The Semantic Variant of Primary Progressive Aphasia: Clinical and Neuroimaging Evidence in Single Subjects

摘要: Background/Aim We present a clinical-neuroimaging study in series of patients with clinical diagnosis semantic variant primary progressive aphasia (svPPA), the aim to provide clinical-functional correlations cognitive and behavioral manifestations at single-subject level. Methods performed neuropsychological investigations, 18F-FDG-PET group analysis, an optimized SPM voxel-based approach, correlation analyses. A measurement white matter integrity by means diffusion tensor imaging (DTI) was also available for subgroup patients. Results Cognitive assessment confirmed presence typical memory deficits all patients, relative sparing executive, attentional, visuo-constructional, episodic domains. showed consistent pattern cerebral hypometabolism across which correlated performance tasks. In addition, majority presented disturbances associated metabolic dysfunction limbic structures. cases DTI analysis FA abnormalities inferior longitudinal uncinate fasciculi. Discussion Each svPPA individual had functional derangement involving extended, connected system within left temporal lobe, crucial part verbal network, as well involvement The latter extended beyond area atrophy shown CT scan. Conclusion Single-subject can account both alterations svPPA. This provides useful support diagnosis.