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Dementia and cognitive impairment in amyotrophic lateral sclerosis: a review

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Abstract

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.

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Acknowledgments

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

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  1. Department of Neuroscience, University of Turin, Via Cherasco 15, 10126, Turin, Italy

    Maria Teresa Giordana, Patrizia Ferrero, Silvia Grifoni, Alessia Pellerino, Andrea Naldi & Anna Montuschi

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  1. Maria Teresa Giordana
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  2. Patrizia Ferrero
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  3. Silvia Grifoni
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  4. Alessia Pellerino
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  5. Andrea Naldi
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Correspondence to Maria Teresa Giordana.

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Giordana, M.T., Ferrero, P., Grifoni, S. et al. Dementia and cognitive impairment in amyotrophic lateral sclerosis: a review. Neurol Sci 32, 9–16 (2011). https://doi.org/10.1007/s10072-010-0439-6

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