Myelination in Children with Partial Deletions of Chromosome 18q

Overview

Journal AJNR Am J Neuroradiol

Specialty Neurology

Date 2005 Mar 12

PMID 15760848

Citations 10

Authors

Jack L Lancaster

Jannine D Cody

Trevor Andrews

L Jean Hardies

Daniel E Hale

Peter T Fox

Affiliations

Soon will be listed here.

Abstract

Background And Purpose: We compared myelin levels in white matter (WM) in typically developing children with those of children with partial deletions of chromosome 18q (18q-).

Methods: Conventional spin-echo MR imaging at 1.9T was used to acquire T1-, T2-, and proton density-weighted images of the brain. From these images, a three-pool model was used to estimate the fraction of water in myelin, myelinated axon, and mixed water compartments (or pools) in six WM regions. A model curve was fit to the pool fractions to model the trend of myelin development by age in each region. Water-pool fractions in children with 18q- aged 5 months to 13 years were compared with those of age-matched, typically developing children.

Results: In children with 18q-, the model predicted later onset of myelination (P <.02), lower myelination rates (P <.001), and smaller equilibrium myelin pool fractions (P <.001). Significant differences were seen between the two groups for all three water pool fractions (P <.001). The mixed pool fraction was larger in children with 18q-. Although the myelin pool fraction was significantly smaller, the myelinated axon pool fraction was only slightly smaller, leading to a significantly smaller estimate of myelin per myelinated axon in children with 18q- (P <.001).

Conclusion: Myelination modeling in 18q- children indicated delayed onset, a lower rate of myelination, and equilibrium myelin levels less than 50% those of age-matched, typically developing children.

Citing Articles

Imaging Findings and MRI Patterns in a Cohort of 18q Chromosomal Abnormalities.

Malik P, Branson H, Yoon G, Shroff M, Blaser S, Krishnan P AJNR Am J Neuroradiol. 2024; 45(10):1578-1585.

PMID: 38816019 PMC: 11448982. DOI: 10.3174/ajnr.A8361.

Hypomyelinating disorders in China: The clinical and genetic heterogeneity in 119 patients.

Ji H, Li D, Wu Y, Zhang Q, Gu Q, Xie H PLoS One. 2018; 13(2):e0188869.

PMID: 29451896 PMC: 5815574. DOI: 10.1371/journal.pone.0188869.

Potassium channel gene associations with joint processing speed and white matter impairments in schizophrenia.

Bruce H, Kochunov P, Paciga S, Hyde C, Chen X, Xie Z Genes Brain Behav. 2017; 16(5):515-521.

PMID: 28188958 PMC: 5457349. DOI: 10.1111/gbb.12372.

A New Strategy for Fast MRI-Based Quantification of the Myelin Water Fraction: Application to Brain Imaging in Infants.

Kulikova S, Hertz-Pannier L, Dehaene-Lambertz G, Poupon C, Dubois J PLoS One. 2016; 11(10):e0163143.

PMID: 27736872 PMC: 5063462. DOI: 10.1371/journal.pone.0163143.

Perfusion shift from white to gray matter may account for processing speed deficits in schizophrenia.

Wright S, Hong L, Winkler A, Chiappelli J, Nugent K, Muellerklein F Hum Brain Mapp. 2015; 36(10):3793-804.

PMID: 26108347 PMC: 4714540. DOI: 10.1002/hbm.22878.

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