Abstract
It is now well recognized that, in addition to motor impairment, amyotrophic lateral sclerosis (ALS) may cause extra-motor clinical signs and symptoms. These can include the alteration of certain cognitive functions, impaired social cognition, and changes in the perception and processing of emotions. Where these extra-motor manifestations occur in ALS, they usually do so from disease onset. In about 10% of cases, the cognitive and behavioral changes meet the diagnostic criteria for frontotemporal dementia. The timecourse of behavioral and cognitive involvement in ALS is unclear. Whereas longitudinal studies have failed to show cognitive decline over time, some cross-sectional studies have demonstrated poorer cognitive performances in the advanced stages of the disease. Neuroimaging studies show that in ALS, extra-motor signs and symptoms are associated with specific brain lesions, but little is known about how they change over time. Finally, patients with ALS appear less depressed than might be expected, given the prognosis. Moreover, many patients achieve satisfactory psychosocial adjustment throughout the course of the disease, regardless of their degree of motor disability. There are scant longitudinal data on extra-motor impairment in ALS, and to our knowledge, no systematic review on this subject has yet been published. Even so, a better understanding of patients’ clinical trajectory is essential if they are to be provided with tailored care and given the best possible support. We therefore undertook to review the evidence for extra-motor changes and their time course in ALS, in both the cognitive, emotional and psychological domains, with a view to identifying mechanisms that may help these patients cope with their disease.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of upper and lower motor neurons
We focused on amyotrophic lateral sclerosis (ALS) cases that do not meet the clinical criteria for dementia, as ALS-dementia syndromes raise different issues, and may not be as similar to ALS without dementia as is usually claimed (Lulé et al, 2018)
Menke et al (2018) assessed brain changes in patients with ALS over 2 years and reported widespread changes in both white and gray matter in the cingulate gyrus, thalami, caudate nuclei, pallidum, hippocampi and parahippocampal gyri, and insula. These results indicate that over time, cerebral changes extend into extra-motor areas, but it remains difficult to draw a link between these changes and clinical cognitive implications
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of upper and lower motor neurons. Depression rates remain low, considering the severity of the disease (Rabkin et al, 2016), and patients’ quality of life (QoL) is not as impaired as might be expected (Roach et al, 2009; Lulé et al, 2012; Jakobsson Larsson et al, 2017). This relatively good psychosocial adjustment raises important questions about the coping strategies used by patients
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