Clinical course of primary progressive aphasia: clinical and FDG-PET patterns

Primary progressive aphasia (PPA) may be the onset of several neurodegenerative diseases. This study evaluates a cohort of patients with PPA to assess their progression to different clinical syndromes, associated factors that modulate this progression, and patterns of cerebral metabolism linked to different clinical evolutionary forms. Thirty-five patients meeting PPA criteria underwent a clinical and neuroimaging (18)F-Fluorodeoxyglucose PET evaluation. Survival analysis was performed using time from clinical onset to the development of a non-language symptom or deficit (PPA-plus). Cerebral metabolism was analyzed using Statistical Parametric Mapping. Patients classified into three PPA variants evolved to atypical parkinsonism, behavioral disorder and motor neuron disease in the agrammatic variant; to behavioral disorder in the semantic; and to memory impairment in the logopenic. Median time from the onset of symptoms to PPA-plus was 36 months (31-40, 95 % confidence interval). Right laterality, and years of education were associated to a lower risk of progression, while logopenic variant to a higher risk. Different regions of hypometabolism were identified in agrammatic PPA with parkinsonism, motor neuron disease and logopenic PPA-plus. Clinical course of PPA differs according to each variant. Left anterior temporal and frontal medial hypometabolism in agrammatic variant is linked to motor neuron disease and atypical parkinsonism, respectively. PPA variant, laterality and education may be associated to the risk of progression. These results suggest the possibility that clinical and imaging data could help to predict the clinical course of PPA.