Neurological Sciences

, Volume 32, Issue 1, pp 9–16 | Cite as

Dementia and cognitive impairment in amyotrophic lateral sclerosis: a review

  • Maria Teresa Giordana
  • Patrizia Ferrero
  • Silvia Grifoni
  • Alessia Pellerino
  • Andrea Naldi
  • Anna Montuschi
Review Article


Amyotrophic lateral sclerosis (ALS) is generally considered to be a paradigm of pure motor neuron disorder; nevertheless, the possible occurrence of cognitive impairment up to a frank dementia in patients affected by ALS is recognized. The appraisal of the cognitive impairment in ALS patients is crucial not only to the therapeutic trials of this incurable disease, but also to the planning of care, compliance to interventions, the end-of-life decisions. The cognitive/behavioral changes of ALS patients are consistent with frontotemporal dysfunctions; the overlap of neuropathological features of ALS and frontotemporal lobe degeneration (FTLD) supports, in addition, the putative spectrum of ALS and FTD. In the present review, the pertinent clinical, genetic, neuropathological, neuropsychological and neuroimaging data of the literature are comprehensively and critically discussed. The distinct and overlapping features of ALS and FTD are pointed out, as well as the undisclosed questions deserving additional studies.


ALS FTD Dementia Neuropathology TDP-43 Genetics Neuropsychological evaluation 



Supported by Compagnia di San Paolo, Torino, Grant Grant 2004.1424, and Regione Piemonte, Ricerca Sanitaria Finalizzata, Grant 466.2008.


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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Maria Teresa Giordana
    • 1
  • Patrizia Ferrero
    • 1
  • Silvia Grifoni
    • 1
  • Alessia Pellerino
    • 1
  • Andrea Naldi
    • 1
  • Anna Montuschi
    • 1
  1. 1.Department of NeuroscienceUniversity of TurinTurinItaly

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