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Huntington's Disease Updates

MedpageToday

Are Movement Disorders to Blame for a Lack of Social Awareness?

—An examination of 50 relevant studies found that individuals with any of four major types of hyperkinetic movement disorders consistently exhibited impaired social cognition. What does this mean for them—and the professionals who care for them?

Individuals with hyperkinetic movement disorders may experience impaired social cognition, which includes a range of intricate mental capacities that allow people to perceive and react to social cues. That was the main finding from a recent systematic review published by an international team of researchers in Social Neuroscience.1

Hyperkinetic movement disorders encompass a group of neurological conditions characterized by excessive, involuntary movements. One of the most well-known hyperkinetic movement disorders is Huntington disease (HD), a rare hereditary condition caused by a mutated HTT gene. HD affects an estimated 2.7 per 100,000 individuals worldwide.2 Other movement disorders include Tourette syndrome (TS), essential tremor (ET), and dystonia, which is characterized by intermittent or sustained muscle contractions that result in abnormal and often repetitive movements or postures.3

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“The brain regions impacted by hyperkinetic movement disorders hold significance for social cognition, even though this connection has not always been acknowledged in the literature,” says Alessio Avenanti, PhD, one of the new study’s authors.

“Our systematic review,” he continues, “sheds light on the functional relevance of subcortical sensorimotor regions and helps to expand our understanding of our social brain.”

‘Valuable implications’ for clinicians

This systematic review screened 1137 studies that analyzed the impact of various hyperkinetic movement disorders on social cognition.1 A total of 50 studies met inclusion criteria. Patients with HD, TS, dystonia, and ET were studied.

Assessments of social perception, theory of mind (ToM), and empathy were used to quantify the severity of social cognition impairment. Social perception involves the awareness and interpretation of social cues. ToM is the ability to gauge our own mental state and the mental states of others. Finally, empathy allows us to understand the perceptions and emotions of other people.1 

In this, individuals from all four groups of hyperkinetic movement disorders exhibited impairment of social perception and ToM. Additionally, people with HD and TS showed empathy impairments, while those with ET and dystonia did not.

“The insights gleaned from our study carry valuable implications for clinicians dedicated to treating individuals with hyperkinetic movement disorders,” says Dr. Avenanti, who is professor of cognitive neuroscience at the University of Bologna, Italy. “Moving beyond the mere mitigation of motor symptoms, enhancing the quality of life for these individuals necessitates a careful consideration and potential rehabilitation of cognitive deficits, particularly those pertinent to social interactions. Regrettably, these cognitive aspects often go unnoticed, emphasizing the pressing need for comprehensive approaches that address not only motor symptoms but also the intricacies of social cognition impairments.”

What are the main take-aways?

For healthcare teams that manage patients with neurological conditions, the authors emphasize the importance of treating nonmotor symptoms of hyperkinetic movement disorders.

“I’d like to underscore the significance of adopting multifaceted approaches in the study and management of hyperkinetic movement disorders,” says Dr. Avenanti. “These approaches should encompass not only motor symptoms but also extend to cognitive and emotional dimensions. By recognizing and addressing the broader spectrum of challenges that these individuals face, we can advance more comprehensive and effective interventions that truly enhance their quality of life.” 

Dr. Avenanti agrees, however, that additional research is needed to further delineate the association between motor symptoms and social cognitive challenges.

“Further investigation should prioritize understanding the influence of disease progression, as well as the effects of standard and combined treatments, on social cognitive functions,” he says. “Exploring the effectiveness of tailored rehabilitation strategies, aimed at addressing social cognition deficits within these disorders, would significantly contribute to improving patient care.”

Published:

Adam Ash is an emergency medicine physician who practices in New York. His 15 years of experience in education, administration, and research allows him to write about a broad spectrum of medical topics.

References

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Juvenile-Onset Huntington’s Disease: A More Nuanced Approach to Monitoring Progression
This study identified specific motor skills as highly sensitive markers of juvenile-onset Huntington’s disease (JOHD) progression, offering more precise tools for tracking the disease and designing future treatments tailored to patients with JOHD.
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In Huntington’s Disease, Exploring the Roles of Race, Ethnicity, and Education
A study identified racial and ethnic disparities in Huntington’s disease, as well as differences based on levels of education of patients, finding that minority patients may be disproportionately affected.
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Neurogenetic Disease Patients and Caregivers May Benefit from End-of-Life Conversations
A French study based on questionnaires and interviews showed a willingness among patients with neurogenetic diseases and their primary caregivers to talk about sensitive issues like advance directives and end-of-life care.
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In Huntington’s Disease, Depression is Different
Investigators call for psychosocial interventions in those with or at risk for Huntington’s disease. Taking a personalized approach to address factors such as concern for the future and sleep disturbances is essential.
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In Early-Stage Huntington’s Disease, Cognitive Trajectories Vary
In a study of adults with early or early-mid Huntington’s disease, two types of patients emerged: those with a mild, slow progression of cognitive decline and those marked by a more rapid and aggressive decline. How did these groups compare with healthy controls?
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Huntington’s Disease: A Look at the Global Pace
This updated systematic review and meta-analysis—the first in at least a decade—analyzed rates of this rare inherited neurological disorder in 21 countries, across multiple continents. Have rates gone up, down, or stayed the same?