Triple pathology in a patient with uncontrolled epilepsy: a case report

Sturge-Weber syndrome is an uncommon neurocutaneous disorder with characteristic vascular lesions, leptomeningeal angiomas, and facial nevi. Seizureis the most prevalent neurological sign of Sturge-Weber syndrome and complications related to seizure control, comorbidities, or outcome can make the way of medical care turbid. Accordingly, the co-occurrence of Sturge-Weber syndrome with mesial temporal lobe sclerosis/mesial temporal sclerosis and low-grade glioma has not been reported in literature.

In this report, we present a 29-year-old Kurdish male with Sturge-Weber syndrome showing evidence of sclerotic changes in the medial part of the right temporal including the hippocampus in magnetic resonance imaging, in addition to a low-grade glioma. He used to have seizures, starting with left dominant somatosensory aura, before puberty. Interestingly, the semiology has changed to an epigastric aura afterward. Therefore, we assumed that there could be a new seizure onset zone. Seizures from the right mesial temporal region were recorded during long-term electroencephalogram monitoring admission. This finding was compatible with lesions found in the right temporal lobe.

Debates arise whether the co-occurrence of mesial temporal sclerosis and low-grade glioma with Sturge-Weber syndrome lesions is incidental or secondary to Sturge-Weber syndrome pathogenesis. Furthermore, this association can be attributed to a common genetic underlying. While there is no compelling evidence in this case to address the exact pathogenicity, the impact of early diagnosis and intense control of primary seizures on preventing downstream effects, such as sclerosis and tumor formations, is considerable. We strongly believe further research is needed to address this concern.