Effectiveness of vagus nerve stimulation for drug-resistant generalized epilepsy in children aged six and younger.

BACKGROUND: To explore a novel scoring system to evaluate the efficacy of vagus nerve stimulation (VNS) in children with drug-resistant generalized epilepsy (DRGE) aged six and younger. BASIC PROCEDURES: The data of twelve children with DRGE under the age of 6 years who accepted VNS and have been followed up for at least 3 years were retrospectively reviewed. The outcome was evaluated with the McHugh Classification System and a novel scoring system we proposed. MAIN FINDINGS: Based on the McHugh Classification System, the total response rate was 91.67% (11/12) and the rate of Grade I was 41.67% (5/12). A novel scoring system involving seizure frequency, seizure duration and quality of life (QOL) was proposed, by which the outcome was scored from -3 to 11 and graded from IV to I. Based on the novel scoring system, the total response rate was 91.67% (11/12) and the rate of Grade I was 33.33% (4/12). The incidence of complication was 16.67% (2/12). The efficacy of VNS appeared a gradually improving trend with plateau or fluctuation over time. Shorter course of epilepsy prior to VNS may be related to better outcome. PRINCIPAL CONCLUSIONS: VNS could effectively reduce the seizure frequency and improve the QOL of children with DRGE aged six and younger. The novel scoring system was comprehensive and feasible to evaluate the efficacy of VNS. The time pattern of the long-term efficacy of VNS requires further investigation.

Whole Exome Sequencing of Multiple Atypical Meningiomas in a Patient without History of Neurofibromatosis Type II: A Case Report.

BACKGROUND The pathogenesis of sporadic multiple meningiomas in the patients without history of neurofibromatosis type II remains unclear. We report whole exome sequencing (WES) of 2 metachronous multiple meningiomas of the same patient. CASE REPORT A 39-year-old female had a 5-month history of headache and her magnetic resonance imaging (MRI) revealed a significantly enhanced intracranial space-occupying pathology with dura tail sign and skull invasion. She had no history of neurofibromatosis type II or other tumors. Tumor resection achieved Simpson grade I and the pathological studies revealed an atypical meningioma. After surgery, she accepted focal external-beam radiation therapy. One year later, MRI showed a significantly enhanced intracranial space-occupying pathology near the primary site of the previous tumor. She had only a mild headache. Simpson grade I resection of the tumor was achieved. The pathological diagnosis was still an atypical meningioma. WES on both tumors identified 220 common somatic gene mutations and 43 different somatic gene mutations. Three deleterious mutated genes including QRICH2, KIF2C, and MUC16 were identified only in the first tumor, and 9 deleterious mutated genes including FCGBP, RPS6KA5, GOLGA6L2, IGHV3-66, RPTN, AGRN, USP6, CLTCL1, and PABPC3 were identified only in the second tumor. As shown by the identical result of 3 prediction tools, RPS6KA5 and AGRN were most likely to be related to the progress of multiple atypical meningiomas. CONCLUSIONS The metachronous meningiomas with same World Health Organization (WHO) grades in the same patient could have distinct genetic aberration patterns. The roles of RPS6KA5 and AGRN in the rapid progress of multiple atypical meningiomas need further studies.

[Changes of endothelial cell function and platelet activation in rabbit spinal cord with ischemia-reperfusion injury].

OBJECTIVE: To study the changes of vascular endothelial cell function and platelet activation in rabbit spinal cord following ischemia-reperfusion (I/R) injury and their roles in the spinal cord injury. METHODS: Rabbit spinal cord I/R injury models were established using Zivin method, and the changes in plasma NO and GMP140 levels were dynamically monitored after the injury. RESULTS: Plasma NO level increased significantly in the I/R group at the end of the ischemia, and reached the peak level at 2 h of reperfusion as compared to that in sham-operated group (P<0.01). Plasma NO level decreased at 6 h of reperfusion, but still significantly higher than the level in the sham-operated group (P<0.05). Plasma GMP140 underwent no significant changes in the sham-operated group, but significantly increased in the I/R group at the end of the ischemia, followed by gradual declination to the normal level at 2 h of reperfusion. CONCLUSION: Spinal cord I/R injury causes overexpressions of NO and GMP140, suggesting the involvement of endothelial cell injury and platelet overactivation in the pathological process and repair of spinal cord I/R injury.

[Visual pathway and pituitary stalk protection in pituitary tumor surgery and the clinical outcome].

OBJECTIVE: To investigate the association of microsurgical anatomy and growth of pituitary tumors with the recovery of visual pathway, and describe the intraoperative protection of the pituitary stalk and visual pathway. METHODS: A total of 113 patients undergoing pituitary tumor surgery were retrospectively analyzed, including 102 with visual disorder and 106 with pituitary dysfunction with the tumor size ranging from 1.9 to 6.8 cm. All the operations were performed via a transpterygoid approach or transfrontal approach. RESULTS: Radical resection of the tumors was performed in 86 cases, subtotal resection in 21 cases, and partial resection in 5 cases. After operation, 133 eyes showed vision improvement (77.8%), 29 showed no vision changes (17%) and 9 had deteriorated vision. Two patients died due to hypothalamic disorder and multiple organ failure. CONCLUSION: The arachnoid barrier between the pituitary tumor and visual pathway is an important structure for visual pathway protection during operation. Total separation of the tumor from the visual pathway allows total removal of the tumor. The preoperative localization and intraoperative identification of the pituitary stalk are critical for pituitary stalk protection.