10 months old male baby with skin lesions and mild seizures.
Loading Stack -
0 images remaining
- multiple bilateral periventricular and subependymal nodular lesions with predominantly high T1 and low to intermediate T2 / FLAIR signal intensity.
- bilateral subcortical patchy areas of variable T1 and high T2 / FLAIR signal intensity with no restricted diffusion. Some subcortical lesions are possibly calcified with low T2 signal.
- streaky linear T2 / Flair hyperintensities (radial bands) along the radial migrations from ventricle to cortex. These bands are oriented perpendicular to the ventricular wall.
- focal deep white matter cyst–like T2 hyperintense lesions (cystoid degenerations) are seen; most evident at the right high posterior frontal lobe.
This case shows typical MRI findings of tuberous sclerosis of the brain with sucortical tubers and subependymal nodules (SEN). Tuberous sclerosis is one of the phakomatoses which are a group of neuroectodermal disorders characterized by coexisting skin lesons and CNS tumors. The typical classic triad of TS is mental retardation, adenoma sebaceoum and seizures, yet it is found in a minority of patients.
- Yilmaz U, Altmeyer K, Meyer S. Tuberous sclerosis complex. Radiologe. 2013;53 (12): 1091-8. doi:10.1007/s00117-013-2553-y - Pubmed citation
- Nandigam K, Mechtler LL, Smirniotopoulos JG. Neuroimaging of neurocutaneous diseases. Neurol Clin. 2014;32 (1): 159-92. doi:10.1016/j.ncl.2013.07.003 - Pubmed citation
- Northrup H, Krueger DA. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 international tuberous sclerosis complex consensus conference. Pediatr. Neurol. 2013;49 (4): 243-54. doi:10.1016/j.pediatrneurol.2013.08.001 - Pubmed citation