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Maria Eugenia Caligiuri¹, Maria Celeste Bonacci¹, Tobias Kober², Domenico Zacà³, Ferdinando Squitieri^4,5, Aldo Quattrone¹, and Umberto Sabatini^1
1Neuroscience Research Center, Università degli Studi Magna Graecia di Catanzaro, Catanzaro, Italy, ²Advanced Clinical Imaging Technology, Siemens Healthcare AG, Lausanne, Switzerland, ³Siemens Healthcare, Milan, Italy, ⁴Italian League for Research on Huntington Disease, Rome, Italy, ⁵IRCSS Casa Sollievo della Sofferenza/CSS-Mendel, Rome, Italy

Keywords: Neurodegeneration, Rare disease, Huntington Disease

T2 mapping is a quantitative MRI technique that may provide further insight regarding the changes that differentially underlie Huntington Disease in two of its clinical forms, adult-onset and juvenile-onset. Here we present pilot evidence that the study of T2 properties of the brain might be of great benefit to the research focused on this rare disease.

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Beatriz Padrela¹, Markus H Sneve², Sanne Zelhorst¹, Mervin Tee³, Amnah Mahroo⁴, Joost Kuijer¹, Kristine Walhovd², Simon Konstandin⁴, Klaus Eickel^4,5, Frederik Barkhof^1,6, Saima Hilal³, Matthias Günther⁴, Henk J.M.M. Mutsaerts¹, and Jan Petr^7
1Radiology and Nuclear Medicine, Amsterdam UMC, Amsterdam, Netherlands, ²Department of Psychology, Center for Lifespan Changes in Brain and Cognition, Oslo, Norway, ³National University of Singapore and National University Health System, Saw Swee Hock School of Public Health, Singapore, Singapore, ⁴Fraunhofer-Insitute for Digital Medicine MEVIS, Bremen, Germany, ⁵mediri GmbH, Heidelberg, Germany, ⁶University College London, Queen Square Institute of Neurology and Centre for Medical Image Computing (CMIC), London, United Kingdom, ⁷Helmholtz-Zentrum Dresden-Rossendorf, Dresden, Germany

Keywords: Neurodegeneration, Arterial spin labelling, Blood-brain barrier

Blood-brain-barrier (BBB) dysfunction is a hallmark of aging-related disorders, including cerebral small vessel disease and Alzheimer’s disease. An emerging biomarker of BBB dysfunction is time of exchange (Tex) of water across the BBB as measured by multi-echo arterial spin labeling MRI. We evaluated Tex across the age spectrum in 40 adults from two cohorts of healthy controls, and demonstrated that Tex is higher in gray than in white matter, higher in females than in males, and that Tex decreases with age. These findings suggest that BBB permeability changes over the lifespan can be investigated using arterial spin labeling approaches.

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Won Jin Moon¹, Younghee Yim², Byeong Kyu Park¹, Jinho Yang¹, Yeonsil Moon³, Seol-Heui Han³, Hee-Jin Kim⁴, and Jongho Lee^5
1Radiology, Konkuk University Medical Center, Seoul, Korea, Republic of, ²Radiology, Chung-Ang University hospital, Seoul, Korea, Republic of, ³Neurology, Konkuk University Medical Center, Seoul, Korea, Republic of, ⁴Neurology, Hanyang University Hospital, Seoul, Korea, Republic of, ⁵Electrical and Computer Engineering, Seoul National University, Seoul, Korea, Republic of

Keywords: Neurodegeneration, Alzheimer's Disease

Prediction or prevention of progression to dementia for non-demented patients is critical for management and prevention strategy of dementia. Recent evidences suggest that larger choroid plexus (CP) volume was associated with the severity of cognitive impairment in Alzheimer disease (AD) spectrum. We evaluated clinical data and the volume, permeability and susceptibility of choroid plexus of 62 consecutive non-demented prospective cohorts with follow-up up to 2 years. Dementia converter group showed larger CP volume than that of non-converters. Thus, CP volume could be utilized as a potential imaging marker for patients who are likely to progress to dementia.

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Denis Peruzzo¹, Tommaso Ciceri¹, Sara Mascheretti², Valentina Lampis³, Filippo Arrigoni⁴, Nivedita Agarwal¹, Alice Giubergia¹, Filippo Maria Villa³, Alessandro Crippa³, Maria Nobile³, Elisa Mani³, Annamaria Russo⁵, and Maria Grazia D'Angelo^5
1Neuroimaging Unit, Scientific Institute IRCCS Eugenio Medea, Bosisio Parini, Italy, ²Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy, ³Child Psychopathology Unit, Scientific Institute IRCCS Eugenio Medea, Bosisio Parini, Italy, ⁴Paediatric Radiology and Neuroradiology Department, V. Buzzi Children’s Hospital, Milano, Italy, ⁵NeuroMuscular Unit, Scientific Institute IRCCS Eugenio Medea, Bosisio Parini, Italy

Keywords: Neurodegeneration, Genetic Diseases

Duchenne Muscular Dystrophy (DMD) is a genetic disease caused by an abnormal dystrophin expression and it is often associated with other cognitive and behavioral impairments, which are an important confounding factor while investigating the effects of dystrophin abnormal expression in the central nervous system. We applied a Machine Learning based analysis to T1-weighted and Diffusion Tensor Imaging data of 36 subjects accounting also for their demographic, cognitive and behavioral profiles. DMD is specifically associated with a reduced microstructural integrity of long fiber bundles and a reduced cortical thickness of the motor cortex, cingulate cortex, hippocampus and insula.

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Aurea Martins Bach¹, Shoshana Spring², Zeinab Ali³, Brian J. Nieman², John Sled², Remya R. Nair^3,4, Elizabeth Fisher⁵, Silvia Corrochano⁶, Thomas Cunningham^3,7, Jason Lerch¹, and Karla Miller^1
1Wellcome Centre for Integrative Neuroimaging, FMRIB, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom, ²Mouse Imaging Centre, The Hospital for Sick Children, Toronto, ON, Canada, ³Mammalian Genetics Unit, MRC Harwell Institute, Oxford, United Kingdom, ⁴Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, United Kingdom, ⁵Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom, ⁶Neurological Disorders Group, Hospital Clínico San Carlos, IdISSC, Madrid, Spain, ⁷MRC Prion Unit and Institute of Prion diseases, University College London, London, United Kingdom

Keywords: Neurodegeneration, Preclinical

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by aggregates of TDP-43 in the brain. The TDP-M323K mouse model of ALS has a mutation in Tardbp and presents progressive motor, neurological and behavioural phenotypes, in addition to widespread changes in brain volume at 12 months of age. Here, we assessed if these volumetric changes are progressive or if they are already present before other symptoms start to present. Post-mortem structural MRI in 3- and 12 months-old TDP-M323K mice revealed that brain volume is already altered in young adults despite the absence of major clinical and pathological phenotypes.

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Natenael B Semmineh¹, Ethan Mathew², Bruce Damon³, and C Chad Quarles^1
1Cancer Systems Imaging, The university of Texas MD Anderson Cancer Center, Houston, TX, United States, ²Barrow Neuroimaging Innovation Center, Barrow Neurological Institute, Phoenix, TX, United States, ³Carle Clinical Imaging Research Program Stephens Family Clinical Research Institute Carle Health, Urbana, IL, United States

Keywords: Neurodegeneration, Microstructure, amyotrophic lateral sclerosis - ALS

Amyotrophic lateral sclerosis (ALS) is a fatal upper and lower motor neuron degradation disease that leads to progressive myofiber abnormalities (e.g., decreased size and distribution). The relaxivity contrast imaging (RCI) parameter, TRATE (tissue transverse relaxivity at tracer equilibrium), has been shown to decrease during ALS induced myofiber degradation. The goal of this study is to expand a validated computational method to investigate the biophysical basis of TRATE in muscles of ALS patients and to optimize RCI acquisition parameters. 

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Yuhan Jiang¹, Yuan Li¹, Qingwei Song¹, and Yanwei Miao^1
1the First Affiliated Hospital of Dalian Medical University, Dalian, China

Keywords: Neurodegeneration, Neurodegeneration

Cognitive impairment is very common in patients with end-stage renal disease (ESRD). Hippocampal atrophy has been proven to be an effective marker for distinguishing the normal population from the cognitively impaired patients. However, there are almost no studies on changes in hippocampal structure, especially the volume of hippocampal subfields, in ESRD patients. In this study, automated volumetry of hippocampal subfields in ESRD patients was performed and compared with healthy controls, and found that ESRD patients have hippocampal subfield atrophy, especially in bilateral fimbria and right hippocampal tail.

YANG MaoJiang¹, Liu JianHao², ANUP Bhetuwal¹, QIONG Xian³, ZHANG HanWen¹, YANG HanFeng¹, Chen Meining⁴, and XU XiaoXue^1
1Affiliated Hospital of North Sichuan Medical College, Nanchong 637000, Nanchong, China, ²The Fifth People's Hospital of Chengdu, Chengdu, China, ³Second Affiliated Hospital of North Sichuan Medical College,Nanchong 637100, Nanchong, China, ⁴MR Scientific Marketing, Siemens Healthcare, Shanghai, China, Shanghai, China

Keywords: Neurodegeneration, Neurodegeneration

At present, the pathogenesis of atypical facial pain is unknown. We conducted pain related analysis on the included patients through magnetic resonance imaging and trigeminal neurolysis, and the results suggest that trigeminal neuropathy may be a pathogenesis of atypical facial pain.

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Prasanna Karunanayaka¹, Biyar Ahmed¹, Rommy Elyan¹, and Qing Yang^1
1Pennsylvania State University College of Medicine, Hershey, PA, United States

Keywords: Neurodegeneration, Neuro, Olfactory fMRI

Both the medial and inferior temporal lobes have been previously implicated in odor-identification. However, the precise neural substrate remains unclear. We used a novel oddball detection olfactory fMRI task to probe the neural basis of odor identification. fMRI activation was detected in the left temporoparietal junction along with known olfactory brain structures. Given the presence of odor identification deficits in early Alzheimer’s disease (AD), our paradigm has the potential to establish relationships between olfactory deficits, neurodegeneration, and memory impairment.

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Guangming Yang¹, Bruce Crosson¹, Robert Haley², Kaundinya Gopinath¹, and Ying Guo^1
1Emory University, Atlanta, GA, United States, ²UT Southwestern Medical Center, Dallas, TX, United States

Keywords: Neurodegeneration, fMRI (resting state)

Around 200,000 veterans of the 1991 Gulf War (GW) suffer from GW illness (GWI). GWI is a poorly understood chronic medical condition, characterized by multiple symptoms. One factor that hampers mechanistic investigations into GWI is that there is considerable heterogeneity in symptoms across the GW veteran population. Only one case definitions of GWI addresses this heterogeneity. The Haley GWI case definition addresses this by further breaking down GWI into three main syndrome variants (Syn1, Syn2, and Syn3) based on factor analysis of symptoms presented by GWI veterans.  In this study, we extracted rsfMRI connectomics biomarkers for different syndromes of GWI.

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Xubin Chai^1,2, Le He¹, Changhao Zhu³, Wanting Hu³, Rong Xue², Xiaolei Song¹, and Li Wang^4
1Center for Biomedical Imaging Research, Department of Biomedical Engineering, School of Medicine, Tsinghua University, Beijing, Beijing, China, ²State Key Laboratory of Brain and Cognitive Science, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China, ³China School of Information Sciences and Technology, Northwest University, Xi'an, China, ⁴Department of Neurology, The First Hospital of Tsinghua University, Beijing, China

Keywords: Neurodegeneration, Molecular Imaging

Cervical dystonia (CD) has been regarded as the most common form of focal dystonia, the affected neck muscles always in sustained situation lead to an abnormal rotation of the head[1]. The mechanisms of CD have not yet been thoroughly illustrated. Botulinum toxin (BT)is considered as the recommended first-line therapeutic method for the focal dystonia[2].Traditional MRI Scan has been used to evaluate any pathophysiological changes of brain anatomy or the affected neck muscles[3].However, seldom MRI have provided the cervical muscles metabolic messages[4].The CEST MRI could provide the metabolic aspects of the cervical muscles before and after the treatment of the BT

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Jack Knight-Scott¹, Marie Caillaud², Isabelle Gallagher², Yanrong Li², and Andreana P. Haley^2
1Radiology, Children's Healthcare of Atlanta, Atlanta, GA, United States, ²Psychology, The University of Texas at Austin, Austin, TX, United States

Keywords: Neurodegeneration, Spectroscopy

Quantification of the combination glutathione (GSH) + γ-aminobutyric acid (GABA) at 3T in medium TE but high signal-noise brain spectra yields a stable concentration with a low coefficient of variation (CV) of only 6%, smaller than individual CV values for GSH or GABA. While an indication of the interdependency of GSH and GABA quantification, [GSH+GABA] also suggests a pathway for the study of perturbations in GABA and GSH at 3T while maintaining the high information content typical of un-edited spectra.

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Sierra Sparks¹, Daniel P. Bulte¹, and Joana Pinto^1
1Institute of Biomedical Engineering, Department of Engineering Science, University of Oxford, Oxford, United Kingdom

Keywords: Neurodegeneration, Alzheimer's Disease, Tracers

Alzheimer’s disease (AD) is the leading cause of dementia, and it is associated with cerebral amyloid beta accumulation and changes in cerebral perfusion. Amyloid PET imaging can be used to measure the amyloid beta load, while ASL-MRI is preferred for cerebral perfusion measurements, which may occur before amyloid beta accumulation. This study compares the amyloid beta load with the CBF in grey matter, in both cognitively healthy and cognitively impaired subjects from the OASIS-3 dataset. A logistic regression model using this data was able to classify the cognitive status of subjects with a 78.48% test accuracy.

Yufan Chen¹, Yishi Wang², Changyuan Xu³, Diwei Shi⁴, and Guangbin Wang^1
1Shandong Provincial Hospital, Shandong University, Jinan, China, ²Philips Healthcare, Beijing, China, ³Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China, ⁴Center for Nano & Micro Mechanics, Department of Engineering Mechanics, Tsinghua University, Beijing, China

Keywords: Neurodegeneration, Degenerative, aging

The aging progress of neuron is characterized by degenerative changes of structure and function. Few studies have focus on the structure in cellular level. In this study, we quantified cell size and cellularity in globus pallidus, putamen and substantia nigra pars compacta using time dependent diffusion imaging. Subgroup analysis by age showed the increased intracellular volume fraction of putamen in older people while diameter does not change over age. These findings might demonstrate the potential of MRI cell size imaging to assess the degeneration of neuron on cellular level.

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Daniel Sare¹, Amartei Brocke¹, and Andrea Kassner^1,2
1Translational Medicine, The Hospital for Sick Children, Toronto, ON, Canada, ²Department of Medical Imaging, The University of Toronto, Toronto, ON, Canada

Keywords: Neurodegeneration, Brain

Up to 60% of obese youths with obstructive sleep apnea (OSA) are afflicted with episodes of nocturnal hypoxia, a known risk factor for structural cerebral alterations and neurocognitive problems leading to cognitive impairment. By applying grey-level co-occurrence-based texture analysis in children with and without OSA, we were able to show that the presence of OSA is associated with microscopic changes in normal appearing white matter in regions impacted by cognitive impairment. The findings support the lower tissue homogeneity, and decrease in cortical density and thickness seen in moderate-severe OSA groups.

Junxia Wang¹ and Bing Zhang^1
1Nanjing Drum Tower Hospital, Nanjing, China

Keywords: Neurodegeneration, Alzheimer's Disease

In this study, we investigated alterations of cortical morphology, subcortical nuclei volume and morphology in MCI using multiple morphological analysis methods. Moreover, we explored these imaging features relationship with cognitive performances and their efficiency in classification of MCI using support vector machine (SVM). We speculate that combining volumetric and morphological analysis methods will outperform than single analysis method in MCI identification.

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Rodolfo G. Gatto¹, Joseph R. Duffy¹, Rene L. Utianski¹, Heather M. Clark¹, Hugo Botha¹, Mary M. Machulda², Val J. Lowe³, Keith A. Josephs¹, and Jennifer L. Whitwell^3
1Division of Neurology, Mayo Clinic at Rochester, Rochester, MN, United States, ²Division of Psychiatry and Psychology, Mayo Clinic at Rochester, Rochester, MN, United States, ³Division of Radiology, Mayo Clinic at Rochester, Rochester, MN, United States

Keywords: Neurodegeneration, Rare disease, Progressive Apraxia of Speech

Progressive apraxia of speech (PAOS) is a tauopathy characterized by difficulties with motor speech programming and planning. Grey and white matter (GM & WM) brain regions were interrogated by diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI). Twenty-three patients with PAOS and 22 matched controls underwent diffusion MRI. Global WM differences in PAOS were better attained by intracellular volume fraction (ICVF), whereas GM global differences were better attained by mean diffusivity (MD) and isotropic volume fraction (isoVF). DTI and NODDI represent unique aspects of brain tissue microstructure and can be used as PAOS biomarkers.

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Stanley D.T. Pham¹, Danique E. Versluis¹, Hilde van den Brink², Nikki Dieleman², Jaco J.M. Zwanenburg¹, Geert Jan Biessels², and Jeroen C.W. Siero^1
1Radiology, UMC Utrecht, Utrecht, Netherlands, ²Neurology, UMC Utrecht, Utrecht, Netherlands

Keywords: Neurodegeneration, White Matter, small vessel disease, white matter hyperintensities

We studied the association between cerebrovascular reactivity measured with blood oxygenation level-dependent (BOLD-CVR) to hypercapnia and progression of white matter hyperintensities (WMH) on 7T MRI in cerebral small vessel disease (SVD). BOLD-CVR was assessed in voxels of four different categories which represent the fate of a voxel after two-year follow-up. The BOLD-CVR in normal appearing white matter (NAWM) that converted into WMH was significantly lower than the BOLD-CVR of voxels that remained WMH (0.39 [0.16-0.63] vs 0.98 [0.75-1.21]) (p<0.0001). The association between BOLD-CVR and WMH progression signifies the potential of BOLD-CVR to be a predictor of disease progression in SVD.

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Yufan Chen¹, Tao Gong², Cong Sun³, Fei Gao², Tong Chen¹, Weibo Chen⁴, and Guangbin Wang^2
1Shandong Provincial Hospital, Shandong University, Jinan, China, ²Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China, ³Department of Radiology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China, ⁴Philips Healthcare, Shanghai, China

Keywords: Neurodegeneration, Aging

The study investigated age-related neuromelanin signal variation and iron content changes in the subregions of substantia nigra simultaneously by magnetization transfer contrast neuromelanin-sensitive multi-echo fast field echo sequence in a normal population. We manually delineated neuromelanin, iron deposition regions and the overlap regions. The correlation analysis revealed contrast ratio increased over age, while volume showed an age-related decline in the overlap region which were similar to those in the neuromelanin region. No significant correlations were found between susceptibility and age in any subregion. This study may provide more insight for the future degenerative elucidations of substantia nigra.

Xiangxiang Wu¹, Zijian Jiang¹, Jiahui Zheng¹, Zhuqing Jiao², Tongqiang Liu¹, Weiqiang Dou³, and Haifeng Shi^1
1Changzhou Second People’s Hospital, Changzhou, China, ²Changzhou University, Changzhou, China, ³GE Healthcare, MR Research China, Beijing, China

Keywords: Neurodegeneration, Diffusion/other diffusion imaging techniques

This study aimed to investigate the clinical value of intravoxel incoherent motion (IVIM) diffusion-weighted imaging in evaluating the brain microstructural and perfusion changes in end-stage renal disease (ESRD) patients. 40 ESRD patients and 30 healthy subjects were recruited in this study and underwent IVIM MRI. The microstructure and perfusion of the brain showed significantly differences in the left frontal lobe, bilateral temporal lobe, left hippocampus, right occipital lobe, represented by IVIM derived parameters of slow apparent diffusion coefficient (ADC_slow) and fast apparent diffusion coefficient (ADC_fast). Therefore, we concluded that the brain microstructure and perfusion were impaired in ESRD patients.