Magnetic resonance imaging evaluation of delayed myelination in Down syndrome: a case report and review of the literature
Abstract
Magnetic resonance imaging has been found to be useful in assessing brain myelination and provides information on brain maturation. The normal pattern of brain myelination conforms to a fixed sequence, with good pathologic and MRI correlation. Neuropathologic analysis of myelination has shown delayed central myelination in Down syndrome. Delayed myelination on MRI in Down syndrome has not previously been reported. We report a case of Down syndrome with a significant delay in myelination as demonstrated on MRI. This 18-month-old infant had brain myelination equivalent to that expected for an 11-month-old infant. To determine the relative incidence, extent of delayed myelination, and time for recovery to full myelination in Down syndrome, more cases require examination and assessment. Magnetic resonance imaging has the advantage of serial assessment of myelination during brain maturation. (J Child Neurol 1992;7:417-421).
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Previous studies show that DS children manifest learning and memory problems in late infancy which often worsen in adolescence.
“This is in agreement both with previous studies showing that DS children manifest learning and memory problems in late infancy which often worsen in adolescence (Koo et al., 1992; Lanfranchi et al., 2010) and with post-mortem studies that show reduced myelin content (Abraham et al., 2012; Wisniewski, 1990; Wisniewski and Schmidt-Sidor, 1989) and fewer oligodendrocytes in DS striatum (Karlsen and P”
From Down Syndrome Developmental Brain Transcriptome Reveals Defective Oligodendrocyte Differentiation and Myelination (2016) · cites this paper as Koo et al., 1992
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Dendritic atrophy in children with down’s syndrome.
Becker, L. E., Armstrong, D. L., & Chan, F. (1986) · Annals of Neurology · doi:10.1002/ana.410200413 · Cited by 279 · see citing works
Self-citationDown's syndrome: Is there a decreased population of neurons?
Ross, M. H., Galaburda, A. M., & Kemper, T. L. (1984) · Neurology · doi:10.1212/wnl.34.7.909 · Cited by 191 · see citing works
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Olmos-Serrano, J. L., Kang, H. J., Tyler, W. A., Silbereis, J. C., Cheng, F., Zhu, Y., Pletikos, M., Jankovic-Rapan, L., Cramer, N. P., Galdzicki, Z., et al. (2016) · Neuron · doi:10.1016/j.neuron.2016.01.042 · Cited by 239 · see citing works
“…both with previous studies showing that DS children manifest learning and memory problems in late infancy, which often worsen in adolescence (Koo et al., 1992; Lanfranchi et al., 2010), and with postmortem studies that show reduced myelin content (Ábrahám et al., 2012; Wisniewski, 1990;…”
Absence of Prenatal Forebrain Defects in the Dp(16)1Yey/+ Mouse Model of down Syndrome.
Goodliffe, J. W., Olmos-Serrano, J. L., Aziz, N. M., Pennings, J. L. A., Guedj, F., Bianchi, D. W., & Haydar, T. F. (2016) · The Journal of Neuroscience · doi:10.1523/jneurosci.2513-15.2016 · Cited by 65 · see citing works
Abnormal myelination in Angelman syndrome
Harting, I., Seitz, A., Rating, D., Sartor, K., Zschocke, J., Janssen, B., Ebinger, F., & Wolf, N. I. (2009) · European Journal of Paediatric Neurology · doi:10.1016/j.ejpn.2008.04.005 · Cited by 58 · see citing works
- Authors
- Koo, B. K. K., Blaser, S., Harwood-Nash, D., Becker, L. E., & Murphy, E. G.
- Affiliations
- Hospital for Sick Children (CA)
- The Division of Neuroradiology, Hospital for Sick Children, Toronto, Ontario
- ORCID
- Murphy, E.G.
- Venue
- Journal of Child Neurology
- Publisher
- SAGE Publications
- Published
- 1992-10
- Volume
- 7
- Issue
- 4
- Pages
- 417-421
- ISSN
- 0883-0738 (ISSN-L)
- 1708-8283
- OpenAlex
- W2029320725
- Cited by
- 59
- References
- 2
- Citations by year
- 2025: 1 · 2024: 1 · 2023: 16 · 2022: 3 · 2021: 2 · 2019: 1 · 2016: 3 · 2014: 5 · 2013: 1 · 2012: 2
- FWCI
- 0.52
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Child Neurology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Keywords
- Down syndrome, delayed myelination, brain myelination, brain maturation, infant brain development, neuropathology
- MeSH terms
- Brain, Brain Diseases, Down Syndrome, Female, Humans, Infant, Magnetic Resonance Imaging, Myelin Sheath
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