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

Developmental Medicine and Child Neurology
Catherine SarretOdile Boespflug-Tanguy

Abstract

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). 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. Age-adjusted multivariate analysis revealed that patients with PMD0-1 had higher-severity 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. Brain atrophy and myelination of anterior cerebral r...Continue Reading

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Citations

Feb 13, 2016·Developmental Medicine and Child Neurology·Ken Inoue
May 29, 2016·Clinical Genetics·A CharzewskaD Hoffman-Zacharska
Jan 9, 2019·The Journal of Clinical Investigation·Devesh C PantAurora Pujol
Aug 11, 2018·Brain Structure & Function·Catrona AndersonSebastian Ocklenburg
May 12, 2017·Neurogenetics·Maher Awni ShahrourOrly Elpeleg
Jan 15, 2019·Movement Disorders Clinical Practice·Nikola KresojevićVladimir Kostić
Sep 30, 2020·Experimental Neurology·Ruili Feng TieqiaoWen
Dec 17, 2020·Nature Reviews. Neurology·Nicole I WolfMarjo S van der Knaap

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