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Huntingtons psychosis

Valeriya Navrotskaya, Artur Wnorowski, Waldemar Turski, Gregory Oxenkrug
Kynurenic acid (KYNA) is one of the metabolites of evolutionary conserved tryptophan (Trp)/kynurenine (Kyn) metabolic pathway. Elevation of KYNA contributes to development of psychosis in schizophrenia but attenuates neurodegeneration in Drosophila model of Huntington's disease. We have reported that KYNA increased lethality of pupae of wild-type flies, but not of vermilion (v) mutants with impaired formation of Kyn from Trp, suggesting that KYNA toxicity depends on its interaction with downstream Kyn metabolites [i...
April 4, 2018: Neurotoxicity Research
Debby W Tsuang, Tiffany A Greenwood, Suman Jayadev, Marie Davis, Andrew Shutes-David, Thomas D Bird
BACKGROUND: Psychotic symptoms of delusions and hallucinations occur in about 5% of persons with Huntington's disease (HD). The mechanisms underlying these occurrences are unknown, but the same symptoms also occur in schizophrenia, and thus genetic risk factors for schizophrenia may be relevant to the development of psychosis in HD. OBJECTIVE: To investigate the possible role of genes associated with schizophrenia in the occurrence of psychotic symptoms in HD. METHODS: DNA from subjects with HD and psychosis (HD+P; n = 47), subjects with HD and no psychosis (HD-P; n = 126), and controls (CTLs; n = 207) was genotyped using the Infinium PsychArray-24 v1...
2018: Journal of Huntington's Disease
Michał Danek, Janusz Danek, Aleksander Araszkiewicz
Many animal models in different species have been developed for mental and behavioral disorders. This review presents large animals (dog, ovine, swine, horse) as potential models of this disorders. The article was based on the researches that were published in the peer-reviewed journals. Aliterature research was carried out using the PubMed database. The above issues were discussed in the several problem groups in accordance with the WHO International Statistical Classification of Diseases and Related Health Problems 10thRevision (ICD-10), in particular regarding: organic, including symptomatic, disorders; mental disorders (Alzheimer's disease and Huntington's disease, pernicious anemia and hepatic encephalopathy, epilepsy, Parkinson's disease, Creutzfeldt-Jakob disease); behavioral disorders due to psychoactive substance use (alcoholic intoxication, abuse of morphine); schizophrenia and other schizotypal disorders (puerperal psychosis); mood (affective) disorders (depressive episode); neurotic, stress-related and somatoform disorders (posttraumatic stress disorder, obsessive-compulsive disorder); behavioral syndromes associated with physiological disturbances and physical factors (anxiety disorders, anorexia nervosa, narcolepsy); mental retardation (Cohen syndrome, Down syndrome, Hunter syndrome); behavioral and emotional disorders (attention deficit hyperactivity disorder)...
December 30, 2017: Psychiatria Polska
Keane Lim, Yuen Mei See, Jimmy Lee
The discovery of endocannabinoid's role within the central nervous system and its potential therapeutic benefits have brought forth rising interest in the use of cannabis for medical purposes. The present review aimed to synthesize and evaluate the available evidences on the efficacy of cannabis and its derivatives for psychiatric, neurodegenerative and movement disorders. A systematic search of randomized controlled trials of cannabis and its derivatives were conducted via databases (PubMed, Embase and the Cochrane Central Register of Controlled Trials)...
November 30, 2017: Clinical Psychopharmacology and Neuroscience: the Official Scientific Journal of the Korean College of Neuropsychopharmacology
Ana Rita Saldanha Ramos, Carolina Garrett
Huntington's disease (HD) is an incurable, neurodegenerative disease, which manifests via a triad of progressive symptoms: motor impairment, psychiatric disorders, and cognitive decline. Conventionally, the HD diagnosis is based on the presence of involuntary choreiform movements and a positive genetic test for the CAG-expanded allele gene. Although the diagnosis focuses on the motor part of the triad, there is increasing evidence that both cognitive and neuropsychiatric symptoms can, and often do, present decades before the onset of motor symptoms...
2017: Neuro-degenerative Diseases
Amelia D Moser, Eric Epping, Patricia Espe-Pfeifer, Erin Martin, Leah Zhorne, Katherine Mathews, Martha Nance, Denise Hudgell, Oliver Quarrell, Peg Nopoulos
AIM: The symptoms of Huntington's disease are well known, yet the symptoms of juvenile Huntington's disease (JHD) are less established due to its rarity. The study examined a cluster of symptoms considered to be common, but under-recognized in JHD: pain, itching, sleeping difficulties, psychosis and tics. MATERIALS & METHODS: A symptom survey was constructed using the online tool Qualtrics and dispersed to JHD caregivers through websites. RESULTS: A total of 33 surveys were completed...
October 18, 2017: Neurodegenerative Disease Management
Erik van Duijn
Almost all patients with Huntington disease (HD) show some form of behavioral changes during the disease progression. These behavioral manifestations may already be present before cognitive or motor symptoms become apparent, and vary from subtle anxiety to severe psychosis and suicide attempts. These behavioral manifestations are related to both psychologic factors, like the burden of having a severe disease, and biologic factors that are related to the disease itself. In this chapter, both nonpharmacologic and pharmacologic interventions are discussed...
2017: Handbook of Clinical Neurology
Seda Koyuncu, Azra Fatima, Ricardo Gutierrez-Garcia, David Vilchez
Huntington's disease (HD) is a fatal neurodegenerative disorder characterized by motor dysfunction, cognitive deficits and psychosis. HD is caused by mutations in the Huntingtin (HTT) gene, resulting in the expansion of polyglutamine (polyQ) repeats in the HTT protein. Mutant HTT is prone to aggregation, and the accumulation of polyQ-expanded fibrils as well as intermediate oligomers formed during the aggregation process contribute to neurodegeneration. Distinct protein homeostasis (proteostasis) nodes such as chaperone-mediated folding and proteolytic systems regulate the aggregation and degradation of HTT...
July 19, 2017: International Journal of Molecular Sciences
Sujita Kumar Kar, Mohit Kumar Shahi, Adarsh Tripathi, Praveen Kumar Sharma
Huntington's disease (HD) is rare variant of progressive neurodegenerative disorder which follows an autosomal dominant pattern. Psychiatric comorbidities are not uncommon with HD. Mood disorder, cognitive disturbances, anxiety disorders, and psychosis are the psychiatric comorbidities reported with HD. We report here a case of HD, where psychosis developed during illness. Prognosis of psychosis in HD is emphasized in this report with review of literature.
July 2017: Journal of Neurosciences in Rural Practice
Navneet Kaur, Puneet Kumar, Sumit Jamwal, Rahul Deshmukh, Vinod Gauttam
BACKGROUND: Tetrabenazine (TBZ) is the only US Food and Drug Administration-approved drug for the treatment of chorea related to Huntington's disease and other hyperkinetic disorders. TBZ was first synthesized in 1950, and was then used for the treatment of psychosis. But later its potential in treating hyperkinetic disorders was proved by its ability to block vesicular monoamine transporters 2 and deplete monoamine stores. There is still lack of awareness about the therapeutic potential of this drug...
September 2016: Annals of Neurosciences
Ana Laura Colín-González, Gabriela Aguilera, Abel Santamaría
The endocannabinoid system (ECS) comprises a complex of receptors, enzymes, and endogenous agonists that are widely distributed in the central nervous system of mammals and participates in a considerable number of neuromodulatory functions, including neurotransmission, immunological control, and cell signaling. In turn, the kynurenine pathway (KP) is the most relevant metabolic route for tryptophan degradation to form the metabolic precursor NAD(+). Recent studies demonstrate that the control exerted by the pharmacological manipulation of the ECS on the glutamatergic system in the brain may offer key information not only on the development of psychiatric disorders like psychosis and schizophrenia-like symptoms, but it also may constitute a solid basis for the development of therapeutic strategies to combat excitotoxic events occurring in neurological disorders like Huntington's disease (HD)...
2016: Advances in Neurobiology
Changqing Xu, Jegan Yogaratnam, Nigel Tan, Kang Sim
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease characterized by a triad of progressive motor dysfunction, cognitive decline and psychiatric disturbances. The hallmark of HD is the distinctive choreiform movement disorder that typically has a subtle, insidious onset in the fourth to fifth decade of life and gradually worsens over 10 to 20 years until death. Notably, two-thirds of HD patients present with chorea and one third with mental changes. The prevalence of psychiatric symptoms is significantly higher than in the general population, and is estimated to be around 66-73%...
August 31, 2016: Clinical Psychopharmacology and Neuroscience: the Official Scientific Journal of the Korean College of Neuropsychopharmacology
Tiago A Mestre, Erik van Duijn, Aileen M Davis, Anne-Catherine Bachoud-Lévi, Monica Busse, Karen E Anderson, Joaquim J Ferreira, Philipp Mahlknecht, Vitor Tumas, Cristina Sampaio, Chris G Goetz, Esther Cubo, Glenn T Stebbins, Pablo Martinez-Martin
Behavioral symptoms are an important feature of Huntington's disease and contribute to impairment in quality of life. The Movement Disorder Society commissioned the assessment of the clinimetric properties of rating scales in Huntington's disease to make recommendations regarding their use, following previously used standardized criteria. A systematic literature search was conducted to identify the scales used to assess behavioral symptoms in Huntington's disease. For the purpose of this review, 7 behavioral domains were deemed significant in Huntington's disease: irritability, anxiety, depression, apathy, obsessive-compulsive behaviors, psychosis, and suicidal ideation...
October 2016: Movement Disorders: Official Journal of the Movement Disorder Society
J D Rollnik
BACKGROUND: Huntington's disease (HD) is a progressive neurodegenerative disorder characterized by hyperkinetic movements, psychiatric (e.g. depression and psychosis) and cognitive symptoms (frontal lobe dementia). In Germany approximately 8000 patients suffer from HD. OBJECTIVES: The paper reviews the clinical course, epidemiology, genetics, differential diagnoses, pathophysiology, symptomatics and causal treatment options. METHODS: Publications on animal and human HD studies and trials and reviews available in Medline have been taken into account...
June 2015: Der Nervenarzt
E van der Weijde, A Schadé
Huntington's disease is characterised by a triad of motor symptoms (choreic movements), psychiatric symptoms and cognitive decline. Much less is known about the psychiatric symptoms such as psychosis and anxiety than about the motor symptoms. We discuss the case of a patient with Huntington's disease and psychosis and comment on the possible types of medication that should be used.
2015: Tijdschrift Voor Psychiatrie
Jonathan Ding, Amin Muhammad Gadit
This is a case of a 60-year-old man who presented with a 6-month history of increasing agitation and emotional volatility. His family brought him to the emergency room as they were concerned about his threatening and aggressive behaviour. The patient was initially incoherent and uncooperative. During the interview, the patient's family revealed that he had a previous diagnosis of Huntington's disease; there was also a family history of personality changes preceding Huntington's chorea. The patient was admitted to the psychiatric inpatient unit and started on low-dose risperidone...
2014: BMJ Case Reports
Leora L Borek, Joseph H Friedman
INTRODUCTION: Psychosis is a major psychiatric problem that often occurs at the interface of psychiatry and the neurological specialty of movement disorders. Psychotic syndromes are common in treated movement disorder patients, and almost all antipsychotic drugs produce movement disorders. There is little published data on psychosis in movement disorders aside from Parkinson's disease (PD). AREAS COVERED: In this review, we focus primarily on PD, in which about 30% of treated patients have visual hallucinations and 5-10% have paranoid delusions; dementia with Lewy bodies, a variant of PD in which dementia occurs early and psychotic symptoms are common; Huntington's disease (HD), an inherited disorder that causes behavioral problems, frequently including psychosis; and tardive dyskinesia (TD), a group of movement disorder syndromes caused by antipsychotic drugs...
August 2014: Expert Opinion on Pharmacotherapy
Erik van Duijn, David Craufurd, Anna A M Hubers, Erik J Giltay, Raphael Bonelli, Hugh Rickards, Karen E Anderson, Marleen R van Walsem, Rose C van der Mast, Michael Orth, G Bernhard Landwehrmeyer
BACKGROUND: The majority of Huntington's disease (HD) mutation carriers experience some psychopathology during their lifetime, varying from irritability to psychosis, but prevalences of particular symptoms vary widely due to diverse study populations in different stages of HD and the use of different assessment methods. METHODS: The study population consisted of 1993 HD mutation carriers from 15 European countries, all participating in the observational REGISTRY study...
December 2014: Journal of Neurology, Neurosurgery, and Psychiatry
Barbara J Zarowitz, Terrence O'Shea, Martha Nance
BACKGROUND: The purpose of this descriptive, retrospective analysis was to develop a demographic and clinical profile of nursing home residents with a diagnosis of Huntington's disease (HD). METHODS: Queries were made of a large data repository of linked and de-identified Minimum Data Set version 3.0 and prescription claims records, for the time period of October 1, 2010 through March 31, 2012. RESULTS: Of 249,811 residents, 340 (0.14%) had a diagnosis of HD; 61% were female and 77...
June 2014: Journal of the American Medical Directors Association
M Nagel, H J Rumpf, M Kasten
Huntington disease (HD) is an inherited, progressive, autosomal dominant disorder. Some patients develop severe chorea or cognitive symptoms. The genetic defect causes progressive atrophy of the striatum, the cortex and extrastriatal structures (Sheperd GM. Corticostriatal connectivity and its role in disease. Nat Rev Neurosci 2013;14:278-91). The precise timing of clinical diagnosis of HD is poorly characterized and is mainly based on motor symptoms (Huntington, Study and Group. Unified Huntington's Disease Rating Scale: reliability and consistency...
May 2014: General Hospital Psychiatry
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