Статті в журналах: "Huntington's disease Magnetic resonance imaging"

1

Sethi, Kapil D. "Magnetic resonance imaging in huntington's disease." Movement Disorders 6, no. 2 (1991): 186. http://dx.doi.org/10.1002/mds.870060223.

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2

Singh, Paramdeep, and Rupinderjeet Kaur. "Magnetic resonance imaging findings in case of Huntington's disease." Journal of Clinical Sciences 17, no. 1 (2020): 9. http://dx.doi.org/10.4103/jcls.jcls_46_19.

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3

Klöppel, S., S. M. Henley, N. Z. Hobbs, R. C. Wolf, J. Kassubek, S. J. Tabrizi, and R. S. J. Frackowiak. "Magnetic resonance imaging of Huntington's disease: preparing for clinical trials." Neuroscience 164, no. 1 (November 2009): 205–19. http://dx.doi.org/10.1016/j.neuroscience.2009.01.045.

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4

Wolf, Robert Christian, Fabio Sambataro, Nenad Vasic, Nadine Donata Wolf, Philipp Arthur Thomann, G. Bernhard Landwehrmeyer, and Michael Orth. "Longitudinal functional magnetic resonance imaging of cognition in preclinical Huntington's disease." Experimental Neurology 231, no. 2 (October 2011): 214–22. http://dx.doi.org/10.1016/j.expneurol.2011.06.011.

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Domínguez, Juan F., Julie C. Stout, Govinda Poudel, Andrew Churchyard, Phyllis Chua, Gary F. Egan, and Nellie Georgiou-Karistianis. "Multimodal imaging biomarkers in premanifest and early Huntington's disease: 30-month IMAGE-HD data." British Journal of Psychiatry 208, no. 6 (June 2016): 571–78. http://dx.doi.org/10.1192/bjp.bp.114.156588.

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BackgroundThe discovery of potential disease-modifying therapies in a neurodegenerative condition like Huntington's disease depends on the availability of sensitive biomarkers that reflect decline across disease stages and that are functionally and clinically relevant.AimsTo quantify macrostructural and microstructural changes in participants with premanifest and symptomatic Huntington's disease over 30 months, and to establish their functional and clinical relevance.MethodMultimodal magnetic resonance imaging study measuring changes in macrostructural (volume) and microstructural (diffusivity) measures in 40 patients with premanifest Huntington's disease, 36 patients with symptomatic Huntington's disease and 36 healthy control participants over three testing sessions spanning 30 months.ResultsRelative to controls, there was greater longitudinal atrophy in participants with symptomatic Huntington's disease in whole brain, grey matter, caudate and putamen, as well as increased caudate fractional anisotropy; caudate volume loss was the only measure to differ between premanifest Huntington's disease and control groups. Changes in caudate volume and fractional anisotropy correlated with each other and neurocognitive decline; caudate volume loss also correlated with clinical and disease severity.ConclusionsCaudate neurodegeneration, especially atrophy, may be the most suitable candidate surrogate biomarker for consideration in the development of upcoming clinical trials.

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Bohanna, India, Nellie Georgiou-Karistianis, Anthony J. Hannan, and Gary F. Egan. "Magnetic resonance imaging as an approach towards identifying neuropathological biomarkers for Huntington's disease." Brain Research Reviews 58, no. 1 (June 2008): 209–25. http://dx.doi.org/10.1016/j.brainresrev.2008.04.001.

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7

Starkstein, S. E., J. Brandt, F. Bylsma, C. Peyser, M. Folstein, and S. E. Folstein. "Neuropsychological correlates of brain atrophy in Huntington's disease: a magnetic resonance imaging study." Neuroradiology 34, no. 6 (1992): 487–89. http://dx.doi.org/10.1007/bf00598956.

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8

Aylward, Elizabeth H. "Magnetic resonance imaging striatal volumes: A biomarker for clinical trials in Huntington's disease." Movement Disorders 29, no. 11 (August 27, 2014): 1429–33. http://dx.doi.org/10.1002/mds.26013.

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9

Wolf, Robert C., Georg Grön, Fabio Sambataro, Nenad Vasic, Nadine D. Wolf, Philipp A. Thomann, Carsten Saft, G. Bernhard Landwehrmeyer, and Michael Orth. "Magnetic resonance perfusion imaging of resting-state cerebral blood flow in preclinical Huntington's disease." Journal of Cerebral Blood Flow & Metabolism 31, no. 9 (May 11, 2011): 1908–18. http://dx.doi.org/10.1038/jcbfm.2011.60.

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Magnetic resonance imaging (MRI) of the brain could be a powerful tool for discovering early biomarkers in clinically presymptomatic carriers of the Huntington's disease gene mutation (preHD). The aim of this study was to investigate the sensitivity of resting-state perfusion MRI in preHD and to identify neural changes, which could serve as biomarkers for future clinical trials. Differences in regional cerebral blood flow (rCBF) in 18 preHD and 18 controls were assessed with a novel MRI method based on perfusion images obtained with continuous arterial spin labeling. High-resolution structural data were collected to test for changes of brain volume. Compared with controls, preHD individuals showed decreased rCBF in medial and lateral prefrontal regions and increased rCBF in the precuneus. PreHD near to symptom onset additionally showed decreased rCBF in the putamen and increased rCBF in the hippocampus. Network analyses revealed an abnormal lateral prefrontal pattern in preHD far and near to motor onset. These data suggest early changes of frontostriatal baseline perfusion in preHD independent of substantial reductions of gray matter volume. This study also shows the feasibility of detecting neural changes in preHD with a robust MRI technique that would be suitable for longitudinal multisite application.

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10

Harris, Gordon J., Godfrey D. Pearlson, Carol E. Peyser, Elizabeth H. Aylward, Joy Roberts, Patrick E. Barta, Gary A. Chase, and Susan E. Folstein. "Putamen volume reduction on magnetic resonance imaging exceeds caudate changes in mild Huntington's disease." Annals of Neurology 31, no. 1 (January 1992): 69–75. http://dx.doi.org/10.1002/ana.410310113.

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11

Puri, BK, GM Bydder, MS Manku, A. Clarke, AD Waldman, and CF Beckmann. "Reduction in Cerebral Atrophy Associated with Ethyl-Eicosapentaenoic Acid Treatment in Patients with Huntington's Disease." Journal of International Medical Research 36, no. 5 (October 2008): 896–905. http://dx.doi.org/10.1177/147323000803600505.

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Ultra-pure ethyl-eicosapentaenoic acid (ethyl-EPA), a semi-synthetic ethyl ester of eicosapentaenoic acid, is associated with clinical improvement in motor functioning in Huntington's disease. The aim was to determine the extent to which it might reduce the rate of progress of cerebral atrophy. High-resolution cerebral magnetic resonance imaging scans were acquired at baseline, 6 months and 1 year in up to 34 patients with stage I or II Huntington's disease who took part in a randomized, double-blind, placebo-controlled trial of ethyl-EPA. For each subject and each pair of structural images, the two-timepoint brain volume change was calculated in a double-blind manner. Significant group-level reductions in brain atrophy were observed in the head of the caudate nucleus and the posterior thalamus. These findings show that treatment with ethyl-EPA is associated with significant reduction in brain atrophy, particularly in the caudate and thalamus. No other drug tested in Huntington's disease has shown this effect.

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12

Zacharoff, Lori, Ivan Tkac, Qingfeng Song, Chuanning Tang, Patrick J. Bolan, Silvia Mangia, Pierre-Gilles Henry, Tongbin Li, and Janet M. Dubinsky. "Cortical Metabolites as Biomarkers in the R6/2 Model of Huntington's Disease." Journal of Cerebral Blood Flow & Metabolism 32, no. 3 (November 2, 2011): 502–14. http://dx.doi.org/10.1038/jcbfm.2011.157.

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To improve the ability to move from preclinical trials in mouse models of Huntington's disease (HD) to clinical trials in humans, biomarkers are needed that can track similar aspects of disease progression across species. Brain metabolites, detectable by magnetic resonance spectroscopy (MRS), have been suggested as potential biomarkers in HD. In this study, the R6/2 transgenic mouse model of HD was used to investigate the relative sensitivity of the metabolite profiling and the brain volumetry to anticipate the disease progression. Magnetic resonance imaging (MRI) and 1H MRS data were acquired at 9.4 T from the R6/2 mice and wild-type littermates at 4, 8, 12, and 15 weeks. Brain shrinkage was detectable in striatum, cortex, thalamus, and hypothalamus by 12 weeks. Metabolite changes in cortex paralleled and sometimes preceded those in striatum. The entire set of metabolite changes was compressed into principal components (PCs) using Partial Least Squares-Discriminant Analysis (PLS-DA) to increase the sensitivity for monitoring disease progression. In comparing the efficacy of volume and metabolite measurements, the cortical PC1 emerged as the most sensitive single biomarker, distinguishing R6/2 mice from littermates at all time points. Thus, neurochemical changes precede volume shrinkage and become potential biomarkers for HD mouse models.

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13

Feki, Fatma, Chahnez Triki, and Nesrine Amara. "Drug-Resistant Myoclonic Epilepsy Revealing Juvenile Huntington's Disease: A Case Report." Journal of Pediatric Epilepsy 07, no. 01 (March 2018): 021–23. http://dx.doi.org/10.1055/s-0038-1641727.

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AbstractJuvenile Huntington's disease (JHD) shares many general clinical features with the adult form. One important difference is that JHD patients experience more epileptic manifestations, sometimes difficult to control. We describe an atypical clinical picture of a genetically confirmed JHD patient diagnosed during evaluation for a progressive myoclonic epilepsy. A female patient with a family history of psychiatric disorders developed recurrent drug-resistant myoclonic seizures at the age of 6 years, followed by extrapyramidal symptoms (rigidity and dystonia). Cognitive impairment, akinetic rigidity syndrome, and dystonia were noticed at the age of 10 years. Epileptiform abnormalities were noted in ictal electroencephalography. Magnetic resonance imaging showed brain atrophy. Genetic testing for HD confirmed the diagnosis. JHD can initially manifest as myoclonic epilepsy. A DNA testing should be performed if clinical history is suggestive.

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14

Georgiou-Karistianis, N., S.-P. Carron, I. Bohanna, J. C. Stout, A. Churchyard, P. Chua, E. Frajman, and G. Egan. "I07 Image-HD: a functional magnetic resonance imaging study of spatial working memory in Huntington's disease." Journal of Neurology, Neurosurgery & Psychiatry 81, Suppl 1 (September 2010): A38.1—A38. http://dx.doi.org/10.1136/jnnp.2010.222679.7.

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Sánchez-Castañeda, Cristina, Ferdinando Squitieri, Margherita Di Paola, Michael Dayan, Martina Petrollini, and Umberto Sabatini. "The role of iron in gray matter degeneration in Huntington's disease: A magnetic resonance imaging study." Human Brain Mapping 36, no. 1 (August 21, 2014): 50–66. http://dx.doi.org/10.1002/hbm.22612.

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16

Sarac, H., and S. Telarovic. "Magnetic resonance imaging and proton spectroscopy in the Huntington disease." Journal of the Neurological Sciences 333 (October 2013): e125. http://dx.doi.org/10.1016/j.jns.2013.07.418.

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Leitão, Ricardo, Carla Guerreiro, Rita G. Nunes, Nilza Gonçalves, Giulia Galati, Madalena Rosário, Leonor Correia Guedes, Joaquim J. Ferreira, and Sofia Reimão. "Neuromelanin Magnetic Resonance Imaging of the Substantia Nigra in Huntington’s Disease." Journal of Huntington's Disease 9, no. 2 (June 5, 2020): 143–48. http://dx.doi.org/10.3233/jhd-190388.

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Sawiak, S. J., N. I. Wood, G. B. Williams, A. J. Morton, and T. A. Carpenter. "Use of magnetic resonance imaging for anatomical phenotyping of the R6/2 mouse model of Huntington's disease." Neurobiology of Disease 33, no. 1 (January 2009): 12–19. http://dx.doi.org/10.1016/j.nbd.2008.09.017.

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19

Gavazzi, Cinzia, Riccardo Della Nave, Raffaele Petralli, Maria Assunta Rocca, Laura Guerrini, Carlo Tessa, Stefano Diciotti, Massimo Filippi, Silvia Piacentini, and Mario Mascalchi. "Combining Functional and Structural Brain Magnetic Resonance Imaging in Huntington Disease." Journal of Computer Assisted Tomography 31, no. 4 (July 2007): 574–80. http://dx.doi.org/10.1097/01.rct.0000284390.53202.2e.

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20

Aggarwal, Manisha, Wenzhen Duan, Zhipeng Hou, Neal Rakesh, Qi Peng, Christopher A. Ross, Michael I. Miller, Susumu Mori, and Jiangyang Zhang. "Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging." NeuroImage 60, no. 4 (May 2012): 2086–95. http://dx.doi.org/10.1016/j.neuroimage.2012.01.141.

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21

Harris, G. J., E. H. Aylward, C. E. Peyser, G. D. Pearlson, J. Brandt, J. V. Roberts-Twillie, P. E. Barta, and S. E. Folstein. "Single Photon Emission Computed Tomographic Blood Flow and Magnetic Resonance Volume Imaging of Basal Ganglia in Huntington's Disease." Archives of Neurology 53, no. 4 (April 1, 1996): 316–24. http://dx.doi.org/10.1001/archneur.1996.00550040044013.

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22

Georgiou-Karistianis, Nellie, Julie C. Stout, Juan F. Domínguez D., Sarah P. Carron, Ayaka Ando, Andrew Churchyard, Phyllis Chua, et al. "Functional magnetic resonance imaging of working memory in Huntington's disease: Cross-sectional data from the IMAGE-HD study." Human Brain Mapping 35, no. 5 (August 2, 2013): 1847–64. http://dx.doi.org/10.1002/hbm.22296.

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23

Cui, Weitong, Wei Fu, Yunfeng Lin, and Tianxu Zhang. "Application of Nanomaterials in Neurodegenerative Diseases." Current Stem Cell Research & Therapy 16, no. 1 (December 1, 2021): 83–94. http://dx.doi.org/10.2174/1574888x15666200326093410.

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Neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and Huntington's disease are very harmful brain lesions. Due to the difficulty in obtaining therapeutic drugs, the best treatment for neurodegenerative diseases is often not available. In addition, the bloodbrain barrier can effectively prevent the transfer of cells, particles and macromolecules (such as drugs) in the brain, resulting in the failure of the traditional drug delivery system to provide adequate cellular structure repair and connection modes, which are crucial for the functional recovery of neurodegenerative diseases. Nanomaterials are designed to carry drugs across the blood-brain barrier for targets. Nanotechnology uses engineering materials or equipment to interact with biological systems at the molecular level to induce physiological responses through stimulation, response and target site interactions, while minimizing the side effects, thus revolutionizing the treatment and diagnosis of neurodegenerative diseases. Some magnetic nanomaterials play a role as imaging agents or nanoprobes for Magnetic Resonance Imaging to assist in the diagnosis of neurodegenerative diseases. Although the current research on nanomaterials is not as useful as expected in clinical applications, it achieves a major breakthrough and guides the future development direction of nanotechnology in the application of neurodegenerative diseases. This review briefly discusses the application and advantages of nanomaterials in neurodegenerative diseases. Data for this review were identified by searches of PubMed, and references from relevant articles published in English between 2015 and 2019 using the search terms “nanomaterials”, “neurodegenerative diseases” and “blood-brain barrier”.

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Moraes, Louise, Andreia Vasconcelos-dos-Santos, Fernando Cleber Santana, Mariana Araya Godoy, Paulo Henrique Rosado-de-Castro, Jasmin, Ricardo Luiz Azevedo-Pereira, et al. "Neuroprotective effects and magnetic resonance imaging of mesenchymal stem cells labeled with SPION in a rat model of Huntington's disease." Stem Cell Research 9, no. 2 (September 2012): 143–55. http://dx.doi.org/10.1016/j.scr.2012.05.005.

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Seliverstov, Yu A., E. V. Seliverstova, R. N. Konovalov, S. A. Klyushnikov, M. V. Krotenkova, and S. N. Illarioshkin. "CLINICAL AND IMAGING ANALYSIS OF HUNTINGTON DISEASE WITH USE OF RESTING-STATE FUNCTIONAL MAGNETIC RESONANCE IMAGING." Neurological Journal 20, no. 3 (July 29, 2015): 11. http://dx.doi.org/10.18821/1560-9545-2015-20-3-11-21.

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Wolf, R. C., F. Sambataro, N. Vasic, M. S. Depping, P. A. Thomann, G. B. Landwehrmeyer, S. D. Süssmuth, and M. Orth. "Abnormal resting-state connectivity of motor and cognitive networks in early manifest Huntington's disease." Psychological Medicine 44, no. 15 (March 27, 2014): 3341–56. http://dx.doi.org/10.1017/s0033291714000579.

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Background.Functional magnetic resonance imaging (fMRI) of multiple neural networks during the brain's ‘resting state’ could facilitate biomarker development in patients with Huntington's disease (HD) and may provide new insights into the relationship between neural dysfunction and clinical symptoms. To date, however, very few studies have examined the functional integrity of multiple resting state networks (RSNs) in manifest HD, and even less is known about whether concomitant brain atrophy affects neural activity in patients.Method.Using MRI, we investigated brain structure and RSN function in patients with early HD (n = 20) and healthy controls (n = 20). For resting-state fMRI data a group-independent component analysis identified spatiotemporally distinct patterns of motor and prefrontal RSNs of interest. We used voxel-based morphometry to assess regional brain atrophy, and ‘biological parametric mapping’ analyses to investigate the impact of atrophy on neural activity.Results.Compared with controls, patients showed connectivity changes within distinct neural systems including lateral prefrontal, supplementary motor, thalamic, cingulate, temporal and parietal regions. In patients, supplementary motor area and cingulate cortex connectivity indices were associated with measures of motor function, whereas lateral prefrontal connectivity was associated with cognition.Conclusions.This study provides evidence for aberrant connectivity of RSNs associated with motor function and cognition in early manifest HD when controlling for brain atrophy. This suggests clinically relevant changes of RSN activity in the presence of HD-associated cortical and subcortical structural abnormalities.

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Jenkins, Bruce G., Emmanuel Brouillet, Yin-Ching I. Chen, Elsdon Storey, Jörg B. Schulz, Pamela Kirschner, M. Flint Beal, and Bruce R. Rosen. "Non-Invasive Neurochemical Analysis of Focal Excitotoxic Lesions in Models of Neurodegenerative Illness Using Spectroscopic Imaging." Journal of Cerebral Blood Flow & Metabolism 16, no. 3 (May 1996): 450–61. http://dx.doi.org/10.1097/00004647-199605000-00011.

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Water-suppressed chemical shift magnetic resonance imaging was used to detect neurochemical alterations in vivo in neurotoxin-induced rat models of Huntington's and Parkinson's disease. The toxins were: N-methyl-4-phenylpyridinium (MPP+), aminooxyacetic acid (AOAA), 3-nitropropionic acid (3-NP), malonate, and azide. Local or systemic injection of these compounds caused secondary excitotoxic lesions by selective inhibition of mitochondrial respiration that gave rise to elevated lactate concentrations in the striatum. In addition, decreased N-acetylaspartate (NAA) concentrations were noted at the lesion site over time. Measurements of lactate washout kinetics demonstrated that t1/2 followed the order: 3-NP ≈ MPP+ » AOAA ≈ malonate, which parallels the expected lifetimes of the neurotoxins based on their mechanisms of action. Further increases in lactate were also caused by intravenous infusion of glucose. At least part of the excitotoxicity is mediated through indirect glutamate pathways because lactate production and lesion size were diminished using unilateral decortectomies (blockade of glutamatergic input) or glutamate antagonists (MK-801). Lesion size and lactate were also diminished by energy repletion with ubiquinone and nicotinamide. Lactate measurements determined by magnetic resonance agreed with biochemical measurements made using freeze clamp techniques. Lesion size as measured with MR, although larger by 30%, agreed well with lesion size determined histologically. These experiments provide evidence for impairment of intracellular energy metabolism leading to indirect excitotoxicity for all the compounds mentioned before and demonstrate the feasibility of small-volume metabolite imaging for in vivo neurochemical analysis.

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28

Hedjoudje, Abderrahmane, Gaël Nicolas, Alice Goldenberg, Catherine Vanhulle, Clémentine Dumant-Forrest, Guillaume Deverrière, Pauline Treguier, et al. "Morphological features in juvenile Huntington disease associated with cerebellar atrophy — magnetic resonance imaging morphometric analysis." Pediatric Radiology 48, no. 10 (June 20, 2018): 1463–71. http://dx.doi.org/10.1007/s00247-018-4167-z.

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Beste, C., C. Konrad, C. Saft, J. Andrich, R. Gold, B. Pfleiderer, M. Hausmann, and M. Falkenstein. "69. Voluntary movement execution in Huntington’s disease – a combined neurophysiological and morphometric magnetic resonance imaging study." Clinical Neurophysiology 120, no. 1 (January 2009): e31. http://dx.doi.org/10.1016/j.clinph.2008.07.068.

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CHUA, P., P. DESMOND, S. CHRISTENSEN, C. STEWARD, D. VELAKOULIS, F. JUDD, E. CHIU, J. LLOYD, and B. TRESS. "Poster 19: Basal Ganglia Pathology in Preclinical and Early Symptomatic Huntington's Disease: Diffusion Tensor Imaging, Magnetic Resonance Spectroscopy, and Volumetric Measures—Which Imaging Modality Is More Sensitive?" Neurotherapeutics 6, no. 1 (January 2009): 210. http://dx.doi.org/10.1016/j.nurt.2008.10.023.

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Teichmann, Marc, Emmanuel Dupoux, Sid Kouider, and Anne-Catherine Bachoud-Lévi. "The Role of the Striatum in Processing Language Rules: Evidence from Word Perception in Huntington's Disease." Journal of Cognitive Neuroscience 18, no. 9 (September 2006): 1555–69. http://dx.doi.org/10.1162/jocn.2006.18.9.1555.

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On the assumption that linguistic faculties reflect both lexical storage in the temporal cortex and combinatorial rules in the striatal circuits, several authors have shown that striatal-damaged patients are impaired with conjugation rules while retaining lexical knowledge of irregular verbs [Teichmann, M., Dupoux, E., Kouider, S., Brugières, P., Boissé, M. F., Baudic, S., Cesaro, P., Peschanski, M., & Bachoud-Lévi, A. C. (2005). The role of the striatum in rule application. The model of Huntington's disease at early stage. Brain, 128, 1155–1167; Ullman, M. T., Corkin, S., Coppola, M., Hickok, G., Growdon, J. H., Koroshetz, W. J., & Pinker, S. (1997). A neural dissociation within language: Evidence that the mental dictionary is part of declarative memory, and that grammatical rules are processed by the procedural system. Journal of Cognitive Neuroscience, 9, 266–276]. Yet, such impairment was documented only with explicit conjugation tasks in the production domain. Little is known about whether it generalizes to other language modalities such as perception and whether it refers to implicit language processing or rather to intentional rule operations through executive functions. We investigated these issues by assessing perceptive processing of conjugated verb forms in a model of striatal dysfunction, namely, in Huntington's Disease (HD) at early stages. Rule application and lexical processes were evaluated in an explicit task (acceptability judgments on verb and nonword forms) and in an implicit task (lexical decision on frequency-manipulated verb forms). HD patients were also assessed in executive functions, and striatal atrophy was evaluated with magnetic resonance imaging (bicaudate ratio). Results from both tasks showed that HD patients were selectively impaired for rule application but lexical abilities were spared. Bicaudate ratios correlated with rule scores on both tasks, whereas executive parameters only correlated with scores on the explicit task. We argue that the striatum has a core function in linguistic rule application generalizing to perceptive aspects of morphological operations and pertaining to implicit language processes. In addition, we suggest that the striatum may enclose computational circuits that underpin explicit manipulation of regularities.

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Adanyeguh, Isaac M., Marie-Lorraine Monin, Daisy Rinaldi, Léorah Freeman, Alexandra Durr, Stéphane Lehéricy, Pierre-Gilles Henry, and Fanny Mochel. "Expanded neurochemical profile in the early stage of Huntington disease using proton magnetic resonance spectroscopy." NMR in Biomedicine 31, no. 3 (January 9, 2018): e3880. http://dx.doi.org/10.1002/nbm.3880.

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Saba, Roberta Arb, James H. Yared, Thomas M. Doring, Med Phys, Vanderci Borges, and Henrique Ballalai Ferraz. "Diffusion tensor imaging of brain white matter in Huntington gene mutation individuals." Arquivos de Neuro-Psiquiatria 75, no. 8 (August 2017): 503–8. http://dx.doi.org/10.1590/0004-282x20170085.

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ABSTRACT Objective To evaluate the role of the involvement of white matter tracts in huntingtin gene mutation patients as a potential biomarker of the progression of the disease. Methods We evaluated 34 participants (11 symptomatic huntingtin gene mutation, 12 presymptomatic huntingtin gene mutation, and 11 controls). We performed brain magnetic resonance imaging to assess white matter integrity using diffusion tensor imaging, with measurement of fractional anisotropy. Results We observed a significant decrease of fractional anisotropy in the cortical spinal tracts, corona radiate, corpus callosum, external capsule, thalamic radiations, superior and inferior longitudinal fasciculus, and inferior frontal-occipital fasciculus in the Huntington disease group compared to the control and presymptomatic groups. Reduction of fractional anisotropy is indicative of a degenerative process and axonal loss. There was no statistically significant difference between the presymptomatic and control groups. Conclusion White matter integrity is affected in huntingtin gene mutation symptomatic individuals, but other studies with larger samples are required to assess its usefulness in the progression of the neurodegenerative process.

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Lee, Wang-Tso, and Chen Chang. "Magnetic resonance imaging and spectroscopy in assessing 3-nitropropionic acid-induced brain lesions: an animal model of Huntington’s disease." Progress in Neurobiology 72, no. 2 (February 2004): 87–110. http://dx.doi.org/10.1016/j.pneurobio.2004.02.002.

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35

Evans, A. C., C. Beil, S. Marrett, C. J. Thompson, and A. Hakim. "Anatomical-Functional Correlation Using an Adjustable MRI-Based Region of Interest Atlas with Positron Emission Tomography." Journal of Cerebral Blood Flow & Metabolism 8, no. 4 (August 1988): 513–30. http://dx.doi.org/10.1038/jcbfm.1988.92.

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A procedure is described for combining anatomical information from magnetic resonance imaging (MRI) or computerized tomography (CT) and functional information from positron emission tomography (PET) in a rapid fashion. MRI data are combined with a procedure for the definition, storage, and recall of anatomically based regions of interest. An atlas of standard regions of interest, defined for a set of 18 parallel planes spaced at 6-mm intervals, provides an initial region of interest template for each patient slice. Global adjustments to scale, orientation, and position are applied to obtain an initial match. Individual regions of interest may then be moved, deleted, or redrawn as needed. The ability to store region of interest templates ensures reproducibility of analysis over long periods and introduces a standardization of analysis technique. In 25 brain structures, the mean coefficient of variation in cerebral glucose utilization rate (CMRGlc) measurements among five neuroanatomically trained observers was reduced from 8.1% for manual region of interest definition to 4.0% using the template approach with MRI. Template analysis for space-occupying lesions such as tumors or infarcts is illustrated with PET data from a stroke study, emphasizing the facility for rapid, reproducible analysis of multifunctional studies. MRI-PET matching for a structurally intact caudate nucleus having reduced CMRGlc in Huntington's disease emphasizes the accuracy of anatomical localization required to quantify small structures.

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Moeller, Ashley A., Marcia V. Felker, Jennifer A. Brault, Laura C. Duncan, Rizwan Hamid, and Meredith R. Golomb. "Patients With Extreme Early Onset Juvenile Huntington Disease Can Have Delays in Diagnosis: A Case Report and Literature Review." Child Neurology Open 8 (January 2021): 2329048X2110361. http://dx.doi.org/10.1177/2329048x211036137.

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Huntington disease (HD) is caused by a pathologic cytosine-adenine-guanine (CAG) trinucleotide repeat expansion in the HTT gene. Typical adult-onset disease occurs with a minimum of 40 repeats. With more than 60 CAG repeats, patients can have juvenile-onset disease (jHD), with symptom onset by the age of 20 years. We report a case of a boy with extreme early onset, paternally inherited jHD, with symptom onset between 18 and 24 months. He was found to have 250 to 350 CAG repeats, one of the largest repeat expansions published to date. At initial presentation, he had an ataxic gait, truncal titubation, and speech delay. Magnetic resonance imaging showed cerebellar atrophy. Over time, he continued to regress and became nonverbal, wheelchair-bound, gastrostomy-tube dependent, and increasingly rigid. His young age at presentation and the ethical concerns regarding HD testing in minors delayed his diagnosis.

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37

Graziola, Federica, Sabrina Maffi, Melissa Grasso, Giacomo Garone, Simone Migliore, Eugenia Scaricamazza, Consuelo Ceccarelli, et al. "“Spazio Huntington”: Tracing the Early Motor, Cognitive and Behavioral Profiles of Kids with Proven Pediatric Huntington Disease and Expanded Mutations > 80 CAG Repeats." Journal of Personalized Medicine 12, no. 1 (January 17, 2022): 120. http://dx.doi.org/10.3390/jpm12010120.

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The “Spazio Huntington—A Place for Children” program was launched in 2019. The aim was to contact at risk kids within Huntington disease (HD) families, to provide counseling to their parents and to start a prospective follow-up of kids suspicious to manifest pediatric HD (PHD). We met 25 at risk kids in two years, four of whom with PHD and highly expanded (HE) mutations beyond 80 CAG repeats. We rated motor, neuropsychological and behavioral changes in all PHD kids by the Unified HD Rating Scale (UHDRS)-total motor score (TMS) and additional measures of (1) cognitive level (Leiter International Performance Scale), (2) adaptive functioning (Adaptive Behavior Assessment Systems), (3) receptive language (Peabody Picture Vocabulary Test) and (4) behavioral abnormalities (Child Behavior Check List and Children’s Yale–Brown Obsessive Compulsive Scale). All PHD kids showed a severe progression of neurological and psychiatric manifestations including motor, cognitive and behavioral changes. The magnetic resonance imaging contributed to confirm the suspicious clinical observation by highlighting very initial striatum abnormalities in PHD. Spazio Huntington is a program to prospectively study PHD, the most atypical face of HD, and may represent the basis to recruit PHD patients in future clinical trials.

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38

Furukawa, Fumiko, Kinya Ishikawa, Takanori Yokota, and Nobuo Sanjo. "Cross-Sectional Area Analysis of the Head of the Caudate Nucleus in Huntington’s Disease." European Neurology 81, no. 1-2 (2019): 13–18. http://dx.doi.org/10.1159/000499909.

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Background: Caudate nucleus atrophy is a well-known neuroimaging feature of Huntington’s disease (HD). Some researchers have reported a decrease in the volume of the striatum on magnetic resonance images (MRIs) even in the presymptomatic stage of the disease. Despite the many neuroimaging studies on HD, the optimal method for measuring the caudate nucleus area on MRIs and the most effective cutoff values for diagnosing HD remain unclear. Objectives and Methods: To define suitable imaging sequences and cutoff values for HD, we measured the area of the head of the caudate nucleus (HCN) in 11 patients with HD, 22 age- and sex-matched individuals without neurodegenerative disorders in the central nervous system, 22 sex-matched patients with Alzheimer’s disease, 22 sex-matched patients with Parkinson’s disease, and 7 patients with dentatorubral-pallidoluysian atrophy. Results: On T2-weighted images (T2WIs), we found significantly reduced HCN area at the rostral level in individuals with HD relative to those of the individuals in the other groups. A significant inverse correlation (ρ = –0.61, p = 0.046) was observed between the HD duration and HCN area at the rostral slice level on T2WIs. The cutoff value for distinguishing patients with HD from healthy individuals and those with other neurodegenerative diseases was 85 mm2 at the rostral level on T2WIs (100% sensitivity and specificity). Conclusions: This cutoff value can be applied clinically to evaluate brain atrophy in HD. Our method is advantageous because it is simple and can be implemented easily in daily clinical practice.

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Byrne, Lauren M., Filipe B. Rodrigues, Eileanor B. Johnson, Peter A. Wijeratne, Enrico De Vita, Daniel C. Alexander, Giuseppe Palermo, et al. "Evaluation of mutant huntingtin and neurofilament proteins as potential markers in Huntington’s disease." Science Translational Medicine 10, no. 458 (September 12, 2018): eaat7108. http://dx.doi.org/10.1126/scitranslmed.aat7108.

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Huntington’s disease (HD) is a genetic progressive neurodegenerative disorder, caused by a mutation in the HTT gene, for which there is currently no cure. The identification of sensitive indicators of disease progression and therapeutic outcome could help the development of effective strategies for treating HD. We assessed mutant huntingtin (mHTT) and neurofilament light (NfL) protein concentrations in cerebrospinal fluid (CSF) and blood in parallel with clinical evaluation and magnetic resonance imaging in premanifest and manifest HD mutation carriers. Among HD mutation carriers, NfL concentrations in plasma and CSF correlated with all nonbiofluid measures more closely than did CSF mHTT concentration. Longitudinal analysis over 4 to 8 weeks showed that CSF mHTT, CSF NfL, and plasma NfL concentrations were highly stable within individuals. In our cohort, concentration of CSF mHTT accurately distinguished between controls and HD mutation carriers, whereas NfL concentration, in both CSF and plasma, was able to segregate premanifest from manifest HD. In silico modeling indicated that mHTT and NfL concentrations in biofluids might be among the earliest detectable alterations in HD, and sample size prediction suggested that low participant numbers would be needed to incorporate these measures into clinical trials. These findings provide evidence that biofluid concentrations of mHTT and NfL have potential for early and sensitive detection of alterations in HD and could be integrated into both clinical trials and the clinic.

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40

Simmons, Danielle A., Brian D. Mills, Robert R. Butler III, Jason Kuan, Tyne L. M. McHugh, Carolyn Akers, James Zhou, et al. "Neuroimaging, Urinary, and Plasma Biomarkers of Treatment Response in Huntington’s Disease: Preclinical Evidence with the p75NTR Ligand LM11A-31." Neurotherapeutics 18, no. 2 (March 30, 2021): 1039–63. http://dx.doi.org/10.1007/s13311-021-01023-8.

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AbstractHuntington’s disease (HD) is caused by an expansion of the CAG repeat in the huntingtin gene leading to preferential neurodegeneration of the striatum. Disease-modifying treatments are not yet available to HD patients and their development would be facilitated by translatable pharmacodynamic biomarkers. Multi-modal magnetic resonance imaging (MRI) and plasma cytokines have been suggested as disease onset/progression biomarkers, but their ability to detect treatment efficacy is understudied. This study used the R6/2 mouse model of HD to assess if structural neuroimaging and biofluid assays can detect treatment response using as a prototype the small molecule p75NTR ligand LM11A-31, shown previously to reduce HD phenotypes in these mice. LM11A-31 alleviated volume reductions in multiple brain regions, including striatum, of vehicle-treated R6/2 mice relative to wild-types (WTs), as assessed with in vivo MRI. LM11A-31 also normalized changes in diffusion tensor imaging (DTI) metrics and diminished increases in certain plasma cytokine levels, including tumor necrosis factor-alpha and interleukin-6, in R6/2 mice. Finally, R6/2-vehicle mice had increased urinary levels of the p75NTR extracellular domain (ecd), a cleavage product released with pro-apoptotic ligand binding that detects the progression of other neurodegenerative diseases; LM11A-31 reduced this increase. These results are the first to show that urinary p75NTR-ecd levels are elevated in an HD mouse model and can be used to detect therapeutic effects. These data also indicate that multi-modal MRI and plasma cytokine levels may be effective pharmacodynamic biomarkers and that using combinations of these markers would be a viable and powerful option for clinical trials.

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Vallès, Astrid, Melvin M. Evers, Anouk Stam, Marina Sogorb-Gonzalez, Cynthia Brouwers, Carlos Vendrell-Tornero, Seyda Acar-Broekmans, et al. "Widespread and sustained target engagement in Huntington’s disease minipigs upon intrastriatal microRNA-based gene therapy." Science Translational Medicine 13, no. 588 (April 7, 2021): eabb8920. http://dx.doi.org/10.1126/scitranslmed.abb8920.

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Huntingtin (HTT)–lowering therapies hold promise to slow down neurodegeneration in Huntington’s disease (HD). Here, we assessed the translatability and long-term durability of recombinant adeno-associated viral vector serotype 5 expressing a microRNA targeting human HTT (rAAV5-miHTT) administered by magnetic resonance imaging–guided convention-enhanced delivery in transgenic HD minipigs. rAAV5-miHTT (1.2 × 1013 vector genome (VG) copies per brain) was successfully administered into the striatum (bilaterally in caudate and putamen), using age-matched untreated animals as controls. Widespread brain biodistribution of vector DNA was observed, with the highest concentration in target (striatal) regions, thalamus, and cortical regions. Vector DNA presence and transgene expression were similar at 6 and 12 months after administration. Expression of miHTT strongly correlated with vector DNA, with a corresponding reduction of mutant HTT (mHTT) protein of more than 75% in injected areas, and 30 to 50% lowering in distal regions. Translational pharmacokinetic and pharmacodynamic measures in cerebrospinal fluid (CSF) were largely in line with the effects observed in the brain. CSF miHTT expression was detected up to 12 months, with CSF mHTT protein lowering of 25 to 30% at 6 and 12 months after dosing. This study demonstrates widespread biodistribution, strong and durable efficiency of rAAV5-miHTT in disease-relevant regions in a large brain, and the potential of using CSF analysis to determine vector expression and efficacy in the clinic.

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Yu, Ji-Hea, Bae-Geun Nam, Min-Gi Kim, Soonil Pyo, Jung-Hwa Seo, and Sung-Rae Cho. "In Vivo Expression of Reprogramming Factor OCT4 Ameliorates Myelination Deficits and Induces Striatal Neuroprotection in Huntington’s Disease." Genes 12, no. 5 (May 10, 2021): 712. http://dx.doi.org/10.3390/genes12050712.

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White matter atrophy has been shown to precede the massive loss of striatal GABAergic neurons in Huntington’s disease (HD). This study investigated the effects of in vivo expression of reprogramming factor octamer-binding transcription factor 4 (OCT4) on neural stem cell (NSC) niche activation in the subventricular zone (SVZ) and induction of cell fate specific to the microenvironment of HD. R6/2 mice randomly received adeno-associated virus 9 (AAV9)-OCT4, AAV9-Null, or phosphate-buffered saline into both lateral ventricles at 4 weeks of age. The AAV9-OCT4 group displayed significantly improved behavioral performance compared to the control groups. Following AAV9-OCT4 treatment, the number of newly generated NSCs and oligodendrocyte progenitor cells (OPCs) significantly increased in the SVZ, and the expression of OPC-related genes and glial cell-derived neurotrophic factor (GDNF) significantly increased. Further, amelioration of myelination deficits in the corpus callosum was observed through electron microscopy and magnetic resonance imaging, and striatal DARPP32+ GABAergic neurons significantly increased in the AAV9-OCT4 group. These results suggest that in situ expression of the reprogramming factor OCT4 in the SVZ induces OPC proliferation, thereby attenuating myelination deficits. Particularly, GDNF released by OPCs seems to induce striatal neuroprotection in HD, which explains the behavioral improvement in R6/2 mice overexpressing OCT4.

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43

Casella, Chiara, Jose Bourbon-Teles, Sonya Bells, Elizabeth Coulthard, Greg D. Parker, Anne Rosser, Derek K. Jones, and Claudia Metzler-Baddeley. "Drumming Motor Sequence Training Induces Apparent Myelin Remodelling in Huntington’s Disease: A Longitudinal Diffusion MRI and Quantitative Magnetization Transfer Study." Journal of Huntington's Disease 9, no. 3 (October 8, 2020): 303–20. http://dx.doi.org/10.3233/jhd-200424.

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Background: Impaired myelination may contribute to Huntington’s disease (HD) pathogenesis. Objective: This study assessed differences in white matter (WM) microstructure between HD patients and controls, and tested whether drumming training stimulates WM remodelling in HD. Furthermore, it examined whether training-induced microstructural changes are related to improvements in motor and cognitive function. Methods: Participants undertook two months of drumming exercises. Working memory and executive function were assessed before and post-training. Changes in WM microstructure were investigated with diffusion tensor magnetic resonance imaging (DT-MRI)-based metrics, the restricted diffusion signal fraction (Fr) from the composite hindered and restricted model of diffusion (CHARMED) and the macromolecular proton fraction (MPF) from quantitative magnetization transfer (qMT) imaging. WM pathways linking putamen and supplementary motor areas (SMA-Putamen), and three segments of the corpus callosum (CCI, CCII, CCIII) were studied using deterministic tractography. Baseline MPF differences between patients and controls were assessed with tract-based spatial statistics. Results: MPF was reduced in the mid-section of the CC in HD subjects at baseline, while a significantly greater change in MPF was detected in HD patients relative to controls in the CCII, CCIII, and the right SMA-putamen post-training. Further, although patients improved their drumming and executive function performance, such improvements did not correlate with microstructural changes. Increased MPF suggests training-induced myelin changes in HD. Conclusion: Though only preliminary and based on a small sample size, these results suggest that tailored behavioural stimulation may lead to neural benefits in early HD, that could be exploited for delaying disease progression.

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O’Connell, Adam B., Timothy R. Kuchel, Sunthara R. Perumal, Victoria Sherwood, Daniel Neumann, John W. Finnie, Kim M. Hemsley, and A. Jennifer Morton. "Longitudinal Magnetic Resonance Spectroscopy and Diffusion Tensor Imaging in Sheep (Ovis aries) With Quinolinic Acid Lesions of the Striatum: Time-Dependent Recovery of N-Acetylaspartate and Fractional Anisotropy." Journal of Neuropathology & Experimental Neurology 79, no. 10 (August 2, 2020): 1084–92. http://dx.doi.org/10.1093/jnen/nlaa053.

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Abstract We created an excitotoxic striatal lesion model of Huntington disease (HD) in sheep, using the N-methyl-d-aspartate receptor agonist, quinolinic acid (QA). Sixteen sheep received a bolus infusion of QA (75 µL, 180 mM) or saline, first into the left and then (4 weeks later) into the right striatum. Magnetic resonance spectroscopy (MRS) and diffusion tensor imaging (DTI) of the striata were performed. Metabolite concentrations and fractional anisotropy (FA) were measured at baseline, acutely (1 week after each surgery) and chronically (5 weeks or greater after the surgeries). There was a significant decrease in the neuronal marker N-acetylaspartate (NAA) and in FA in acutely lesioned striata of the QA-lesioned sheep, followed by a recovery of NAA and FA in the chronically lesioned striata. NAA level changes indicate acute death and/or impairment of neurons immediately after surgery, with recovery of reversibly impaired neurons over time. The change in FA values of the QA-lesioned striata is consistent with acute structural disruption, followed by re-organization and glial cell infiltration with time. Our study demonstrates that MRS and DTI changes in QA-sheep are consistent with HD-like pathology shown in other model species and that the MR investigations can be performed in sheep using a clinically relevant human 3T MRI scanner.

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Spronck, Elisabeth, Astrid Vallès, Margit Lampen, Paula Montenegro-Miranda, Sonay Keskin, Liesbeth Heijink, Melvin Evers, et al. "Intrastriatal Administration of AAV5-miHTT in Non-Human Primates and Rats Is Well Tolerated and Results in miHTT Transgene Expression in Key Areas of Huntington Disease Pathology." Brain Sciences 11, no. 2 (January 20, 2021): 129. http://dx.doi.org/10.3390/brainsci11020129.

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Huntington disease (HD) is a fatal, neurodegenerative genetic disorder with aggregation of mutant Huntingtin protein (mutHTT) in the brain as a key pathological mechanism. There are currently no disease modifying therapies for HD; however, HTT-lowering therapies hold promise. Recombinant adeno-associated virus serotype 5 expressing a microRNA that targets HTT mRNA (AAV5-miHTT) is in development for the treatment of HD with promising results in rodent and minipig HD models. To support a clinical trial, toxicity studies were performed in non-human primates (NHP, Macaca fascicularis) and Sprague-Dawley rats to evaluate the safety of AAV5-miHTT, the neurosurgical administration procedure, vector delivery and expression of the miHTT transgene during a 6-month observation period. For accurate delivery of AAV5-miHTT to the striatum, real-time magnetic resonance imaging (MRI) with convection-enhanced delivery (CED) was used in NHP. Catheters were successfully implanted in 24 NHP, without neurological symptoms, and resulted in tracer signal in the target areas. Widespread vector DNA and miHTT transgene distribution in the brain was found, particularly in areas associated with HD pathology. Intrastriatal administration of AAV5-miHTT was well tolerated with no clinically relevant changes in either species. These studies demonstrate the excellent safety profile of AAV5-miHTT, the reproducibility and tolerability of intrastriatal administration, and the delivery of AAV5-miHTT to the brain, which support the transition of AAV5-miHTT into clinical studies.

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Kronenbuerger, Martin, Jun Hua, Jee Y. A. Bang, Kia E. Ultz, Xinyuan Miao, Xiaoyu Zhang, James J. Pekar, et al. "Differential Changes in Functional Connectivity of Striatum-Prefrontal and Striatum-Motor Circuits in Premanifest Huntington’s Disease." Neurodegenerative Diseases 19, no. 2 (2019): 78–87. http://dx.doi.org/10.1159/000501616.

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Background: Huntington’s disease (HD) is a progressive neurodegenerative disorder. The striatum is one of the first brain regions that show detectable atrophy in HD. Previous studies using functional magnetic resonance imaging (fMRI) at 3 tesla (3 T) revealed reduced functional connectivity between striatum and motor cortex in the prodromal period of HD. Neuroanatomical and neurophysiological studies have suggested segregated corticostriatal pathways with distinct loops involving different cortical regions, which may be investigated using fMRI at an ultra-high field (7 T) with enhanced sensitivity compared to lower fields. Objectives: We performed fMRI at 7 T to assess functional connectivity between the striatum and several chosen cortical areas including the motor and prefrontal cortex, in order to better understand brain changes in the striatum-cortical pathways. Method: 13 manifest subjects (age 51 ± 13 years, cytosine-adenine-guanine [CAG] repeat 45 ± 5, Unified Huntington’s Disease Rating Scale [UHDRS] motor score 32 ± 17), 8 subjects in the close-to-onset premanifest period (age 38 ± 10 years, CAG repeat 44 ± 2, UHDRS motor score 8 ± 2), 11 subjects in the far-from-onset premanifest period (age 38 ± 11 years, CAG repeat 42 ± 2, UHDRS motor score 1 ± 2), and 16 healthy controls (age 44 ± 15 years) were studied. The functional connectivity between the striatum and several cortical areas was measured by resting state fMRI at 7 T and analyzed in all participants. Results: Compared to controls, functional connectivity between striatum and premotor area, supplementary motor area, inferior frontal as well as middle frontal regions was altered in HD (all p values <0.001). Specifically, decreased striatum-motor connectivity but increased striatum-prefrontal connectivity were found in premanifest HD subjects. Altered functional connectivity correlated consistently with genetic burden, but not with clinical scores. Conclusions: Differential changes in functional connectivity of striatum-prefrontal and striatum-motor circuits can be found in early and premanifest HD. This may imply a compensatory mechanism, where additional cortical regions are recruited to subserve functions that have been impaired due to HD pathology. Our results suggest the potential value of functional connectivity as a marker for future clinical trials in HD.

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Hwang, S. U., J. D. Yoon, K. Eun, H. Kim, and S. H. Hyun. "25 PRODUCTION OF TRANSGENIC PIGS WITH CreER-MEDIATED ASTROCYTIC-SPECIFIC RECOMBINATION SYSTEM FOR NEUROLOGICAL DISEASE MODELS." Reproduction, Fertility and Development 29, no. 1 (2017): 120. http://dx.doi.org/10.1071/rdv29n1ab25.

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Pigs are one of the most suitable alternative laboratory models than other animals, because they have similar cardiovascular, renal and gastrointestinal organs with those of human. However, in the case of genetically engineered animals, early development of embryos is inhibited by expression of foreign genes, there are many cases of miscarriage or birth early mortality. To overcome these problems, we constructed pig glial fibrillary acidic protein (GFAP) promoter-Cre recombinase fused to a mutated ligand-binding domain of the human oestrogen receptor (CreERT2) and enhanced green fluorescent protein (EGFP)-LoxP transgenes for tamoxifen(TM)-inducible CreERT2-mediated recombination. We then established donor transgenic pig fibroblasts with pGFAP-CreERT2; LCMV-EGFPLoxP transgenes for somatic cell nuclear transfer (SCNT). We produced the SCNT embryos using a Cloud male #5 pGFAP-CreERT2+LCMV-EGFPLoxP donor cell line that was verified in vitro. It was transferred into a surrogate mother and then 5 pGFAP-CreERT2; EGFPLoxP TG piglets were born. By immunofluorescence staining and semi-nested PCR analysis, it was proved that CreER-mediated astrocytic-specific recombination system was operated in some cerebral astrocytic cells after TM-administration to TG pig #4. Additionally, we obtained brain magnetic resonance imaging (MRI) images using 3T-tesla MRI. Brain compartment volume (total brain, grey matter, white matter, cerebellum, brainstem, lateral ventricle, thalamus, midbrain, pons, medulla oblongata, hypophysis) was no significant differences between normal pig and pGFAP-CreERT2; EGFPLoxP transgenic (TG) pig. In summary, we verified the pGFAP promoter-driven CreERT2-LoxP recombination system in TG pig generated by SCNT depending on the TM administration. We suggest that this technology will be a useful tool for studying physiology of astrocytes and generating TG pig model of neurological disease such as Huntington’s disease, Alzheimer’s disease and brain tumour.

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48

Puri, Basant K. "High-resolution magnetic resonance imaging sinc-interpolation-based subvoxel registration and semi-automated quantitative lateral ventricular morphology employing threshold computation and binary image creation in the study of fatty acid interventions in schizophrenia, depression, chronic fatigue syndrome and Huntington's disease." International Review of Psychiatry 18, no. 2 (January 2006): 149–54. http://dx.doi.org/10.1080/09540260600583015.

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49

Sarac, Helena, David Ozretic, Neven Henigsberg, Pero Hrabac, Ivan Bagaric, and Lucija Bagaric-Krakan. "PROTON MAGNETIC RESONANCE SPECTROSCOPY IN HUNTINGTON'S DISEASE ACCOMPANYING NEUROBORRELIOSIS." Psychiatria Danubina 29, no. 2 (June 26, 2017): 226–30. http://dx.doi.org/10.24869/psyd.2017.226.

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50

Sturrock, Aaron, Corree Laule, Katy Wyper, Ruth A. Milner, Joji Decolongon, Rachelle Dar Santos, Allison J. Coleman, et al. "A longitudinal study of magnetic resonance spectroscopy Huntington's disease biomarkers." Movement Disorders 30, no. 3 (February 18, 2015): 393–401. http://dx.doi.org/10.1002/mds.26118.

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