The association of affective disorder with Huntington's Disease in a case series and in families

1983 ◽  
Vol 13 (3) ◽  
pp. 537-542 ◽  
Author(s):  
Susan E. Folstein ◽  
Margaret H. Abbott ◽  
Gary A. Chase ◽  
Barbara A. Jensen ◽  
Marshal F. Folstein
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Affective Disorder ◽  
Genetic Heterogeneity ◽  
Geographical Area ◽  
Case Series ◽  
Consecutive Case ◽  
Multiple Sources

SynopsisMajor affective disorder clinically similar to the disorder found in conditions other than Huntington's Disease (HD) was found in 41% of patients with HD in a consecutive case series ascertained through multiple sources in a defined geographical area. The association appears to be confined to certain families, and affective disorder may appear as long as 20 years before the onset of chorea and dementia. The association may represent genetic heterogeneity in HD.

M6 Nabilone in huntington’s disease: a case series of five patients

2016 ◽  
Vol 87 (Suppl 1) ◽  
pp. A103.2-A103 ◽  
Author(s):  
Beatrice Heim ◽  
Sweta Bajaj ◽  
Roberto De Marzi ◽  
Stephanie Mangesius ◽  
Atbin Djamshidian ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Case Series

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

CNS Spectrums ◽  
2018 ◽  
Vol 24 (04) ◽  
pp. 413-418 ◽  
Author(s):  
Yael Manor ◽  
Yael Oestreicher-Kedem ◽  
Alona Gad ◽  
Jennifer Zitser ◽  
Achinoam Faust-Socher ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Oropharyngeal Dysphagia ◽  
Case Series ◽  
Solid Food ◽  
Self Report ◽  
Endoscopic Evaluation ◽  
Retrospective Case ◽  
Tertiary Referral Center

BackgroundHuntington’s disease (HD) is a neurodegenerative disease characterized by increasing dysphagia as the disease progresses. Specific characteristics of the HD dysphagia are not well defined.ObjectiveTo characterize the swallowing disturbances of HD patients, to evaluate the feasibility of Fiberoptic Endoscopic Evaluation of Swallowing (FEES) in assessing dysphagia in HD patients, and to discern the relation between FEES findings and patients’ self-report on dysphagia symptoms and swallowing related quality of life (SWAL-QOL).MethodA retrospective case series in a tertiary referral center. All recruited HD patients underwent Bed Side Swallowing Evaluation (BSE), FEES, the Unified Huntington’s Disease Rating Scale (UHDRS), and the Montreal Cognitive Assessment (MoCA). All completed the Swallowing Disturbances Questionnaire (SDQ) and the SWAL-QOL questionnaire.ResultsFourteen HD patients were recruited. All were able to complete the FEES study. The FEES demonstrated delayed swallowing reflex, solid food residues, and pre/post swallowing spillage in most patients (50%, 53.5%, 83.3%, and 87.5%, respectively). The mean SDQ score was 13.2. Significant correlations were found between the SWAL-QOL fear of eating score; the SDQ oral, pharyngeal, and total scores; and the FEES parameters of pureed and solid food bolus flow time. Significant correlations were also found between the total UHDRS score, the volitional cough score, and the SWAL-QOL disease burden score.ConclusionHD patients exhibit prominent unique oropharyngeal dysphagia features that may serve as a marker of disease progression. The FEES and the SDQ are valuable tools for detecting these features in HD patients with swallowing disturbance.

Conduct disorder and affective disorder among the offspring of patients with Huntington's Disease

1983 ◽  
Vol 13 (1) ◽  
pp. 45-52 ◽  
Author(s):  
Susan E. Folstein ◽  
Mary Lousie Franz ◽  
Barbara A. Jensen ◽  
Gary A. Chase ◽  
Marshal F. Folstein
Keyword(s):  
Conduct Disorder ◽  
Psychiatric Disorder ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Affective Disorder ◽  
Genetic Counselling ◽  
Early Onset ◽  
Affective Illness ◽  
Hd Gene ◽  
Early Manifestation

SynopsisThe rate of occurrence of conduct disorder and affective illness was studied for a sample of 112 offspring of 34 Huntington's Disease (HD) patients. Psychiatric disorder in the offspring was assessed as a function of: (1) age of the parent at the onset of symptoms of HD; (2) family disorganization; and (3) psychiatric disorder in either parent. The findings indicated an increased frequency of conduct disorder in disrupted families, most especially in those where the HD parent had an early onset of symptoms and the non-HD parent showed psychiatric disorder. Affective disorder in the offspring was most strongly associated with the presence of similar symptoms in the HD parent. Affective disorder, but not conduct disorder, may be an early manifestation of the HD gene. The implication of these findings for genetic counselling is discussed.

Depression and Huntington's Disease: Prevalence, Clinical Manifestations, Etiology, and Treatment

CNS Spectrums ◽  
2001 ◽  
Vol 6 (4) ◽  
pp. 306-308,325-326 ◽  
Author(s):  
James R. Slaughter ◽  
Matthew P. Martens ◽  
Kathleen A. Slaughter
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Selective Serotonin Reuptake Inhibitors ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Case Series ◽  
Convulsive Therapy ◽  
Number Of Factors ◽  
Literature Searches ◽  
Increased Risk

ABSTRACTIn order to determine the extent to which depression complicates Huntington's disease (HD), we have analyzed the existing literature on depression in HD in order to report the prevalence, clinical manifestations, and treatment of HD depression. By means of MEDLINE literature searches and reviews of HD articles' bibliographies, we identified for our analysis 16 HD depression studies. Our results indicate that the prevalence of depression is 30% for all HD patients. Clinical manifestations of HD depression include a marked increased risk for suicide. The etiology of HD depression is unclear, but may be due to a number of factors, such as dysfunction in the caudate nucleus, dysfunction in the ventral striatum, and various genetic factors that are discussed in this review. Case reports and case series support the efficacy of standard antidepressant interventions in resolving symptoms of depression. Efficacious treatments reported in the literature include tricyclic antidepressants, monoamine oxidase inhibitors, selective serotonin reuptake inhibitors, and electro-convulsive therapy. In this study, the successful anecdotal treatment of seven consecutive HD depressed patients with sertraline suggests that sertraline may be a safe and efficacious treatment of HD depression.

scholarly journals Dose-dependent improvement of myoclonic hyperkinesia due to Valproic acid in eight Huntington's Disease patients: a case series

BMC Neurology ◽  
2006 ◽  
Vol 6 (1) ◽  
Author(s):  
Carsten Saft ◽  
Thorsten Lauter ◽  
Peter H Kraus ◽  
Horst Przuntek ◽  
Juergen E Andrich
Keyword(s):  
Valproic Acid ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Case Series ◽  
Dose Dependent

scholarly journals Psychological Interventions for People with Huntington’s Disease: A Call to Arms

2020 ◽  
Vol 9 (3) ◽  
pp. 231-243
Author(s):  
Nicolò Zarotti ◽  
Maria Dale ◽  
Fiona Eccles ◽  
Jane Simpson
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Case Series ◽  
Psychological Theory ◽  
Randomised Control Trial ◽  
Cochrane Library ◽  
Future Research ◽  
Psychological Interventions ◽  
Wide Range ◽  
Psychological Difficulties

Background: Although Huntington’s disease (HD) can cause a wide range of psychological difficulties, no review has ever been carried out on the range of psychological interventions adopted with this population. Objective: To scope the literature on psychological interventions for psychological difficulties in people affected by HD. Methods: A systematic scoping review was performed across MEDLINE, PsycINFO, CINAHL, Academic Search Ultimate, and Cochrane Library up to 1 March 2020. Results: From an initial return of 1579 citations, a total of nine papers were considered eligible for review. These included a qualitative investigation, three case studies, two case series, two uncontrolled pretest-posttest designs, and only one randomised control trial (RCT). Despite the wide range of psychological difficulties which can be experienced by people affected by the HD gene expansion, the adopted interventions only accounted for five main psychological outcomes (anxiety, apathy, depression, irritability, and coping). Further discussion and suggestions for future research are provided for each outcome. Conclusion: The current literature on psychological interventions in people affected by HD is extremely limited both in terms of methods and addressed clinical outcomes. Consequently, no conclusions can be offered yet as to which psychological therapy may help this population. As further more comprehensive research is urgently needed for this group, the ultimate aim of the present review is to act as a call to arms for HD researchers worldwide to help shed light on the most effective way to translate psychological theory into practice for the benefit of people affected by HD.

scholarly journals Neuropsychiatry of Huntington's disease

2007 ◽  
Vol 9 (2) ◽  
pp. 191-197 ◽  
Keyword(s):  
Major Depression ◽  
General Population ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical Experience ◽  
Psychiatric Diagnosis ◽  
Case Series ◽  
Executive Dysfunction ◽  
Psychiatric Conditions ◽  
Psychiatric Manifestations

Psychiatric manifestations are an integral part of Huntington's disease. They may be divided into those syndromes which resemble idiopathic disorders, but for which HD patients may be particularly at risk, those constellations which are peculiar to HD and related conditions, such as the executive dysfunction syndrome, and those symptoms that can truly be regarded as nonspecific, such as delirium. Most of these problems are believed to arise from subcortical neuropathologic changes. Major depression is a common psychiatric diagnosis, but the executive dysfunction syndrome, a difficult-to-define condition characterized by often simultaneous apathy and disinhibition, may be even more widespread. There are no large controlled studies of psychiatric treatments in HD, but case series, anecdotal reports, and clinical experience indicate that many of these syndromes respond readily to treatment. Further study of the neuropsychiatry of HD may help to reveal the underpinnings of psychiatric conditions found in the general population.

scholarly journals Neuropsychological functioning in juvenile/adolescent onset Huntington's disease: A case series

1997 ◽  
Vol 12 (4) ◽  
pp. 421-421
Author(s):  
J. J. Vasterling ◽  
R. F. White ◽  
K. Marans ◽  
R. Myers ◽  
D. Sax
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuropsychological Functioning ◽  
Case Series
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