Frontotemporal lobar degeneration (FTLD) comprises two main classes of neurodegenerative diseases characterized by neuronal/glial proteinaceous inclusions (i.e., proteinopathies) including tauopathies (i.e., FTLD-Tau) and TDP-43 proteinopathies (i.e., FTLD-TDP) while other very rare forms of FTLD are known such as FTLD with FUS pathology (FTLD-FUS). This review focuses mainly on FTLD-Tau and FLTD-TDP, which may present as several clinical syndromes: a behavioral/dysexecutive syndrome (behavioral variant frontotemporal dementia); language disorders (primary progressive aphasia variants); and motor disorders (amyotrophic lateral sclerosis, cortico-basal syndrome, progressive supranuclear palsy syndrome). There is considerable heterogeneity in clinical presenta-tions of underlying neuropathology and current clinical criteria do not reliably predict underlying proteinopathies ante-mortem. In contrast, molecular etiologies of hereditary FTLD are consistently associated with speciﬁc proteinopa-thies. These include MAPT mutations with FTLD-Tau and GRN, C9orf72, VCP and TARDBP with FTLD-TDP. The last decade has seen a rapid expansion in our knowledge of the molecular pathologies associated with this clinically and neuropathologically heterogeneous group of FTLD dis-eases. Moreover, in view of current limitations to reliably diagnose speciﬁc FTLD neuropathologies prior to autopsy, we summarize the current state of the science in FTLD biomarker research including neuroimaging, bioﬂuid and genetic analyses. We propose that combining several of these biomarker modalities will improve diagnostic speci-ﬁcity in FTLD through a personalized medicine approach. The goals of these efforts are to enhance power for clini-cal trials focused on slowing or preventing progression of spread of tau, TDP-43 and other FTLD-associated patholo-gies and work toward the goal of deﬁning clinical endophe-notypes of FTD.
FTD Caregiver, Jamie Arking, shares the story of his father's experience with Frontotemporal degeneration (FTD).
FTD Caregiver, Sandy Karger, shares the story of her husband's experience with Frontotemporal degeneration (FTD).
FTD Caregiver, Diane Fehon, shares the story of her husband's experience with Frontotemporal degeneration (FTD).
Frontotemporal degeneration (FTD) is a debilitating neurodegenerative disease that dramatically affects the lives of both the patient and their loved ones. Hear the stories of three dedicated FTD caregivers and their experience with the Penn Frontotemporal Degeneration Center.
William Reiter of the Alzheimer’s Association Delaware Valley Chapter wrapped up the conference with a presentation on community resources.
Matthew Sharp, MSS of the Association for Frontotemporal Degeneration (AFTD) discusses the importance of FTD Advocacy.
Paul L. Feldman, a founding partner of the law firm of Feldman and Feldman, stressed that it is never too early to talk about and plan for the future.
The Penn FTD Center's Lauren Massimo, PhD, CRNP discusses decision making in advanced illness, focussing on interventions to promote quality of life
Dr. Corey McMillan discusses the importance of biomarkers.
Dr. David Irwin of the Penn FTD Center discusses how improving the ability to distinguish between FTD subtypes is crucial to both clinical treatment and research
Diane Fehon talks about her FTD caregiving journey
Murray Grossman, MD, EdD Intrdoces the premiere of Through the Eyes of the Caregiver: The Penn Frontotemporal Degeneration (FTD) Center
Alison Lynn, MSW, LSW of the Penn Memory Center discusses how the diagnosis of neurodegenerative disease in a loved one impacts children and teens.
Kaylee Faulkner Naczi, MS, CGC of the Penn Telegenetics Program educates the audience about the genetics of FTD
Dr. Roy Hamilton discusses strategies for improving Primary Progressive Aphasia
Anna Yung, BSN, RN presents practical strategies for managing symptoms at home
Dr. H. Branch Coslett from the Penn Neuroscience Center presents on Corticobasal Syndrome & Progressive Supranuclear Palsy
Dr. Colin Quinn who treats patients at the Veteran's Association and the Penn Neuroscience Center presents on Amyotrophic Lateral Sclerosis
Dr. Murray Grossman presents an overview of behavioral-variant Frontotemporal Degeneration (bvFTD) and Primary Progressive Aphasia (PPA).
Murray Grossman, MD, EdD Penn Frontotemporal Degeneration Center
Susan L-J Dickinson, MS, CGC Association for Frontotemporal Degeneration
William Reiter Alzheimer’s Association, Delaware Valley Chapter
Kathryn Jedrziewski, PhD Institute on Aging, University of Pennsylvania