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Background and Importance: Surgery of jugular foramen tumors (JFTs) often requires vascular control by means of ligating the internal jugular vein and sigmoid sinus (SS) to allow intrabulbar access. Occlusion of the SS traditionally involves presigmoid and retrosigmoid durotomies allowing introduction of ligature devices, predisposing to cerebrospinal fluid (CSF) leakage and pseudomeningoceles. We describe a simple and novel endoluminal sigmoid sinus occlusion (ESSO) technique with Gelfoam that is entirely extradural., Clinical Presentation: An extended anterolateral infralabyrinthine approach with ESSO was performed in 33 patients with JFTs. After ligating the internal jugular vein, the SS is opened and Gelfoam is placed endoluminally into the proximal SS. Care is taken to avoid occlusion of the venous outflow of the vein of Labbe to avoid temporal lobe venous infarction. Hemostatic gelatin matrix is injected distally to stop venous backflow from the inferior petrosal sinus. The jugular venous system is isolated, and the outer jugular wall can be opened to expose the JFT for resection. There were no complications of temporal lobe venous infarction or postoperative hematoma observed. Four patients with intradural tumor extension developed pseudomeningoceles. For patients with purely extradural JFTs, none developed postoperative incisional CSF leaks and one had pseudomeningocele., Conclusion: This ESSO technique is fast and effective, permitting occlusion of the SS during JFT surgery. It has the advantage of being entirely extradural, avoiding durotomy which can result in postoperative CSF leak. It is important to keep the Gelfoam distal to the transverse-sigmoid junction to avoid occlusion of the vein of Labbe inlet and temporal lobe venous infarction., (Copyright © Congress of Neurological Surgeons 2024. All rights reserved.)

Dejerine-Sottas syndrome (DSS) is the earlier onset, more severe form of Charcot-Marie-Tooth (CMT) disease with heterogenous neurologic manifestations in addition to the peripheral neuropathy depending not only on the underlying causative gene but also the specific mutation. The Trembler mutation is an uncommon missense mutation in the PMP22 gene, the most commonly mutated gene responsible for CMT. We report two cases of DSS in a mother and son with the Trembler mutation, with associated findings of hearing loss and cognitive impairment. The mother had developmental gait abnormalities and became wheelchair bound in adolescence. She displayed impairment on cognitive and audiologic testing. Her son had similar developmental gait abnormalities and became wheelchair bound at age 19. Cognitive function showed an earlier decline in the son as compared to his mother. This report extends the clinical spectrum of the Trembler mutation in humans to include associated hearing loss with cognitive impairment., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Glucagon-like peptide-1 (GLP-1) is a gut-derived peptide that has various physiological actions. One of its main actions is the regulation of blood glucose level when it is elevated as it potentiates insulin release. It is also known that GLP-1 protects neurons from damage caused by neurodegenerative diseases. Lixisenatide is one of the GLP-1 analogues that has a strong affinity to the GLP-1 receptor. Experimental animal studies have shown that it holds a neuroprotective effect in Parkinson, myocardial, and cerebral ischemic disease animal models. The beneficial effect of lixisenatide on the brain after cerebral ischemia-reperfusion (I/R) is not clarified yet; thus, it needs further explanatory studies. Our research is the first to study the effect of lixisenatide on myeloperoxidase (MPO) and toll-like receptors (TLRs)/mitogen-activated protein kinase (MAPK) pathway in a rat model of cerebral I/R. Lixisenatide with 2 doses 0.7 and 7 nmol/kg was given intraperitoneal in 2 different groups for 14 days; then, the bilateral common carotid artery was occluded for 1 h followed by reperfusion for 1 h. Examination of hippocampus CA1 neurons by Nissl stain showed that the number of intact neurons was elevated in the lixisenatide-treated group related to the control group (I/R group). Lixisenatide exhibited neuroprotection action possibly via downregulation of MPO, TLR2/4, nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB), and pP38 and upregulation of phosphorylated extracellular signal-regulated kinase (pERK1/2); thus, this study gives possible link between lixisenatide and TLR/MAPK pathway following cerebral I/R and supports the use of lixisenatide for neuroprotection against stroke.