Citations

BACKGROUND: Surgical intervention remains an important option in the management of vestibular schwannoma (VSs). Development of a systematic approach to choose the most appropriate route for this intervention, based on existing published evidence, is an important goal. OBJECTIVE: To review the literature published since the 2018 Congress of Neurological Surgeons Guideline on surgical intervention for patients with sporadic VSs and use this information to update that set of recommendations. METHODS: The literature in the PubMed and MEDLINE databases from January 2015 through May 20, 2022, was searched for manuscripts pertaining to surgical intervention for VSs. Those manuscripts meeting inclusion criteria were then analyzed for creation of recommendations in response to a set of updated questions. RESULTS: The resultant findings included a considerable amount of data that did not alter the recommendations form the 2018 publication on this topic. Thus, recommendations stating hearing preservation surgery through the middle fossa or retrosigmoid approach may be considered in individuals with good preoperative hearing as an alternative to simple observation remain. In addition, if microsurgical resection is necessary after stereotactic radiosurgery, it is recommended that patients be counseled that there is an increased likelihood of a subtotal resection and decreased facial nerve function. In some questions, insufficient data were present to create an answer and that is stated. CONCLUSION: This guideline demonstrates surgical intervention for VSs and represents a range of options, and the choice of the intervention depends on the specific aspects of the lesion and the individual that harbors them. Objective refinement of those choices will require thoughtful research design by investigations that wish to address those items for which we still have insufficient information. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/7-surgical-resection-treatment-of-patients-with-ve-2

BACKGROUND: Imaging is a critical aspect of vestibular schwannoma (VS) management, influencing essentially every aspect of care including diagnosis, surveillance, treatment decision making, and follow-up after either resection or stereotactic radiosurgery. Despite this, treatment protocols are heterogeneous, and frequently based on historical practices, or low-quality evidence. OBJECTIVE: To update evidence-based guidelines for the use of imaging in the clinical management of patients with VS published by the Congress of Neurological Surgeons in 2018. METHODS: Systematic review of the literature published from 1/1/2015 to 5/20/2022 regarding imaging protocols for VS management. Salient questions were identified by a writing group of diverse individuals with topic-specific expertise. Questions were validated by the Congress of Neurological Surgeons Guidelines Committee. Following systematic review, literature tables and summary statements pertinent to the study questions were generated by the writing group, which underwent subsequent evaluation and revision by the task force before formalization. RESULTS: Seven questions were formulated; adequate literature was identified to formulate updated recommendations for 6 of these. Search strategy identified 1143 unique records, of which 109 underwent full-text review, and 57 were included in this study. Most studies provided level III evidence, with rare level II studies noted, yielding level III recommendations. CONCLUSION: The current evidence base for imaging protocols in VS clinical management is broad, diverse, low certainty, and low quality. This in part reflects a heterogeneous disease, although variability in treatment philosophies may also influence local decision making. Key areas for future study include the clinical utility of advanced imaging techniques and head-to-head comparisons of imaging protocols for patients in common initial VS management pathways (eg, observation, resection, or stereotactic radiosurgery). The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/5-role-of-imaging-in-management-of-patients-with-v

BACKGROUND: Given the increasing prevalence and shifting disease demographic of vestibular schwannoma toward smaller tumors in people with less advanced symptoms, increasing emphasis has been placed on functional hearing preservation. OBJECTIVE: To provide an update to the 2018 CNS Guideline on hearing preservation outcomes in patients with sporadic vestibular schwannoma. METHODS: Systematic review and clinical practice guideline summarizing data pertaining to hearing preservation outcomes within the first 10 years after contemporary stereotactic radiation, microsurgery, or observation with serial imaging. The analysis incorporates studies included in the 2018 guideline and additional literature published up to May 20, 2022, providing a comprehensive up-to-date review of published clinical outcome data over time. RESULTS: Pooled estimated rates of serviceable hearing preservation are presented for observation, radiosurgery, and microsurgery for adults with sporadic vestibular schwannoma who have documented serviceable hearing in the ipsilateral ear at the time of diagnosis. Overall estimated rates of maintaining serviceable hearing are 78% at 2 years, 59% at 5 years, and 47% at 10 years during observation; 71% at 2 years, 59% at 5 years, and 38% at 10 years after radiosurgery; and 48% at 2 years, 40% at 5 years, and 32% at 10 years after microsurgery. In addition, features portending hearing outcome among management modalities are reported to guide accurate patient counseling. CONCLUSION: Regardless of treatment modality, fewer than half of patients with sporadic vestibular schwannoma who present with serviceable hearing will maintain useful hearing by 10 years. Across all studies, microsurgery and radiosurgery seem to accelerate this decline over the natural history, although further research is needed given limitations of available evidence. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/4-hearing-preservation-outcomes-in-patients-with-s

BACKGROUND: Intraoperative neuromonitoring (IONM) has become vital in the management of vestibular schwannoma (VS) with the paradigm shift from tumor eradication to functional preservation. Several facial nerve (FN) monitoring strategies have been explored over the past few decades ranging from free-running electromyography, direct nerve stimulation, continuous nerve stimulation, facial motor evoked potentials, blink reflex, and others. Hearing preservation surgery is guided primarily by far-field auditory brainstem response and real-time cochlear nerve action potentials. Given the heterogeneity in tumor and patient factors, it remains very difficult to accurately predict cranial nerve outcomes, regardless of the monitoring strategy. OBJECTIVE: To critically appraise literature regarding IONM during VS surgery and update the previous evidence-based clinical practice guideline. METHODS: This is a systematic review of the literature, incorporating articles from March 2015 to May 2022. Literature published before 2015 that would have been included in the previous Congress of Neurological Surgeons guideline was not searched again in this update. RESULTS: FN monitoring provides better functional outcomes compared with anatomic dissection alone and may guide the extent of tumor resection. While facial motor evoked potentials and free-running electromyography can provide continuous noninvasive FN monitoring, there are insufficient data to determine which more strongly correlates with facial function outcomes. Both electrophysiological data and tumor size correlate with facial function outcomes. The ideal hearing monitoring strategy remains unclear as there are insufficient data comparing cochlear nerve action potentials with far-field auditory brainstem response. All studies were graded as Class III evidence. CONCLUSION: IONM should be used in all VS cases. While the optimal FN and hearing monitoring strategy remains elusive, available data support the use of a combination of strategies, including preoperative tumor size, to maximize sensitivity and specificity. There remains a significant need for high-quality comparative studies to determine which intraoperative monitoring scheme can provide intraoperative guidance and predict postoperative outcome. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/3-intraoperative-cranial-nerve-monitoring-in-manag

BACKGROUND: Stereotactic radiosurgery (SRS) is an established modality for treatment of adult patients with vestibular schwannomas (VS). The aim of this work was to provide an updated literature review on this topic. OBJECTIVE: To review the literature published since the last guideline on this topic. METHODS: OVID MEDLINE and Embase were searched for the period January 1, 2015, to May 20, 2022, using search terms and search strategies to identify pertinent abstracts. These were then screened using published exclusion/inclusion criteria to identify full-text review articles. Evidence tables were constructed using data derived from full-text reviews and recommendations made from the evidence derived. RESULTS: From the total 1035 abstracts identified, 26 full-text articles met inclusion/exclusion criteria and were included in this update. Four new level III recommendations stemmed from this work. In adult patients with sporadic intracanalicular or <2 cm VS, SRS should not be recommended as superior to observation alone for hearing preservation. In adult patients with sporadic VS treated with SRS, cochlear dose constraint should be considered because it provides better hearing preservation than no constraint. In the same population, single fraction SRS should be recommended rather than hypofractionated SRS (>1 and ≤5 fractions) because it results in decreased cranial nerve dysfunction. Finally, adult patients with sporadic VS undergoing SRS should be informed that SRS does not result in an increased number of secondary malignancies compared with the rate expected in the overall population. CONCLUSION: Recent published literature provides new recommendations for the treatment of adult patients with VS with SRS. The full guideline can be seen online at https://www.cns.org/guidelines/treatment-adults-vestibular-schwannoma/6-role-of-radiosurgery-srs-radiation-therapy-in-ma

Background/Objectives: The Visual Function and Corneal Health Status (V-FUCHS) questionnaire assesses vision-related quality of life in patients affected by Fuchs endothelial corneal dystrophy (FECD) through 15 items, which are divided into the “visual acuity (VA) Factor” and “glare Factor” domains. The purpose of this study was to translate and validate the Italian version of the V-FUCHS instrument. Methods: The original V-FUCHS questionnaire was translated into Italian using certified forward and backward translation methods and administered to patients with FECD undergoing a unilateral or bilateral Descemet membrane endothelial keratoplasty (DMEK) for FECD and healthy controls. Its test–retest reliability was assessed by administering the questionnaire twice, four weeks apart. Modified Krachmer grade, best-corrected visual acuity (BCVA) and central corneal thickness (CCT) were also measured. Results: A total of 74 participants, aged 45 to 83 years, were prospectively enrolled and divided into five subgroups: mild-to-moderate FECD (n = 18), advanced FECD (n = 15), unilateral DMEK (n = 9), bilateral DMEK (n = 12), and healthy controls (n = 20). Retest reliability confirmed the consistency and agreement of their responses (intraclass correlation coefficient > 0.90 for both factors). The Italian V-FUCHS effectively discriminated between different stages of FECD severity, with significant differences in VA and glare factor scores across all subgroups (p < 0.001). While both BCVA and CCT showed correlations with V-FUCHS scores, only the association between the VA factor and BCVA was statistically significant (p < 0.05), indicating that the VA factor meaningfully reflects patients’ measured VA. Conclusions: The proposed Italian version of the V-FUCHS questionnaire is a valid and reliable tool for assessing visual disability in patients with FECD before and after DMEK. This instrument may aid in optimizing endothelial keratoplasty timings and evaluating postoperative symptomatic improvements in FECD patients.

Rashes are a common issue in primary care, encompassing various differential diagnoses. Chilblain lupus erythematosus (CHLE), a subset of cutaneous lupus erythematosus (CLE), is a rare skin condition with limited documented cases. A young male patient presented with purple discoloration of his toes and fingers, along with violaceous skin scaling after exposure to cold temperatures. A positive antinuclear antibody test led to the diagnosis of CHLE and the initiation of treatment, resulting in a positive response.

Abstract How do brain networks limit seizure activity? In the interictal suppression hypothesis, we recently postulated that high inward connectivity to seizure onset zones (SOZs) from non-involved zones (NIZs) is a sign of broader network suppression. If broad networks appear to be responsible for interictal SOZ suppression, what changes during seizure initiation, spread and termination? For patients with drug-resistant epilepsy, intracranial monitoring offers a view into the electrographic networks that organize around and in response to the SOZ. In this manuscript, we investigate network dynamics in the peri-ictal periods to assess possible mechanisms of seizure suppression and the consequences of this suppression being overwhelmed. Peri-ictal network dynamics were derived from stereo electroencephalography recordings from 75 patients with drug-resistant epilepsy undergoing presurgical evaluation at Vanderbilt University Medical Center. We computed directed connectivity from 5 s windows in the periods between, immediately before, during and after 668 seizures. We aligned connectivity matrices across seizures and patients, then calculated net connectivity changes from the SOZ, propagative zone and NIZ. Across all seizure types, we observed two phases: a rapid increase in directed communication towards the SOZ, followed by a collapse in network connectivity. During the first phase, SOZs could be distinguished from all other regions (one-way ANOVA, P = 8.32 × 10−19–2.22 × 10−7). In the second phase and post-ictal period, SOZ inward connectivity decreased yet remained distinct (one-way ANOVA, P = 2.58 × 10−10–1.66 × 10−2). NIZs appeared to drive increased SOZ connectivity while intra-NIZ connectivity decreased concordantly. Stratifying by seizure subtype, we found that consciousness-impairing seizures decrease inward connectivity from the NIZ earlier than consciousness-sparing seizures (one-way ANOVA, P &lt; 0.01 after false discovery correction). Tracking network reorganization against a surrogate for seizure involvement highlighted a possible antagonism between seizure propagation and the ability of the NIZ to maintain high connectivity to the SOZ. Finally, we found that inclusion of peri-ictal connectivity improved the accuracy of SOZ classification from previous models to a combined area under the curve of 93%. Overall, NIZs appear actively to increase inhibitory signaling towards the SOZ during seizure onset, possibly to thwart seizure activity. Although inhibition appears insufficient to prevent seizure onset, the inability to restrict seizure propagation might contribute to loss of consciousness during larger seizures. Dynamic connectivity patterns uncovered in this work might: (i) facilitate accurate delineation of surgical targets in focal epilepsy; (ii) reveal why interictal suppression of SOZs might be insufficient to prevent all seizures; and (iii) provide insight into mechanisms of loss of consciousness during certain seizures.

Abstract Background Cardiovascular guidelines have long recommended low-density lipoprotein-cholesterol (LDL-C) as the primary target for lipid-lowering therapy. Recent guidelines have emphasized the importance of achieving low LDL-C levels; hence, the accurate measurement of low LDL-C is increasingly clinically relevant. Methods Using lipid panel test results from the Mayo Clinic (n = 24 590) and the FOURIER clinical trial of evolocumab (n = 9605), the following modified Sampson equation was developed by least-squares regression to match LDL-C (mg/dL) by the β-quantification reference method, by combining terms into non High Density Lipoprotein Cholesterol (nonHDLC = Total Cholesterol – High Density Lipoprotein Cholesterol) and forcing the coefficient to be one: Results The modified Sampson equation demonstrated significant improvement in its concordance to the reference method compared to other equations (the Lin Concordance Correlation Coefficient 0.992, P &lt; 0.001). By overall kappa analysis, it showed the best agreement to the reference method at the 55 mg/dL cutpoint (1.4 mmol/L, 0.98 [P &lt; 0.001], Sampson–NIH: 0.96, Martin–Hopkins: 0.96, Friedewald: 0.94) and the 70 mg/dL cutpoint (1.8 mmol/L, 0.97 [P &lt; 0.001], Sampson–NIH: 0.94, Martin–Hopkins: 0.95, Friedewald: 0.92). The false classification rate of the modified Sampson equation was also significantly lower compared to the other equations at 55 mg/dL (15%, [P &lt; 0.001], Sampson–NIH: 29%, Martin–Hopkins: 28%, Friedewald: 37%) and 70 mg/dL (18%, [P &lt; 0.001]; Sampson–NIH: 30%, Martin–Hopkins: 28.%, Friedewald: 34%). The new equation increases the percentage of correctly classified patients with low LDL-C by approximately 10% to 20% over the other equations based on its net reclassification index. Conclusions The modified Sampson equation shows improved accuracy compared to other equations for low LDL-C. It more accurately identifies high-risk patients, who are not at their LDL-C goals and could benefit from more intensive lipid-lowering therapy.

Abstract Cadaveric dissections, which are considered the most realistic model to study neuroanatomy, are expensive and not readily available in all centers. Given the surge of technological advances, incorporation of three‐dimensional (3D) scanning technologies and 3D models has gained popularity, both in the educational and clinical settings. We present our institutional experience in creating high‐fidelity neuroanatomical 3D models using three 3D scanning techniques: structured light 3D scanning, “manual” photogrammetry with a single DSLR camera, and “automatic” photogrammetry using a scanner equipped with five vertically arranged DSLR cameras and an automatic turntable within a square box. A survey study was conducted with 20 neurosurgical residents to assess the quality of the three resulting 3D models. In the study, “manual” photogrammetry was determined to be the most cost‐effective technique, while “automatic” photogrammetry was the most time‐effective and user‐friendly technique. The best visual quality was obtained using “manual” photogrammetry, as determined from survey results of 20 neurosurgical residents. While structured light 3D scanning had the lowest quality of resolution of the texture map, this technique was the most accurate to use for determining measurements, with a fixed accuracy of 0.05 mm. Overall, “manual” photogrammetry can allow for the most detailed 3D models and is the most cost‐effective strategy, while structured light 3D scanning is most suitable for obtaining clinically relevant measurements given the high degree of structural accuracy. Alternatively, “automatic” photogrammetry can serve as a hybrid between obtaining relatively high‐quality models in a time‐effective and user‐friendly manner.