Tuberous sclerosis

Case contributed by Abhishek Bhargava
Diagnosis certain

Presentation

The patient presented with seizures and headaches.

Patient Data

Age: 2 years
Gender: Female
mri

Multiple T2 hyperintensities are seen in cortical and subcortical white matter of both cerebral hemispheres likely representing cortical tubers. Few cortical tubers are showing a hypointense central region with a hyperintense rim possibly epileptogenic cortical tubers foci.

Multiple subependymal nodules are present in both the lateral ventricles. Extensive Subependymal calcification is seen along the dilated posterior horn of the left lateral ventricle.

Case Discussion

Cortical tubers are typically benign pathological entities. However, the extent of cerebral dysfunction, including intractable epilepsy and mental retardation, may be related to the burden from cortical tubers, especially when there is bilateral hemisphere involvement.

Cortical tubers that demonstrate heterogeneous signal intensity characterized by a hypointense central region surrounded by a hyperintense rim on FLAIR MR images are associated with epileptic seizures.Imaging may be particularly helpful, serving as a road map for localizing the epileptogenic tubers 1.

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