Authors :
Presenting Author: Vishal Shah, MD – KUMC
Joseph Newman, APRN – KUMC; Daniel Mafla, MD – KUMC; maryam Matloub, MD – KUMC; Ryan Lay, MD – KUMC; Nancy Hammond, MD – KUMC; Murtaza Khan, MD – KUMC; Carol Ulloa, MD – KUMC; Jennifer Cheng, MD – KUMC; Michael Abraham, MD – KUMC; Utku Uysal, MD – KUMC; Patrick Landazuri, MD – KUMC
Rationale: Temporal lobe encephaloceles are recognized as an etiology of drug resistant epilepsy (DRE). Neuroimaging findings associated with idiopathic intracranial hypertension (IIH) and cerebral venous sinus stenosis (CVSS)
are known to be associated with cerebral encephaloceles. Management of CVSS and its effect on seizure control is unknown. We aim to describe the clinical profile, radiological findings, and neuro-interventional approaches and outcomes for patients with CVSS, encephaloceles and outcomes on seizure control.
Methods: We retrospectively reviewed an existing database of patients at the University of Kansas Comprehensive Epilepsy Center with DRE and encephaloceles. Pre-surgical clinical profile, IIH symptoms, cerebral venous sinus stenosis, and treatment outcomes were analyzed. As part of IIH evaluation, patients underwent further testing including ophthalmic evaluation, lumbar puncture, CTV/MRV, and venogram studies. Patients with significant CVSS were offered venous stenting. We assessed seizure outcomes after CVSS venous stenting.
Results: Twenty seven (43%) of 64 patients undergoing evaluations for DRE with identified concomitant encephalocele(s) underwent further work up. No patient had papilledema on the exam. Six patients had elevated opening pressures during between 22 and 46 cm H
2O. Seven patients underwent non-invasive venography and 15 patients underwent conventional venogram study. Nine patients underwent CVSS stenting due to elevated mean pressure gradient (MPG) of > 8 mmHg across the stenotic region. All patients had MPG normalization after stenting. Two additional patients are scheduled for stent placement. Seven of nine patients reported improvement in seizure frequency immediately after venous stent placement with two patients reporting seizure freedom > 1 year. Two patients report seizure freedom > 4 months. Two patients have undergone temporal lobe resection for seizure recurrence > 6 months later. Two patients had gradual return to baseline seizure frequency following initial improvement. One patient saw no improvement after CVSS stenting.
Conclusions: Endovascular stenting was performed to prevent further complications of IIH / CVSS including the development of more encephaloceles and to assess hypothesis of resultant improvement in seizure frequency. Most patients undergoing endovascular intervention reported improvement of seizure frequency. Patients with continued seizure freedom have continued anti-seizure medications. We present these data as a potential treatment strategy, particularly for patients who may be reticent to consider epilepsy surgery. As our cohort is small and this is a newly described entity, approach, and treatment, we present this data to introduce this concept and invite collaboration to better understand this disease process.
Funding: None