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The 'double cortex' syndrome on MRI

R Hashimoto1, T Seki, Y Takuma

  • 1Department of Pediatrics, Shizuoka Red Cross Hospital, Japan.

Brain & Development
|January 1, 1993
PubMed
Summary
This summary is machine-generated.

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This study describes a rare brain malformation called diffuse subcortical heterotopia, or double cortex syndrome, in a young girl. This condition involves abnormal gray matter organization, presenting unique diagnostic challenges.

Area of Science:

  • Neuroscience
  • Radiology
  • Genetics

Background:

  • Diffuse subcortical heterotopia (DSH), also known as band heterotopia, is a rare neuronal migration disorder.
  • It is characterized by the presence of a band of heterotopic gray matter beneath the cerebral cortex.
  • This condition is typically associated with epilepsy, cognitive impairment, and behavioral issues, predominantly affecting females.

Observation:

  • A 6-year-old girl presented with diffuse subcortical heterotopia, exhibiting a distinctive 'double cortex' appearance on magnetic resonance imaging (MRI).
  • Her cerebral parenchyma displayed four distinct layers: cortex, outer white matter, heterotopic gray matter band, and inner white matter.
  • Clinical observations included mild developmental delay, clumsiness, emotional lability, and abnormal movements during sleep, though daily functioning was largely preserved.

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Findings:

  • MRI revealed a 'double cortex' pattern, a radiological hallmark of laminar heterotopia.
  • Electroencephalography (EEG) showed high-voltage spindles in bilateral frontal areas during sleep, but no definitive epileptic discharges were identified.
  • The patient's neurological presentation, while severe on imaging, had a relatively mild impact on her daily life.

Implications:

  • The 'double cortex' appearance on MRI is a key radiological marker for laminar heterotopia.
  • Establishing double cortex syndrome as a distinct clinico-radiological entity is crucial for understanding its spectrum.
  • Further research is needed to clarify the etiology, long-term prognosis, and precise management of this rare neurological anomaly.