Frontotemporal Lobar Degeneration

  • Enrico Premi
  • Alessandro Padovani
  • Barbara BorroniEmail author
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 724)


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.


Amyotrophic Lateral Sclerosis Neurodegenerative Disease Neurol Neurosurg Psychiatry Motor Neuron Disease Semantic Dementia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Landes Bioscience and Springer Science+Business Media 2012

Authors and Affiliations

  • Enrico Premi
    • 1
  • Alessandro Padovani
    • 1
  • Barbara Borroni
    • 1
    Email author
  1. 1.Centre for Ageing Brain and Neurodegenerative Disorders, Neurology UnitUniversity of BresciaBresciaItaly

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