Abstract
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.
Highlights
Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease (MND), a progressive neurodegenerative condition defined by concomitant lower and upper motor neuron degeneration [1]
Mentioned in early descriptions of ALS [3, 4], cognitive and behavioral deficits and frank dementia were previously considered atypical of ALS
While hexanucleotide repeat expansions in C9ORF72 are often associated with ALS-frontotemporal dementia (FTD) [14], extra-motor symptoms are not unique to this mutation and extra-motor neuroimaging findings can be readily identified in a significant proportion of C9 negative patients [15, 16]
Summary
Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease (MND), a progressive neurodegenerative condition defined by concomitant lower and upper motor neuron degeneration [1]. The identification of TAR DNA-binding protein 43 (TDP-43) positive ubiquitinated cytoplasmic inclusions in almost all patients with ALS and more than half of patients with frontotemporal dementia (FTD) has placed ALS on the so-called “ALS-FTD continuum,” highlighting the considerable clinical, pathophysiological, and neuroimaging overlap between the two neurodegenerative conditions [2]. Mentioned in early descriptions of ALS [3, 4], cognitive and behavioral deficits and frank dementia were previously considered atypical of ALS. It is not until the end of the twentieth century that clinical and research interest shifted to the extra-motor features of ALS and it has been gradually recognized as a genuine multisystem disease [5,6,7,8]. ALS is crucial due to its impact on functional decline [17], survival [18], compliance with assistive devices [19], decisionmaking, and engagement in end-of-life and legal decisions [20]
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