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PLoS One. 2015 Mar 10;10(3):e0120197. doi: 10.1371/journal.pone.0120197. eCollection 2015.

The semantic variant of primary progressive aphasia: clinical and neuroimaging evidence in single subjects.

Author information

1
Vita-Salute San Raffaele University and Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy.
2
Vita-Salute San Raffaele University and Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy; CERMAC, Vita-Salute San Raffaele University, Milan, Italy.
3
Istituto di Bioimmagini e Fisiologia Molecolare C.N.R., Segrate, Italy.
4
Istituto Universitario degli Studi Superiori-IUSS, Pavia, Italy.
5
Department of Clinical Neurosciences, San Raffaele Hospital, Milan, Italy.
6
IRCCS Foundation "Carlo Besta" Neurological Institute, Milan, Italy.
7
Departments of Neurology, San Raffaele Hospital, Milan, Italy.
8
Istituto Universitario degli Studi Superiori-IUSS, Pavia, Italy; CERMAC, Vita-Salute San Raffaele University, Milan, Italy.
9
Vita-Salute San Raffaele University and Division of Neuroscience, San Raffaele Scientific Institute, Milan, Italy; CERMAC, Vita-Salute San Raffaele University, Milan, Italy; Istituto di Bioimmagini e Fisiologia Molecolare C.N.R., Segrate, Italy; Nuclear Medicine Unit, San Raffaele Hospital, Milan, Italy.

Abstract

BACKGROUND/AIM:

We present a clinical-neuroimaging study in a series of patients with a clinical diagnosis of semantic variant of primary progressive aphasia (svPPA), with the aim to provide clinical-functional correlations of the cognitive and behavioral manifestations at the single-subject level.

METHODS:

We performed neuropsychological investigations, 18F-FDG-PET single-subject and group analysis, with an optimized SPM voxel-based approach, and correlation analyses. A measurement of white matter integrity by means of diffusion tensor imaging (DTI) was also available for a subgroup of patients.

RESULTS:

Cognitive assessment confirmed the presence of typical semantic memory deficits in all patients, with a relative sparing of executive, attentional, visuo-constructional, and episodic memory domains. 18F-FDG-PET showed a consistent pattern of cerebral hypometabolism across all patients, which correlated with performance in semantic memory tasks. In addition, a majority of patients also presented with behavioral disturbances associated with metabolic dysfunction in limbic structures. In a subgroup of cases the DTI analysis showed FA abnormalities in the inferior longitudinal and uncinate fasciculi.

DISCUSSION:

Each svPPA individual had functional derangement involving an extended, connected system within the left temporal lobe, a crucial part of the verbal semantic network, as well as an involvement of limbic structures. The latter was associated with behavioral manifestations and extended beyond the area of atrophy shown by CT scan.

CONCLUSION:

Single-subject 18F-FDG-PET analysis can account for both cognitive and behavioral alterations in svPPA. This provides useful support to the clinical diagnosis.

PMID:
25756991
PMCID:
PMC4354903
DOI:
10.1371/journal.pone.0120197
[Indexed for MEDLINE]
Free PMC Article

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