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Huntington disease is devastating to patients and their families - with autosomal dominant inheritance, onset typically in the prime of adult life, progressive course, and a combination of motor, cognitive and behavioural features. The disease is caused by an expanded CAG trinucleotide repeat (of variable length) in HTT, the gene that encodes the ...
Michael R Hayden +2 more
exaly +9 more sources
Bariatric surgery and semaglutide in a youth with juvenile Huntington disease and severe obesity: a case report [PDF]
Background Managing severe obesity in youth with chronic, progressive conditions, such as juvenile Huntington disease, presents unique challenges. Juvenile Huntington disease, is characterized by rapid neurodegeneration, with most patients experiencing a
Alaina P. Vidmar +7 more
doaj +2 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
Huntington's disease is a complex degenerative disorder that affects the central nervous system. Although it is a rare condition, nurses are ideally placed to assess and manage patients with the disease, while also providing information and support to family members.
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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 +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
Huntington's disease (HD) is the most common inherited neurodegenerative disease and is characterized by uncontrolled excessive motor movements and cognitive and emotional deficits. The mutation responsible for HD leads to an abnormally long polyglutamine (polyQ) expansion in the huntingtin (Htt) protein, which confers one or more toxic functions to ...
openaire +4 more sources
Background: Unawareness of Huntington disease is prevalent where patients might have a denial of illness, less reporting of symptoms such as changes in behavior or cognitive impairment, or poor coping with the disease.
Nawal H Alharthi +2 more
doaj +1 more source
Huntington disease exacerbates action impulses
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 +1 more source
Huntington's disease (HD) is a late-onset degenerative disorder of the central nervous system, caused by a dominantly inherited mutation in a gene on chromosome 4p. The identification of the trinucleotide repeat mutation responsible for this disorder has been an important step towards understanding the molecular pathology of HD, but in the meantime has
Shutish C. Patel +2 more
+8 more sources

