Huntington's disease, behavioral disturbances, and kynurenines: Preclinical findings and therapeutic perspectives

1996 ◽  
Vol 39 (12) ◽  
pp. 1061-1063 ◽  
Author(s):  
László Vécsei ◽  
M. Flint Beal
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Behavioral Disturbances

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 Clinical and genetic analysis of 29 Brazilian patients with Huntington's disease-like phenotype

2011 ◽  
Vol 69 (3) ◽  
pp. 419-423 ◽  
Author(s):  
Guilherme Riccioppo Rodrigues ◽  
Ruth H. Walker ◽  
Benedikt Bader ◽  
Adrian Danek ◽  
Alexis Brice ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Spinocerebellar Ataxia ◽  
Neurodegenerative Disorder ◽  
African Ancestry ◽  
Behavioral Disturbances

Huntington's disease (HD) is a neurodegenerative disorder characterized by chorea, behavioral disturbances and dementia, caused by a pathological expansion of the CAG trinucleotide in the HTT gene. Several patients have been recognized with the typical HD phenotype without the expected mutation. The objective of this study was to assess the occurrence of diseases such as Huntington's disease-like 2 (HDL2), spinocerebellar ataxia (SCA) 1, SCA2, SCA3, SCA7, dentatorubral-pallidoluysian atrophy (DRPLA) and chorea-acanthocytosis (ChAc) among 29 Brazilian patients with a HD-like phenotype. In the group analyzed, we found 3 patients with HDL2 and 2 patients with ChAc. The diagnosis was not reached in 79.3% of the patients. HDL2 was the main cause of the HD-like phenotype in the group analyzed, and is attributable to the African ancestry of this population. However, the etiology of the disease remains undetermined in the majority of the HD negative patients with HD-like phenotype.

Huntington Disease

2017 ◽  
Author(s):  
Charles S. Venuto ◽  
Karl Kieburtz
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Supportive Therapy ◽  
Off Label Use ◽  
Behavioral Disturbances ◽  
Pharmacologic Agent ◽  
Off Label ◽  
Progression Of Disease ◽  
Specific Symptoms

The clinical management of Huntington’s disease entails pharmacologic interventions and nonpharmacologic supportive therapy. There are no treatments that can halt or alter the progression of disease, therefore the goal is to maximize function and optimize quality of life. Tetrabenazine is the only pharmacologic agent with regulatory approval for Huntington’s disease chorea; however, off-label use of antidopaminergic agents is common. Treatment of behavioral disturbances can be tailored to the specific symptoms by using antidepressant, antipsychotic, and anxiolytic agents. Clinical trials testing therapeutic strategies for motor, behavioral, and cognitive aspects of disease and delaying progression are ongoing.

Monitoring Huntington's Disease Mortality across a 30-Year Period: Geographic and Temporal Patterns

2016 ◽  
Vol 47 (3-4) ◽  
pp. 155-163 ◽  
Author(s):  
Germán Sánchez-Díaz ◽  
Greta Arias-Merino ◽  
Ana Villaverde-Hueso ◽  
Antonio Morales-Piga ◽  
Ignacio Abaitua-Borda ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Health Care Disparities ◽  
European Population ◽  
Geographical Differences ◽  
Behavioral Disturbances ◽  
Disease Mortality ◽  
Spatial Changes ◽  
Gender And Age ◽  
Standardized Mortality Ratios

Background: Huntington's disease (HD) is a progressive neurodegenerative condition characterized by chorea, dystonia, behavioral disturbances and cognitive decline. The aim of this study is to assess temporal and spatial changes on mortality attributable to HD over 30 years in Spain. Methods: HD data were extracted from the nationwide mortality registry for the period 1984-2013. Annual and 5-year gender- and age-specific rates adjusted for the standard European population were calculated. Geographic analysis was performed by districts from 1999 through 2013, and then estimated standardized mortality ratios (SMRs) and smoothed SMRs. Results: There were 1,556 HD-related deaths across the study period. An increasing trend in age-adjusted HD mortality was in evidence, specifically from 1994 through 1998. On a year-by-year basis, age-adjusted mortality rates increased from 0.076 per 100,000 population in 1984 to 0.157 in 2013. Geographical differences among districts were evident in specific areas and in the southwest of Spain with a significantly higher HD mortality risk. Conclusion: HD mortality rising trends in Spain might be attributable to improvements in diagnosis leading to a rise in prevalence. Geographical variability in HD mortality could be related to regional differences in disease prevalence, health-care disparities, or other factors which call for in-depth assessment in future studies.

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