MRI based composite parameter of multiple tissue types for improved patient-level hemispheric and regional level lateralization in pediatric epilepsy.

Although voxel-based morphometry (VBM) of gray matter (GM), white matter (WM) and cerebrospinal fluid (CSF) changes aid in epileptic seizure lateralization, type of T1 pulse sequence, preprocessing steps and tissue segmentation methods lead to variation in tissue classification. Here, we test the prediction accuracy of individual MRI based tissue types and a novel composite ratio parameter [(GM + WM)/CSF], sensitive to parenchymal changes and independent of tissue classification variations. Pediatric patients with partial seizures (both simple and complex), but normal and lesion-free MRI were considered (33 patients; unilateral EEG; 17 female / 16 male; age mean ± SD = 11.5 ± 5 years). MRI based seizure lateralization was performed for each patient and verified with EEG findings alone or in combination with seizure semiology. T1 weighted MRI from patients and normal control subjects was spatially transformed to the Talairach atlas and automatically segmented into GM, WM and CSF tissue types. 41 age matched normal controls (11 female / 30 male; age mean ± SD = 14.6 ± 3 years) served as the null distribution to test tissue type deviations across each epilepsy patient. When verified with the patient EEG prediction, WM, GM and CSF had a hemispheric match of 76%, 70% and 55% respectively, while the composite ratio [(GM + WM)/CSF)] showed the highest accuracy of 85%. When EEG findings and seizure semiology were combined, MRI predictions using the composite ratio improved further to 88%. To further localize the epileptic focus, regional level (frontal, temporal, parietal and occipital) MRI predictions were obtained. The composite ratio performed at 88-91% accuracy, revealing regional MRI changes, not predictable with EEG. The results show inconsistent changes in GM and WM in majority of the pediatric epilepsy patients and demonstrate the applicability of the composite ratio [(GM + WM)/CSF)] as a superior predictor, independent of tissue classification variations. Clinical EEG findings combined with seizure semiology, can overcome scalp EEG's limitations and lean towards the MRI lateralization in specific cases.

Deciphering the surgical treatment gap for drug-resistant epilepsy (DRE): A literature review.

Patients with drug-resistant epilepsy (DRE) rarely achieve seizure freedom with medical therapy alone. Despite being safe and effective for select patients with DRE, epilepsy surgery remains heavily underutilized. Multiple studies have indicated that the overall rates of surgery in patients with DRE have stagnated in recent years and may be decreasing, even when hospitalizations for epilepsy-related problems are on the rise. Ultimately, many patients with DRE who might otherwise benefit from surgery continue to have intractable seizures, lacking access to the full spectrum of available treatment options. In this article, we review the various factors accounting for the persistent underutilization of epilepsy surgery and uncover several key themes, including the persistent knowledge gap among physicians in identifying potential surgical candidates, lack of coordinated patient care, patient misconceptions of surgery, and socioeconomic disparities impeding access to care. Moreover, factors such as the cost and complexity of the preoperative evaluation, a lack of federal resource allocation for the research of surgical therapies for epilepsy, and difficulties recruiting patients to clinical trials all contribute to this multifaceted dilemma.

Early-onset epileptic encephalopathy with myoclonic seizures related to 9q33.3-q34.11 deletion involving STXBP1 and SPTAN1 genes.

We describe a 10-month-old boy with early-onset epileptic encephalopathy who was found to have a hemizygous deletion in 9q33.3-q34.11 involving STXBP1 and SPTAN1 genes. He presented at the age of 2.5 months with frequent upper extremity myoclonus, hypotonia, and facial dysmorphisms. Interictal EEG showed multifocal polyspike and wave during wakefulness and sleep. Ictal EEG revealed low-amplitude generalized sharp slow activity, followed by diffuse attenuation. Metabolic testing was unrevealing. Brain MRI showed thinning of the corpus callosum with an absence of rostrum. This patient is the second reported case with 9q33.3-q34.11 deletion involving STXBP1 and SPTAN1 genes associated with epileptic encephalopathy and myoclonic seizures. Larger case series are needed to better delineate this association.

Vagal nerve stimulation for medically refractory epilepsy in Angelman syndrome: a series of three cases.

BACKGROUND: We describe three children with Angelman syndrome and medically refractory epilepsy. METHODS: Case series of three pediatric patients with Angelman syndrome and medically refractory epilepsy. All three patients failed medical treatment and were recommended for vagal nerve stimulator (VNS) implantation. RESULTS: Following VNS implantation, all three patients experienced reduction in seizure frequency greater than that afforded by medication alone. CONCLUSION: We present vagal nerve stimulator implantation as a viable treatment option for medically refractory epilepsy associated with Angelman syndrome.

Frameless stereotactic placement of depth electrodes in epilepsy surgery.

OBJECT: Depth electrodes are useful in the identification of deep epileptogenic foci. Computerized tomography-magnetic resonance (CT/MR)- and angiography-guided frame-based techniques are safe and accurate but require four-point skull fixation that limits cranial access for the placement of additional grids and strips. The authors investigated the viability and accuracy of placing depth electrodes by using a commercially available frameless system. METHODS: A slotted, custom-designed adapter was built to interface with the StealthStation Guide Frame-DT and 960-525 StealthFighter. The Cranial Navigation software was used to plan the trajectory and entry site based on preoperative spoiled gradient MR imaging studies. Forty-one depth electrodes were placed in 51 targets in 20 patients. Thirty-one of these electrodes were inserted through the temporal neocortex following craniotomy and placement of subdural grids, whereas 10 were placed through burr holes. All electrodes had contact either within (71%) or touching (29%) the target, 50 of which (98%) provided adequate recordings. Although the mean distance of the distal electrode contact from the intended target was 3.1 +/- 0.5 mm, the mean distance to the edge of the anatomical structure was 0.4 +/- 0.9 mm. Placement via the laterotemporal approach was significantly (p < 0.001) more accurate than that via the occipitotemporal approach. No complication occurred. CONCLUSIONS: Depth electrodes can be placed safely and accurately by using a commercially available frameless stereotactic navigation system and a custom-made adapter. Depth electrode placement to record ictal onsets during epilepsy surgery only requires the contacts to touch rather than to reside within the intended structure. The laterotemporal approach is a more accurate method of placing electrodes than is the occipitotemporal one, likely due to the increased distance from the entry point to the target.

Standardization of amygdalohippocampectomy with intraoperative magnetic resonance imaging: preliminary experience.

PURPOSE: Intraoperative magnetic resonance imaging (IMRI) is an extremely useful neurosurgical tool in surgeries in which the extent of resection is known to have a significant impact on outcome. Residual hippocampus is the most common cause of recurrent seizures after temporal lobectomy for medial temporal lobe epilepsy. Although the risk/benefit ratio of a policy of universal radical hippocampal resection is not known, we hypothesized that IMRI would aid in the intraoperative assessment of the extent of hippocampal resection and assist in accomplishing a complete hippocampectomy. METHODS: Five consecutive patients with medically intractable medial temporal lobe epilepsy underwent a radical amygdalohippocampectomy as part of the their surgery for epilepsy. IMRI was used before surgery and after an initial resection. The quality of images was assessed. Postoperative MR images were evaluated by a radiologist to determine the extent of resection of the amygdala, hippocampus, and parahippocampal gyrus. RESULTS: There were no perioperative infections. After a mean follow-up of 10 months, all patients are seizure free. T(1)-weighted coronal intraoperative images were judged adequate at visualizing the medial structures in all patients. T(2) and fluid-attenuated inversion recovery (FLAIR) images did not provide useful information. Postoperative MR images indicated that a complete hippocampectomy had been achieved in all patients. CONCLUSIONS: IMRI is a useful adjunct in the surgical treatment of medial temporal lobe epilepsy and perhaps the most reliable method of standardizing a complete hippocampectomy. T(1)-weighted coronal images are the most helpful sequence.