Management of multiple cervical neurofibromas with myelopathy in neurofibromatosis type 1: A systematic review, case report and technical note

Objective: Neurofibromatosis type 1 (NF1) is a common disorder in which affected individuals uncommonly develop cervical neurofibromas. The presentation of cervical neurofibroma with myelopathy is clinically challenging. Available data of NF1 patients with cervical cord compression secondary to multiple neurofibromas remain scarce in the literature. To this end, we sought to address this limitation. Methods: Case presentation: We report a case of a 22-year-old man, recently diagnosed with NF1, who presented with progressive cervical myelopathy over the course of 12 months. Imaging revealed multiple cervical neurofibromas with significant spinal cord compression. The patient underwent a C3 to C7 decompressive laminectomy and subtotal resection of the bilateral neurofibromas and instrumented fusion. During the postoperative period, he experienced transient bilateral weakness in C5 and C6 muscle groups that gradually resolved, and his weakness and spasticity significantly improved thereafter.Systematic review: We performed a systematic review of PubMed and Scopus in English-language literature dated between 1960 and December 2019 for studies that included cervical neurofibromas presenting with myelopathy in patients with NF1. Results: Fifty-seven articles were identified for full-text examination, of which 19 articles were included in the systematic review; 10 involved studies on surgical treatment, and nine on other treatment modalities. Twelve studies were retrospective, 3 involved prospective cohorts, and 4 were case reports. Most studies included various types of spinal cord tumors with or without neurofibromatosis. Only two studies exclusively involved neurofibromas in NF patients. There was wide variation in surgical and radiation therapy techniques and outcome measures reported. Conclusion: Surgical decompression is the primary treatment strategy for multiple cervical neurofibromas that cause a progressive neurological deficit. Fusion is recommended to avoid late kyphotic deformity. Data describing the management plan and long-term outcomes in this group of patients remain scarce in the literature, and no standardized treatment strategy is available

Indications, technique, and safety profile of insular stereoelectroencephalography electrode implantation in medically intractable epilepsy

OBJECTIVEInsular epilepsy is relatively rare; however, exploring the insular cortex when preoperative workup raises the suspicion of insular epilepsy is of paramount importance for accurate localization of the epileptogenic zone and achievement of seizure freedom. The authors review their clinical experience with stereoelectroencephalography (SEEG) electrode implantation in patients with medically intractable epilepsy and suspected insular involvement.METHODSA total of 198 consecutive cases in which patients underwent SEEG implantation with a total of 1556 electrodes between June 2009 and April 2013 were reviewed. The authors identified patients with suspected insular involvement based on seizure semiology, scalp EEG data, and preoperative imaging (MRI, PET, and SPECT or magnetoencephalography [MEG]). Patients with at least 1 insular electrode based on the postoperative 3D reconstruction of CT fused with the preoperative MRI were included.RESULTSOne hundred thirty-five patients with suspected insular epilepsy underwent insular implantation of a total of 303 electrodes (1–6 insular electrodes per patient) with a total of 562 contacts. Two hundred sixty-eight electrodes (88.5%) were implanted orthogonally through the frontoparietal or temporal operculum (420 contacts). Thirty-five electrodes (11.5%) were implanted by means of an oblique trajectory either through a frontal or a parietal entry point (142 contacts). Nineteen patients (14.07%) had insular electrodes placed bilaterally. Twenty-three patients (17.04% of the insular implantation group and 11.6% of the whole SEEG cohort) were confirmed by SEEG to have ictal onset zones in the insula. None of the patients experienced any intracerebral hemorrhage related to the insular electrodes. After insular resection, 5 patients (33.3%) had Engel Class I outcomes, 6 patients (40%) had Engel Class II, 3 patients (20%) had Engel Class III, and 1 patient (6.66%) had Engel Class IV.CONCLUSIONSInsula exploration with stereotactically placed depth electrodes is a safe technique. Orthogonal electrodes are implanted when the hypothesis suggests opercular involvement; however, oblique electrodes allow a higher insular sampling rate.</jats:sec

Outcome of Anterior and Posterior Endoscopic Procedures for Cervical Radiculopathy Due to Degenerative Disk Disease: A Systematic Review and Meta-Analysis

Study design: Systematic review and meta-analysis. Objectives: Cervical spine endoscopic discectomy and decompression have gained popularity in the last decade. This review aimed to shed light on the current outcomes of cervical spine endoscopic procedures for degenerative disc disease (DDD) and to calculate a pooled estimate of various outcome measures. Methods: We retrieved articles published in English related to endoscopic cervical spine procedures from 3 central databases from inception until September 2020. A subgroup analysis based on the anterior versus the posterior approach was performed. Results: Thirty-one articles fulfilled the eligibility criteria and included 1,410 patients. A successful outcome was observed in 91.3% (88.6-93.4%, P = 0.000). This percentage was lower for the anterior approach (89.6% [85.8-92.5%], P = 0.000) than for the posterior approach (94.2% [90.4-96.5%], P = 0.000). A higher percentage of poor outcomes was reported for the anterior approach (5.7% [3.2-10.1%], P = 0.000 vs. 2.3% [1-5.5%], P = 0.000 for the posterior approach). The overall complication rate was 7.2% (5.2-9.8%, P = 0.000). There was a slightly higher complication rate for the anterior approach (7.9% [4.5-13.3%], P = 0.000) than for the posterior approach (6.7% [4.4-10%], P = 0.000). The revision rate was 4.2% (2.6-6.8%, P = 0.000); and 4.2% (1.8-9.7%, P = 0.000) for the anterior approach and 4.00% (2.2-7.4%, P = 0.000) for the posterior approach. Conclusions: There is a higher success rate and lower complication rate with the posterior approach than with the anterior approach. However, high-quality randomized controlled trials are vital to evaluate the efficacy of these procedures. </jats:sec