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