Time-course of myelination and atrophy on cerebral imaging in 35 patients with PLP1 -related disorders

Autor: Jerome Coste, Davide Tonduti, Catherine Sarret, Jean-Jacques Lemaire, Odile Boespflug-Tanguy, Basile Roche, Fabien Feschet, Bruno Pereira, Anna Sontheimer
Přispěvatelé: Image Guided Clinical Neurosciences and Connectomics (IGCNC), Université d'Auvergne - Clermont-Ferrand I (UdA), Département de Pédiatrie [Clermont-Ferrand], CHU Clermont-Ferrand, Génétique, Reproduction et Développement (GReD), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])-Centre National de la Recherche Scientifique (CNRS), Service de Neurochirurgie [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, Institut Pascal (IP), SIGMA Clermont (SIGMA Clermont)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])-Centre National de la Recherche Scientifique (CNRS), Neuroprotection du Cerveau en Développement / Promoting Research Oriented Towards Early Cns Therapies (PROTECT), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Istituti di Ricovero e Cura a Carattere Scientifico (IRCCS), service de Biostatistiques, DRCI, Hôpital Robert Debré, Centre National de la Recherche Scientifique (CNRS)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Neurochirurgie [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Gabriel Montpied [Clermont-Ferrand], SIGMA Clermont (SIGMA Clermont)-Centre National de la Recherche Scientifique (CNRS)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])
Jazyk: angličtina
Rok vydání: 2016
Předmět:
Adult
Male
0301 basic medicine
Cerebellum
Pathology
medicine.medical_specialty
Adolescent
Pelizaeus-Merzbacher Disease
[SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/Imaging
[SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology
Grey matter
Corpus callosum
Severity of Illness Index
Statistics
Nonparametric
Cohort Studies
White matter
Young Adult
03 medical and health sciences
0302 clinical medicine
Atrophy
Developmental Neuroscience
Image Processing
Computer-Assisted
medicine
Spastic
Humans
Child
Myelin Sheath
Cerebral Cortex
Movement Disorders
medicine.diagnostic_test
Age Factors
Brain
Infant
Magnetic resonance imaging
Middle Aged
medicine.disease
Magnetic Resonance Imaging
030104 developmental biology
medicine.anatomical_structure
Child
Preschool
Pediatrics
Perinatology and Child Health
Disease Progression
Neurology (clinical)
Paraplegia
Psychology
030217 neurology & neurosurgery
Zdroj: Developmental Medicine and Child Neurology
Developmental Medicine and Child Neurology, 2016, 58 (7), pp.706-713. ⟨10.1111/dmcn.13025⟩
Developmental Medicine and Child Neurology, Wiley-Blackwell, 2016, 58 (7), pp.706-713. ⟨10.1111/dmcn.13025⟩
ISSN: 0012-1622
Popis: International audience; Aim: Brain magnetic resonance imaging (MRI) motor development score (MDS) correlations were used to analyze the natural time-course of hypomyelinating PLP1-related disorders (Pelizaeus-Merzbacher disease [PMD] and spastic paraplegia type 2).Method: Thirty-five male patients (ranging from 0.7-43.5y at the first MRI) with PLP1-related disorder were prospectively followed over 7 years. Patients were classified according to best motor function acquired before 5 years (MDS) into five categories (from PMD0 without motor acquisition to PMD4 with autonomous walking). We determined myelination and atrophy scores and measured corpus callosum area, volume of cerebellum, white matter and grey matter on 63 MRI.Results: Age-adjusted multivariate analysis revealed that patients with PMD0-1 had higherseverity atrophy scores and smaller corpus callosum area than did patients with PMD2 and PMD3-4. Myelination score increased until 12 years. There was evidence that the mean myelination differed in frontal white matter, arcuate fibres, and internal capsules among the groups. Most patients showed worsening atrophy (brain, cerebellum, corpus callosum), whereas grey matter and white matter proportions did not change.Interpretation: Brain atrophy and myelination of anterior cerebral regions appear to be pertinent biomarkers of motor development. The time-course of inter-and intra-individual cerebral white matter and grey matter atrophy suggests that both oligodendrocytes and neurons are involved in the physiopathology of PLP1-related disorders
Databáze: OpenAIRE
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