Article Text

Download PDFPDF
Curious case of FTD-ALS
  1. K A Josephs
  1. Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr K A Josephs, Mayo Clinic, Rochester, MN 55905, USA; josephs.keith{at}

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Do behavioural and cognitive deficits support FTD and ALS being on a spectrum or FTD-ALS being a unique entity?

The frontotemporal dementias (FTDs) are a group of disorders linked by presentation features combining executive, language and behavioural dysfunction. FTD includes three main clinical syndromes: behavioural variant FTD (bvFTD), semantic dementia and progressive agrammatic/non-fluent aphasia. Over the past two decades there have been significant advancements in our understanding of the biology, genetics and molecular pathology of FTD. One such advancement was the identification of TAR DNA binding protein 43 (TDP-43) in the brains of FTD patients.1 Another was the discovery that FTD can be associated with expansions in the C9ORF72 gene.2 What is intriguing is that both TDP-43 and C9ORF72 mutations are also strongly linked to another neurodegenerative disease, …

View Full Text


  • Contributors KAJ wrote the Editorial Commentary.

  • Funding The author has not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; internally peer reviewed.

Linked Articles

  • Neurodegeneration
    Jennifer A Saxon Jennifer C Thompson Jennifer M Harris Anna M Richardson Tobias Langheinrich Sara Rollinson Stuart Pickering-Brown Amina Chaouch John Ealing Hisham Hamdalla Carolyn A Young Dan Blackburn Tahir Majeed Claire Gall Matthew Jones Julie S Snowden