scholarly journals Neuropsychiatry of Huntington's disease

2007 ◽  
Vol 9 (2) ◽  
pp. 191-197 ◽  

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.

2016 ◽  
Vol 87 (Suppl 1) ◽  
pp. A103.2-A103 ◽  
Author(s):  
Beatrice Heim ◽  
Sweta Bajaj ◽  
Roberto De Marzi ◽  
Stephanie Mangesius ◽  
Atbin Djamshidian ◽  
...  

2009 ◽  
Vol 24 (S1) ◽  
pp. 1-1
Author(s):  
J. Maia

Huntington's Disease (HD) is an inherited autosomal dominant disorder characterized by motor, cognitive and psychiatric symptomatology, being considered a paradigmatic neuropsychiatric disorder that includes all three components of the "Triadic Syndromes": dyskinesia, dementia and depression.Firstly described in 1872 as an "Hereditary Chorea" by George Huntington only in 1993 was its responsible gene identified. A person who inherits the HD gene will sooner or later develop the disease. the age of onset, early signs and rate of disease progression vary greatly from person to person.Neuropsychiatric symptoms are an integral part of HD and have been considered the earliest markers of the disease, presenting sometimes more than 10 years before a formal diagnosis is done. Patients may experience dysphoria, mood swings, agitation, irritability, hostile outbursts, psychotic symptoms and deep bouts of depression with suicidal ideation. Personality change is reported in 48% of the cases, with the paranoid subtype being described as the most prevalent. the clinical case presented illustrates a case of HD which started with insidious psychiatric symptoms and an important personality change.Despite a wide number of medications being prescribed to help control emotional, movement and behaviour problems, there is still no treatment to stop or reverse the course of the disease. Furthermore, psychiatric manifestations are often amenable to treatment, and relief of these symptoms may provide significant improvement in patient's and caregivers quality of life.A greater awarness of psychiatric manifestations of HD is essential to an earlier diagnosis and an optimized therapeutic approach.


1983 ◽  
Vol 13 (3) ◽  
pp. 537-542 ◽  
Author(s):  
Susan E. Folstein ◽  
Margaret H. Abbott ◽  
Gary A. Chase ◽  
Barbara A. Jensen ◽  
Marshal F. Folstein

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.


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