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OBJECTIVE Drug-resistant epilepsy (DRE) affects many children. Vagus nerve stimulation (VNS) may improve seizure control; however, its role in children with genetic etiologies of epilepsy is not well described. The authors systematically reviewed the literature to examine the effectiveness of VNS in this cohort. METHODS In January 2021, the authors performed a systematic review of the PubMed/MEDLINE, SCOPUS/Embase, Cochrane, and Web of Science databases to investigate the impact of VNS on seizure outcomes in children with genetic etiologies of epilepsy. Primary outcomes included seizure freedom rate, ≥ 90% seizure reduction rate, and ≥ 50% seizure reduction rate. Secondary outcomes were seizure severity and quality of life (QOL), including cognitive, functional, and behavioral outcomes. A random-effects meta-analysis was performed. RESULTS The authors identified 125 articles, of which 47 with 216 nonduplicate patients were analyzed. Common diagnoses were Dravet syndrome (DS) (92/216 patients [42.6%]) and tuberous sclerosis complex (TSC) (63/216 [29.2%]). Seizure freedom was not reported in any patient with DS; the pooled proportion (95% CI) of patients with ≥ 50% seizure reduction was 41% (21%–58%). Secondary cognitive outcomes of VNS were variable in DS patients, but these patients demonstrated benefits in seizure duration and status epilepticus. In TSC patients, the pooled (95% CI) seizure freedom rate was 40% (12%–71%), ≥ 90% seizure reduction rate was 31% (8%–56%), and ≥ 50% reduction rate was 68% (48%–91%). Regarding the secondary outcomes of VNS in TSC patients, several studies reported decreased seizure severity and improved QOL outcomes. There was limited evidence regarding the use of VNS to treat patients with other genetic etiologies of epilepsy, such as mitochondrial disease, Rett syndrome, Doose syndrome, Landau-Kleffner syndrome, Aicardi syndrome, Angelman syndrome, ring chromosome 20 syndrome, and lissencephaly; variable responses were reported in a limited number of cases. CONCLUSIONS The authors conducted a systematic review of VNS outcomes in children with genetic etiologies of DRE. Among the most studied conditions, patients with TSC had substantial seizure reduction and improvements in QOL, whereas those with DS had less robust seizure reduction. Increased testing, diagnosis, and long-term follow-up studies are necessary to better characterize VNS response in these children.

Background: Primary central nervous system lymphoma (PCNSL) is an aggressive extranodal subtype of nonHodgkin’s lymphoma. Ventricle-predominant PCNSL, arising in the CNS ventricular system, is a rare entity. In over 90% of cases, PCNSL is classified as diffuse large B-cell lymphoma. Rarely, PCNSL may be classified as marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue (MALT). Taken together, a primary MALT-type MZBCL arising in a cerebral ventricle is an extremely rare presentation. Case Description: A 69-year-old female presented with a persistent left frontal headache for 1 year. Magnetic resonance imaging revealed an enhancing soft-tissue lesion within the left lateral ventricle, with associated periventricular edema. We performed an excisional biopsy of the tumor, which grossly had the appearance of a meningioma. Histopathology of the tumor was consistent with MZBCL of the MALT type. The patient was treated with Rituximab and Ibrutinib. Six months after surgery, she remained neurologically intact and free of disease. Conclusion: We report the case of a primary MALT-type MZBCL arising in the CNS ventricular system, with characteristics mimicking meningioma. This lymphoma involved the lateral ventricle and likely originated from the choroid plexus. Meningothelial cells and epithelial cells in the choroid plexus may acquire MALT in response to chronic inflammatory stimuli, such as infection or autoimmune disease. In rare cases, MALT lymphoma may develop as part of this pathogenesis.