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Huntingtons psychosis

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
Johnathan Cooper-Knock, Pamela J Shaw, Janine Kirby
The GGGGCC (G4C2) repeat expansion in C9ORF72 is the most common cause of familial amyotrophic lateral sclerosis (ALS), frontotemporal lobar dementia (FTLD) and ALS-FTLD, as well as contributing to sporadic forms of these diseases. Screening of large cohorts of ALS and FTLD cohorts has identified that C9ORF72-ALS is represented throughout the clinical spectrum of ALS phenotypes, though in comparison with other genetic subtypes, C9ORF72 carriers have a higher incidence of bulbar onset disease. In contrast, C9ORF72-FTLD is predominantly associated with behavioural variant FTD, which often presents with psychosis, most commonly in the form of hallucinations and delusions...
March 2014: Acta Neuropathologica
B K Puri, S W Lewis
Cranial single-photon emission computerized tomography (SPECT or SPET) can now give regional cerebral blood flow images with a resolution approaching that of positron emission tomography (PET). In this paper, the use of high resolution SPECT neuroimaging in neuropsychiatric disorders, including Alzheimer's disease, multi-infarct dementia, Pick's disease, progressive supranuclear palsy, Korsakoff's psychosis, Creutzfeld-Jakob disease, Parkinson's disease, Huntington's disease, schizophrenia, mood disorders, obsessive-compulsive disorder, HIV infection and AIDS is reviewed...
1992: Behavioural Neurology
Samuel Frank
Huntington's disease (HD) is a dominantly inherited progressive neurological disease characterized by chorea, an involuntary brief movement that tends to flow between body regions. HD is typically diagnosed based on clinical findings in the setting of a family history and may be confirmed with genetic testing. Predictive testing is available to family members at risk, but only experienced clinicians should perform the counseling and testing. Multiple areas of the brain degenerate, mainly involving the neurotransmitters dopamine, glutamate, and γ-aminobutyric acid...
January 2014: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Hyun Ah Kim, Luning Jiang, Heather Madsen, Clare L Parish, Jim Massalas, Arthur Smardencas, Claire O'Leary, Ilse Gantois, Colm O'Tuathaigh, John L Waddington, Michelle E Ehrlich, Andrew J Lawrence, John Drago
Progressive cell loss is observed in the striatum, cerebral cortex, thalamus, hypothalamus, subthalamic nucleus and hippocampus in Huntington disease. In the striatum, dopamine-responsive medium spiny neurons are preferentially lost. Clinical features include involuntary movements, gait and orofacial impairments in addition to cognitive deficits and psychosis, anxiety and mood disorders. We utilized the Cre-LoxP system to generate mutant mice with selective postnatal ablation of D1 dopamine receptor-expressing striatal neurons to determine which elements of the complex Huntington disease phenotype relate to loss of this neuronal subpopulation...
February 2014: Neurobiology of Disease
Sandor Kantor, Lajos Szabo, Janos Varga, Marc Cuesta, A Jennifer Morton
Sleep disturbances in Huntington's disease may be deleterious to the cognitive performance, affective behaviour, and general well-being of patients, but a comprehensive description of the progression of changes in sleep and electroencephalogram in Huntington's disease has never been conducted. Here we studied sleep and electroencephalogram disturbances in a transgenic mouse model of Huntington's disease (R6/2 mice). We implanted 10 R6/2 mice and five wild-type littermates with electromyography electrodes, frontofrontal and frontoparietal electroencephalogram electrodes and then recorded sleep/wake behaviour at presymptomatic, symptomatic and late stages of the disease...
July 2013: Brain: a Journal of Neurology
Shaheen E Lakhan, Mario Caro, Norell Hadzimichalis
N-Methyl-d-aspartate (NMDA) receptors play a variety of physiologic roles and their proper signaling is essential for cellular homeostasis. Any disruption in this pathway, leading to either enhanced or decreased activity, may result in the manifestation of neuropsychiatric pathologies such as schizophrenia, mood disorders, substance induced psychosis, Huntington's disease, Alzheimer's disease, and neuropsychiatric systemic lupus erythematosus. Here, we explore the notion that the overlap in activity of at least one biochemical pathway, the NMDA receptor pathway, may be the link to understanding the overlap in psychotic symptoms between diseases...
2013: Frontiers in Psychiatry
Monika Edlinger, Klaus Seppi, Wolfgang Fleischhacker, Alex Hofer
We report on the successful use of a combined psychopharmacological treatment in a patient with Huntington's disease, at age 39, suffering from severe psychotic and behavioral symptoms. He presented with a schizophreniform psychosis accompanied by aggressive behavior leading to admission to the locked ward of our hospital. After unsatisfactory control of the psychiatric symptoms with olanzapine, risperidone, and amisulpride, we introduced aripiprazole. This did not affect the psychotic symptoms; however, led to an improvement in aggressive behavior, motivation, and even chorea...
July 2013: International Clinical Psychopharmacology
Haidy E Michel, Mariane G Tadros, Ashraf B Abdel-Naim, Amani E Khalifa
Liquorice extract was reported to have nootropic and/or antiamnestic effects. Prepulse inhibition (PPI) of startle response is a multimodal, cross-species phenomenon used as a measure of sensorimotor gating. Previous studies indicated that liquorice/its constituents augmented mouse brain monoamine levels. Increased brain monoamines' transmission was suggested to underlie PPI disruption. However, the effect of antiamnestic dose(s) of the extract on PPI has not been investigated despite the coexistence of impaired memory and PPI deficit in some neurological disorders...
June 7, 2013: Neuroscience Letters
Cristina Cusin, Felipe Boschini Franco, Carlos Fernandez-Robles, Christina M DuBois, Charles A Welch
Many patients with Huntington's disease (HD) develop psychiatric symptoms such as depression and psychosis. For severe symptoms, electroconvulsive therapy (ECT) can be a valuable treatment. In this case series, we identified seven patients with HD who received ECT at Massachusetts General Hospital in the past 20 years. In all cases, ECT was well tolerated and produced improvement in psychiatric and behavioral symptoms. Our case series supports the hypothesis of a positive risk-benefit ratio for ECT in patients with HD and severe depression or psychosis...
November 2013: General Hospital Psychiatry
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