Proper Therapy Selection Improves Epilepsy Outcomes in Patients With Multilobar Sturge-Weber Syndrome.

BACKGROUND: Hemispherectomy is an optimal treatment for patients with Sturge-Weber syndrome (SWS) affecting the whole hemisphere; however, a consensus has not been reached regarding therapeutic choices for those with involvement of two to three lobes. In this study, we compared seizure and cognitive outcomes between medical and surgical treatment groups in patients with multilobar involvement. METHODS: We evaluated 50 patients with multilobar involvement. Surgical indications included (1) antiepileptic drug (AED)-resistant seizures; (2) developmental delay; and (3) cortical atrophy. Twenty-nine patients were classified in the medical treatment group (MTG), and 21 patients were in the surgical treatment group (STG). Seizure type and frequency, SWS electroencephalography score (SWS-EEGS), and pretherapeutic and posttherapeutic SWS neurological scores (SWS-NS) were compared between groups. Median ages at the initial evaluation of the MTG and STG were 4 and 2 years, and at the final evaluation were 13 and 17 years, respectively. RESULTS: The STG had a higher incidence (76.2%) of focal to bilateral tonic-clonic seizures and status epilepticus, although no difference in SWS-EEGS. Seizure and cognitive subcategories of SWS-NS at initial evaluation were worse in the STG (P = 0.025 and P = 0.007). The seizure subcategory in MTG and STG improved after therapy (P = 0.002 and P = 0.001). Cognition was maintained in MTG and improved in STG (P = 0.002). The seizure-free rates in MTG and STG were 58.6% and 85.7%, respectively. CONCLUSIONS: Appropriate therapeutic choices improved seizure outcomes. Although patients who required surgery had more severe epilepsy and cognitive impairment, surgery improved both.

Epilepsy surgery without lipoma removal for temporal lobe epilepsy associated with lipoma in the Sylvian fissure.

Epileptic seizure is the common symptom associated with lipomas in the Sylvian fissure (Sylvian lipomas). Removal of these lipomas carries risks of hemorrhage and brain damage. We report a surgical strategy of not removing the lipoma in a case of intractable temporal lobe epilepsy associated with Sylvian lipoma. We performed anterior temporal lobectomy with preservation of the pia mater of the Sylvian fissure and achieved seizure freedom. Focal cortical dysplasia type 1 of the epileptic neocortex adjacent to the Sylvian lipoma was pathologically diagnosed. We recommend our surgical procedure in similar cases to avoid complications and achieve adequate seizure control.

Can intraoperative electrocorticography be used to minimize the extent of resection in patients with temporal lobe epilepsy associated with hippocampal sclerosis?

OBJECTIVE: Tailored surgery to extensively resect epileptogenic lesions using intraoperative electrocorticography (ioECoG) may improve seizure outcomes. However, resection of large areas is associated with decreased memory function postoperatively. The authors assessed whether ioECoG could provide useful information on how to minimize the focus resection and obtain better seizure outcomes without memory deterioration. They examined the postoperative seizure-free period and memory alteration in a retrospective cohort of patients with mesial temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) in whom the extent of removal was determined using ioECoG findings. METHODS: The authors enrolled 82 patients with TLE associated with HS who were treated surgically. Transsylvian amygdalohippocampectomy was indicated as the first step. When visual inspection identified interictal epileptic discharges from the lateral temporal lobe on ioECoG, anterior temporal lobectomy (ATL) was eventually performed. The patients were divided into the selective amygdalohippocampectomy (SA, n = 40) and ATL (n = 42) groups. Postoperative seizure outcomes were assessed at 1, 2, 3, 5, and 7 years postoperatively using the International League Against Epilepsy classification. The Kaplan-Meier survival analysis was applied to evaluate the period of seizure recurrence between the SA and ATL groups. Factors attributed to seizure recurrence were analyzed using the Cox proportional hazards model, and they were as follows: epileptic focal laterality; age at seizure onset (< 10 or ≥ 10 years old); seizure frequency (more than weekly or less than weekly seizures); history of focal to bilateral tonic-clonic seizure; infectious etiology; and surgical procedure. The Wechsler Memory Scale-Revised was used to evaluate memory function pre- and postoperatively. RESULTS: Seizure outcomes were significantly worse in the SA group than in the ATL group at 2 years postoperatively (p = 0.045). The International League Against Epilepsy class 1 outcomes at 7 years postoperatively in the SA and ATL groups were 63% and 81%, respectively. Kaplan-Meier analysis showed that seizure recurred significantly earlier in the SA group than in the ATL group (p = 0.031). The 2-way ANOVA analysis was used to compare the SA and ATL groups in each memory category, and revealed that there was no significant difference regardless of the side of surgery. CONCLUSIONS: Visual assessment of ioECoG cannot be used as an indicator to minimize epileptic focus resection in patients with TLE associated with HS. ATL is more effective in obtaining seizure-free outcomes; however, both ATL and SA can preserve memory function.

Clinicopathological Significance of Autophagy-related Proteins and its Association With Genetic Alterations in Gliomas.

AIM: To investigate clinicopathological significance of autophagy and its association with genetic alterations in gliomas. MATERIALS AND METHODS: The expression of three autophagy-related proteins, light chain-3 (LC3), beclin 1, and p62 was immunohistochemically analyzed in 32 low-grade gliomas and 65 high-grade gliomas. RESULTS: LC3, beclin 1, and p62 expression was positive in 70/94 (74%), 51/94 (54%) and 55/96 (57%) gliomas, respectively. High expression of LC3, beclin 1 and p62 was significantly more frequent in high-grade gliomas than in low-grade. Positive expression of LC3, beclin 1 and p62 were significantly positively correlated with overall survival, methylation of O6-methylyguanine-DNA methyltransferase (MGMT) promoter, mutations of isocitrate dehydrogenase 1 (IDH1) and telomerase reverse transcriptase (TERT) promoter, and 1p/19q co-deletion. Kaplan-Meier analyses revealed that LC3, p62 and autophagy status (positivity for at least two of the three proteins) were significantly associated with poorer survival. CONCLUSION: Autophagy might be associated with the progression of glioma, particularly high-grade, and thus might be a useful prognostic factor in patients with glioma.

Oligodendrocytes Up-regulate the Invasive Activity of Glioblastoma Cells via the Angiopoietin-2 Signaling Pathway.

BACKGROUND/AIM: Glioblastoma (GBM) is one of the most lethal solid cancers due to its highly invasive nature. The malignant potential of GBM cells might be partially regulated by surrounding normal cells, such as oligodendrocytes or fibroblasts. The aim of this study was to examine the interaction between stromal cells and GBM cells. MATERIALS AND METHODS: Two GBM cell lines were used. The effect of stromal cells, oligodendrocytes or fibroblasts, on the invasive ability of GBM cells was examined by wound-healing assay and invasion assay. RESULTS: Oligodendrocytes, in contrast to fibroblasts, significantly increased the migration and invasive ability of GBM cells. Angiopoietin-2 levels were high in the conditioned medium obtained from oligodendrocytes. Angiopoietin-2 significantly increased the motility of GBM, and the motility-stimulating activity of the oligodendrocytes-derived conditioned medium was significantly decreased by anti-angiopoietin-2-neutralizing antibody. CONCLUSION: Glioma stromal cells, oligodendrocytes, might up-regulate the invasiveness of GBM cells via angiopoietin-2 signaling.

Safety and accuracy of spinal instrumentation surgery in a hybrid operating room with an intraoperative cone-beam computed tomography.

Although spinal instrumentation technique has undergone revolutionary progress over the past few decades, it may still carry significant surgery-related risks. The purpose of the present study was to assess the radiological accuracy of spinal screw instrumentation using a hybrid operating room (OR) and quantify the related radiation exposure. This retrospective study included 33 cases of complex spine fusion surgeries that were conducted using a hybrid OR with a flat panel detector (FPD) angiography system. Twelve cases (36.4%) were cervical, and 21 (63.6%) were thoracolumbar. The average number of spine fusion levels was 3 and 4.8, respectively, at the cervical and thoracolumbar spine levels. A FPD angiography system was used for intraoperative cone-beam computed tomography (CBCT) to obtain multi-slice spine images. All operations were conducted under optimized radiation shielding. Entrance surface doses (ESDs) and exposure times were recorded in all cases. A total of 313 screws were placed. Satisfactory screw insertion could be achieved in all cases with safe screw placement in 97.4% and acceptable placement in 2.6%. None of the cases showed any significant anatomical violation by the screws. The radiation exposure to the patients was absolutely consistent with the desired ESD value, and that to the surgeons, under the annual dose limit. These results suggest that the hybrid OR with a FPD angiography system is helpful to achieve safe and precise spinal fusion surgery, especially in complex cases.

Aggressive surgery based on an anatomical subclassification of craniopharyngiomas.

OBJECTIVE Craniopharyngiomas remain a particularly formidable challenge in the neurosurgical field. Because these lesions involve the hypothalamus and ophthalmological systems, their resection is associated with either higher rates of mortality and recurrence or a lower rate of radical resection. The authors report the outcomes of aggressive surgeries based on an anatomical subclassification of craniopharyngiomas. METHODS Clinical and ophthalmological examinations, imaging studies, endocrinological studies, neuropsychological function, and surgical complications in all patients who had undergone microsurgical resection for craniopharyngioma at Osaka City University hospital between January 2000 and December 2014 were retrospectively reviewed through the medical records. Radical resections were planned in all of the patients. To help choose the correct surgical approach, craniopharyngiomas were classified based on tumor origin. The 4 possible groups included the intrasellar type, prechiasmatic type, retrochiasmatic type, and intra-third ventricle type. A multistage surgery was planned in some cases. RESULTS Seventy-two cases of craniopharyngioma were resected. Thirty-two patients (44.4%) had undergone previous surgical procedures at other institutions. Thirty-five cases (48.6%) were classified as retrochiasmatic, 19 (26.4%) as prechiasmatic, 12 (16.7%) as intra-third ventricle, and 6 (8.3%) as intrasellar. In 26 cases (36.1%), multistage surgery was required to complete the radical resection. Overall, 41 cases involved an orbitozygomatic approach; 21, a transpetrosal approach; 21, an interhemispheric approach; and 14, a transsphenoidal approach. In 3 cases, other approaches were applied. Gross-total resection was achieved in 43 patients (59.7%), near-total resection in 28 (38.9%), and partial resection in only 1 patient (1.4%). The mean follow-up period after resection was 4.7 years. Tumor recurrence or regrowth occurred in 15 (20.8%) of the 72 patients, with 14 of the 15 cases successfully controlled after additional resections and stereotactic radiosurgery. However, 1 patient died of uncontrollable tumor progression, and 2 patients died of unrelated diseases during the follow-up. Overall, disease in 69 (95.8%) of 72 patients was well controlled at the last follow-up. CONCLUSIONS Aggressive tumor resection is the authors' treatment policy for craniopharyngioma. Using an anatomical subclassification of craniopharyngioma to choose the most appropriate surgical approach is helpful in achieving that goal of aggressive resection.

Anatomic Understanding of Vertical Hemispherotomy With Cadaveric Brains and Intraoperative Photographs.

BACKGROUND: Vertical hemispherotomy is performed in hemispheric epilepsy to disconnect commissural fibers, projecting fibers, and limbic system from the affected side of the brain with minimal parenchyma removal. However, anatomic understanding of this surgery is generally difficult. OBJECTIVE: To present the vertical hemispherotomy procedures using cadaveric brains and intraoperative photographs. METHODS: Two formalin-fixed adult cadaveric brains were used to demonstrate vertical hemispherotomy. Intraoperative photographs were taken of a 19-year-old man with intractable epilepsy due to head trauma in infancy. RESULTS: After coronal skin incision along the coronal suture, bifrontal craniotomy and a C-shaped dural incision from lateral to medial to the midline are performed. The interhemispheric fissure is dissected from anterior to posterior. Interhemispheric total corpus callosotomy is performed to disconnect commissural fibers. Corticotomy on the cingulate gyrus is performed to approach the lateral ventricle. The lateral border of the thalamus is cut from posterior to anterior until exposing the inferior horn of the lateral ventricle and hippocampal head to disconnect projecting fibers. At the anteromedial side of the hippocampus, the inferior part of the amygdala and uncal gyrus is removed, exposing the basal cistern to disconnect the hippocampus and amygdala. The posterior column of the fornix at the trigone of the lateral ventricle is resected to disconnect the limbic system. Projecting fibers from the anterior frontal lobe are disconnected. CONCLUSION: A step-by-step procedure using cadaveric brains and intraoperative photographs provide a better anatomic understanding of vertical hemispherotomy.

An ischemic stroke patient with free floating thrombus in carotid artery, successfully treated by open carotid thrombectomy: a first case report in Nepal.

Free floating thrombus in the carotid artery is a well-known phenomenon, though relatively rare. We present a case in which we performed open surgery and achieved successful retrieval of the thrombus. A 40 year-old male patient presented with ischemic stroke and mild left hemiparesis. Computerized tomography and magnetic resonance imaging showed infarction in the right parieto-occipital area. Carotid Doppler study showed carotid stenosis on the right side. Further investigation with CT angiography of the neck vessels confirmed significant carotid artery occlusion with a free-floating thrombus in the internal carotid artery. Carotid endarterectomy was planned under EEG monitoring. The right carotid artery was exposed with a vertical incision along the medial margin of the sternocleido-mastoid muscle. The carotid artery was opened and, as expected, showed a soft, mobile thrombus. Thus thrombectomy was planned. A 2 Fr fogarty catheter was introduced distal to the thrombus, the balloon was inflated and pulled back gently, which removed the thrombus completely. There was no postoperative complication and the patient is fine at 1 year follow-up.