Tag: Guidelines

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Guidelines for the Treatment of Adults With WHO Grade II Diffuse Glioma: Update

Neurosurgery 98:984–991, 2026

These updated CNS evidence-based guidelines consolidate current literature on the management of WHO grade II diffuse gliomas, covering imaging, surgical strategies, neuropathology, radiotherapy, chemotherapy, and recurrence. They summarize evidence levels, provide topic-specific recommendations, and identify areas needing higher-quality research to inform clinical decisions.

The document details unchanged, updated, and new recommendations—ranging from MRI protocols and intraoperative mapping to molecular testing and adjuvant therapy—while describing methodology, conflict-of-interest procedures, and plans for periodic revision to ensure clinical relevance.

Scope: Updated evidence-based recommendations for adult WHO grade II diffuse glioma across imaging, surgery, neuropathology/molecular markers, radiotherapy, chemotherapy, and recurrence management.

Methods: Systematic search of MEDLINE/Embase/Cochrane covering Jan 1, 2013–Jan 31, 2020, with recommendations based on evidence rather than consensus.

Imaging (minimum MRI): Minimum evaluation includes T2-weighted and pre- and post-gadolinium T1-weighted MRI; serial imaging is used to detect new enhancement or significant growth suggesting higher-grade transformation.

Imaging (advanced/PET): Diffusion plus perfusion techniques (e.g., DSC/DCE/ASL) are suggested when standard MRI cannot distinguish grade II from higher-grade gliomas; amino-acid PET (FET or FDOPA) may be added when not evident on MRI to help with grading, prognosis (FET), or progression detection (FDOPA).

Surgery: When safe/feasible, resection (gross total or subtotal) is recommended over biopsy alone to reduce progression; extent of resection should be maximized when safely possible (including for IDH-mutant and IDH–wild-type tumors).

Pathology/molecular testing: Diagnosis relies on histopathology from representative tissue; IDH mutation testing is recommended for classification/prognosis, and 1p/19q loss-of-heterozygosity testing is recommended in oligodendroglial cases for prognosis/treatment planning.

Adjuvant therapy (high-risk): For high-risk grade II diffuse glioma (age >40 or <40 without gross total resection), adding chemotherapy to radiotherapy is recommended to improve overall survival (including procarbazine/PCV-based approaches) and is also recommended without decline in neurocognitive function.

Recurrence: At suspected recurrence, advanced imaging (MRS/perfusion/diffusion/PET) is suggested; temozolomide is suggested (often initial choice) and procarbazine may help (strongest evidence in oligodendroglioma); radiation is suggested if not previously given and reirradiation may be considered.

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Guidelines for the Diagnosis and Clinical Management of Cavernous Malformations of the Brain and Spinal Cord

Neurosurgery 98:3–22, 2026

These guidelines present updated, evidence-based recommendations for diagnosing and managing cavernous malformations (CMs) of the brain and spinal cord, produced by an expert multidisciplinary panel convened by the Alliance to Cure Cavernous Malformation. The document summarizes systematic literature review methods, evidence ratings, and 53 consensus recommendations across epidemiology, genetics, imaging, neurosurgery, and neurology.

Key clinical guidance addresses diagnostic MRI protocols, genetic testing for familial CM (KRIT1/CCM2/PDCD10), surgical/radiosurgical indications including pediatric and geriatric considerations, seizure and headache management, and lifestyle and medication factors potentially affecting hemorrhage risk. Recommendations highlight evidence limitations and prioritize further research.

Diagnosis: MRI with susceptibility-weighted sequences is the gold standard for diagnosing cavernous malformations (CMs); CT may be used in emergencies, but MRI is preferred for follow-up and detailed assessment.

Genetic Testing: Genetic testing for KRIT1 (CCM1), CCM2, and PDCD10 (CCM3) is recommended for individuals with multiple CMs, a family history, or suspected familial CM (FCM); founder mutations exist in certain populations.

Hemorrhage Risk: Annual risk of symptomatic intracranial hemorrhage (ICH) from CMs ranges from 0.7% to 7.5%, with higher risk for recurrent bleeds and in familial cases, especially with CCM3 mutations.

Surgical Management: Surgery is generally reserved for symptomatic, accessible CMs or those causing drug-resistant epilepsy; conservative management is preferred for asymptomatic or deep/eloquent area lesions unless recurrent bleeds occur.

Medical Management: Antiseizure medication is recommended after a first CM-related seizure; standard migraine therapy applies for nonhemorrhagic CMs, and nonaspirin NSAIDs can be used cautiously.

Pregnancy: Pregnancy does not increase the risk of CM hemorrhage compared to nonpregnant states; MRI without contrast is advised for new neurological symptoms during pregnancy.

Medication Risks: Antithrombotic agents (e.g., aspirin) do not increase and may actually lower CM hemorrhage risk, while female hormones may increase risk; thrombolytic use remains controversial and should be considered on a case-by-case basis.

Lifestyle and Emerging Therapies: Vitamin D supplementation, aerobic activity, and a diet low in processed foods are reasonable lifestyle recommendations; propranolol and statins show potential but lack definitive evidence for reducing hemorrhage risk.

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Evolution and Impact of the Brain Trauma Foundation Guidelines

Neurosurgery 89:1148–1156, 2021

The Brain Trauma Foundation (BTF) Guidelines for the Management of Severe Head Injury were the first clinical practice guidelines published by any surgical specialty. These guidelines have earned a reputation for rigor and have been widely adopted around the world. Implementation of these guidelines has been associated with a 50% reduction in mortality and reduced costs of patient care.

Over their 25-yr history the traumatic brain injury (TBI) guidelines have been expanded, refined, and made increasingly more rigorous in conjunction with new clinical evidence and evolving methodologic standards.

Here, we discuss the history and accomplishments of BTF guidelines for TBI as well as their limitations. We also discuss planned changes to future TBI guidelines intended to increase their utility and positive impact in an evolving medical landscape. Perhaps the greatest limitation of TBI guidelines now is the lack of high-quality clinical research as well as novel diagnostics and treatments with which to generate substantially new recommendations.

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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on Neuroablative Procedures for Patients With Cancer Pain

Neurosurgery 88:437–442, 2021

Managing cancer pain once it is refractory to conventional treatment continues to challenge caregivers committed to serving those who are suffering from a malignancy. Although neuromodulation has a role in the treatment of cancer pain for some patients, these therapies may not be suitable for all patients. Therefore, neuroablative procedures,whichwere once a mainstay in treating intractable cancer pain, are again on the rise. This guideline serves as a systematic review of the literature of the outcomes following neuroablative procedures.

OBJECTIVE: To establish clinical practice guidelines for the use of neuroablative procedures to treat patients with cancer pain.

METHODS: A systematic review of neuroablative procedures used to treat patients with cancer pain from 1980 to April 2019 was performed using the United States National Library of Medicine PubMed database, EMBASE, and Cochrane CENTRAL. After inclusion criteria were established, full text articles that met the inclusion criteria were reviewed by 2 members of the task force and the quality of the evidence was graded.

RESULTS: In total, 14 646 relevant abstracts were identified by the literature search, from which 189 met initial screening criteria. After full text review, 58 of the 189 articles were included and subdivided into 4 different clinical scenarios. These include unilateral somatic nociceptive/neuropathic body cancer pain, craniofacial cancer pain,midline subdiaphragmatic visceral cancer pain, and disseminated cancer pain. Class II and III evidence was available for these 4 clinical scenarios. Level III recommendations were developed for the use of neuroablative procedures to treat patients with cancer pain.

CONCLUSION: Neuroablative procedures may be an option for treating patients with refractory cancer pain. Serious adverse events were reported in some studies, but were relatively uncommon. Improved imaging, refinements in technique and the availability of new lesioning modalities may minimize the risks of neuroablation even further. The full guidelines can be accessed at https://www.cns.org/guidelines/browse-guidelinesdetail/ guidelines-on-neuroablative-procedures-patients-wi.

 

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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Treatment of Pediatric Hydrocephalus: Update of the 2014 Guidelines

Neurosurgery 87:1071–1075, 2020

The Congress of Neurological Surgeons reviews its guidelines according to the Institute of Medicine’s recommended best practice of reviewing guidelines every 5 yrs. The authors performed a planned 5-yr review of the medical literature used to develop the “Pediatric hydrocephalus: systematic literature review and evidence-based guidelines” and determined the need for an update to the original guideline based on new available evidence.

OBJECTIVE: To perform an update to include the current medical literature for the “Pediatric hydrocephalus: systematic literature review and evidence-based guidelines”, originally published in 2014.

METHODS: The Guidelines Task Force used the search terms and strategies consistent with the original guidelines to search PubMed and Cochrane Central for relevant literature published between March 2012 and November 2019. The same inclusion/exclusion criteria were also used to screen abstracts and to perform the full-text review. Full text articles were then reviewed and when appropriate, included as evidence and recommendations were added or changed accordingly.

RESULTS: A total of 41 studies yielded by the updated search met inclusion criteria and were included in this update.

CONCLUSION: New literature resulting from the update yielded a new recommendation in Part 2, which states that neuro-endoscopic lavage is a feasible and safe option for the removal of intraventricular clots and may lower the rate of shunt placement (Level III). Additionally a recommendation in part 7 of the guideline now states that antibiotic- impregnated shunt tubing reduces the risk of shunt infection compared with conventional silicone hardware and should be used for children who require placement of a shunt (Level I). <https://www.cns.org/guidelines/browse-guidelines-detail/pediatric- hydrocephalus-guideline>

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Stereotactic Radiosurgery for Spetzler-Martin Grade I and II Arteriovenous Malformations: International Society of Stereotactic Radiosurgery (ISRS) Practice Guideline

Neurosurgery 87:442–452, 2020

No guidelines have been published regarding stereotactic radiosurgery (SRS) in the management of Spetzler-Martin grade I and II arteriovenous malformations (AVMs).

OBJECTIVE: To establish SRS practice guidelines for grade I-II AVMs on the basis of a systematic literature review.

METHODS: Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-compliant search of Medline, Embase, and Scopus, 1986-2018, for publications reporting post-SRS outcomes in ≥ 10 grade I-II AVMs with a follow-up of ≥ 24 mo. Primary endpoints were obliteration and hemorrhage; secondary outcomes included SpetzlerMartin parameters, dosimetric variables, and “excellent” outcomes (defined as total obliteration without new post-SRS deficit).

RESULTS: Of 447 abstracts screened, 8 were included (n = 1, level 2 evidence; n = 7, level 4 evidence), representing 1102 AVMs, of which 836 (76%) were grade II. Obliteration was achieved in 884 (80%) at a median of 37 mo; 66 hemorrhages (6%) occurred during a median follow-up of 68 mo. Total obliteration without hemorrhage was achieved in 78%. Of 836 grade II AVMs, Spetzler-Martin parameters were reported in 680: 377 were eloquent brain and 178 had deep venous drainage, totaling 555/680 (82%) high-risk SRS-treated grade II AVMs.

CONCLUSION: The literature regarding SRS for grade I-II AVM is low quality, limiting interpretation. Cautiously, we observed that SRS appears to be a safe, effective treatment for grade I-II AVM and may be considered a front-line treatment, particularly for lesions in deep or eloquent locations. Preceding publications may be influenced by selection bias, with favorable AVMs undergoing resection, whereas those at increased risk of complications and nonobliteration are disproportionately referred for SRS.

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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for the Treatment of Adults With Metastatic Brain Tumors: Executive Summary

Neurosurgery 84:550–552, 2019

The Congress of Neurological Surgeons systematic review and evidencebased clinical practice parameter guidelines for the treatment of adults with metastatic brain tumorswas first published in 2010. Because of the time elapsed since that publication, an update of this set of guidelines based on literature published since is now indicated.

OBJECTIVE: To establish the best evidence-based management of metastatic brain tumors over all commonly used diagnostic and treatment modalities in regularly encountered clinical situations.

METHODS: Literature searches regarding management of metastatic brain tumors with whole brain radiation therapy, surgery, stereotactic radiosurgery, chemotherapy, prophylactic anticonvulsants, steroids, instances of multiple brain metastases, and emerging and investigational therapies were carried out to answer questions designed by consensus of a multidisciplinary writing group.

RESULTS: Recommendations were created and their strength linked to the quality of the literature data available thus creating an evidence-based guideline. Importantly, shortcomings and biases to the literature data are brought out so as to provide guidance for future investigation and improvements in the management of patients with metastatic brain tumors.

CONCLUSION: This series of guidelines was constructed to assess the most current and clinically relevant evidence for management of metastatic brain tumors. They set a benchmark regarding the current evidence base for this management while also highlighting important key areas for future basic and clinical research, particularly on those topics for which no recommendations could be formulated.

The full guideline can be found here.

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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on Subthalamic Nucleus and Globus Pallidus Internus Deep Brain Stimulation for the Treatment of Patients With Parkinson’s Disease

Neurosurgery 82:753–756, 2018

QUESTION 1: Is bilateral subthalamic nucleus deep brain stimulation (STN DBS) more, less, or as effective as bilateral globus pallidus internus deep brain stimulation (GPi DBS) in treating motor symptoms of Parkinson’s disease, as measured by improvements in Unified Parkinson’s Disease Rating Scale, part III (UPDRS-III) scores?

RECOMMENDATION: Given that bilateral STN DBS is at least as effective as bilateral GPi DBS in treating motor symptoms of Parkinson’s disease (as measured by improvements in UPDRS-III scores), consideration can be given to the selection of either target in patients undergoing surgery to treat motor symptoms. (Level I)

QUESTION 2: Is bilateral STN DBS more, less, or as effective as bilateral GPi DBS in allowing reduction of dopaminergic medication in Parkinson’s disease?

RECOMMENDATION: When the main goal of surgery is reduction of dopaminergic medications in a patient with Parkinson’s disease, then bilateral STN DBS should be performed instead of GPi DBS. (Level I)

QUESTION 3: Is bilateral STN DBSmore, less, or as effective as bilateral GPi DBS in treating dyskinesias associated with Parkinson’s disease?

RECOMMENDATION: There is insufficient evidence to make a generalizable recommendation regarding the target selection for reduction of dyskinesias. However, when the reduction of medication is not anticipated and there is a goal to reduce the severity of “on” medication dyskinesias, the GPi should be targeted. (Level I)

QUESTION 4: Is bilateral STN DBS more, less, or as effective as bilateral GPi DBS in improving quality of life measures in Parkinson’s disease?

RECOMMENDATION:When considering improvements in quality of life in a patient undergoing DBS for Parkinson’s disease, there is no basis to recommend bilateral DBS in 1 target over the other. (Level I) QUESTION 5: Is bilateral STN DBS associated with greater, lesser, or a similar impact on neurocognitive function than bilateral GPi DBS in Parkinson disease?

RECOMMENDATION: If there is significant concern about cognitive decline, particularly in regards to processing speed and working memory in a patient undergoing DBS, then the clinician should consider using GPi DBS rather than STN DBS, while taking into consideration other goals of surgery. (Level I)

QUESTION 6: Is bilateral STN DBS associated with a higher, lower, or similar risk of mood disturbance than GPi DBS in Parkinson’s disease?

RECOMMENDATION: If there is significant concern about the risk of depression in a patient undergoing DBS, then the clinician should consider using pallidal rather than STN stimulation, while taking into consideration other goals of surgery. (Level I)

QUESTION 7: Is bilateral STN DBS associated with a higher, lower, or similar risk of adverse events compared to GPi DBS in Parkinson’s disease?

RECOMMENDATION: There is insufficient evidence to recommend bilateral DBS in 1 target over the other in order to minimize the risk of surgical adverse events.

The full guideline can be found at: https://www.cns.org/guidelines/deep-brainstimulation- parkinsons-disease.

 

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Guidelines for the Management of Severe Traumatic Brain Injury, Fourth Edition

Neurosurgery 80:6–15, 2017

The scope and purpose of this work is 2-fold: to synthesize the available evidence and to translate it into recommendations. This document provides recommendations only when there is evidence to support them. As such, they do not constitute a complete protocol for clinical use.

Our intention is that these recommendations be used by others to develop treatment protocols, which necessarily need to incorporate consensus and clinical judgment in areas where current evidence is lacking or insu cient.

We think it is important to have evidence-based recommendations to clarify what aspects of practice currently can and cannot be supported by evidence, to encourage use of evidence-based treatments that exist, and to encourage creativity in treatment and research in areas where evidence does not exist.

The communities of neurosurgery and neuro-intensive care have been early pioneers and supporters of evidence-based medicine and plan to continue in this endeavor. The complete guideline document, which summarizes and evaluates the literature for each topic, and supplemental appendices (A-I) are available online at https://www.braintrauma.org/coma/guidelines.

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Occipital Nerve Stimulation for the Treatment of Patients With Medically Refractory Occipital Neuralgia

Occipital Nerve Stimulation for the Treatment of Patients With Medically Refractory Occipital Neuralgia

Neurosurgery 77:332–341, 2015

Occipital neuralgia (ON) is a disorder characterized by sharp, electrical, paroxysmal pain, originating from the occiput and extending along the posterior scalp, in the distribution of the greater, lesser, and/or third occipital nerve. Occipital nerve stimulation (ONS) constitutes a promising therapy for medically refractory ON because it is reversible with minimal side effects and has shown continued efficacy with long-term follow-up.

OBJECTIVE: To conduct a systematic literature review and provide treatment recommendations for the use of ONS for the treatment of patients with medically refractory ON.

METHODS: A systematic literature search was conducted using the PubMed database and the Cochrane Library to locate articles published between 1966 and April 2014 using MeSH headings and keywords relevant to ONS as a means to treat ON. A second literature search was conducted using the PubMed database and the Cochrane Library to locate articles published between 1966 and June 2014 using MeSH headings and keywords relevant to interventions that predict response to ONS in ON. The strength of evidence of each article that underwent full text review and the resulting strength of recommendation were graded according to the guidelines development methodology of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Guidelines Committee.

RESULTS: Nine studies met the criteria for inclusion in this guideline. All articles provided Class III Level evidence.

CONCLUSION: Based on the data derived from this systematic literature review, the following Level III recommendation can be made: the use of ONS is a treatment option for patients with medically refractory ON.

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Deep Brain Stimulation for Obsessive-Compulsive Disorder

DBS

Neurosurgery 75:327–333, 2014

It is estimated that 40% to 60% of patients with obsessive-compulsive disorder (OCD) continue to experience symptoms despite adequate medical management. For this population of treatment-refractory patients, promising results have been reported with the use of deep brain stimulation (DBS).

OBJECTIVE: To conduct a systematic review of the literature and develop evidencebased guidelines on DBS for OCD.

METHODS: A systematic literature search was undertaken using the PubMed database for articles published between 1966 and October 2012 combining the following words: “deep brain stimulation and obsessive-compulsive disorder” or “electrical stimulation and obsessivecompulsive disorder.” Of 353 articles, 7 were retrieved for full-text review and analysis. The quality of the articles was assigned to each study and the strength of recommendation graded according to the guidelines development methodology of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Guidelines Committee.

RESULTS: Of the 7 studies, 1 class I and 2 class II double-blind, randomized, controlled trials reported that bilateral DBS is more effective in improving OCD symptoms than sham treatment.

CONCLUSION: Based on the data published in the literature, the following recommendations can be made: (1) There is Level I evidence, based on a single class I study, for the use of bilateral subthalamic nucleus DBS for the treatment of medically refractory OCD. (2) There is Level II evidence, based on a single class II study, for the use of bilateral nucleus accumbens DBS for the treatment of medically refractory OCD. (3) There is insufficient evidence to make a recommendation for the use of unilateral DBS for the treatment of medically refractory OCD.

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