Cognitive and social cognition deficits in Huntington’s disease differ between the prodromal and the manifest stages of the condition: A scoping review of recent evidence

Abstract

Objective. Huntington's disease (HD) is a dramatic neurodegenerative disorder encompassing severe motor symptoms coupled to significant cognitive and social cognition deficits. However, it is not clear whether and how patients' neuropsychological profile changes between the prodromal and the manifest stages of the condition. The aim of the present in-depth review is to consider cognitive and social cognition impairment in HD patients by differentiating deficits arising before diagnosis from those evident from the manifest phase onwards.Methods. Electronic databases were searched between January 1 st , 2010 and December 31 st , 2020 by using multiple combinations of keywords related to the investigation of neuropsychological profile in HD for preliminary search, and by defining strict selection criteria for studies to be included. Results. Forty-two studies were included. Evidence suggests that the neuropsychological profile in HD reflects a complex pathological spectrum of deficits. It includes impairment in the realms of executive functions, memory, attention, information processing, and social cognition. Interestingly, patients' profiles differ significantly between the manifest and the prodromal stages of their condition, not only in quantitative terms but also from a qualitative point of view.Conclusions. Researchers and clinicians should thus include in clinical routine timely and specific neuropsychological assessments in order to monitor patients' cognitive status as time goes by, with the ultimate goal to implement effective clinical management strategies.

Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

“…Recently, investigations described irritability in HD as a multidimensional construct associated with other psychiatric behavioral symptoms, such as anxiety and depression [ 19 ]. Additionally, an impaired ability to reflect on the mental states of the self and others (theory of mind; ToM), impaired recognition of emotions and social cognitive deficits might impair communication and living with those individuals affected by HD [ 20 , 21 , 22 , 23 , 24 ].…”

Pharmacological characterization of SAGE‐718, a novel positive allosteric modulator of N‐methyl‐d‐aspartate receptors

“…Subchronic PCP administration is an established model of schizophrenia, in particular of the negative and cognitive symptoms (G. Lee & Zhou, 2019), and prolonged inhibition of NMDA receptors with PCP produces long‐lasting neural maladaptations and concomitant cognitive deficits (Dawson et al, 2014; Jenkins et al, 2008; McKibben et al, 2010). In Huntington's disease, cognitive impairment can include deficits in social cognition, such as recognition of emotions in response to facial and vocal stimuli, which can precede the onset of motor symptoms (Bora et al, 2016; Cavallo et al, 2022; Henley et al, 2012). Furthermore, in individuals with Huntington's disease, 24(S)‐HC levels are correlated with several cognitive tasks, including negative emotional processing in the Eckman faces task (Lewis et al, 2020).…”

A Practical Guide for Clinical Approach to Patients With Huntington’s Disease in Korea

“…47 Cognitive features of HD begin with nonamnestic mild cognitive impairment and progress into a broad range of cognitive deficits in the executive, learning and memory, attention, perception, and language domains. 48 In South Korea, the prevalence of dementia in HD patients is approximately 40% and gradually increases to 80% in patients over 80 years of age. 5 Cognitive performance is poorer in the parkinsonism-dominant group than in the chorea-dominant and mixed-motor phenotype groups, independent of disease duration and severity.…”