This application combines cutting-edge clinical phenotyping, imaging, pathology, and genetics to move the FID and dementia field forward. With this renewal we will test and, if indicated, modify new international research criteria for bvFTD and PPA developed in this PPG, and determine the added value of imaging and other biomarkers for diagnosis. While establishing parameters for recognizing bvFTD, PPA, CBS, and PSPS patients with AD versus FTLD pathology, we will improve antemortem recognition of molecular subtypes that are associated with FID, CBS, and PSP-S. Imaging is a focus in renewal, and multimodality imaging and ICN fMRI will be used to detect early changes in FTD and facilitate diagnosis. We believe that identifying molecular subtypes in vivo will be improved by the use of biomarkers from CSF and genetics. These new approaches come at a time when preventive and disease-modifying clinical trials are beginning in FTD and related disorders, trials that will require knowledge of the underlying molecular etiology for each clinical syndrome. While improving differential diagnosis, we hope to further understanding of the puzzling and troubling socio-emotional, executive control, and language problems that occur with FTD. In our first eight years we developed new cognitive and emotional tasks in Core A and in Dr. Levenson's Emotion Project 3. In Project 4, Drs. Miller and Gorno-Tempini employed novel and sophisticated approaches to studying different components of behavior in bvFTD and language in PPA. Project 5 developed tasks that probe executive control and self-awareness. We will continue to study these tasks for their underlying anatomy and diagnostic value. Beyond their relevance to patients with dementia, the tasks used in the PPG offer a new way to probe conditions with anatomy that overlaps with FTD, including autism, attention deficit disorder, schizophrenia, bipolar illness, obsessive-compulsive disorder and dyslexia.
The Program will benefit the public health by advancing the understanding of front temporal dementia and identify the best diagnostic tools and potentially uncover treatment targets. If successful, this work could accelerate the search for new therapies for prevalent age-related neurodegnerative diseases.
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| Rojas, Julio C; Stephens, Melanie L; Rabinovici, Gil D et al. (2018) Multiproteinopathy, neurodegeneration and old age: a case study. Neurocase 24:1-6 |
| Dijkstra, Anke A; Lin, Li-Chun; Nana, Alissa L et al. (2018) Von Economo Neurons and Fork Cells: A Neurochemical Signature Linked to Monoaminergic Function. Cereb Cortex 28:131-144 |
| Mok, Sue-Ann; Condello, Carlo; Freilich, Rebecca et al. (2018) Mapping interactions with the chaperone network reveals factors that protect against tau aggregation. Nat Struct Mol Biol 25:384-393 |
| Erkkinen, Michael G; Zúñiga, Raquel Gutiérrez; Pardo, Cristóbal Carnero et al. (2018) Artistic Renaissance in Frontotemporal Dementia. JAMA 319:1304-1306 |
| Schneider, Raphael; McKeever, Paul; Kim, TaeHyung et al. (2018) Downregulation of exosomal miR-204-5p and miR-632 as a biomarker for FTD: a GENFI study. J Neurol Neurosurg Psychiatry 89:851-858 |
| Ljubenkov, Peter A; Staffaroni, Adam M; Rojas, Julio C et al. (2018) Cerebrospinal fluid biomarkers predict frontotemporal dementia trajectory. Ann Clin Transl Neurol 5:1250-1263 |
| La Joie, Renaud; Bejanin, Alexandre; Fagan, Anne M et al. (2018) Associations between [18F]AV1451 tau PET and CSF measures of tau pathology in a clinical sample. Neurology 90:e282-e290 |
| Kim, Eun-Joo; Brown, Jesse A; Deng, Jersey et al. (2018) Mixed TDP-43 proteinopathy and tauopathy in frontotemporal lobar degeneration: nine case series. J Neurol 265:2960-2971 |
| Henry, Maya L; Hubbard, H Isabel; Grasso, Stephanie M et al. (2018) Retraining speech production and fluency in non-fluent/agrammatic primary progressive aphasia. Brain 141:1799-1814 |
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