Ep. 204: Social Cognition in ALS – beyond motor neurons

00:00:00: Welcome to EANcast, your weekly source for education research and updates from the European Academy of Neurology.

00:00:15: Hello!

00:00:15: And welcome to the EANCast Weekly Neurology.

00:00:19: My name is Elisa Canu.

00:00:21: I am a neuropsychologist and researcher at San Rafael Hospital in Milan currently serving as co-chair of the Ean Scientific Panel on Higher Cortical Functions.

00:00:33: My research focuses on the structural and functional correlates of cognitive and behavioral features in neurodegenerative disorders with particular focus on frontotemporal lower degeneration.

00:00:47: This month's topic is ALS and Frontotemporale Dementia, And In todays episode we will talk about social co-opinion in ALS beyond motor neurons!

00:01:02: She is a neuropsychologist and postdoctoral researcher at the Neuroimaging Unit of Neurodegenerative Diseases, of some Raffaele Hospital in Milan.

00:01:12: A research focus on the neural correlates of social cognition with particular emphasis on emotional reserve across different variants from frontotemporal dementia.

00:01:24: Welcome Anna!

00:01:25: And thank you for joining us today.

00:01:28: Thank You Elisa and hello everybody.

00:01:33: So we can start with the first question.

00:01:37: An ALS is often thought to be a poorly motor disease, Is that really the case?

00:01:45: It's definitely not the case.

00:01:47: over the last two decades who have seen a paradigm shift?

00:01:51: We now surely establish ALS as a multi-system disorder.

00:01:55: Research highlighted that ALS exists on clinical pathological continuum which frontotemporal lover degeneration.

00:02:03: About fifty percent of our patients can experience some form of cognitive or behavioral change, while ten-fifteen percent meet the full criteria of FTD.

00:02:14: A much larger group exhibits milder cognitive deficits.

00:02:18: These no matter features are critical because they're intrinsic components in disease pathology not just secondary complications and a whole significant prognostic implication.

00:02:30: So Hannah Among the main cognitive abilities, there is also social cognition.

00:02:36: But what does this domain?

00:02:39: Think of Social Cognition as an umbrella term for the mental processes that allows us to navigate a human world.

00:02:46: It's what allows us develop civilization.

00:02:50: We break it down into four inter-independent domains.

00:02:54: The first is the theory of mind which has the ability attribute mental states thoughts, motives and intention to ourselves another.

00:03:03: It has a cognitive component which is knowing what someone thinks an unaffected component understanding what they feel.

00:03:11: The second domain is empathy Which is the ability To understand Another person's feeling through a vicarious emotion experience.

00:03:19: Third domain is social perception.

00:03:24: It's our ability to distinguish a person identity, gender and emotional status from just a brief glance at their face.

00:03:32: And the last is social behavior which is hand results The observable action an interaction shaped by all processes above.

00:03:42: How do social cognition impairments manifest in ALS?

00:03:47: What are their early clinical signs through which they can be recognized?

00:03:53: So, in ALS these impermanence often manifests as a progressive blunting of social awareness.

00:04:00: One of the early signs is a diminished psychophysiological arousal to emotional stimuli.

00:04:06: Clinically we see that patients often struggle to recognize and attribute specific emotions particularly disgusts and surprise followed by fear anger and sadness.

00:04:17: A key sign is the breakdown effect theory.

00:04:20: The patient might understand what you are thinking logically, but they can no longer process how your feeling.

00:04:27: Family often notice increased apathy or a perceived coldness.

00:04:32: This is an involuntary personality change by the direct result of disruption in neural pathway responsible for emotional processing.

00:04:43: So I think the people which is listening are curious about, what are their main tools?

00:04:48: The main neuropsychological tool used to assess social cognition in ALS patients.

00:04:56: How can they assess this domain of ALS?

00:05:00: Sure!

00:05:00: Thank you for your question Lisa.

00:05:03: When assessing social cognitions at ALS the primary requirement is that tools must account for physical limitations particularly motor speed and speech impairment.

00:05:16: We prioritise brief, validated batteries that specifically adjust for this disability.

00:05:21: to avoid overestimating cognitive decline due to physical weakness.

00:05:26: A very effective approach involves tasks where patients are asked to infer the intention or emotion of a character depicting a series of ingests which allows us to evaluate with the cognitive and affective component Additionally, Emotion Recognition tasks based on the facial expressions such as the classic Pollack Man faces are commonly used to measure patient's ability to identify specific states like disgusts fear or sadness.

00:05:55: We also evaluate perception of prosody and vocal tone together a more complete picture of social processing.

00:06:03: The main advantage is that they require only simple vocal responses.

00:06:10: These ensure that even as the disease progresses, we can accurately monitor the patient's social cognitive health.

00:06:19: But very clear on thanks.

00:06:23: I was wondering why a social cognition is affected in ALS?

00:06:28: What are the neurobiological mechanisms underlying social cognition in patients with ALS?

00:06:36: So this is a really complicated question, but these deficits are driven by the degeneration of frontostriatal and temporal circuits caused by the accumulation of TDP-vIII protein in key empathy related areas like orbital frontal cortex and anterior cingulate.

00:06:55: Research shows that rain-matter loss in these regions directly correlates with difficulties in attributing emotions, while white matter alteration disrupts the communication between the limbic system and frontal lobes.

00:07:10: Using functional MRI research discovered changes in resting state connectivity can predict social deficits six months in advance.

00:07:19: Even in purely motor cases, damage to structures such as the left pallidum affected a perception of specific emotions like disgust confirming the multi-system nature of disease.

00:07:34: So why is so important to detect very early this social cognition impairment in ILS?

00:07:44: What are clinical and prognostic implications for social cognition deficits in these patients.

00:07:52: So, it's fundamentally changed the trajectory of care.

00:07:56: Patients with this deficit often have poor prognosis.

00:08:00: These impairments are directly linked to reduced compliance With critical life-sustaining interventions such as noninvasive ventilation.

00:08:09: If you can't perceive emotional logic or a treatment Or distress from your caregiver You're less likely follow through with accomplished care plans.

00:08:18: It also leads to a faster functional decline and increase curgiven burnout, making social cognition a vital factor in predicting the disease overall impact.

00:08:30: So what intervention strategies if there is any are currently available for social cognitive disturbances?

00:08:41: This is a really interesting question, Elisa.

00:08:43: Thank you!

00:08:44: So given the progressive nature of ALS our current approach focuses on compensatory strategies rather than restorative one.

00:08:53: first and foremost it's psychoeducation with caregivers.

00:08:57: we must explain that the family that the patient lack of empathy is a biological symptom not a choice.

00:09:04: this reframing is incredibly powerful.

00:09:07: Second, we use environmental modification.

00:09:10: We teach family to use explicit communication If the patient cannot longer read a supposed said expression.

00:09:17: this post must learn to say I'm sad and i need you listen.

00:09:22: You moved from a subtle social cues to explicit verbal data To reduce the cognitive load on patients

00:09:30: So understand.

00:09:32: can social cognition be effectively integrated into a multidisciplinary care of ILS?

00:09:41: It needs to be part of the routine checkup.

00:09:43: We shouldn't just measure lung capacity and lung score strength, we should be screening for social cognition at that time of diagnosis.

00:09:52: These allow the multidisciplinary team, neurologists, psychologists and speech therapists to tailor their communication and set realistic expectations for the family regarding patients' decision-banking capacity.

00:10:07: Social cognition impairments are often invisible symptoms.

00:10:12: How do these changes in personality and social interaction reshape the family dynamic?

00:10:21: And how can caregivers be supported in managing this emotional toll?

00:10:28: So, coming to like the most critical part of ALS care.

00:10:34: When a patient loses mother function, this pose become a caregiver.

00:10:38: but when the patients lose social cognition, this post feels they've lost their soulmates.

00:10:44: So we support them by validating their grief and acknowledging that caring for someone who doesn't seem to care back is uniquely exhausting.

00:10:53: But then, we provide clear communication tools giving them a manual on how to interact with the brain which can no longer process social signals.

00:11:03: And then we provide psychological support helping them navigate change in personality as an neurobiological reality of disease which can help reduce feeling of resentment and burnout.

00:11:18: So thanks Anna for your impressive preparation on the field, it was very nice listening to you!

00:11:28: And thank all people who are listening with this episode.

00:11:34: Thank You for your attention!

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00:12:21: Thanks for listening!