The neurosurgical treatment of skull base temporal encephalocele for patients with epilepsy is variable. We describe two adult cases of temporal lobe epilepsy (TLE) with spheno-temporal encephalocele, currently seizure-free for more than two years after anterior temporal lobectomy (ATL) and lesionectomy sparing the hippocampus without long-term intracranial electroencephalogram (EEG) monitoring. Encephaloceles were detected by magnetic resonance imaging (MRI) and confirmed by maxillofacial head computed tomography (CT) scans. Seizures were captured by scalp video-EEG recording. One case underwent intraoperative electrocorticography (ECoG) with pathology demonstrating neuronal heterotopia. We propose that in some patients with skull base temporal encephaloceles, minimal surgical resection of herniated and adjacent temporal cortex (lesionectomy) is sufficient to render seizure freedom. In future cases, where an associated malformation of cortical development is suspected, newer techniques such as minimally invasive EEG monitoring with stereotactic-depth EEG electrodes should be considered to tailor the surrounding margins of the resected epileptogenic zone.

Purpose: Parry-Romberg syndrome (PRS) is a rare disorder characterized by slowly progressive hemifacial atrophy. Although the underlying etiology is unknown, proposed disease mechanisms which include autoimmune, infection, trauma, and other causes have been proposed as underlying disease mechanisms. Approximately, 10–20% of PRS patients have neurologic manifestations such as epilepsy, headaches, or associated vascular malformations. There are reports of PRS responsive to immunosuppressive medications, supporting the autoimmune hypothesis. Currently, the neuropathologic findings in patients with PRS are not well documented. Methods: Herein, we present a case of a 19-year-old female with PRS, who underwent partial frontal lobectomy for intractable epilepsy. Results: The resection specimen showed multifocal active lymphocytic (T-cell mediated) arteritis in midsized cortical arterioles, with the adjacent meninges showing fibrosis involving both the meningeal tissue and its vascular network. In addition, neuronal loss and gliosis were evident in the cortex, likely due to the associated vascular injury. Conclusions: This report is the first to demonstrate active cerebral vasculitis in a patient with PRS, supporting the previous suspicion of inflammatory etiology in this disease. In addition, the widespread vascular fibrosis in the meningeal vessels and an area of cortical ischemia support the presence of previous inflammatory vascular processes in the area.