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Case of the Week 6

*A 10 year-old child presented with seizure.

What is the most likely diagnosis?

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

Answer


Case Discussion:

MRI shows a mass which projects into the posterior of the right lateral ventricle. It has signal intensity which match those of cortex on all sequences

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

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