Hippocampal sclerosis in association with Sturge-weber syndrome: An intertwining of 2 epileptogenic lesions

Epilepsy is a common neurological disease that to this day presents a significant neurological challenge worldwide. It has diverse etiologies with different manifestations associated with complex progressive brain alterations. Secondary epileptogenesis was a concept that emerged from the study of drug-refractory focal epilepsy subjects. It implies that repetitive seizure activity originating from a primary source could transform a previously normal cortical region into a secondary epileptogenic focus. Experimental and clinical studies have been searching for this phenomenon and its effects on neural circuitry for a long time. However, despite achieved advancements, the exact mechanisms of underlying secondary epileptogenicity remain a controversial field of study. Our case involves a 34-year-old female with a history of epilepsy, initially presenting with manageable epilepsy in infancy that gradually escalated to drug-refractory epilepsy. Magnetic resonance imaging (MRI) of the patient revealed 2 epileptogenic focuses, left-sided Sturge-Weber syndrome (SWS) and ipsilateral hippocampal sclerosis (HS). Furthermore, the term "Dual pathology" was used in the simultaneous presence of HS with other potential extra-hippocampal epileptogenic lesions and is often used by previous studies as a way of understanding secondary epileptogenicity. Thereby, this newly presented case of dual pathology will be addressed within the framework of secondary epileptogenicity in the review of current literature.