title: Frontotemporal networks and behavioral symptoms in primary progressive aphasia.
abstract: To determine if behavioral symptoms in patients with primary progressive aphasia (PPA) were associated with degeneration of a ventral frontotemporal network.
  
  
  
  We used diffusion tensor imaging tractography to quantify abnormalities of the uncinate fasciculus that connects the anterior temporal lobe and the ventrolateral frontal cortex. Two additional ventral tracts were studied: the inferior fronto-occipital fasciculus and the inferior longitudinal fasciculus. We also measured cortical thickness of anterior temporal and orbitofrontal regions interconnected by these tracts. Thirty-three patients with PPA and 26 healthy controls were recruited.
  
  
  
  In keeping with the PPA diagnosis, behavioral symptoms were distinctly less prominent than the language deficits. Although all 3 tracts had structural pathology as determined by tractography, significant correlations with scores on the Frontal Behavioral Inventory were found only for the uncinate fasciculus. Cortical atrophy of the orbitofrontal and anterior temporal lobe cortex was also correlated with these scores.
  
  
  
  Our findings indicate that damage to a frontotemporal network mediated by the uncinate fasciculus may underlie the emergence of behavioral symptoms in patients with PPA.
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France. m.catani@iop.kcl.ac.uk lucio.d'anna@kcl.ac.uk.
authors.forename: Lucio
authors.initials: L
authors.lastname: D'Anna
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Marsel M
authors.initials: MM
authors.lastname: Mesulam
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Thiebaut de Schotten
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Flavio
authors.initials: F
authors.lastname: Dell'Acqua
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Declan
authors.initials: D
authors.lastname: Murphy
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Christina
authors.initials: C
authors.lastname: Wieneke
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Adam
authors.initials: A
authors.lastname: Martersteck
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Derin
authors.initials: D
authors.lastname: Cobia
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France.
authors.forename: Emily
authors.initials: E
authors.lastname: Rogalski
authors.affiliation: From Natbrainlab, Department of Forensic and Neurodevelopmental Sciences (L.D., M.T.d.S., F.D., M.C.), Department of Neuroimaging (F.D.), and Sackler Institute of Translational Neurodevelopment (D.M.), Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, UK; Neurology Clinic, Department of Experimental and Clinical Medical Sciences (L.D.), University of Udine Medical School; Department of Neurosciences (L.D.), "S. Maria della Misericordia" University Hospital, Udine, Italy; Cognitive Neurology and Alzheimer's Disease Center (M.M.M., C.W., A.M., D.C., E.R.) and Department of Neurology (M.M.M., A.M., D.C.), Northwestern University Feinberg School of Medicine, Chicago, IL; and Brain Connectivity and Behaviour, Brain and Spine Institute (M.T.d.S.), CNRS UMR 7225 INSERM-UPMC UMRS 1127 La Salpêtrière, Paris, France. m.catani@iop.kcl.ac.uk lucio.d'anna@kcl.ac.uk.
authors.forename: Marco
authors.initials: M
authors.lastname: Catani
mesh_terms: D000368:Aged; D018888:Aphasia, Primary Progressive / Q000000981:diagnostic imaging* / Q000523:psychology; D001284:Atrophy; D001526:Behavioral Symptoms / Q000000981:diagnostic imaging*; D038524:Diffusion Magnetic Resonance Imaging; D056324:Diffusion Tensor Imaging; D005260:Female; D005625:Frontal Lobe / Q000000981:diagnostic imaging*; D006801:Humans; D007807:Language Tests; D008032:Limbic System / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D009434:Neural Pathways / Q000000981:diagnostic imaging; D009778:Occipital Lobe / Q000000981:diagnostic imaging*; D009929:Organ Size; D012720:Severity of Illness Index; D013702:Temporal Lobe / Q000000981:diagnostic imaging*; D066127:White Matter / Q000000981:diagnostic imaging