Tuberous sclerosis complex with subependymal giant cell astrocytoma

Case contributed by Dr Mustafa Takesh

Presentation

Intractable epilepsy.

Patient Data

Age: 4 years
Gender: Male

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

Nuclear medicine

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.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.