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Front Neurol. 2018 Nov 22;9:1005. doi: 10.3389/fneur.2018.01005. eCollection 2018.

Clinical and Radiological Markers of Extra-Motor Deficits in Amyotrophic Lateral Sclerosis.

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First Department of Neurology, Aeginition Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Second Department of Radiology, University General Hospital Attikon, National and Kapodistrian University of Athens, Athens, Greece.
Computational Neuroimaging Group, Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland.


Amyotrophic lateral sclerosis (ALS) is now universally recognized as a complex multisystem disorder with considerable extra-motor involvement. The neuropsychological manifestations of frontotemporal, parietal, and basal ganglia involvement in ALS have important implications for compliance with assistive devices, survival, participation in clinical trials, caregiver burden, and the management of individual care needs. Recent advances in neuroimaging have been instrumental in characterizing the biological substrate of heterogeneous cognitive and behavioral deficits in ALS. In this review we discuss the clinical and radiological aspects of cognitive and behavioral impairment in ALS focusing on the recognition, assessment, and monitoring of these symptoms.


amyotrophic lateral sclerosis; behavior; cognition; extra-motor involvement; neuroimaging; neuropsychological deficits

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