Tuberous sclerosis complex with subependymal giant cell astrocytoma
Presentation
Intractable epilepsy.
Patient Data
Selected images demonstrating numerous supratentorial cortical tubers in form of non-enhancing areas of T2/FLAIR hyperintensity involving the cortical and subcortical white matter throughout bilateral cerebral hemispheres. In addition, there is a large right cerebellar cortical tuber.
Multiple enhancing subependymal nodules, the largest seen close to the right foramen of Monro, suggestive of SEGA. No associated ventricular obstruction.
18F-FDG PET/CT
Selected images demonstrating multiple areas of hypometabolism throughout the cerebral hemispheres, corresponding to the cortical tubers seen in MRI. In addition, multiple calcified subependymal nodules.
Case Discussion
A 4-year-old male with drug-resistant focal epilepsy, secondary to tuberous sclerosis. His seizures started at 3 months of age with generalized epileptic spasms and evolved to stiffening of left arm, smiling and eye movements. Frequency ranges from 3-4 times per week to 1-2 per day. No status epilepticus was documented.
On EEG, the vast majority of electrographic and electroclinical seizures were captured from the right frontal or frontocentral or central-parietal regions.
MRI showed a high burden of tubers, corresponding to hypometabolic areas in 18F-FDG-PET, with no sure localization of the epileptogenic focus. So, stereo EEG implantation was considered for surgical planning.
Courtesy of Dr. Christine Saint-Martin, Associate Professor in the Neuroradiology and Pediatric Radiology at McGill University.