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Heterotopic Gray Matter

Last Updated: October 1, 2018

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

    • Seve...