Top Ad unit 728 × 90

Radiology News


Tuberous Sclerosis with SGCA

14 yr old male child has seizures and mental retardation. MRI shows non enhancing cortical  hyperintensities on T2 and FLAIR.  In addition, a large intensely and heterogenously enhancing intraventricular, foramen of Monroe Space occupying lesion with obstructive hydrocephalus  with calcification. Features possibly  represent  cortical tubers with subependymal giant cell astrocytoma. Case submitted by-Dr MGK Murthy.

Tuberous sclerosis for the Radiologist

Synonym:  Bournveilles disease. Genetic disease – mutation of TSC1and TSC2, which encode for proteins hamartin and Tuberin (act as tumour growth suppressive factors )

Dignostic  criteria=   11 Major criteria and 9 minor criteria

Brain lesions
Tubers- triangular in shape with apex towards ventricles  and look hyperintense on T2. It may subside with age , but histopathology will still reveal it. Represents neuronal migration disorder. Other MR findings –Radial white matter tracts hyperintense on  T2. Heterotopic grey matter-Subependymal  nodules—abnormal  swollen glial cells and bizarre multinucleated cells . These could turn in to subependymal giant cell astrocytoma (SGCA). Ventricular enlargement

Tuberous Sclerosis with SGCA Reviewed by Sumer Sethi on Monday, April 25, 2011 Rating: 5

No comments:

All Rights Reserved by Sumer's Radiology Blog © 2014 - 2015
Powered By Blogger,

Contact Form


Email *

Message *

All contents copyrights with Sumer Sethi. Powered by Blogger.