Abstract

Even though increasing literature describes changes in emotional processing in Amyotrophic Lateral Sclerosis (ALS), efforts to summarize relevant findings are lacking in the field. A systematic literature review was performed to provide a critical and up-to-date account of emotional abilities in ALS. References were identified by searches of PubMed, Web of Science and Scopus (1980–2021, English literature), with the following key terms: (“Amyotrophic Lateral Sclerosis” or “Primary Lateral Sclerosis” or “Motor Neuron”) and “Emotion*” and (“Processing” or “Attribution” or “Elaboration” or “Perception” or “Recognition”). Studies concerning only caregivers, pseudobulbar affect, and social cognition were excluded. Forty-one articles were included, all concerning ALS, and seven topics were identified: Emotion recognition, Emotional responsiveness, Emotional reactivity, Faces approachability rating, Valence rating, Memory for emotional materials and Alexithymia. The majority of these aspects have only been sparsely addressed. The evidence confirms altered emotional processing in ALS. The most consistent findings regard the recognition of facial expressions for negative emotions, but also alterations in the subjective responsiveness to emotional stimuli (arousal, valence and approachability), in psychophysiological and cerebral reactivity and in emotional memory, together with alexithymia traits, were reported. According to this evidence, emotional abilities should be included in the clinical assessment and therapeutic interventions.

Highlights

  • The present review aims to focus on emotional processing and recognition and to summarize available findings

  • A total of 41 papers met the eligibility criteria for review and their characteristics are depicted in Supplementary Table S2

  • One study describes a patient with anterior temporal lobe degeneration and Amyotrophic Lateral Sclerosis (ALS) [20]

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Summary

Introduction

The literature provided consistent evidence of social cognition impairment as a cognitive manifestation of ALS, in particular in the domains of basic emotion recognition and Theory of Mind (ToM), i.e., the ability to infer others’ mental and emotional states [1]. Social cognition abilities are often considered in association with a dysexecutive syndrome and one of the hallmark features of frontotemporal degeneration (FTD). The emotional and social cognition abilities are relevant issues in neurodegenerative disorders, according to their potential effects on patients’ quality of life and ability to engage in end-of-life decisions [5,6]. Emotional abilities help patients to maintain positive and satisfying relationships with their caregivers and relatives and sustain treatment decisions along the course of the disease [7]

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