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Maffucci Syndrome
Joseph Junewick, MD FACR
over 10 years ago
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Band Heterotopia

Case Detail

Anatomy: Brain-Spine
Heather Borders, MD
Diagnostic Category: Developmental or Congenital
Created: over 10 years ago
Updated: over 10 years ago
Tags: PEDS
Modality/Study Types: MRI
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Three year old patient with seizures.

Case Images


Band Heterotopia


A thick band of gray matter (parallels the intensity of gray matter on all sequences) separated from the outer cortex and ventricles by layers of white matter. The outer cortex shows subjective mildly shallow sulci.


Band Heterotopia typically presents in childhood due to developmental delay and mixed seizures. There is a female preponderance due to the location of DCX on the X chromosome.

On imaging, there is a homogeneous band of gray matter located between the ventricles and the cerebral cortex and separated from both by layers of white matter. Overlying cortex is usually normal in thickness and there is variablility in the degree of shallowness of the sulci.

This may be mistaken for myelinating white matter in a neonate unless the cell sparse zone is visible.



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