The Case History of Lucy
Lucy, age 12, tuberous sclerosis complex (TSC) presenting with status epilepticus and a large subependymal giant cell astrocytoma (SEGA).
Lucy is a young lady with tuberous sclerosis complex (TSC) who presented with two medical problems that required treatment. She was transferred in status epilepticus and with a large SEGA. Her medical and surgical management involved several steps. First, using scalp video EEG, MRI and FDG-PET we determined that her status was originating from the left temporal lobe where a large cortical tuber was located (see FDG-PET/MRI co-registration pictures). We removed the left temporal lobe which stopped the status epilepticus.
We followed her for several months with serial MRI scans. With these scans it became apparent that the right SEGA was growing. We tried rapamycin for several months which reduced the size of the SEGA, but the tumor quickly grew when the family elected to stop the medication.
A year and a half after her "epilepsy surgery" she went to surgery for successful removal of the SEGA. Her seizures returned, although less severe than before, so she underwent a third procedure to implant a vagus nerve stimulator when another presurgical evaluation failed to determine another cortical tuber for resection. While not completely seizure free, she is independent, talks about many things, and attends school.
Lucy's case represents what can be done for patients with complex problems, like those with TSC, using multi-disciplinary medical teams at a major medical center. Lucy's care involved UCLA's TSC center of excellence, and included a pediatric neurologist (Joyce Wu, MD) and pediatric oncologist (Joseph Lasky, MD) along with a host of others to diagnosis and treat her condition.
Neurosurgeon: Gary W. Mathern, MD