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      Huntington’s disease: a forensic risk factor in women

      case-report

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          Abstract

          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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          Most cited references31

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          Dysfunction in the neural circuitry of emotion regulation--a possible prelude to violence.

          Emotion is normally regulated in the human brain by a complex circuit consisting of the orbital frontal cortex, amygdala, anterior cingulate cortex, and several other interconnected regions. There are both genetic and environmental contributions to the structure and function of this circuitry. We posit that impulsive aggression and violence arise as a consequence of faulty emotion regulation. Indeed, the prefrontal cortex receives a major serotonergic projection, which is dysfunctional in individuals who show impulsive violence. Individuals vulnerable to faulty regulation of negative emotion are at risk for violence and aggression. Research on the neural circuitry of emotion regulation suggests new avenues of intervention for such at-risk populations.
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            Gender and Crime: A General Strain Theory Perspective

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              Neuropsychiatric aspects of Huntington's disease.

              Neuropsychiatric symptoms are common in Huntington's disease and have been considered its presenting manifestation. Research characterising these symptoms in Huntington's disease is variable, however, encumbered by limitations within and across studies. Gaining a better understanding of neuropsychiatric symptoms is essential, as these symptoms have implications for disease management, prognosis, and quality of life for patients and caregivers. Fifty two patients with Huntington's disease were administered standardised measures of cognition, psychiatric symptoms, and motor abnormalities. Patient caregivers were administered the neuropsychiatric inventory. Ninety eight per cent of the patients exhibited neuropsychiatric symptoms, the most prevalent being dysphoria, agitation, irritability, apathy, and anxiety. Symptoms ranged from mild to severe and were unrelated to dementia and chorea. Neuropsychiatric symptoms are prevalent in Huntington's disease and are relatively independent of cognitive and motor aspects of the disease. Hypothesised links between neuropsychiatric symptoms of Huntington's disease and frontal-striatal circuitry were explored. Findings indicate that dimensional measures of neuropsychiatric symptoms are essential to capture the full range of pathology in Huntington's disease and are vital to include in a comprehensive assessment of the disease.
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                Author and article information

                Contributors
                e.chu@queensu.ca
                MariONeill@priorygroup.com
                debasishdp@googlemail.com
                caroline.knight@elysiumhealthcare.co.uk
                Journal
                J Clin Mov Disord
                J Clin Mov Disord
                Journal of Clinical Movement Disorders
                BioMed Central (London )
                2054-7072
                24 July 2019
                24 July 2019
                2019
                : 6
                : 3
                Affiliations
                [1 ]ISNI 0000000121901201, GRID grid.83440.3b, Huntington’s Disease Centre, UCL Institute of Neurology, ; 2nd Floor Russell Square House, London, WC1B 5EH UK
                [2 ]ISNI 0000 0004 1936 8331, GRID grid.410356.5, Department of Psychiatry, , Queen’s University, ; Kingston, ON Canada
                [3 ]Clinical Psychology, Priory Group, Melton Mowbray, UK
                [4 ]General Adult Psychiatry, Northamptonshire Healthcare Foundation Trust, Northampton, UK
                [5 ]Elysium Neurological Services, Elysium Healthcare, St Neots Hospital, St Neots, Cambridgeshire UK
                [6 ]The Oakleaf Group, Hartwell, Northamptonshire UK
                [7 ]ISNI 0000 0004 1936 8411, GRID grid.9918.9, School of Psychology, , University of Leicester, ; Leicester, Leicestershire UK
                Author information
                http://orcid.org/0000-0001-5739-3229
                Article
                78
                10.1186/s40734-019-0078-x
                6657174
                7670772e-0e6b-4010-ab8c-3db0ab5e6347
                © The Author(s). 2019

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 10 October 2018
                : 14 July 2019
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2019

                huntington’s disease,neuropsychiatry,forensic risk

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