PET Imaging in Huntington’s Disease

Roussakis, Andreas–Antonios; Piccini, Paola

doi:10.3233/jhd-150171

Cited by 16 publications

(13 citation statements)

References 55 publications

(48 reference statements)

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“…In this approach, methods such as PET, functional MRI (fMRI) and magnetic resonance spectroscopy (MRS) are used to quantify the neuronal activity and functioning of specific brain regions by measuring specific metabolites. An example is 18F-fluorodeoxyglucose (FDG)-PET analysis used to quantify glucose hypometabolism in the striatum in HD patients [ 42 ].…”

Section: Biomarkers—general Information and Types Applicable For Hmentioning

confidence: 99%

What, When and How to Measure—Peripheral Biomarkers in Therapy of Huntington’s Disease

Przybył

Woźna-Wysocka

Kozłowska

et al. 2021

IJMS

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Among the main challenges in further advancing therapeutic strategies for Huntington’s disease (HD) is the development of biomarkers which must be applied to assess the efficiency of the treatment. HD is a dreadful neurodegenerative disorder which has its source of pathogenesis in the central nervous system (CNS) but is reflected by symptoms in the periphery. Visible symptoms include motor deficits and slight changes in peripheral tissues, which can be used as hallmarks for prognosis of the course of HD, e.g., the onset of the disease symptoms. Knowing how the pathology develops in the context of whole organisms is crucial for the development of therapy which would be the most beneficial for patients, as well as for proposing appropriate biomarkers to monitor disease progression and/or efficiency of treatment. We focus here on molecular peripheral biomarkers which could be used as a measurable outcome of potential therapy. We present and discuss a list of wet biomarkers which have been proposed in recent years to measure pre- and postsymptomatic HD. Interestingly, investigation of peripheral biomarkers in HD can unravel new aspects of the disease pathogenesis. This especially refers to inflammatory proteins or specific immune cells which attract scientific attention in neurodegenerative disorders.

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Section: Biomarkers—general Information and Types Applicable For Hmentioning

confidence: 99%

What, When and How to Measure—Peripheral Biomarkers in Therapy of Huntington’s Disease

Przybył

Woźna-Wysocka

Kozłowska

et al. 2021

IJMS

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“…183 Different radiotracers have been used with PET to measure brain metabolism, dopaminergic function, neuro-inflammation, phosphodiesterases, and other targets in HD. 183 However, few adenosine analog radiotracers have been developed and employed to noninvasively image A 1 R and A 2A R occupancy.…”

Section: Pet Imaging For Adenosine Receptor Occupancy In Hdmentioning

confidence: 99%

The Role of Adenosine Tone and Adenosine Receptors in Huntington's Disease

Blum

Chern

Domenici

et al. 2018

Journal of Caffeine and Adenosine Research

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Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by a mutation in the IT15 gene that encodes for the huntingtin protein. Mutated hungtingtin, although widely expressed in the brain, predominantly affects striato-pallidal neurons, particularly enriched with adenosine A 2A receptors (A 2A R), suggesting a possible involvement of adenosine and A 2A R is the pathogenesis of HD. In fact, polymorphic variation in the ADORA2A gene influences the age at onset in HD, and A 2A R dynamics is altered by mutated huntingtin. Basal levels of adenosine and adenosine receptors are involved in many processes critical for neuronal function and homeostasis, including modulation of synaptic activity and excitotoxicity, the control of neurotrophin levels and functions, and the regulation of protein degradation mechanisms. In the present review, we critically analyze the current literature involving the effect of altered adenosine tone and adenosine receptors in HD and discuss why therapeutics that modulate the adenosine system may represent a novel approach for the treatment of HD.Keywords: Huntington's disease, adenosine, adenosine receptors, equilibrative nucleoside transporter, animal models Pathogenic Mechanisms of Huntington's DiseaseOverview H untington's disease (HD) is an inherited neurodegenerative disorder that is caused by a single, autosomal dominant gene mutation. HD generally affects young adults and is characterized by involuntary, abnormal movements and postures (chorea, dyskinesia, dystonia), psychiatric disturbances, and cognitive alterations.1,2 It is estimated that 1 in 10,000 people have HD and this disorder is fatal within 15-20 years after the onset of symptoms. Multiple brain regions, including several cortical and subcortical regions (cerebral cortex, layers III, V, and VI; pallidum, sub-thalamic nucleus, cerebellum), show signs of neurodegeneration, but the most pronounced neuronal loss is present in the caudate nucleus and the putamen of the striatum.3 Within the striatum, the striato-pallidal, medium spiny neurons (MSN) that express enkephalin, dopamine D 2 receptors (D 2 R) and adenosine A 2A receptors (A 2A R), 4,5 appear to be the most vulnerable.6,7

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“…[42][43][44] Overall, there is increasing evidence of reduced glucose metabolism in the striatum, and frontal and temporal cortices, which seem to be reliable predictors of disease progression in HD. [42][43][44] There have been no PET studies performed to date that specifically focused on the glucose metabolism of the visual cortex in HD patients. However, an interesting finding was observed by a study group that examined spatial covariance patterns between different networks of regions with altered glucose metabolism using PET imaging.…”

Section: Cerebral Metabolismmentioning

confidence: 99%

The visual cortex and visual cognition in Huntington’s disease: An overview of current literature

Coppen

Hart

Lakke

et al. 2018

Behavioural Brain Research

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The processing of visual stimuli from retina to higher cortical areas has been extensively studied in the human brain. In Huntington's disease (HD), an inherited neurodegenerative disorder, it is suggested that visual processing deficits are present in addition to more characteristic signs such as motor disturbances, cognitive dysfunction, and behavioral changes. Visual deficits are clinically important because they influence overall cognitive performance and have implications for daily functioning. The aim of this review is to summarize current literature on clinical visual deficits, visual cognitive impairment, and underlying visual cortical changes in HD patients. A literature search was conducted using the electronic database of PubMed/Medline. This review shows that changes of the visual system in patients with HD were not the primary focus of currently published studies. Still, early atrophy and alterations of the posterior cerebral cortex was frequently observed, primarily in the associative visual cortical areas such as the lingual and fusiform gyri, and lateral occipital cortex. Changes were even present in the premanifest phase, before clinical onset of motor symptoms, suggesting a primary region for cortical degeneration in HD. Although impairments in visuospatial processing and visual perception were reported in early disease stages, heterogeneous cognitive batteries were used, making a direct comparison between studies difficult. The use of a standardized battery of visual cognitive tasks might therefore provide more detailed information regarding the extent of impairments in specific visual domains. Further research could provide more insight into clinical, functional, and pathophysiological changes of the visual pathway in HD.

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PET Imaging in Huntington’s Disease

Cited by 16 publications

References 55 publications

What, When and How to Measure—Peripheral Biomarkers in Therapy of Huntington’s Disease

What, When and How to Measure—Peripheral Biomarkers in Therapy of Huntington’s Disease

The Role of Adenosine Tone and Adenosine Receptors in Huntington's Disease

The visual cortex and visual cognition in Huntington’s disease: An overview of current literature

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