Understanding Others with ALS: A Few Words on Social Cognition

On the occasion of World Amyotrophic Lateral Sclerosis (ALS) Day, celebrated every year on June 21st, we interviewed Dr. Elisa Canu, researcher at the Neuroimaging of Neurodegenerative Disorders laboratory at IRCCS San Raffaele Hospital, led by Prof. Massimo Filippi, to explore the cognitive aspects of a disease often thought to affect only motor abilities.

Doctor, what is ALS?

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that primarily affects motor neurons, the nerve cells responsible for muscle control. Almost all ALS patients primarily present motor symptoms, such as weakness, stiffness, or muscle atrophy (loss of muscle mass), but also – in more aggressive forms of the disease – difficulties with breathing, swallowing, and dysarthria, which is difficulty articulating words clearly and understandably. In Italy alone, the disease affects between one and three new individuals per 100,000 people each year, raising important clinical concerns. To date, no targeted treatments exist that slow or stop the disease’s progression, which has a very short average duration of about 3-4 years.

It is often thought that ALS is purely a motor disease. Is that really the case?

Although the onset of the disease is characterized by motor difficulties, ALS can also present cognitive and behavioral symptoms. These symptoms occur in 30-50% of patients, who may experience difficulties in executive functions such as the ability to plan, inhibit automatic or inappropriate responses, and adapt strategies based on situational needs, and/or behavioral issues like emotional detachment or disinhibition.

Why has the cognitive dimension of these patients been largely overlooked?

Until a few years ago, the focus was primarily on the motor difficulties of the disease, which are the main symptoms prompting patients to see a specialist. However, in-depth study of this disease has progressively allowed researchers and clinicians to recognize clinical, pathological, and genetic features shared within the spectrum of frontotemporal diseases—a group of neurodegenerative disorders that cause progressive deterioration of cognitive and behavioral functions.
Thorough neuropsychological evaluation of these patients in cognitive and behavioral domains has revealed subtle and silent alterations that often go unrecognized by both the patient and caregiver. Moreover, cognitive and behavioral changes in ALS patients are frequently associated with anosognosia, a condition in which the person is unaware of their impairments, which may therefore go unnoticed. This differs, for example, from patients with Alzheimer’s disease, who often are aware of their memory impairments.

Among the main cognitive abilities is social cognition: what is it and how does it manifest in ALS patients?

One of the newest branches of neuropsychology is precisely social cognition. This term refers to the ability to understand others’ intentions, desires, and emotions, comprehend collective social norms and rules, and behave accordingly. Until recently, social cognition had not been clinically studied; only in the past decade has its importance become clear. Social cognition requires the so-called ‘theory of mind’ and is divided into two main components: a cognitive dimension, which involves understanding others’ mental states such as intentions and desires, and an affective dimension, which involves understanding others’ emotions and feelings.
Thus, social cognition includes social perception—the ability to recognize not only faces but also the emotions they express, along withv body language and prosody. Finally, a crucial aspect is social behavior, or the ability to act appropriately according to the emotional tone of a given situation. ALS patients generally show difficulty recognizing facial emotions, especially negative ones like sadness, anger, and disgust, as well as in inferring others’ cognitive states.

What is your research focus in the field of social cognition?

At the Neuroimaging of Neurodegenerative Disorders group, we also address this topic. In collaboration with the Auxological Institute of Milan and UCSF (University of California, San Francisco), we conducted a study using the ‘Ekman faces’—images of faces expressing the universal expressions of basic emotions, namely joy, sadness, anger, surprise, fear, and disgust. These images are commonly used to study emotional recognition in a given sample of people by asking them, for example, to indicate which face, among five possible options, expresses a certain emotion. Our study showed how these types of tests can discriminate not only healthy individuals from those affected by ALS but also distinguish which patients among the ill exhibit cognitive impairment and which do not. However, it is important to emphasize that while some tests can differentiate healthy from affected patients, they are not always optimal at detecting early signs of cognitive impairment. For this reason, a thorough neuropsychological evaluation remains necessary.

What is the most important message to convey, in your opinion, when talking about ALS?

In my view, the most important message is about the complexity of the disease. ALS is therefore a disease that involves motor abilities but sometimes also the cognitive and behavioral dimension. For this reason, it is important to overcome the conception of ALS as an exclusively motor pathology, recognizing that it requires a care approach that includes not only physical support but also cognitive and behavioral assistance. Research into cognitive and behavioral abilities is fundamental for clinical prognosis and because it concerns patients who are asked to make decisions about therapeutic strategies, so we need to know that these patients are capable of deciding for themselves.

Interview by Andrea Iotti