Frontotemporal Lobar Degeneration

13Citations
Citations of this article
31Readers
Mendeley users who have this article in their library.

You may have access to this PDF.

Abstract

Frontotemporal Lobar Degeneration (FTLD) is an heterogeneous neurodegenerative disorder characterized by behaviour and language disturbances, associated with degeneration of the frontal and temporal lobes. Three different clinical presentations have been described, namely behavioural variant Frontotemporal Dementia (bvFTD), Semantic Dementia (SD) and Progressive Non-Fluent Aphasia (PNFA). The associated histopathology includes different neuropathological hallmarks, the most frequent being tau-positive inclusions (FTLD-TAU) or tau-negative and TDP-43 positive inclusions (FTLD-TDP). The majority of familial FTLD cases are caused by mutations within Microtubule-Associated Protein Tau (MAPT) gene, leading to FTLD-TAU, or Progranulin (PGRN) gene, leading to FTLD-TDP. In the last few years, imaging, biological and genetic biomarkers have been developed, helping in clinical evaluation and diagnostic accuracy. Though current pharmacologic interventions are only symptomatic, recent research argues for possible disease-modifying strategies in the near future. © 2012 Landes Bioscience and Springer Science+Business Media.

Cite

CITATION STYLE

APA

Premi, E., Padovani, A., & Borroni, B. (2012). Frontotemporal Lobar Degeneration. Advances in Experimental Medicine and Biology, 724, 114–127. https://doi.org/10.1007/978-1-4614-0653-2_9

Register to see more suggestions

Mendeley helps you to discover research relevant for your work.

Already have an account?

Save time finding and organizing research with Mendeley

Sign up for free