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Neural and biomarker correlates of the Parkinson's Disease–Cognitive Rating Scale in Huntington's disease [PDF]
NeuroImage: ReportsBackground: Cognitive decline is a core feature of Huntington's disease (HD), often preceding motor symptoms and progressing with disease severity. While several neuropsychological tests track cognitive changes, few studies have examined the biological ...
Saul Martinez-Horta +11 more
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Assessing the prognostic value of early oculomotor abnormalities in Huntington’s disease [PDF]
Frontiers in NeurologyBackgroundAbnormalities of eye movements occur early in the disease course of Huntington’s disease, often preceding clinical motor diagnosis. The increasing availability of digital eye tracking tools indicate a potential role for eye movements as ...
Ahmad Kaddoura +6 more
doaj +2 more sources
Nature Reviews Disease Primers, 2015 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 ...
Matthew P. Parsons, Lynn A. Raymond
+7 more sources
EBioMedicine, 2021 Background: Pathological processes in Huntington's disease (HD) begin many years prior to symptom onset. Recently we demonstrated that in a premanifest cohort approximately 24 years from predicted disease onset, despite intact function, there was ...
Eileanoir B. Johnson +13 more
doaj +1 more source
Progressive alterations in white matter microstructure across the timecourse of Huntington's disease
Brain and Behavior, 2023 Background Whole‐brain longitudinal diffusion studies are crucial to examine changes in structural connectivity in neurodegeneration. Here, we investigated the longitudinal alterations in white matter (WM) microstructure across the timecourse of ...
Carlos Estevez‐Fraga +13 more
doaj +1 more source
Neuroprotection, 2023 Background Huntington's disease is a progressive neurodegenerative disorder. Brain atrophy, as measured by volumetric magnetic resonance imaging (MRI), is a downstream consequence of neurodegeneration, but microstructural changes within brain tissue are ...
Ashwinee Manivannan +5 more
doaj +1 more source
Nursing Standard, 2004 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.
+6 more sources
Prenatal Diagnosis, 1996 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
Cold Spring Harbor Perspectives in Biology, 2011 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
Case report: Cerebellar sparing in juvenile Huntington's disease
Frontiers in Neurology, 2023 Juvenile Huntington's disease is an early-onset variant of Huntington's disease, generally associated with large CAG repeats and distinct clinical symptoms. The role of the cerebellum in Huntington's disease has been reevaluated, based on the presence of
Bruno Lopes Santos-Lobato +3 more
doaj +1 more source