Successful surgery in late onset epilepsy with tuberous sclerosis complex

Area of Science:

• Neurology
• Genetics
• Neurosurgery

Background:

• Tuberous sclerosis complex (TSC) is a genetic disorder characterized by benign tumor formation, frequently associated with epilepsy.
• Epilepsy in TSC often presents as medically intractable seizures, posing significant challenges for treatment and presurgical evaluation.
• Identifying the precise epileptogenic zone is crucial for surgical planning in TSC patients with refractory epilepsy.

Observation:

• A 21-year-old woman with TSC experienced medically intractable epilepsy since age 15.
• Multiple potentially epileptogenic lesions were visible on MRI, complicating presurgical evaluation.
• A multi-stage evaluation including video-EEG, high-resolution MRI, SPECT, PET, and invasive recordings was performed.

Findings:

• The comprehensive evaluation successfully identified a concordant epileptogenic tuber and seizure onset zone in the right temporal lobe.
• A tailored surgical resection targeting the identified tuber was performed.
• The patient achieved excellent surgical outcomes, with seizure freedom classified as Engel class I at 12-month follow-up.

Implications:

• Multimodal presurgical evaluation is critical for defining the epileptogenic zone in TSC patients with complex epilepsy.
• Tailored surgical resection based on concordant findings can lead to successful seizure control in TSC.
• These findings highlight the importance of a multidisciplinary approach in managing intractable epilepsy associated with TSC.