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)
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...
Genetic risk factors for the posterior cortical atrophy variant of Alzheimer's disease.
Alzheimer's & Dementia 12(8):862 (2016)
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...
Predicting disease progression in progressive supranuclear palsy in multicenter clinical trials.
Parkinsonism & Related Disorders 28:41 (2016)
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...
Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration.
JAMA Neurology 73(6):733 (2016)
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 ...
Association Between Genetic Traits for Immune-Mediated Diseases and Alzheimer Disease.
JAMA Neurology 73(6):691 (2016)
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...
Structural connectivity of the human anterior temporal lobe: A diffusion magnetic resonance imaging study.
Human Brain Mapping 37(6):2210 (2016)
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...
Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation.
Cell 165(4):921 (2016)
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...
Tau PET patterns mirror clinical and neuroanatomical variability in Alzheimer's disease.
Brain 139(Pt 5):1551 (2016)
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...
The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry.
Journal of Neurology, Neurosurgery & Psychiatry 87(5):501 (2016)
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...
Dominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia.
PNAS 113(17):E2430 (2016)
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...
Selective Frontoinsular von Economo Neuron and Fork Cell Loss in Early Behavioral Variant Frontotemporal Dementia.
Cerebral Cortex 26(4):1843 (2016)
Plasma neurofilament light chain predicts progression in progressive supranuclear palsy.
Annals of clinical and translational neurology 3(3):216 (2016)
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...
Cognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage.
Neurology 86(7):600 (2016)
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...
Deep clinical and neuropathological phenotyping of Pick disease.
Annals of Neurology 79(2):272 (2016)
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...
Frontotemporal Dementia and Psychiatric Illness: Emerging Clinical and Biological Links in Gene Carriers.
American Journal of Geriatric Psychiatry 24(2):107 (2016)
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...
Cross-species translation of the Morris maze for Alzheimer's disease.
Journal of Clinical Investigation 126(2):779 (2016)
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...
Association of traumatic brain injury with subsequent neurological and psychiatric disease: a meta-analysis.
Journal of Neurosurgery 124(2):511 (2016)
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...
Neuroeconomic dissociation of semantic dementia and behavioural variant frontotemporal dementia.
Brain 139(Pt 2):578 (2016)
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...
C9orf72 repeat expansions that cause frontotemporal dementia are detectable among patients with psychosis.
Psychiatry Research 235:200 (2016)
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...
Early-onset Alzheimer's disease versus frontotemporal dementia: resolution with genetic diagnoses?
Neurocase 22(2):161 (2016)
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...