated with these variants.
Does surface dyslexia dysgraphia relate to semantic deficits in the semantic variant of primary progressive aphasia?
Results showed that sv-PPA patients are impaired at reading and writing irregular words, and that they have impaired performance in both the semantic and the lexical task. Reading/writing scores with irregulars correlated significantly with performance in the lexical but not the semantic task.
Is primary progressive aphasia fluent or nonfluent?
Lopogenic progressive aphasia may cause difficulty finding the right words or understanding others. Progressive non-fluent aphasia may cause poor grammar or difficulty talking fluently. Semantic dementia may cause difficulty naming objects or understanding the meaning of stand-alone words.
What does the word Logopenic mean?
The term Logopenic comes from a combination of two ancient Greek words: logo meaning speech and penic meaning ‘lack of’. Progressive refers to the fact that the disease gets worse over time. Aphasia is a word that also refers to changes in a person’s speech. LPA is one of three language based dementias.
Can MRI visual assessment differentiate the variants of primary progressive aphasia?
MR imaging was least sensitive for the diagnosis of nonfluent-variant primary-progressive aphasia. Intrarater agreement analysis showed mean κ values above the widely accepted threshold of 0.6 (mean, 0.63 ± 0.16).
How rare is primary progressive aphasia?
PPA is classified as a rare disease, however, many may be undiagnosed since they might not seek medical help or be misdiagnosed due to the unfamiliarity with PPA. 3 Interestingly, about twice as many men than women develop PPA. The average age of onset is between 50 and 70.
Can PPA be misdiagnosed?
Neuroimaging signatures may also be discriminative in differential diagnosis. We present two cases in which PPA was initially misdiagnosed, and in which misinterpretation of neuroimaging contributed to delayed diagnosis.
How long do you live with primary progressive aphasia?
The typical life expectancy from onset of the disease is 3 to 12 years. 9 Often, complications from PPA, such as swallowing difficulties, often lead to the eventual decline.
Is primary progressive aphasia hereditary?
In the vast majority of individuals, PPA is not genetic. However, in a small number of families, PPA can be caused by hereditary forms of FTLD. The most common gene implicated in these families is the progranulin gene (GRN).
Does PPA show on MRI?
Patients with a rare neurodegenerative brain disorder called Primary Progressive Aphasia, or PPA, show abnormalities in brain function in areas that look structurally normal on an MRI scan.
Does aphasia show up on MRI?
Magnetic resonance imaging (MRI) scans can help diagnose primary progressive aphasia, detect shrinking of certain areas of the brain and show which area of the brain might be affected. MRI scans can also detect strokes, tumors or other conditions that affect brain function.
What is the life expectancy for someone with PPA?