Determinants of Onset of Huntington’s Disease with Behavioral Symptoms: Insight from 92 Patients

2015 ◽  
Vol 4 (4) ◽  
pp. 319-324 ◽  
Author(s):  
Abhishek Lenka ◽  
Nitish L. Kamble ◽  
V. Sowmya ◽  
Ketan Jhunjhunwala ◽  
Ravi Yadav ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Behavioral Symptoms

scholarly journals Attenuation of Rhes Activity Significantly Delays the Appearance of Behavioral Symptoms in a Mouse Model of Huntington's Disease

PLoS ONE ◽  
2013 ◽  
Vol 8 (1) ◽  
pp. e53606 ◽  
Author(s):  
Brandon A. Baiamonte ◽  
Franklin A. Lee ◽  
Steve T. Brewer ◽  
Daniela Spano ◽  
Gerald J. LaHoste
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Behavioral Symptoms

Response to letter by Saenz‐Farret et al. on “Rating scales for behavioral symptoms in Huntington's disease: Critique and recommendations”

2017 ◽  
Vol 32 (3) ◽  
pp. 482-482
Author(s):  
Tiago A. Mestre ◽  
Erik van Duijn ◽  
Aileen M. Davis ◽  
Anne‐Catherine Bachoud‐Lévi ◽  
Monica Busse ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scales ◽  
Behavioral Symptoms

scholarly journals Revisiting the neuropsychiatry of Huntington's disease

2016 ◽  
Vol 10 (4) ◽  
pp. 261-266 ◽  
Author(s):  
Antonio Lucio Teixeira ◽  
Leonardo Cruz de Souza ◽  
Natalia Pessoa Rocha ◽  
Erin Furr-Stimming ◽  
Edward C. Lauterbach
Keyword(s):  
Cognitive Impairment ◽  
Neurodegenerative Disease ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Functional Capacity ◽  
Autosomal Dominant ◽  
Neuropsychiatric Symptoms ◽  
Executive Dysfunction ◽  
Behavioral Symptoms ◽  
Motor Symptoms

ABSTRACT Huntington's disease (HD) is an autosomal dominant neurodegenerative disease classified under the choreas. Besides motor symptoms, HD is marked by cognitive and behavioral symptoms, impacting patients' functional capacity. The progression of cognitive impairment and neuropsychiatric symptoms occur in parallel with neurodegeneration. The nature of these symptoms is very dynamic, and the major clinical challenges include executive dysfunction, apathy, depression and irritability. Herein, we provide a focused updated review on the cognitive and psychiatric features of HD.

scholarly journals Clinical overview and therapeutic management of the cognitive and behavorial aspects of Huntington’s disease

2021 ◽  
Author(s):  
Giovanna de Camargo Innocencio ◽  
Juliana de Souza Rosa ◽  
Patrick de Abreu Cunha Lopes ◽  
Paulo Roberto Hernandes Júnior ◽  
Jhoney Francieis Feitosa
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Treatment Success ◽  
Executive Dysfunction ◽  
Therapeutic Management ◽  
Behavioral Symptoms ◽  
Clinical Aspects ◽  
Attention Disorders ◽  
Positive Effects ◽  
Pubmed Database

Background: Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease classified among chorea that, in addition to motor symptoms, is characterized by neuropsychiatric disorders. Objectives: to analyze the cognitive and behavioral clinical aspects of Huntington’s disease and the therapeutic management of these symptoms. Methods: a literature review was carried out from the Scielo and PubMed database, using “Huntington’s disease”, “Cognition”, “Behavior” and “Treatment” as descriptors, where 11 articles were selected between 2001 and 2018. Results: the nature of cognitive and behavioral symptoms in HD are very dynamic, and cognitive dysfunctions are present prior to diagnosis. The most common clinical challenges include executive dysfunction, mainly slow thinking and attention disorders, apathy, depression and irritability. One study used data from the European Huntington’s Disease Network and evaluated almost 2.000 carriers of the mutation, in which 47.4% reported apathy, while depression and irritability occurred in 42.1%, and aggression and psychosis occurred in 38.6%. Other studies have confirmed apathy as an early manifestation related to its progression. For the therapeutic management of chorea, tetrabenazine is used, while antidepressants can be effective in mood symptoms. On the other hand, antipsychotics can lead to the advance and rapid progress of the disease. Small controlled studies with atomoxetine, donepezil and rivastigmine have found no positive effects on patients’ cognition. Recent publications have shown that circulating levels of brain-derived neutrotrophic factors in HD correlate with mood, cognition and motor function and can serve as markers of treatment success, while growth factor I is associated with cognitive decline and can provide biomarker targets for treatment validation. Conclusion: cognitive and behavioral symptoms in HD are very diversified and some strategies may have potential therapies and/or deleterious ones.

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

2005 ◽  
Vol 490 (4) ◽  
pp. 354-370 ◽  
Author(s):  
Edward C. Stack ◽  
James K. Kubilus ◽  
Karen Smith ◽  
Kerry Cormier ◽  
Steven J. Del Signore ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Behavioral Symptoms

scholarly journals Rating scales for behavioral symptoms in Huntington's disease: Critique and recommendations

2016 ◽  
Vol 31 (10) ◽  
pp. 1466-1478 ◽  
Author(s):  
Tiago A. Mestre ◽  
Erik van Duijn ◽  
Aileen M. Davis ◽  
Anne-Catherine Bachoud-Lévi ◽  
Monica Busse ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scales ◽  
Behavioral Symptoms

Poster 28: Delphi Process for the Development of Treatment Guidelines for Behavioral Symptoms and Chorea in Huntington's Disease

2010 ◽  
Vol 7 (1) ◽  
pp. 147-147
Author(s):  
E. van Duijn ◽  
M. Groves ◽  
D. Craufurd ◽  
K. Anderson ◽  
M. Guttman ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Treatment Guidelines ◽  
Delphi Process ◽  
Behavioral Symptoms

scholarly journals Huntington’s disease: Pathophysiology and therapeutic intervention

2021 ◽  
Vol 15 (2) ◽  
pp. 171-184
Author(s):  
Payal B Kshirsagar ◽  
Hemant S Kanhere ◽  
Pallavi C Bansinge ◽  
Sawan K Rathod ◽  
Vrushali S Khandare ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Deficit ◽  
Autosomal Dominant ◽  
Behavioral Symptoms ◽  
Dominant Trait ◽  
Autosomal Dominant Trait ◽  
Juvenile Onset ◽  
Psychiatric Disturbances ◽  
European Populations

Huntington's disease [HD] is a progressive neurodegenerative condition characterized by movement disorder, cognitive impairment, and behavioral symptoms. It is inherited as an autosomal-dominant trait and normally manifests in mid-adulthood. HD is common in India and parts of Central Asia, with a prevalence rate of 4–8 per 100 000 in most European populations. Juvenile onset affects around 5–10% of cases, with signs appearing before the age of 20. Patients may show more parkinsonian symptoms such as bradykinesia, dystonia, tremors and a cognitive deficit in place of chorea. There is no therapy that can completely stop the condition from progressing. There are medications that can help to regulate chorea, dystonia, mental, and psychiatric disturbances. The study covers the disease's pathophysiology, as well as plants and phytochemicals that have been shown to be beneficial.

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