1. Reading words and other people: A comparison of exception word, familiar face and affect processing in the left and right temporal variants of primary progressive aphasia.

    Cortex 82:147 (2016) PMID 27389800 PMCID PMC4969161

    Semantic variant primary progressive aphasia (svPPA) typically presents with left-hemisphere predominant rostral temporal lobe (rTL) atrophy and the most significant complaints within the language domain. Less frequently, patients present with right-hemisphere predominant temporal atrophy couple...
  2. Genetic risk factors for the posterior cortical atrophy variant of Alzheimer's disease.

    Alzheimer's & Dementia 12(8):862 (2016) PMID 26993346

    The genetics underlying posterior cortical atrophy (PCA), typically a rare variant of Alzheimer's disease (AD), remain uncertain. We genotyped 302 PCA patients from 11 centers, calculated risk at 24 loci for AD/DLB and performed an exploratory genome-wide association study. We confirm that varia...
  3. Predicting disease progression in progressive supranuclear palsy in multicenter clinical trials.

    Parkinsonism & Related Disorders 28:41 (2016) PMID 27172829 PMCID PMC4914418

    Clinical and MRI measurements can track disease progression in PSP, but many have not been extensively evaluated in multicenter clinical trials. We identified optimal measures to capture clinical decline and predict disease progression in multicenter PSP trials. Longitudinal clinical rating scal...
  4. Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration.

    JAMA Neurology 73(6):733 (2016) PMID 27111692 PMCID PMC4924620

    We provide novel evidence of specific clinical and neuroimaging features that may help for the in vivo prediction of underlying pathology in patients with nonfluent/agrammatic primary progressive aphasia (nfvPPA) and progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD) proved ...
  5. Association Between Genetic Traits for Immune-Mediated Diseases and Alzheimer Disease.

    JAMA Neurology 73(6):691 (2016) PMID 27088644 PMCID PMC4905783

    Late-onset Alzheimer disease (AD), the most common form of dementia, places a large burden on families and society. Although epidemiological and clinical evidence suggests a relationship between inflammation and AD, their relationship is not well understood and could have implications for treatm...
  6. Structural connectivity of the human anterior temporal lobe: A diffusion magnetic resonance imaging study.

    Human Brain Mapping 37(6):2210 (2016) PMID 26945805 PMCID PMC4922800

    The anterior temporal lobes (ATL) have been implicated in a range of cognitive functions including auditory and visual perception, language, semantic knowledge, and social-emotional processing. However, the anatomical relationships between the ATLs and the broader cortical networks that subserve...
  7. Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation.

    Cell 165(4):921 (2016) PMID 27114033 PMCID PMC4860138

    Microglia maintain homeostasis in the brain, but whether aberrant microglial activation can cause neurodegeneration remains controversial. Here, we use transcriptome profiling to demonstrate that deficiency in frontotemporal dementia (FTD) gene progranulin (Grn) leads to an age-dependent, progre...
  8. Tau PET patterns mirror clinical and neuroanatomical variability in Alzheimer's disease.

    Brain 139(Pt 5):1551 (2016) PMID 26962052

    SEE SARAZIN ET AL DOI101093/BRAIN/AWW041 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: The advent of the positron emission tomography tracer (18)F-AV1451 provides the unique opportunity to visualize the regional distribution of tau pathology in the living human brain. In this study, we tested the...
  9. The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry.

    Journal of Neurology, Neurosurgery & Psychiatry 87(5):501 (2016) PMID 26216940 PMCID PMC4755931

    The primary goal of this article is to critically discuss the syndromic overlap that exists between early behavioural variant frontotemporal dementia (bvFTD)--the most common clinical syndrome associated with frontotemporal lobar degeneration (FTLD)--and several primary psychiatric disorders. We...
  10. Dominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia.

    PNAS 113(17):E2430 (2016) PMID 27071080 PMCID PMC4855566

    The brain continuously influences and perceives the physiological condition of the body. Related cortical representations have been proposed to shape emotional experience and guide behavior. Although previous studies have identified brain regions recruited during autonomic processing, neurologic...
  11. Selective Frontoinsular von Economo Neuron and Fork Cell Loss in Early Behavioral Variant Frontotemporal Dementia.

    Cerebral Cortex 26(4):1843 (2016) PMID 26796215

  12. Plasma neurofilament light chain predicts progression in progressive supranuclear palsy.

    Annals of clinical and translational neurology 3(3):216 (2016) PMID 27042681 PMCID PMC4774256

    Blood-based biomarkers for neurodegenerative conditions could improve diagnosis and treatment development. Neurofilament light chain (NfL), a marker of axonal injury, is elevated in cerebrospinal fluid (CSF) of patients with progressive supranuclear palsy (PSP). The goal of this study was to det...
  13. Cognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage.

    Neurology 86(7):600 (2016) PMID 26802093 PMCID PMC4762418

    To characterize the cognitive and neuropsychiatric symptoms of patients with behavioral variant frontotemporal dementia (bvFTD) over the natural course of the disease. We examined the initial and subsequent neuropsychological test performance and neuropsychiatric symptoms in a large cohort of pa...
  14. Deep clinical and neuropathological phenotyping of Pick disease.

    Annals of Neurology 79(2):272 (2016) PMID 26583316 PMCID PMC4755803

    To characterize sequential patterns of regional neuropathology and clinical symptoms in a well-characterized cohort of 21 patients with autopsy-confirmed Pick disease. Detailed neuropathological examination using 70μm and traditional 6μm sections was performed using thioflavin-S staining and imm...
  15. Frontotemporal Dementia and Psychiatric Illness: Emerging Clinical and Biological Links in Gene Carriers.

    American Journal of Geriatric Psychiatry 24(2):107 (2016) PMID 26324540 PMCID PMC4686378

    To describe psychiatric presentations in individuals with genetic mutations causing frontotemporal dementia (FTD). Case descriptions from five carriers of FTD-related gene mutations with symptoms associated with non-neurodegenerative psychiatric disease. A comprehensive research program investig...
  16. Cross-species translation of the Morris maze for Alzheimer's disease.

    Journal of Clinical Investigation 126(2):779 (2016) PMID 26784542 PMCID PMC4731157

    Analogous behavioral assays are needed across animal models and human patients to improve translational research. Here, we examined the extent to which performance in the Morris water maze - the most frequently used behavioral assay of spatial learning and memory in rodents - translates to human...
  17. Association of traumatic brain injury with subsequent neurological and psychiatric disease: a meta-analysis.

    Journal of Neurosurgery 124(2):511 (2016) PMID 26315003 PMCID PMC4751029

    Mild traumatic brain injury (TBI) has been proposed as a risk factor for the development of Alzheimer's disease, Parkinson's disease, depression, and other illnesses. This study's objective was to determine the association of prior mild TBI with the subsequent diagnosis (that is, at least 1 year...
  18. Neuroeconomic dissociation of semantic dementia and behavioural variant frontotemporal dementia.

    Brain 139(Pt 2):578 (2016) PMID 26667277 PMCID PMC4861653

    Many neuropsychiatric disorders are marked by abnormal behaviour and decision-making, but prevailing diagnostic criteria for such behaviours are typically qualitative and often ambiguous. Behavioural variant frontotemporal dementia and semantic variant primary progressive aphasia (also called se...
  19. C9orf72 repeat expansions that cause frontotemporal dementia are detectable among patients with psychosis.

    Psychiatry Research 235:200 (2016) PMID 26723138 PMCID PMC4724461

    A pathologic hexanucleotide repeat expansion in C9orf72 causes frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). Behavioral abnormalities can also occur among mutation carriers with FTD, but it is uncertain whether such mutations occur among persons with psychoses per se. Amo...
  20. Early-onset Alzheimer's disease versus frontotemporal dementia: resolution with genetic diagnoses?

    Neurocase 22(2):161 (2016) PMID 26304661 PMCID PMC4733403

    We report a diagnostically challenging case of a 64-year-old man with a history of remote head trauma who developed mild behavioral changes and dyscalculia. He was diagnosed with clinical Alzheimer's disease (AD), with additional features consistent with behavioral variant frontotemporal dementi...