Dementia and cognitive impairment in amyotrophic lateral sclerosis: a review.
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.
Department of Neuroscience, University of Turin, Via Cherasco 15, 10126, Turin, Italy. mariateresa.giordana@unito.it
Maria Teresa
MT
Giordana
Patrizia
P
Ferrero
Silvia
S
Grifoni
Alessia
A
Pellerino
Andrea
A
Naldi
Anna
A
Montuschi
D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000235:genetics; D001526:Behavioral Symptoms / Q000175:diagnosis / Q000209:etiology; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology*; D003704:Dementia / Q000175:diagnosis* / Q000209:etiology*; D006801:Humans