Selective Amygdalohippocampectomy.

Mesial temporal lobe epilepsy is a common condition that is frequently drug resistant. Anterior temporal lobectomy has been shown to be effective in controlling seizures but entails resecting anterior and lateral temporal lobe regions that are not necessarily included in the epileptogenic zone. Selective amygdalohippocampectomy spares uninvolved structures while providing the same benefit as anterior temporal lobectomy. This article describes the 3 most common surgical approaches for performing selective amygdalohippocampectomy and discusses their relative merits and risks

Immediate serial postoperative de novo psychogenic nonepileptic seizures after selective amygdalohippocampectomy

De novo psychogenic nonepileptic seizures (PNES) following epilepsy surgery in patients without a prior history of nonepileptic seizure are well-described, but uncommon events. Recent large retrospective studies indicate a prevalence of less than 4%, and while the majority suffer a PNES event in the first year after surgery, they seldom occur within weeks of surgery. This report illustrates the case of a 57-year-old man with medically intractable localization related epilepsy secondary to mesial temporal sclerosis. Three separate inpatient video electroencephalogram (EEG) studies over the course of 10 years had not revealed PNES and had localized the epileptogenic zone to the left temporal lobe. He underwent a left subtemporal selective amygdalohippocampectomy (SAH) without complication and was maintained on his pre-operative antiseizure medications. Within 12 h of surgery, the patient began exhibiting episodes with a different semiology than the preoperatively documented seizures, increasing in frequency until the 4th postoperative day, when video-EEG revealed clear PNES devoid of electrographic correlation. More than 25 events occurred, with 11 captured on video-EEG. Interventions including reassurance of the patient and family by the epileptologist and surgeon, as well as psychiatry consultation, resulted in immediate cessation of the events. He has remained seizure-free at the time of this report, consistent with an Engel class 1 outcome. While rare, concentrated episodes of PNES activity may arise immediately after epilepsy surgery in patients without a prior history of PNES. This clinical vignette suggests that a suspicion of PNES must be taken into consideration should a patient develop a cluster of seizures immediately after epilepsy surgery

Leukoencephalopathy, calcifications, and cysts: Labrune syndrome

Labrune syndrome is an extremely rare disorder characterized by a radiological triad of leukoencephalopathy, cerebral calcifications, and cysts. The condition is the result of an autosomal mutation in the SNORD118 gene, a non-protein encoding gene that mediates rRNA synthesis. The mutation results selectively in cerebral microangiopathy through an unknown mechanism. Radiological imaging is central to diagnosing the condition, but, because the condition is so rare, there is no standard treatment paradigm. We describe the longitudinal progression of a case of Labrune syndrome, including the radiological diagnosis and imaging and surgical management

Cerebellar anaplastic ganglioglioma in a septuagenarian

Ganglioglioma is a rare neoplasm most common in children and adolescents. It is typically located in the supratentorial compartment, with the temporal lobe being the most common tumor location. Anaplastic ganglioglioma is a WHO grade III ganglioglioma, a rare subtype accounting for a small minority of ganglioglioma cases. Posterior fossa anaplastic ganglioglioma in an adult is incredibly rare; only 3 prior cases have been reported. Only 1 adult anaplastic ganglioglioma in the cerebellum has been reported. We present the second reported adult cerebellar anaplastic ganglioglioma

Analysis of Chemopredictive Assay for Targeting Cancer Stem Cells in Glioblastoma Patients

Introduction: The prognosis of glioblastoma (GBM) treated with standard-of-care maximal surgical resection and concurrent adjuvant temozolomide (TMZ)/radiotherapy remains very poor (less than 15 months). GBMs have been found to contain a small population of cancer stem cells (CSCs) that contribute to tumor propagation, maintenance, and treatment resistance. The highly invasive nature of high-grade gliomas and their inherent resistance to therapy lead to very high rates of recurrence. For these reasons, not all patients with similar diagnoses respond to the same chemotherapy, schedule, or dose. Administration of ineffective anticancer therapy is not only costly but more importantly burdens the patient with unnecessary toxicity and selects for the development of resistant cancer cell clones. We have developed a drug response assay (ChemoID) that identifies the most effective chemotherapy against CSCs and bulk of tumor cells from of a panel of potential treatments, offering great promise for individualized cancer management. Providing the treating physician with drug response information on a panel of approved drugs will aid in personalized therapy selections of the most effective chemotherapy for individual patients, thereby improving outcomes. A prospective study was conducted evaluating the use of the ChemoID drug response assay in GBM patients treated with standard of care. Methods: Forty-one GBM patients (mean age 54 years, 59% male), all eligible for a surgical biopsy, were enrolled in an Institutional Review Board–approved protocol, and fresh tissue samples were collected for drug sensitivity testing. Patients were all treated with standard-of-care TMZ plus radiation with or without maximal surgery, depending on the status of the disease. Patients were prospectively monitored for tumor response, time to recurrence, progression-free survival (PFS), and overall survival (OS). Odds ratio (OR) associations of 12-month recurrence, PFS, and OS outcomes were estimated for CSC, bulk tumor, and combined assay responses for the standard-of-care TMZ treatment; sensitivities/specificities, areas under the curve (AUCs), and risk reclassification components were examined. Results: Median follow-up was 8 months (range 3-49 months). For every 5% increase in in vitro CSC cell kill by TMZ, 12-month patient response (nonrecurrence of cancer) increased two-fold, OR = 2.2 (P = .016). Similar but somewhat less supported associations with the bulk tumor test were seen, OR = 2.75 (P = .07) for each 5% bulk tumor cell kill by TMZ. Combining CSC and bulk tumor assay results in a single model yielded a statistically supported CSC association, OR = 2.36 (P = .036), but a much attenuated remaining bulk tumor association, OR = 1.46 (P = .472). AUCs and [sensitivity/specificity] at optimal outpoints (>40% CSC cell kill and >55% bulk tumor cell kill) were AUC = 0.989 [sensitivity = 100/specificity = 97], 0.972 [100/89], and 0.989 [100/97] for the CSC only, bulk tumor only, and combined models, respectively. Risk categorization of patients was improved by 11% when using the CSC test in conjunction with the bulk test (risk reclassification nonevent net reclassification improvement [NRI] and overall NRI = 0.111, P = .030). Median recurrence time was 20 months for patients with a positive (>40% cell kill) CSC test versus only 3 months for those with a negative CSC test, whereas median recurrence time was 13 months versus 4 months for patients with a positive (>55% cell kill) bulk test versus negative. Similar favorable results for the CSC test were observed for PFS and OS outcomes. Panel results across 14 potential other treatments indicated that 34/41 (83%) potentially more optimal alternative therapies may have been chosen using CSC results, whereas 27/41 (66%) alternative therapies may have been chosen using bulk tumor results. Conclusions: The ChemoID CSC drug response assay has the potential to increase the accuracy of bulk tumor assays to help guide individualized chemotherapy choices. GBM cancer recurrence may occur quickly if the CSC test has a low in vitro cell kill rate even if the bulk tumor test cell kill rate is high