scholarly journals Huntington's disease — the experiences of voluntary organisations

1989 ◽  
Vol 13 (8) ◽  
pp. 409-411 ◽  
Author(s):  
Brian O'Shea
Keyword(s):  
High Risk ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Medical Management ◽  
Psychiatric Morbidity ◽  
Genetic Research ◽  
Morbidity And Mortality ◽  
Psychosocial Dysfunction ◽  
The Face ◽  
Voluntary Organisations

Huntington's disease is associated with a considerable psychiatric morbidity and mortality. These risks are not confined to the sufferer. The relations of victims, both biological and legal, are also at high risk for significant psychosocial dysfunction (O'Shea & Falvey, 1988). Despite recent advances in genetic research, there is still no cure for this chronic choreodementia syndrome. Medical management is confined to palliation of symptoms. The doctor and the patient's family, in the face of this unpleasant disease, are wont to exercise a variety of psychological defences which may hinder the delivery and receipt of adequate support (Martindale, 1987).

scholarly journals The Spectrum of Psychiatric Pathology in a Patient with Genetically Verified Huntington’s Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Samir Alkabie ◽  
Daljinder Singh ◽  
Amy Hernandez ◽  
Rhaisa Dumenigo
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical Spectrum ◽  
Morbidity And Mortality ◽  
Illustrative Case ◽  
Psychiatric Condition ◽  
Behavioral Disturbances ◽  
Psychiatric Hospitalizations

Psychiatric and behavioral disturbances are common in Huntington’s disease (HD) and contribute significantly to its morbidity and mortality. We herein present the case of a 43-year-old woman with genetically verified HD, whose deteriorating psychiatric condition necessitated multiple inpatient psychiatric hospitalizations and featured a clinical spectrum of neuropsychiatric disturbances classically associated with HD. This paper reviews the literature concerning Huntington’s psychopathology and provides an illustrative case example of its clinical nature.

scholarly journals The Allure of High-Risk Rewards in Huntington’s disease

2015 ◽  
Vol 22 (4) ◽  
pp. 426-435 ◽  
Author(s):  
Nelleke C. van Wouwe ◽  
Kristen E. Kanoff ◽  
Daniel O. Claassen ◽  
K. Richard Ridderinkhof ◽  
Peter Hedera ◽  
...  
Keyword(s):  
Decision Making ◽  
High Risk ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurodegenerative Disorder ◽  
Low Risk ◽  
Decision Bias ◽  
Clear Preference ◽  
High Reward ◽  
Risk Choice

AbstractObjectives: Huntington’s disease (HD) is a neurodegenerative disorder that produces a bias toward risky, reward-driven decisions in situations where the outcomes of decisions are uncertain and must be discovered. However, it is unclear whether HD patients show similar biases in decision-making when learning demands are minimized and prospective risks and outcomes are known explicitly. We investigated how risk decision-making strategies and adjustments are altered in HD patients when reward contingencies are explicit. Methods: HD (N=18) and healthy control (HC; N=17) participants completed a risk-taking task in which they made a series of independent choices between a low-risk/low reward and high-risk/high reward risk options. Results: Computational modeling showed that compared to HC, who showed a clear preference for low-risk compared to high-risk decisions, the HD group valued high-risks more than low-risk decisions, especially when high-risks were rewarded. The strategy analysis indicated that when high-risk options were rewarded, HC adopted a conservative risk strategy on the next trial by preferring the low-risk option (i.e., they counted their blessings and then played the surer bet). In contrast, following a rewarded high-risk choice, HD patients showed a clear preference for repeating the high-risk choice. Conclusions: These results indicate a pattern of high-risk/high-reward decision bias in HD that persists when outcomes and risks are certain. The allure of high-risk/high-reward decisions in situations of risk certainty and uncertainty expands our insight into the dynamic decision-making deficits that create considerable clinical burden in HD. (JINS, 2016, 22, 426–435)

scholarly journals Psychiatric morbidity and poor follow-up underlie suboptimal functional and survival outcomes in Huntington's disease

2020 ◽  
Author(s):  
Nikhil Ratna ◽  
Nitish L Kamble ◽  
Sowmya D V ◽  
Meera Purushottam ◽  
Pramod K Pal ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurodegenerative Disorder ◽  
Psychiatric Symptoms ◽  
Tertiary Care ◽  
Psychiatric Morbidity ◽  
Cag Repeat ◽  
Duration Of Illness ◽  
Low Middle Income Countries

Abstract BACKGROUND: Huntington’s disease (HD), an inherited, often late-onset, neurodegenerative disorder, is considered to be a rare, orphan disease. Research into its genetic correlates and services for those affected are inadequate in most low-middle income countries, including India. The apparent ‘incurability’ often deters symptomatic and rehabilitative care, resulting in poor quality of life and sub-optimal outcomes. There are no studies assessing disease burden and outcomes from India. METHODS: We attempted to evaluate individuals diagnosed to have HD at our tertiary-care center between 2013 and 2016 for clinical symptoms, functionality, mortality, follow up status through a structured interview, clinical data from medical records and UHDRS-TFC scoring. RESULTS: Of the 144 patients, 25% were untraceable, and another 17 (11.8%) had already died. Mean age at death and duration of illness at the time of death, were 53 years and 7 years respectively, perhaps due to suicides and other comorbidities at an early age. The patients who could be contacted (n=81) were assessed for morbidity and total functional capacity (TFC). Mean CAG repeat length and TFC score were 44.2 and 7.5 respectively. Most individuals (66%) were in TFC stage I and II and could perhaps benefit from several interventions. The TFC score correlated inversely with duration of illness (p<0.0001). The majority were being taken care of at home, irrespective of the physical and mental disability. There was a high prevalence of psychiatric morbidity (91%) including suicidal tendency (22%). Three of the 17 who died had committed suicide, and several other families reported suicidal history in other family members. Only about half the patients (57%) maintained a regular clinical follow-up. CONCLUSIONS: This study demonstrates the poor follow-up rates, significant suicidality and other psychiatric symptoms, sub-optimal survival durations and functional outcomes highlighting the need for holistic care for the majority who appear to be amenable to interventions.

Child Abuse in Huntington's Disease

PEDIATRICS ◽  
1982 ◽  
Vol 70 (4) ◽  
pp. 630-632
Author(s):  
Louis S. Pearlstein ◽  
Charles B. Brill ◽  
Elliott L. Mancall
Keyword(s):  
Child Abuse ◽  
High Risk ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Family Dynamics

A case of child abuse occurring in a family in which the mother had Huntington's disease is presented. Families affected by Huntington's disease are at high risk in this regard. The literature and family dynamics are reviewed.

scholarly journals Psychiatric morbidity and poor follow-up underlie suboptimal functional and survival outcomes in Huntington’s disease

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Nikhil Ratna ◽  
Nitish L. Kamble ◽  
Sowmya D. Venkatesh ◽  
Meera Purushottam ◽  
Pramod K. Pal ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Psychiatric Morbidity ◽  
Survival Outcomes

scholarly journals A case-note review of continued pregnancies found to be at a high risk of Huntington’s disease: considerations for clinical practice

2019 ◽  
Vol 27 (8) ◽  
pp. 1215-1224
Author(s):  
Felicity Wadrup ◽  
◽  
Simon Holden ◽  
Rhona MacLeod ◽  
Zosia Miedzybrodzka ◽  
...  
Keyword(s):  
Clinical Practice ◽  
High Risk ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Case Note ◽  
Case Note Review

Which Diagnostic Procedures in the Elderly? The Case of Late-Onset Huntington's Disease

1997 ◽  
Vol 10 (1) ◽  
pp. 39-46 ◽  
Author(s):  
Ildebrando Appollonio ◽  
Giovanni B. Frisoni ◽  
Natale Curtò ◽  
Marco Trabucchi ◽  
Lodovico Frattola
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Late Onset ◽  
Genetic Research ◽  
Cognitive Disorders ◽  
High Technology ◽  
Diagnostic Procedures ◽  
The Elderly ◽  
Diagnostic Process ◽  
Adult Onset

The typical adult-onset form of Huntington's disease (HD) is a clinical condition in which the latest advances of genetic research can be usefully applied during the course of the diagnostic process; not so clear are the guidelines for the much less frequent late-onset variant. We have recently seen three patients in their late sixties who had been misdiagnosed for up to 10 years due to the apparently isolated, mild, and slowly progressive nature of their hyperkinetic movements or cognitive disorders. Only after the results of DNA sequencing on a blood sample became available could the appropriate diagnosis of late-onset HD be reached. By contrast, neuroimaging studies lacked sufficient sensitivity and specificity. Appropriate neurogeriatric assessment in these cases should follow specific guidelines and should always include selected high-technology procedures.

scholarly journals Alzheimer's disease – the experiences of national voluntary organisations

1992 ◽  
Vol 16 (11) ◽  
pp. 689-690 ◽  
Author(s):  
Brian O'Shea
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
New Zealand ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Medical Profession ◽  
The Usa ◽  
Australian Experience ◽  
The Impact ◽  
Voluntary Organisations

I previously reported (O'Shea, 1989) on the experiences of national Huntington's disease (HD) organisations in Britain, Holland, Ireland, New Zealand, and the USA. The Australian experience was discussed later (Kapp, 1990; O'Shea, 1990). The earlier paper supported the contention (Black, 1988) that the medical profession in general was not supportive of HD voluntary groups. I also expressed concern that the lobbying of voluntary groups representing far more common disorders was “bound to dilute the impact that Huntington societies may have.”

A Study of Psychiatric Morbidity in Patients with Huntington's Disease, Their Relatives, and Controls

1993 ◽  
Vol 163 (6) ◽  
pp. 790-797 ◽  
Author(s):  
Per Jensen ◽  
Sven Asger Sørensen ◽  
Kirsten Fenger ◽  
Tom G. Bolwig
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Matched Control ◽  
Psychiatric Morbidity ◽  
Disease Process ◽  
Growing Up ◽  
Control Groups ◽  
First Degree Relatives ◽  
Central Register ◽  
Second Degree

Psychiatric morbidity among 74 non-affected first-degree relatives and 93 non-affected second-degree relatives of patients with Huntington's disease (HD) was compared with that of 37 patients with HD and with matched control groups. Due to specific age criteria, the first-degree relatives were at decreased risk and the second-degree relatives at negligible risk of being carriers of the gene for HD. Information on admissions to departments of psychiatry and diagnoses at discharge were obtained for all subjects from a nationwide central register. Psychiatric morbidity was no greater among relatives than among controls, whereas HD patients had significantly more admissions and psychiatric diagnoses than relatives. Growing up with a risk of developing HD does not itself increase the risk of developing psychiatric illness resulting in hospital admission. Severe psychiatric disorders in HD patients were thus most likely to be aetiologically related to the disease process, possibly through a genetic mechanism.

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