The Functional Implications of Motor, Cognitive, Psychiatric, and Social Problem-Solving States in Huntington's Disease

Liew, Charles Van; Gluhm, Shea; Goldstein, Jody; Cronan, Terry A.; Corey–Bloom, Jody

doi:10.1521/psyc.2013.76.4.323

Cited by 7 publications

(8 citation statements)

References 39 publications

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“…Both of these findings make a vital contribution to the design of swallowing therapy for HD patients by recommending that swallowing therapy approaches and swallowing compensatory techniques should be adjusted to the patient's cognitive level. Given that progressive decline in cognitive level is expected with HD progression, [25][26][27] frequent visits to the SLP may be indicated.…”

Section: Discussionmentioning

confidence: 99%

Dysphagia characteristics in Huntington’s disease patients: insights from the Fiberoptic Endoscopic Evaluation of Swallowing and the Swallowing Disturbances Questionnaire

et al. 2018

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Section: Discussionmentioning

confidence: 99%

Dysphagia characteristics in Huntington’s disease patients: insights from the Fiberoptic Endoscopic Evaluation of Swallowing and the Swallowing Disturbances Questionnaire

et al. 2018

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“…In the prodromal phase the impairment is more subtle but abnormalities in psychomotor processing speed, verbal fluency and the recognition of negative emotions are common ( Tabrizi et al, 2013, 2012; Begeti et al, 2013 ). The direct functional implications of these cognitive changes are still unclear ( Kirkwood et al, 2002; Duff et al, 2010; Van Liew et al, 2013 ) but, reduced occupational performance and difficulty managing finances can be seen in pre-manifest HD gene carriers (pre-HD) who are approaching diagnosis ( Beglinger et al, 2010 ). Furthermore, changes in personality and difficulties with social interaction are key features of early HD.…”

Section: Introductionmentioning

confidence: 99%

The role of the amygdala during emotional processing in Huntington's disease: From pre-manifest to late stage disease

et al. 2015

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BackgroundDeficits in emotional processing can be detected in the pre-manifest stage of Huntington's disease and negative emotion recognition has been identified as a predictor of clinical diagnosis. The underlying neuropathological correlates of such deficits are typically established using correlative structural MRI studies. This approach does not take into consideration the impact of disruption to the complex interactions between multiple brain circuits on emotional processing. Therefore, exploration of the neural substrates of emotional processing in pre-manifest HD using fMRI connectivity analysis may be a useful way of evaluating the way brain regions interrelate in the period prior to diagnosis.MethodsWe investigated the impact of predicted time to disease onset on brain activation when participants were exposed to pictures of faces with angry and neutral expressions, in 20 pre-manifest HD gene carriers and 23 healthy controls. On the basis of the results of this initial study went on to look at amygdala dependent cognitive performance in 79 Huntington's disease patients from a cross-section of disease stages (pre-manifest to late disease) and 26 healthy controls, using a validated theory of mind task: “the Reading the Mind in the Eyes Test” which has been previously been shown to be amygdala dependent.ResultsPsychophysiological interaction analysis identified reduced connectivity between the left amygdala and right fusiform facial area in pre-manifest HD gene carriers compared to controls when viewing angry compared to neutral faces. Change in PPI connectivity scores correlated with predicted time to disease onset (r=0.45, p<0.05). Furthermore, performance on the “Reading the Mind in the Eyes Test” correlated negatively with proximity to disease onset and became progressively worse with each stage of disease.ConclusionAbnormalities in the neural networks underlying social cognition and emotional processing can be detected prior to clinical diagnosis in Huntington's disease. Connectivity between the amygdala and other brain regions is impacted by the disease process in pre-manifest HD and may therefore be a useful way of identifying participants who are approaching a clinical diagnosis. Furthermore, the “Reading the Mind in the Eyes Test” is a surrogate measure of amygdala function that is clinically useful across the entire cross-section of disease stages in HD.

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“…Aggressive behaviour can be observed, even in premanifest HD when no motor symptoms are observed as described in case B. Dysfunction in the prefrontal cortex brain circuits that normally inhibit emotional impulses is a prelude to violent outbursts [9]. A lack of social problem solving skills may be reflective of cognitive deficits rather than psychiatric mental state [13] with irritability giving rise to outbursts of aggression and violence due to inability to generate any further solutions to problems faced [14].…”

Section: Discussionmentioning

confidence: 99%

Huntington’s disease: a forensic risk factor in women

et al. 2019

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Background Huntington’s disease (HD) is an autosomal dominant, neurodegenerative disorder. Associated cognitive deficits including impulsivity and disinhibition are the same factors that also predispose to forensic risk. Men tend to be perpetrators of more severe violent behaviours than women and women are less likely than men to be arrested for violence. This finding is not applicable in the case of women with Huntington’s disease and explored in the three clinical cases of women with HD and their forensic histories that are subsequently described. Case presentation ‘A’ was admitted from court following a charge of arson and reckless behavior, with increasing severity and frequency of self-harm and attempted suicide. This case demonstrates someone who had previously presented to psychiatric services on multiple occasions for various reasons, culminating in a serious criminal charge of arson due to psychiatric symptoms associated with HD. ‘B’ was arrested and imprisoned after having been charged with actual bodily harm (ABH) for assaulting her partner and young daughter then breaking her bail conditions. Although she was gene positive for HD she had no neurological symptoms of the disease. B was given leave but needed to be recalled to hospital by police. Six weeks later the medical recommendation for a court imposed hospital order was overturned as B presented and articulated her case so convincingly in court. This case demonstrates that even in the absence of psychiatric history or movement disorder there may be substantial forensic risk indicated by subtle underlying cognitive deficits due to changes in executive function affecting the frontal lobes. ‘C’ was admitted to acute psychiatric services after being found wandering in traffic wanting to die. She had been diagnosed with HD in the previous year and had a long criminal record on a background of alcohol dependency. Following transfer to a specialist psychiatric unit, she engaged well with a neurobehavioural levels system which rewards desirable and appropriate behaviours and she responded well to a highly structured environment resulting in discharge to a community placement. Conclusions These three case studies aim to highlight the need to raise awareness of the increased forensic risk in women with HD. Although criminal behaviour is less frequently observed in women than men and usually violence is less severe in women, HD may cause or contribute to criminal behaviour that can be violent in nature in women who are gene carriers for HD even in the absence of movement disorder, psychiatric symptoms or overt cognitive deficits. Assessment and earlier treatment in appropriate hospital settings may successfully contain and modify behaviours leading to reduced levels of risk and recidivism in this vulnerable patient group.

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The Functional Implications of Motor, Cognitive, Psychiatric, and Social Problem-Solving States in Huntington's Disease

Cited by 7 publications

References 39 publications

Dysphagia characteristics in Huntington’s disease patients: insights from the Fiberoptic Endoscopic Evaluation of Swallowing and the Swallowing Disturbances Questionnaire

Dysphagia characteristics in Huntington’s disease patients: insights from the Fiberoptic Endoscopic Evaluation of Swallowing and the Swallowing Disturbances Questionnaire

The role of the amygdala during emotional processing in Huntington's disease: From pre-manifest to late stage disease

Huntington’s disease: a forensic risk factor in women

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