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White matter abnormalities in tuberous sclerosis complex

P D Griffiths1, P Bolton, C Verity

  • 1Academic Department of Radiology, University of Sheffield, UK.

Acta Radiologica (Stockholm, Sweden : 1987)
|October 2, 1998
PubMed
Summary
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White matter abnormalities are common in children with tuberous sclerosis complex (TSC), often linked to cortical tubers. MR imaging reveals diverse TSC-related white matter changes, including gliosis and hypomyelination.

Area of Science:

  • Pediatric Neurology
  • Neuroimaging
  • Genetics

Background:

  • Tuberous sclerosis complex (TSC) is a genetic disorder.
  • TSC can affect multiple organs, including the brain.
  • White matter abnormalities are a known feature of TSC.

Purpose of the Study:

  • To investigate and describe the spectrum of white matter abnormalities in pediatric TSC patients.
  • To correlate imaging findings with the disease.

Main Methods:

  • Retrospective cross-sectional study.
  • MR imaging analysis of 20 pediatric TSC cases (age ≤ 17 years).

Main Results:

  • 95% of TSC patients showed white matter abnormalities.
  • Abnormalities were most frequent near supratentorial cortical tubers.

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  • Other findings included radial migration lines, white matter cysts, and stroke.

    • Conclusions:

    • MR imaging demonstrates a range of white matter abnormalities in pediatric TSC.
    • Gliosis and hypomyelination near tubers are common.
    • Radial migration lines may represent heterotopic glia/neurons.