Theory of Mind and Its Neuropsychological and Quality of Life Correlates in the Early Stages of Amyotrophic Lateral Sclerosis

Abstract

This study aims to explore the potential impairment of Theory of Mind (ToM; i.e., the ability to represent cognitive and affective mental states to both self and others) and the clinical, neuropsychological and Quality of Life (QoL) correlates of these cognitive abnormalities in the early stages of amyotrophic lateral sclerosis (ALS), a multisystem neurodegenerative disease recently recognized as a part of the same clinical and pathological spectrum of frontotemporal lobar degeneration. Twenty-two consecutive, cognitively intact ALS patients, and 15 healthy controls, underwent assessment of executive, verbal comprehension, visuospatial, behavioral, and QoL measures, as well as of the ToM abilities by Emotion Attribution Task (EAT), Advanced Test of ToM (ATT), and Eyes Task (ET). ALS patients obtained significantly lower scores than controls on EAT and ET. No significant difference was found between the two groups on ATT. As regard to type of ALS onset, patients with bulbar onset performed worse than those with spinal onset on ET. Correlation analysis revealed that EAT and ET were positively correlated with education, memory prose, visuo-spatial performances, and “Mental Health” scores among QoL items. Our results suggest that not only “cognitive” but also “affective” subcomponents of ToM may be impaired in the early stages of ALS, with significant linkage to disease onset and dysfunctions of less executively demanding conditions, causing potential impact on patients’ “Mental Health.”

Social Cognition Dysfunctions in Neurodegenerative Diseases: Neuroanatomical Correlates and Clinical Implications

“…On the other hand, social cognition is included in an ALS cognitive screening testing (i.e., Edinburg Cognitive and Behavioral ALS Screen (ECAS); [ 133 ]) and in the “Axis II: Defining the neuropsychological deficits” of the recently suggested diagnostic criteria for ALS-frontotemporal spectrum disorders (ALS-FTSD) [ 67 ]. Moreover, the evaluation of social cognition in ALS-FTSD may have an utmost importance in clinical settings, given the potential effects of its impairment on patients' quality of life and ability to engage in end-of-life decisions [ 134 - 136 ]. However, it is still debatable whether social cognition deficits are independent of other cognitive deficits in ALS or are part of the executive deficits or not [ 137 - 143 ] and to what degree they are associated with other cognitive deficits, including memory function [ 136 ].…”

“…Moreover, the evaluation of social cognition in ALS-FTSD may have an utmost importance in clinical settings, given the potential effects of its impairment on patients' quality of life and ability to engage in end-of-life decisions [ 134 - 136 ]. However, it is still debatable whether social cognition deficits are independent of other cognitive deficits in ALS or are part of the executive deficits or not [ 137 - 143 ] and to what degree they are associated with other cognitive deficits, including memory function [ 136 ]. Of note, a subgroup of ALS patients without dementia has been found to present impaired social cognition without executive dysfunction [ 140 ].…”

“…Even though previous evidence revealed a more pronounced impairment in affective rather than cognitive component of ToM [ 157 ], Trojsi et al [ 136 ] found that both cognitive and affective ToM may be impaired in the early disease stages by simultaneously comparing both ToM components [ 136 ]. Of note, cognitive ToM impairments have been mostly linked to a more general executive dysfunction [ 154 ].…”

The cognitive profile of behavioural variant FTD and its similarities with ALS: a systematic review and meta-analysis

“…One could argue that social cognition deficits are therefore likely to occur in early stages of bv-FTD and ALS and may show progression over time. 32 This should be investigated in a longitudinal study including only patients with early stage bv-FTD and ALS. If confirmed, this could attribute to the early detection of cognitive impairment in patients with ALS.…”

Deficits in Emotion Recognition as Markers of Frontal Behavioral Dysfunction in Amyotrophic Lateral Sclerosis

“…While executive impairment is the most common cognitive dysfunction in ALS 3 , social cognition deficits have also been reported 1, 4 , with decline in abilities such as theory of mind and emotional processing 1,2 . In this latter regard, deficits in recognition of facial emotion expressions are present in ALS 1,[5][6][7][8][9][10] . ALS patients also exhibit behavioral symptoms, such as apathy, loss of empathy and personality changes 11 , even at early stages of the disease and before motor symptoms 12 .…”