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Wearable Sensors in Huntington Disease: A Pilot Study [PDF]

open access: yesJournal of Huntington's Disease, 2016
Background: The Unified Huntington’s Disease Rating Scale (UHDRS) is the principal means of assessing motor impairment in Huntington disease but is subjective and generally limited to in-clinic assessments.
K. Andrzejewski   +11 more
semanticscholar   +3 more sources

Clinical presentation of juvenile Huntington disease [PDF]

open access: goldArquivos de Neuro-Psiquiatria, 2006
OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner.
Ruocco Heloísa H.   +4 more
doaj   +2 more sources

Huntington disease exacerbates action impulses [PDF]

open access: yesFrontiers in Psychology, 2023
BackgroundImpulsivity is a common clinical feature of Huntington disease (HD), but the underlying cognitive dynamics of impulse control in this population have not been well-studied.ObjectiveTo investigate the temporal dynamics of action impulse control ...
Shuhei Shiino   +4 more
doaj   +2 more sources

Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease [PDF]

open access: bronzeHuman Molecular Genetics, 2003
An expanded CAG repeat is the underlying genetic defect in Huntington disease, a disorder characterized by motor, psychiatric and cognitive deficits and striatal atrophy associated with neuronal loss.
Elizabeth Slow
openalex   +2 more sources

Predictors of persistence and adherence to deutetrabenazine among patients with Huntington disease or tardive dyskinesia [PDF]

open access: yesMental Health Clinician, 2023
Introduction Deutetrabenazine is approved for treatment of Huntington disease (HD)-related chorea and tardive dyskinesia (TD) in adults. Factors associated with deutetrabenazine persistence and adherence are not well understood.
Claassen Daniel O. MD   +4 more
doaj   +2 more sources

Cortical and Striatal Circuits in Huntington’s Disease

open access: yesFrontiers in Neuroscience, 2020
Huntington’s disease (HD) is a hereditary neurodegenerative disorder that typically manifests in midlife with motor, cognitive, and/or psychiatric symptoms.
Sonja Blumenstock, I. Dudanova
semanticscholar   +2 more sources

In vivo evidence for NMDA receptor mediated excitotoxicity in a murine genetic model of Huntington Disease [PDF]

open access: gold, 2008
N-methyl-D-aspartate receptor (NMDAR) mediated excitotoxicity is implicated as a proximate cause of neurodegeneration in Huntington Disease (HD). However, this hypothesis has not been tested rigorously in vivo. NMDAR NR2B-subunits are the predominant NR2
Mary Y. Heng   +4 more
openalex   +4 more sources

Exploring genetic factors involved in huntington disease age of onset. E2F2 as a new potential modifier gene [PDF]

open access: yes, 2015
Age of onset (AO) of Huntington disease (HD) is mainly determined by the length of the CAG repeat expansion (CAGexp) in exon 1 of the HTT gene. Additional genetic variation has been suggested to contribute to AO, although the mechanism by which it could ...
Aguirre, Ana   +17 more
core   +14 more sources

The BACHD Rat Model of Huntington Disease Shows Signs of Fronto-Striatal Dysfunction in Two Operant Conditioning Tests of Short-Term Memory. [PDF]

open access: yesPLoS ONE, 2017
The BACHD rat is a recently developed transgenic animal model of Huntington disease, a progressive neurodegenerative disorder characterized by extensive loss of striatal neurons.
Erik Karl Håkan Clemensson   +3 more
doaj   +1 more source

Brain hypometabolism in rare genetic neurodegenerative disease: Niemann-Pick disease type C, spinocerebellar ataxia and Huntington disease assessed by FDG PET [PDF]

open access: yesAsia Oceania Journal of Nuclear Medicine and Biology, 2021
Brain metabolic imaging using 18F-fluorodeoxyglucose (FDG) Positron Emission Tomography (PET) with contemporaneous low-dose CT may be used to assess neurodegenerative diseases.
Yung Hsiang Kao   +4 more
doaj   +1 more source

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