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Case of the Week 20 2016

*21-month-old girl presented with seizure.

What is the most likely diagnosis?

1. Subependymal giant cell astrocytoma
2. Subependymal grey matter heterotopia
3. Tuberous sclerosis
4. Subependymal haemorrhage

Answer

Answer: Subependymal grey matter heterotopia

Case Discussion:

MR images show masses which projects into the posterior of the bilateral lateral ventricle. It has signal intensity which match those of cortex on all sequences.

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This is the most common form of grey matter heterotopia. Subependymal grey matter heterotopia can be divided according to morphology into:

-unilateral focal
-bilateral focal
-bilateral diffuse: a band of grey matter surrounding the ventricles

Subependymal grey matter heterotopias consist of clusters of neurons and glial cells. They generally are associated with epilepsy and occasionally developmental delay.

References:
1. Abdel Razek AA, Kandell AY, Elsorogy LG et-al. Disorders of cortical formation: MR imaging features. AJNR Am J Neuroradiol. 2009;30 (1): 4-11.doi:10.3174/ajnr.A1223 – Pubmed citation.