Disorders of Emotional Processing in ALS
Disorders of Emotional Processing in ALS
Purpose of review Amyotrophic lateral sclerosis (ALS) is a degenerative brain disease characterized by motor, behavioural and cognitive deficits. Only recently, emotional processing disorders have been shown in this disease. The interest in affective processing in ALS is growing given that basic emotion impairments could impact copying strategies and mood.
Recent findings Studies explore both basic emotion recognition and social cognition. Results are congruent on arousal and valence detection impairments, independently from the stimulus modality (verbal or visual). Further, recognition of facial expressions of anger, sadness and disgust is impaired in ALS, even when cognition is preserved. Clinical features such as type of onset and severity of the disease could be the cause of the heterogeneity in emotional deficits profiles between patients. Finally, a study employing diffusion tensor imaging showed that emotional dysfunctions in ALS are related to right hemispheric connective bundles impairments, involving the inferior longitudinal fasciculus and the inferior frontal occipital fasciculus.
Summary Research on emotional processing in ALS is still in its infancy and results are mixed. Future research including more detailed clinical profiles of patients and measures of brain connectivity will provide useful information to understand heterogeneity of results in ALS.
Along with cognitive deficits, emotional impairments undermine personal identity and hamper the ability to cope with everyday life social tasks. Emotional processing requires the integrity of sensory systems and higher cortical areas that link online information to the knowledge we already possess and that modulate emotions. Studies on healthy individuals and patient works show that the emotional network comprises both cortical, such as insular, temporal and orbitofrontal cortices, and subcortical structures, including the amygdala.
Consequently, a number of necessary 'stations' to process emotions exists within the brain that are susceptible to damage. Not surprisingly, research in the past years provided convincing evidence that degenerative diseases, such as Alzheimer disease and frontotemporal dementia (FTD), are characterized not only by cognitive deficits, but also by widespread impairments in basic emotion recognition and in higher emotional abilities.
Less intuitively, very recent studies have proven that even degenerative brain diseases primarily impacting motor abilities can be characterized by emotional impairments. A striking example is amyotrophic lateral sclerosis (ALS). Initially depicted as a pure motor neuron disease, ALS is now known to cause also nonmotor impairments such as cognitive deficits. In some cases, symptoms include deregulated behavioural manifestations that in around 15% of patients occur as a frank manifestation of dementia.
Anatomical and structural studies on ALS in the 1990s highlighted pathological findings (such as ubiquitin immunoreactive inclusions, neuronal loss and gliosis, hypomethabolism) in the amygdala, the parahippocampal cortex and in the insula, suggesting that ALS might also feature emotional processing impairments. However, the first experimental investigations on basic emotion recognition and social cognition are dated back to 2005. Thus, research on emotional processing in ALS is quite young, with only 13 studies published in 9 years (see Table 1 for a summary of the studies).
This review provides a summary of the findings concerning emotional processing in ALS, concentrating on basic recognition and theory of mind-related studies. It is of uttermost importance to understand whether emotional processing is intact in ALS, as this factor might affect directly disease copying strategies and quality of life.
Abstract and Introduction
Abstract
Purpose of review Amyotrophic lateral sclerosis (ALS) is a degenerative brain disease characterized by motor, behavioural and cognitive deficits. Only recently, emotional processing disorders have been shown in this disease. The interest in affective processing in ALS is growing given that basic emotion impairments could impact copying strategies and mood.
Recent findings Studies explore both basic emotion recognition and social cognition. Results are congruent on arousal and valence detection impairments, independently from the stimulus modality (verbal or visual). Further, recognition of facial expressions of anger, sadness and disgust is impaired in ALS, even when cognition is preserved. Clinical features such as type of onset and severity of the disease could be the cause of the heterogeneity in emotional deficits profiles between patients. Finally, a study employing diffusion tensor imaging showed that emotional dysfunctions in ALS are related to right hemispheric connective bundles impairments, involving the inferior longitudinal fasciculus and the inferior frontal occipital fasciculus.
Summary Research on emotional processing in ALS is still in its infancy and results are mixed. Future research including more detailed clinical profiles of patients and measures of brain connectivity will provide useful information to understand heterogeneity of results in ALS.
Introduction
Along with cognitive deficits, emotional impairments undermine personal identity and hamper the ability to cope with everyday life social tasks. Emotional processing requires the integrity of sensory systems and higher cortical areas that link online information to the knowledge we already possess and that modulate emotions. Studies on healthy individuals and patient works show that the emotional network comprises both cortical, such as insular, temporal and orbitofrontal cortices, and subcortical structures, including the amygdala.
Consequently, a number of necessary 'stations' to process emotions exists within the brain that are susceptible to damage. Not surprisingly, research in the past years provided convincing evidence that degenerative diseases, such as Alzheimer disease and frontotemporal dementia (FTD), are characterized not only by cognitive deficits, but also by widespread impairments in basic emotion recognition and in higher emotional abilities.
Less intuitively, very recent studies have proven that even degenerative brain diseases primarily impacting motor abilities can be characterized by emotional impairments. A striking example is amyotrophic lateral sclerosis (ALS). Initially depicted as a pure motor neuron disease, ALS is now known to cause also nonmotor impairments such as cognitive deficits. In some cases, symptoms include deregulated behavioural manifestations that in around 15% of patients occur as a frank manifestation of dementia.
Anatomical and structural studies on ALS in the 1990s highlighted pathological findings (such as ubiquitin immunoreactive inclusions, neuronal loss and gliosis, hypomethabolism) in the amygdala, the parahippocampal cortex and in the insula, suggesting that ALS might also feature emotional processing impairments. However, the first experimental investigations on basic emotion recognition and social cognition are dated back to 2005. Thus, research on emotional processing in ALS is quite young, with only 13 studies published in 9 years (see Table 1 for a summary of the studies).
This review provides a summary of the findings concerning emotional processing in ALS, concentrating on basic recognition and theory of mind-related studies. It is of uttermost importance to understand whether emotional processing is intact in ALS, as this factor might affect directly disease copying strategies and quality of life.
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