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Tuberous sclerosis

Presentation

Seizure.

Patient Data

Age: 2 years
Gender: Male
MRI

MRI Brain

MRI shows multiple areas of cortical/subcortical hyperintensities on T2-WI and FLAIR sequences compatible with tubers. There are multiple subependymal hamartomas and characteristic linear white matter bands (radial bands).

Case Discussion

Tuberous sclerosis complex (Bourneville-Pringle disease) is a multisystem congenital syndrome. Tubers most commonly found in frontal lobes (90%). Cerebellar tubers are less commonly seen, where they may become apparent only at histologic examination. Magnetization transfer T1- weighted imaging can be superior to FLAIR imaging for detection of subtle tubers. Radial bands that believed to represent heterotopic glial and neurons along the expected path of cortical migration, are located in subcortical white matter and are thought to be specific for TSC.

Case courtesy of Prof. Ali Hekmatnia.

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Case information

rID: 66692
Published: 4th Mar 2019
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Isfahan University of Medical Sciences

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