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The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study

التفاصيل البيبلوغرافية
العنوان: The spinal and cerebral profile of adult spinal-muscular atrophy: A multimodal imaging study
المؤلفون: Rabab Debs, Jean-Yves Hogrel, Gaëlle Bruneteau, Tanya Stojkovic, Maria del Mar Amador, Julien Cohen-Adad, Lucette Lacomblez, David Devos, Nadine Le Forestier, Peter Bede, Timothée Lenglet, Menghan Li, Sophie Blancho, Pierre-François Pradat, Pascal Laforêt, Anthony Behin, François Salachas, Habib Benali, Martin Catala, Véronique Marchand-Pauvert, Mohamed-Mounir El Mendili, Stéphane Lehéricy, Vincent Meininger, Giorgia Querin
المساهمون: Laboratoire d'Imagerie Biomédicale (LIB), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Sorbonne Université (SU), Icahn School of Medicine at Mount Sinai [New York] (MSSM), Service de Neuroradiologie [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centres de référence pour la sclérose latérale amyotrophique [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut de Myologie, Centre National de la Recherche Scientifique (CNRS)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Association française contre les myopathies (AFM-Téléthon)-Sorbonne Université (SU), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Troubles cognitifs dégénératifs et vasculaires - U 1171 (TCDV), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Département de Recherche en Ethique, Université Paris-Sud - Paris 11 (UP11), École Polytechnique de Montréal (EPM), Université de Montréal (UdeM), Unité de Neuroimagerie Fonctionnelle [Montréal] (UNF-CRIUGM), Université de Montréal (UdeM)-Centre de Recherche de l'Institut Universitaire de Gériatrie de Montréal (CRIUGM), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre de Neuro-Imagerie de Recherche (CENIR), Hôpital Raymond Poincaré [AP-HP], Handicap neuromusculaire : Physiopathologie, Biothérapie et Pharmacologies appliquées (END-ICAP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Institut National de la Santé et de la Recherche Médicale (INSERM), Concordia University [Montreal], Institut de Biologie Paris Seine (IBPS), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Trinity College Dublin, University of Ulster, CHU Lille, CNRS, Inserm, Université de Lille, Troubles cognitifs dégénératifs et vasculaires - U1171, Lille Neurosciences & Cognition (LilNCog) - U 1172, Laboratoire d'Imagerie Biomédicale [Paris] (LIB), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Troubles cognitifs dégénératifs et vasculaires - U 1171 - EA 1046 (TCDV), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Center for NeuroImaging Research-Human MRI Neuroimaging core facility for clinical research [ICM Paris] (CENIR), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Gestionnaire, Hal Sorbonne Université, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)
المصدر: Neuroimage-Clinical
Neuroimage-Clinical, Elsevier, 2019, 21, pp.101618. ⟨10.1016/j.nicl.2018.101618⟩
Neuroimage-Clinical, 2019, 21, pp.101618. ⟨10.1016/j.nicl.2018.101618⟩
NeuroImage : Clinical
NeuroImage: Clinical, Vol 21, Iss, Pp-(2019)
بيانات النشر: HAL CCSD, 2019.
سنة النشر: 2019
مصطلحات موضوعية: Male, Pathology, UMN, Upper motor neuron, [SDV]Life Sciences [q-bio], LL, Lower limb, RD, Radial diffusivity, Spinal Muscular Atrophies of Childhood, lcsh:RC346-429, ALS, Amyotrophic lateral sclerosis, 0302 clinical medicine, Grey matter and white matter degeneration, Spinal cord MRI, Multimodal MRI, SMA, Spinal muscular atrophy, Medicine, FA, Fractional anisotropy, Gray Matter, SMA, Spinal muscular atrophy, GM, Grey matter, UL, Upper limb, HC, Healthy control, 05 social sciences, Brain, Middle Aged, Magnetic Resonance Imaging, White Matter, FWE, Familywise error, TR, repetition time, [SDV] Life Sciences [q-bio], Diffusion Tensor Imaging, medicine.anatomical_structure, Spinal Cord, Neurology, FOV, Field-of-view, WM, White matter, lcsh:R858-859.7, Female, [SDV.IB]Life Sciences [q-bio]/Bioengineering, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], EPI, Echo-planar imaging, Motor cortex, Adult, CST, Corticospinal tract, medicine.medical_specialty, CSA, cross-sectional area, Grey matter, Adolescent, ROI, Region-of-interest, MNI, Montreal Neurological Institute, Cognitive Neuroscience, LMN, Lower motor neuron, SC, Spinal cord, lcsh:Computer applications to medicine. Medical informatics, Lower motor neuron, Article, 050105 experimental psychology, MND, Motor neuron disease, Muscular Atrophy, Spinal, White matter, Young Adult, 03 medical and health sciences, AD, Axial diffusivity, Neuroimaging, TE, Echo time, Humans, 0501 psychology and cognitive sciences, Radiology, Nuclear Medicine and imaging, [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], lcsh:Neurology. Diseases of the nervous system, Aged, [SDV.IB] Life Sciences [q-bio]/Bioengineering, MD, mean diffusivity, SMAFRS, SMA functional rating scale, business.industry, VBM, Voxel-based morphometry, SMN1, Survival-motor-neuron 1 gene, MRC, Medical Research Council, TFCE, Threshold-free cluster enhancement, medicine.disease, Spinal cord, white matter degeneration, FDR, False discovery rate, DTI, diffusion tensor imaging, Neurology (clinical), 6MWT, Six-minute walk test, business, TBSS, Tract-based spatial statistics, 030217 neurology & neurosurgery
الوصف: Spinal muscular atrophy (SMA) type III and IV are autosomal recessive, slowly progressive lower motor neuron syndromes. Nevertheless, wider cerebral involvement has been consistently reported in mouse models. The objective of this study is the characterisation of spinal and cerebral pathology in adult forms of SMA using multimodal quantitative imaging. Methods Twenty-five type III and IV adult SMA patients and 25 age-matched healthy controls were enrolled in a spinal cord and brain imaging study. Structural measures of grey and white matter involvement and diffusion parameters of white matter integrity were evaluated at each cervical spinal level. Whole-brain and region-of-interest analyses were also conducted in the brain to explore cortical thickness, grey matter density and tract-based white matter alterations. Results In the spinal cord, considerable grey matter atrophy was detected between C2-C6 vertebral levels. In the brain, increased grey matter density was detected in motor and extra-motor regions of SMA patients. No white matter pathology was identified neither at brain and spinal level. Conclusions Adult forms of SMA are associated with selective grey matter degeneration in the spinal cord with preserved white matter integrity. The observed increased grey matter density in the motor cortex may represent adaptive reorganisation.
Highlights • (SMA) type 3 and 4 is a lower motor neuron syndrome. Nevertheless, wider involvement of the nervous system might be possible. • 25 adults type 3 and 4 SMA patients were studied using brain and cervical spinal cord neuroimaging techniques. • Grey matter atrophy was observed in the spinal cord. No white matter degeneration was present at brain and spinal level. • Increased grey matter density was detected in cerebral motor regions and explained as compensatory mechanism.
وصف الملف: application/octet-stream; application/pdf
اللغة: English
تدمد: 2213-1582
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a6d677f059efc03030b5919d29d82dbeTest
https://hal.sorbonne-universite.fr/hal-02090969Test
حقوق: OPEN
رقم الانضمام: edsair.doi.dedup.....a6d677f059efc03030b5919d29d82dbe
قاعدة البيانات: OpenAIRE
الوصف
تدمد:22131582