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Heterotopic Gray Matter

Last Updated: October 1, 2018

Open Table of Contents: Heterotopic Gray Matter

Figure 1: The phase sensitive (T1) inversion recovery (top row left) and FLAIR (top row right) sequences demonstrate a cluster of nodules along the ependymal surface of the posterior horn of the right lateral ventricle. They have signal characteristics matching gray matter. The T1 weighted image (bottom row) in this patient also demonstrated a band of heterotopic gray matter within the right perirolandic white matter.

Description

  • Disrupted migration of neurons from periventricular germinal zone to the cortex

Pathology

  • Periventricular nodular type
    • FLNA gene commonly involved on Xq28
  • Band like heterotopia/lissencephaly

    • Deletion of LIS1 on 17p13.3 or DCX on Xq22.3-q23

Clinical Features

  • Symptoms
    • Young child with variable developmental delay and seizures
  • Age

    • Severe cases present earlier in life
    • Typically present by the 3rd decade of life
  • Gender

    • M > F
  • Males have worse outcomes

Imaging

  • General
    • Abnormal gray matter nodules or ribbons within the white matter anywhere from ventricles to cortex
  • Modality Specific

    • CT and MR
      • Nonenhancing masses that follow the density or intensity of gray matter on all images
  • Imaging Recommendations

    • MR with contrast
  • Mimic

    • Low grade gliomas may have a similar appearance, but usually do not match gray matter so closely on all MR sequences.

For more information, please see the corresponding chapter in Radiopaedia.

Contributor: Sean Dodson, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.02.16

References

Barkovich AJ. Morphologic Characteristics of Subcortical Heterotopia: MR Imaging Study. AJNR. 2000; 21:290-95.

Barkovic AJ and Kjos BO. Gray Matter Heterotopias: MR Characteristics and Correlation with Developmental and Neurologic Manifestations. Radiology. 1992; 182(2):493-99.

Donkol RH, et al. Assessment of Gray Matter Heterotopia by Magnetic Resonance Imaging. World J Radiol. 2012; 4(3):90-6.

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