Frontotemporal dementia and motor neurone disease: overlapping clinic-pathological disorders

J Clin Neurosci. 2009 Sep;16(9):1131-5. doi: 10.1016/j.jocn.2009.03.005. Epub 2009 Jun 24.


Advances in genetics and pathology have supported the idea of a continuum between frontotemporal dementia (FTD) and motor neurone disease (MND), which is strengthened by the discovery of the trans-activating responsive (Tar) sequence DNA binding protein (TDP-43) as a key component in the underlying pathology of FTD, FTD-MND and sporadic and familial MND patients. MND is a multisystem disorder associated with cognitive and behavioural changes which in some instances reaches the criteria for FTD, while a proportion of patients with FTD develop frank MND. We review the overlap between FTD and MND, emphasizing areas of controversy and uncertainty.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / physiology
  • Dementia / genetics
  • Dementia / pathology*
  • Dementia / psychology
  • Humans
  • Motor Neuron Disease / genetics
  • Motor Neuron Disease / pathology*
  • Motor Neuron Disease / psychology


  • DNA-Binding Proteins