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OBJECTIVE The role of endovascular treatment in the management of patients with brain arteriovenous malformations (AVMs) remains uncertain. AVM embolization can be offered as stand-alone curative therapy or prior to surgery or stereotactic radiosurgery (SRS) (pre-embolization). The Treatment of Brain AVMs Study (TOBAS) is an all-inclusive pragmatic study that comprises two randomized trials and multiple registries. METHODS Results from the TOBAS curative and pre-embolization registries are reported. The primary outcome for this report is death or dependency (modified Rankin Scale [mRS] score > 2) at last follow-up. Secondary outcomes include angiographic results, perioperative serious adverse events (SAEs), and permanent treatment-related complications leading to an mRS score > 2. RESULTS From June 2014 to May 2021, 1010 patients were recruited in TOBAS. Embolization was chosen as the primary curative treatment for 116 patients and pre-embolization prior to surgery or SRS for 92 patients. Clinical and angiographic outcomes were available in 106 (91%) of 116 and 77 (84%) of 92 patients, respectively. In the curative embolization registry, 70% of AVMs were ruptured, and 62% were low-grade AVMs (Spetzler-Martin grade I or II), while the pre-embolization registry had 70% ruptured AVMs and 58% low-grade AVMs. The primary outcome of death or disability (mRS score > 2) occurred in 15 (14%, 95% CI 8%–22%) of the 106 patients in the curative embolization registry (4 [12%, 95% CI 5%–28%] of 32 unruptured AVMs and 11 [15%, 95% CI 8%–25%] of 74 ruptured AVMs) and 9 (12%, 95% CI 6%–21%) of the 77 patients in the pre-embolization registry (4 [17%, 95% CI 7%–37%] of 23 unruptured AVMs and 5 [9%, 95% CI 4%–20%] of 54 ruptured AVMs) at 2 years. Embolization alone was confirmed to occlude the AVM in 32 (30%, 95% CI 21%–40%) of the 106 curative attempts and in 9 (12%, 95% CI 6%–21%) of 77 patients in the pre-embolization registry. SAEs occurred in 28 of the 106 attempted curative patients (26%, 95% CI 18%–35%, including 21 new symptomatic hemorrhages [20%, 95% CI 13%–29%]). Five of the new hemorrhages were in previously unruptured AVMs (n = 32; 16%, 95% CI 5%–33%). Of the 77 pre-embolization patients, 18 had SAEs (23%, 95% CI 15%–34%), including 12 new symptomatic hemorrhages [16%, 95% CI 9%–26%]). Three of the hemorrhages were in previously unruptured AVMs (3/23; 13%, 95% CI 3%–34%). CONCLUSIONS Embolization as a curative treatment for brain AVMs was often incomplete. Hemorrhagic complications were frequent, even when the specified intent was pre-embolization before surgery or SRS. Because the role of endovascular treatment remains uncertain, it should preferably, when possible, be offered in the context of a randomized trial.

OBJECTIVE The Treatment of Brain Arteriovenous Malformations Study (TOBAS) is a pragmatic study that includes 2 randomized trials and registries of treated or conservatively managed patients. The authors report the results of the surgical registry. METHODS TOBAS patients are managed according to an algorithm that combines clinical judgment and randomized allocation. For patients considered for curative treatment, clinicians selected from surgery, endovascular therapy, or radiation therapy as the primary curative method, and whether observation was a reasonable alternative. When surgery was selected and observation was deemed unreasonable, the patient was not included in the randomized controlled trial but placed in the surgical registry. The primary outcome of the trial was mRS score > 2 at 10 years (at last follow-up for the current report). Secondary outcomes include angiographic results, perioperative serious adverse events, and permanent treatment-related complications leading to mRS score > 2. RESULTS From June 2014 to May 2021, 1010 patients were recruited at 30 TOBAS centers. Surgery was selected for 229/512 patients (44%) considered for curative treatment; 77 (34%) were included in the surgery versus observation randomized trial and 152 (66%) were placed in the surgical registry. Surgical registry patients had 124/152 (82%) ruptured and 28/152 (18%) unruptured arteriovenous malformations (AVMs), with the majority categorized as low-grade Spetzler-Martin grade I–II AVM (118/152 [78%]). Thirteen patients were excluded, leaving 139 patients for analysis. Embolization was performed prior to surgery in 78/139 (56%) patients. Surgical angiographic cure was obtained in 123/139 all-grade (89%, 95% CI 82%–93%) and 105/110 low-grade (95%, 95% CI 90%–98%) AVM patients. At the mean follow-up of 18.1 months, 16 patients (12%, 95% CI 7%–18%) had reached the primary safety outcome of mRS score > 2, including 11/16 who had a baseline mRS score ≥ 3 due to previous AVM rupture. Serious adverse events occurred in 29 patients (21%, 95% CI 15%–28%). Permanent treatment-related complications leading to mRS score > 2 occurred in 6/139 patients (4%, 95% CI 2%–9%), 5 (83%) of whom had complications due to preoperative embolization. CONCLUSIONS The surgical treatment of brain AVMs in the TOBAS registry was curative in 88% of patients. The participation of more patients, surgeons, and centers in randomized trials is needed to definitively establish the role of surgery in the treatment of unruptured brain AVMs. Clinical trial registration no.: NCT02098252 (ClinicalTrials.gov)

The Comprehensive Aneurysm Management (CAM) study is a pragmatic trial designed to manage unruptured intracranial aneurysm (UIA) patients within a care research framework.CAM is an all-inclusive study. Management options are allocated according to an algorithm combining pre-randomization and clinical judgment. Eligible patients are offered 1:1 randomized allocation of intervention versus conservative management and 1:1 randomization allocation of surgical versus endovascular treatment. Ineligible patients are registered. The primary outcome is survival without dependency (modified Rankin Scale score3) at 10 years. All UIA patients at 1 center are reported.Between February 2020 and July 2021, 403 UIA patients were recruited: 179 (44%) in one of the randomized controlled trials (RCTs) and 224 (56%) in one of the registries. Conservative management was recommended for 205 of 403 patients (51%); of 198 (49%) patients considered for curative treatment, 159 (80%) were randomly allocated conservative (n = 81) or curative treatment (n = 78). These patients were younger and had larger aneurysms than those in the observation registry (P = 0.004). In 39 of 198 patients (20%), conservative management was not considered reasonable (17 patients were recommended endovascular, 2 surgery, and 20 the RCT comparing endovascular with surgical treatment). In total, 70 patients were recruited in the RCT comparing surgery and endovascular treatment. After informed discussion at time of consent, 141 of 159 patients (89%) agreed with the randomly allocated management plan, while 11% crossed over to the alternative management option.CAM was successfully integrated into routine practice. Meaningful conclusions can be obtained if multiple centers actively participate in the trial.

The anterior cerebral artery (ACA) and the anterior communicating artery (AComA) are important arteries of the telencephalon and are prominent location for cerebral aneurysms. Their embryological development is closely linked, and explains the possible variants seen in adults. In this paper, we present details related to the development of these two arteries, focusing on some variants such as the infra-optic course of the ACA, the unpaired ACA, and the persistent primitive olfactory artery.

Background Aneurysms of spinal arteries not associated with any known predisposing condition are referred to as isolated spinal aneurysms (SAs). In our series, an SA was found in 2 patients during the postpartum period. The goal of this study is to determine whether an occurrence of an SA may be related to puerperium. Methods In a retrospective analysis of our consecutive series of 10 cases of SAs from 2008 to 2020, we identified 2 cases of SAs during puerperium. Patients' charts and imaging were reviewed, for potential predisposing factors. Results In both cases, angiography showed fusiform aneurysms of the anterior SA with concomitant bilateral vertebral artery (VA) dissections. Serum vasculitis and inflammatory panel and genetic testing for collagen disorders were negative in both cases. Review of the literature showed that pregnancy is associated with an increased risk of arterial dissections in various locations and supports the hypothesis that hemodynamic and hormonal changes may play a role in the formation of SAs. Conclusions Pregnancy and peripartum state may be a distinct cause of the formation of SAs, possibly as a result of increased hemodynamic stress and hormonal changes that may alter the arterial wall. It would be appropriate to add pregnancy as a subgroup in the classification of SAs. In our series, both cases were associated with bilateral VA dissections; it is possible that the bilateral VA stenosis may have contributed to the formation of the SAs. It is important to recognize this possibility when considering the occlusion of a dissected VA.

BACKGROUND AND PURPOSE: Noninvasive angiography is commonly used to assess the outcome of surgical or endovascular treatment of intracranial aneurysms in clinical series or randomized trials. We sought to assess whether a standardized 3-grade classification system could be reliably used to compare the CTA and MRA results of both treatments. MATERIALS AND METHODS: An electronic portfolio composed of CTAs of 30 clipped and MRAs of 30 coiled aneurysms was independently evaluated by 24 raters of diverse experience and training backgrounds. Twenty raters performed a second evaluation 1 month later. Raters were asked which angiographic grade and management decision (retreatment; close or long-term follow-up) would be most appropriate for each case. Agreement was analyzed using the Krippendorff α (α(K)) statistic, and the relationship between angiographic grade and clinical management choice, using the Fisher exact and Cramer V tests. RESULTS: Interrater agreement was substantial (α(K) = 0.63; 95% CI, 0.55–0.70); results were slightly better for MRA results of coiling (α(K) = 0.69; 95% CI, 0.56–0.76) than for CTA results of clipping (α(K) = 0.58; 95% CI, 0.44–0.69). Intrarater agreement was substantial to almost perfect. Interrater agreement regarding clinical management was moderate for both clipped (α(K) = 0.49; 95% CI, 0.32–0.61) and coiled subgroups (α(K) = 0.47; 95% CI, 0.34–0.54). The choice of clinical management was strongly associated with the size of the residuum (mean Cramer V = 0.77 [SD, 0.14]), but complete occlusions (grade 1) were followed more closely after coiling than after clipping (P = .01). CONCLUSIONS: A standardized 3-grade scale was found to be a reliable and clinically meaningful tool to compare the results of clipping and coiling of aneurysms using CTA or MRA.

Background Rupture of spinal aneurysms is a rare cause of subarachnoid hemorrhage. These aneurysms are often associated with a variety of vascular malformations that increase blood flow in the spinal circulation or with disorders that compromise the vessel wall. However, spinal aneurysms may be isolated, not associated with any known predisposing condition. The objective of this study is to explore the possible mechanisms associated with the formation and rupture of isolated spinal aneurysms (ISAs). Methods We conducted a retrospective review of a series of consecutive patients admitted for a ruptured ISA. In all cases, spinal angiography confirmed the presence of a spinal aneurysm responsible for the bleeding. Particular attention was paid to medical history and symptoms before bleeding, for potential factors predisposing to their formation and rupture. Results Between 2008 and 2020, ten cases of spinal aneurysms were seen at our institution including 4 cases of ISA. All patients with ISA were female, and in 3 cases the aneurysm involved the territory of the posterior spinal artery. In 3 of these 4 (75%) ISA cases, there was a strikingly similar history of retching and vomiting preceding the thunderclap headache. In 1 patient, the aneurysm was surgically resected; pathologic analysis revealed a fusiform dissecting aneurysm. All 4 patients had a favorable outcome. Conclusions We suggest that the straining during prolonged retching and vomiting plays a role in the formation and rupture of some ISAs, possibly because of pressure spikes, increased transmural arterial pressure, and increased wall shear stress during straining.

Our knowledge of the vascularization of the precentral gyrus by branches of the anterior cerebral artery (ACA) relies mainly on anatomical cadaveric dissection. A distal branch of the ACA known as the posterior internal frontal artery (PIFA) is thought to vascularize the precentral gyrus responsible for proximal arm movement; however, no clinical correlation has yet been reported to confirm this relation. In this manuscript, we report a case of coil migration in the PIFA, causing proximal arm weakness in a 58-year-old woman treated for aneurysmal subarachnoid hemorrhage. The occurrence of clinical signs immediately following coil migration into the PIFA, combined with evidence of stroke in the cortical territory related to arm movement as seen on imaging, indicates that the PIFA indeed can vascularize this lateral portion of the precentral gyrus. This case confirms our current understanding of the vascularization of the precentral gyrus by distal ACA branches, in particular the PIFA.

P2-P3 junction aneurysms are challenging to treat surgically because of their frequent complex morphology and their location deep in close proximity to the midbrain. The sub-temporal route requires significant retraction of the temporal lobe in addition to potential injury to the vein of Labbe. We describe the technique for treating such aneurysms via a lateral supra-cerebellar infra-tentorial (LSCIT) approach, which eliminates manipulation of the temporal lobe. Cadaveric dissection provided comprehensive understanding of relevant anatomy. Intraoperative video shows clipping of the aneurysm using a LSCIT approach. LSCIT approach allows safe clipping of P2-P3 aneurysms with minimal brain manipulation.

Background Scleroderma, or systemic sclerosis, is an autoimmune disorder affecting connective tissues, including blood vessels. Although the exact mechanism is not understood, it results in the production of an abnormal amount of collagen. Cases have been reported in which patients with scleroderma also had intracranial aneurysms. We wish to gain insight into any potential association between the 2 diseases. Case Description We reviewed the literature of scleroderma cases with cerebral aneurysms and added our own case, focusing on patient and aneurysm characteristics. Including the present case, this paper pertains to 11 cases with 26 aneurysms. Fifty-five percent had multiple aneurysms, of which two thirds had >2. When data were available, 35% of aneurysms were ≥1 cm, 12% of which were giant. Four aneurysms were fusiform (15%). Patients presented with subarachnoid hemorrhage in 45% of cases. Conclusions Scleroderma may lead to the formation of an aneurysm, and the abnormal growth of collagen may protect it from early rupture, allowing it to reach a large size.

Background:Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumor-like lesion with unknown pathogenesis. It is likely under-reported due to diagnostic challenges including the nonspecific radiographic features, lack of diagnostic markers, and often asymptomatic nature of the lesions.Methods:We performed detailed examination of 11 CAPNON specimens diagnosed by histopathology, with the help of electron microscopy and immunohistochemistry.Results:Electron microscopy revealed the presence of fibrillary materials consistent with neurofilaments. In addition to some entrapped axons at the periphery of CAPNONs, we discovered that all specimens stained positive for neurofilament-light (NF-L) within the granular amorphous cores, but not neurofilament-phosphorylated (NF-p). CAPNONs also showed variable infiltration of CD8+ T-cells and a decreased ratio of CD4/CD8+ T-cells, suggesting an immune-mediated process in the pathogenesis of CAPNON.Conclusion:NF-L and CD4/CD8 immunostains may serve as diagnostic markers for CAPNON and shed light on its pathogenesis.

Posterior fossa arteriovenous malformations (pfAVMs) can be challenging lesions to manage. AVMs in this location may have distinct features compared with supratentorial AVMs. Our aim was to systematically review the literature on the presenting characteristics of pfAVMs and compare clinical and angiographic outcomes after the various types of treatment employed. The review was conducted according to the Cochrane Collaboration guidelines. Electronic databases from 1900 to March 2018 were searched and complemented by hand-searching and cross-referencing. Articles were categorized into (i) AVM studies that included those in the posterior fossa, (ii) those that focused exclusively on pfAVM, and (iii) those that further specified a cerebellar or brainstem location of the AVM. Seventy-seven articles with 4512 pfAVM patients were retained for analyses. Compared with historical supratentorial controls, pfAVMs were reported to more frequently present with rupture, to more commonly have associated arterial aneurysms, and to more frequently lead to poor clinical and angiographic outcomes. The quality of the literature and lack of standardization of outcome reporting precluded performing a meta-analysis on the results of the various different treatment modalities. Posterior fossa AVMs may have some distinct features compared with supratentorial AVMs. The available reports on pfAVMs are not sufficiently standardized to provide reliable guidance for patient management decisions. This goal will require future studies to be multicentric and to focus on standardized, repeatable clinical and angiographic outcomes.

Whether the best management of middle cerebral artery (MCA) aneurysm patients is surgical or endovascular remains uncertain, with little evidence to guide decision-making. A randomized care trial offering MCA aneurysm patients a 50% chance of surgical and a 50% chance of endovascular management may optimize outcomes in the presence of uncertainty.The Middle Cerebral Artery Aneurysm Trial (MCAAT) is an investigator-initiated, multicenter, parallel group, prospective, 1:1 randomized controlled clinical trial. All adult patients with MCA aneurysms, ruptured or unruptured, amenable to surgical and endovascular treatment can be included. The composite primary outcome is "Treatment Success": (i) occlusion or exclusion of the aneurysm using the allocated treatment modality; (ii) no intracranial hemorrhage during follow-up; (iii) no retreatment of the target aneurysm during follow-up, (iv) no residual aneurysm on angiographic follow-up; and (v) independence (mRS3) at 1 year. The trial tests 2 versions of the same hypothesis (one for ruptured and one for unruptured MCA aneurysm patients): Surgical management will lead to a 15% absolute increase in the proportion of patients reaching Treatment Success from 55% to 70% (ruptured) or from 75% to 90% (unruptured aneurysm patients) compared with endovascular treatment (any method). In this pragmatic trial, outcome evaluations are by treating physicians, except for 1-year angiographic results which will be core lab assessed. The trial will be monitored by an independent data safety monitoring committee to assure safety of participants. MCAAT is registered at clinicaltrials.gov: NCT05161377.Patients with MCA aneurysms can be optimally managed within a care trial protocol.

Operculo-insular epilepsy (OIE) is an under-recognized condition that can mimic temporal and extratemporal epilepsies. Previous studies have revealed structural connectivity changes in the epileptic network of focal epilepsy. However, most reports use the debated streamline-count to quantify ‘connectivity strength’ and rely on standard tracking algorithms. We propose a sophisticated cutting-edge method that is robust to crossing fibers, optimizes cortical coverage, and assigns an accurate microstructure-reflecting quantitative conectivity marker, namely the COMMIT (Convex Optimization Modeling for Microstructure Informed Tractography)-weight. Using our pipeline, we report the connectivity alterations in OIE. COMMIT-weighted matrices were created in all participants (nine patients with OIE, eight patients with temporal lobe epilepsy (TLE), and 22 healthy controls (HC)). In the OIE group, widespread increases in ‘connectivity strength’ were observed bilaterally. In OIE patients, ‘hyperconnections’ were observed between the insula and the pregenual cingulate gyrus (OIE group vs. HC group) and between insular subregions (OIE vs. TLE). Graph theoretic analyses revealed higher connectivity within insular subregions of OIE patients (OIE vs. TLE). We reveal, for the first time, the structural connectivity distribution in OIE. The observed pattern of connectivity in OIE likely reflects a diffuse epileptic network incorporating insular-connected regions and may represent a structural signature and diagnostic biomarker.

The ophthalmic artery (OA) has one of the most complex anatomy and the most fascinating embryological development. The complexity of the embryologic development of the OA resides in the implication of three different embryological systems: the carotid system, the stapedial system, and the ventral pharyngeal system. This explains very well the numerous variations in origin of the OA and the importance of vascular anastomoses developed with branches of the external carotid artery and with the middle meningeal artery. In this review, authors propose a comprehensive description of different hypotheses on the embryologic development of the OA and, in a second part, explain all anatomical variations and clinical implications of this artery.

Background:Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumefactive lesion. CAPNONs can mimic calcified meningiomas at the skull base.Methods:Here, we report two cases of CAPNON and present a systematic review of the literature on skull base CAPNONs, to compare CAPNONs with calcified meningiomas.Results:Case 1: A 57-year-old man presented with right-sided lower cranial neuropathies and gait ataxia. He underwent a subtotal resection of a right cerebellopontine angle lesion, with significant improvement of his gait ataxia. However, his cranial neuropathies persisted. Pathological examination of the lesion was diagnostic of CAPNON, with the entrapped nerve fibers identified at the periphery of the lesion, correlating with the patient’s cranial neuropathy. Case 2: A 70-year-old man presented with progressive headache, gait difficulty, and cognitive impairment. He underwent a frontotemporal craniotomy for a near-total resection of his right basal frontal CAPNON. He remained neurologically stable 7 years after the initial resection without evidence of disease recurrence. We analyzed 24 reported CAPNONs at the skull base in our systematic review of the literature. Cranial neuropathies were present in 11 (45.8%) patients. Outcomes regarding cranial neuropathies were documented in six patients: two had sacrifice of the nerve function with surgical approaches and four had persistent cranial neuropathies.Conclusion:While CAPNON can radiologically and grossly mimic calcified meningiomas, they are two distinctly different pathologies. CAPNONs located at the skull base are commonly associated with cranial neuropathies, which may be difficult to reverse despite surgical intervention.

Background Aneurysmal subarachnoid hemorrhage (SAH) is a catastrophic disease with devastating consequences, including a high mortality rate and severe disabilities among survivors. Inflammation is induced following SAH, but the exact role and phenotype of innate immune cells remain poorly characterized. We investigated the inflammatory components of the early brain injury in an animal model and in SAH patients. Method SAH was induced through injection of blood in the subarachnoid space of C57Bl/6 J wild-type mice. Prospective blood collections were obtained at 12 h, days 1, 2, and 7 to evaluate the systemic inflammatory consequences of SAH by flow cytometry and enzyme-linked immunosorbent-assay (ELISA). Brains were collected, enzymatically digested, or fixed to characterize infiltrating inflammatory cells and neuronal death using flow cytometry and immunofluorescence. Phenotypic evaluation was performed at day 7 using the holding time and footprint tests. We then compared the identified inflammatory proteins to the profiles obtained from the plasma of 13 human SAH patients. Results Following SAH, systemic IL-6 levels increased rapidly, whereas IL-10 levels were reduced. Neutrophils were increased both in the brain and in the blood reflecting local and peripheral inflammation following SAH. More intracerebral pro-inflammatory monocytes were found at early time points. Astrocyte and microglia activation were also increased, and mice had severe motor deficits, which were associated with an increase in the percentage of caspase-3-positive apoptotic neurons. Similarly, we found that IL-6 levels in patients were rapidly increased following SAH. ICAM-1, bFGF, IL-7, IL-12p40, and MCP-4 variations over time were different between SAH patients with good versus bad outcomes. Moreover, high levels of Flt-1 and VEGF at admission were associated with worse outcomes. Conclusion SAH induces an early intracerebral infiltration and peripheral activation of innate immune cells. Furthermore, microglia and astrocytic activation are present at later time points. Our human and mouse data illustrate that SAH is a systemic inflammatory disease and that immune cells represent potential therapeutic targets to help this population of patients in need of new treatments.

Background and purpose Appropriate management of ruptured intracranial aneurysm (RIA) in patients eligible for surgical clipping but under-represented in or excluded from previous randomized trials remains undetermined. Methods The International Subarachnoid Aneurysm Trial-2 (ISAT-2) is a randomized care trial comparing surgical versus endovascular treatment (EVT) of RIA. All patients considered for surgical clipping but eligible for endovascular treatment can be included. The primary endpoint is death or dependency on modified Rankin score (mRS > 2) at 1 year. Secondary endpoints are 1 year angiographic results and length of hospital stay. Results An interim analysis was performed after 103 patients were treated from November 2012 to July 2017 in 4 active centers. Fifty-two of the 55 patients allocated to surgery were treated by clipping, and 45 of the 48 allocated to EVT were treated by coiling, with 3 crossovers in each arm. The main endpoint (1 year mRS > 2), available for 76 patients, was reached in 16/42 patients allocated to clipping (38%; 95%CI: 25%–53%), and 10/34 patients allocated to coiling (29%; 17%–46%). One year imaging results were available in 54 patients: complete aneurysm occlusion was found in 23/27 patients allocated to clipping (85%; 67%–94%), and 18/27 patients allocated to coiling (67%; 47%–81%). Hospital stay exceeding 20 days was more frequent in surgery (26/55 [47%; 34%–60%]) than EVT (9/48 [19%; 10%–31%]). Conclusion Ruptured aneurysm patients for whom surgical clipping may still be best can be managed in a randomized care trial, which is feasible in some centers. More participating centers are needed.

Ultrasound (US) examination of the eye can be used to detect and monitor elevated intracranial pressure (ICP) and its consequences. Elevated ICP is transmitted to the contiguous optic nerve and its sheath (dura mater), thus underlying the development of papilledema and a widened sheath diameter. The US measurement of the optic nerve sheath diameter (ONSD) has previously been validated to diagnose and monitor raised ICP. The occurrence of vitreous hemorrhage in association with subarachnoid hemorrhage (SAH)—i.e., Terson syndrome—can also be easily diagnosed using ophthalmic US. Because of its relevance in anesthesia and critical care, we describe how to perform the technique illustrated by two cases. A 72-yr-old man with hydrocephalus secondary to a SAH developed raised ICP following the removal of an external ventricular drainage (EVD) system. Daily ONSD measurements using handheld US allowed us to diagnose and monitor the progression and resolution of the intracranial hypertension following the placement of a second EVD system. We also describe the steps used to obtain ONSD measurements during the ophthalmic US examination of a 53-yr-old woman who presented with a stage IV SAH with concomitant bilateral vitreous hemorrhages or Terson syndrome. Ophthalmic US using a handheld device to measure and monitor ONSD at the bedside is useful in diagnosing and monitoring the progression of intracranial hypertension following EVD removal in a patient with hydrocephalus secondary to SAH. Ophthalmic US can also be used to identify concomitant vitreous hemorrhage that is associated with a worse prognosis.

Vertebro-PICA aneurysms may be challenging because of their relationship with the brainstem and the lower cranial nerves, especially when the vertebral artery is tortuous and the aneurysm is located in front of the brainstem, contralaterally to the parent vertebral artery. We describe the surgical technique for safe approach. Cadaveric dissection performed by the authors, provided comprehensive understanding of relevant anatomy. Intraoperative photos and videos show clipping of the aneurysm using a combined midline and far-lateral suboccipital craniotomy with a para-condylar extension. The literature reviews potential complications. This combined approach allows safe clipping of such PICA aneurysms.

Objective There are few randomized data comparing clipping and coiling for middle cerebral artery (MCA) aneurysms. We analyzed results from patients with MCA aneurysms enrolled in the CURES (Collaborative UnRuptured Endovascular vs. Surgery) and ISAT-2 (International Subarachnoid Aneurysm Trial II) randomized trials. Methods Both trials are investigator-led parallel-group 1:1 randomized studies. CURES includes patients with 3-mm to 25-mm unruptured intracranial aneurysms (UIAs), and ISAT-2 includes patients with ruptured aneurysms (RA) for whom uncertainty remains after ISAT. The primary outcome measure of CURES is treatment failure: 1) failure to treat the aneurysm, 2) intracranial hemorrhage during follow-up, or 3) residual aneurysm at 1 year. The primary outcome of ISAT-2 is death or dependency (modified Rankin Scale score >2) at 1 year. One-year angiographic outcomes are systematically recorded. Results There were 100 unruptured and 71 ruptured MCA aneurysms. In CURES, 90 patients with UIA have been treated and 10 await treatment. Surgical and endovascular management of unruptured MCA aneurysms led to treatment failure in 3/42 (7%; 95% confidence interval [CI], 0.02–0.19) for clipping and 13/48 (27%; 95% CI, 0.17–0.41) for coiling (P = 0.025). All 71 patients with RA have been treated. In ISAT-2, patients with ruptured MCA aneurysms managed surgically had died or were dependent (modified Rankin Scale score >2) in 7/38 (18%; 95% CI, 0.09–0.33) cases, and 8/33 (24%; 95% CI, 0.13–0.41) for endovascular. One-year imaging results were available in 80 patients with UIA and 62 with RA. Complete aneurysm occlusion was found in 30/40 (75%; 95% CI, 0.60–0.86) patients with UIA allocated clipping, and 14/40 (35%; 95% CI, 0.22–0.50) patients with UIA allocated coiling. Complete aneurysm occlusion was found in 24/34 (71%; 95% CI, 0.54–0.83) patients with RA allocated clipping, and 15/28 (54%; 95% CI, 0.36–0.70) patients with RA allocated coiling. Conclusions Randomized data from 2 trials show that better efficacy may be obtained with surgical management of patients with MCA aneurysms.

The persistent trigeminal artery is the most frequent of the persistent embryonic carotid-basilar artery anastomoses. In the literature, it has most often been described in relation to cerebrovascular pathologies such as aneurysms, vascular nerve compression, trigeminal cavernous fistulas, and thromboembolic ischemia. Its role as collateral circulation, thus supplying brain perfusion during main arterial trunk occlusion, has seldom been described. We describe the case of a patient who presented with a stroke due to a traumatic dissection of the internal carotid artery at the neck, in which the infarction may have been limited by a persistent trigeminal artery.

In the absence of randomized evidence, the optimal management of patients with unruptured intracranial aneurysms (UIA) remains uncertain.Comprehensive Aneurysm Management (CAM) is an all-inclusive care trial combined with a registry. Any patient with a UIA (no history of intracranial hemorrhage within the previous 30 days) can be recruited, and treatment allocation will follow an algorithm combining clinical judgment and randomization. Patients eligible for at least 2 management options will be randomly allocated 1:1 to conservative or curative treatment. Minimization will be used to balance risk factors, using aneurysm size (≥7 mm), location (anterior or posterior circulation), and age60 years.The CAM primary outcome is survival without neurologic dependency (modified Rankin Scale [mRS] score3) at 10 years. Secondary outcome measures include the incidence of subarachnoid hemorrhage during follow-up and related morbidity and mortality; morbidity and mortality related to endovascular treatment or surgical treatment of the UIA at 1 year; overall morbidity and mortality at 1, 5, and 10 years; when relevant, duration of hospitalization; and, when relevant, discharge to a location other than home. The primary hypothesis for patients randomly allocated to at least 2 options, 1 of which is conservative management, is that active UIA treatment will reduce the 10-year combined neurologic morbidity and mortality (mRS score2) from 24% to 16%. At least 961 patients recruited from at least 20 centers over 4 years will be needed for the randomized portion of the study.Patients with unruptured intracranial aneurysms can be comprehensively managed within the context of an all-inclusive care trial.

Objectives: With the current trend of aging of the population, neurosurgeons will be more and more confronted to surgical decision-making involving the elderly. Faced with this increasing demand and frailty of aged patients, a better understanding on the post-operative outcome of this growing population is warranted. The objective of the present study is to assess the post-operative outcome in regard of complications of elderly patients undergoing a craniotomy. Patients and methods: The files of consecutive patients aged 80 years old and more who underwent a craniotomy at a single institution were retrospectively reviewed. Data on demographics, surgical indication, length of surgery, operative blood loss, urgency of surgery, comorbidities using the Elixhauser comorbidity index and post-operative complications were gathered. We performed a multivariate analysis in search of risk factors for post-operative complications. Results: A total of 53 patients were included in the study. The mean age of all patients was 84 years old with the main indication for surgery being subdural hematoma. The overall complication rate was 62%, with 34% of patients suffering from a major complication and 47% from a minor complication. The mean Elixhauser comorbidity index, operative time and operative blood loss were similar to those reported in adult craniotomy series. None of the studied variables were statistically associated with the occurrence of complications in the multivariate analysis. Conclusion: Patients 80 years-old and more were found to harbour a high complication rate following craniotomy when compared to literature. Our study suggests increasing age itself remains an important risk factor for postoperative complications. Keywords: Comorbidity, Complication, Craniotomy, Elderly, Outcome

OBJECTIVEThe management of brain arteriovenous malformations (bAVMs) remains controversial. The Treatment of Brain AVMs Study (TOBAS) was designed to manage patients with bAVMs within a clinical research framework. The objective of this study was to study trial feasibility, recruitment rates, patient allocation to the various management groups, and compliance with treatment allocation.METHODSTOBAS combines two randomized care trials (RCTs) and a registry. Designed to be all-inclusive, the study offers randomized allocation of interventional versus conservative management to patients eligible for both options (first RCT), a second RCT testing the role of preembolization as an adjunct to surgery or radiotherapy, and a registry of patients managed using clinical judgment alone. The primary outcome of the first RCT is death from any cause or disabling stroke (modified Rankin Scale score > 2) at 10 years. A pilot phase was initiated at one center to test study feasibility, record the number and characteristics of patients enrolled in the RCTs, and estimate the frequency of crossovers.RESULTSAll patients discussed at the multidisciplinary bAVM committee between June 2014 and June 2016 (n = 107) were recruited into the study; 46 in the randomized trials (23 in the first RCT with 21 unruptured bAVMs, 40 in the second RCT with 17 unruptured bAVMs, and 17 in both RCTs), and 61 patients in the registry. Three patients crossed over from surgery to observation (first RCT).CONCLUSIONSClinical research was successfully integrated with normal practice using TOBAS. Recruitment rates in a single center are encouraging. Whether the trial will provide meaningful results depends on the recruitment of a sufficient number of participating centers.Clinical trial registration no.: NCT02098252 (clinicaltrials.gov)

Objective To our knowledge, there have not been any reported cases of a meningioma of the craniocervical region presenting solely with syncope as its initial symptom. Only 1 case of meningioma presenting with syncope has been published, but it was associated with hydrocephalus. We report 2 cases of syncope caused by a craniocervical junction meningioma, with syncope being the sole presenting symptom and without hydrocephalus. We discuss the possible pathophysiology, as well as the clinical relevance of this type of presentation. Methods We reviewed the charts, operative details, and imagery of 2 cases of meningioma in the region of the craniocervical junction, with syncope as their sole presenting feature. We also reviewed the literature. Results In 1 case the syncope occurred spontaneously. In the other, it occurred during a Valsalva maneuver. Both meningiomas were surgically removed via a retromastoid approach. There was no recurrence of syncope following surgery. Following a literature review, we found 1 case of posterior fossa meningioma presenting with syncope, but hydrocephalus was also present. Conclusion Syncope can be the sole manifestation of a meningioma of the craniocervical junction. Such syncopes are a consequence of transient dysfunction of the autonomous pathways in the medulla and/or of the medulla's output. In the absence of other causes of syncope, a meningioma in this region, even in the absence of hydrocephalus, should not be considered as fortuitous, but rather as the actual cause of syncope. Recognizing this possibility offers the potential for proper diagnosis and appropriate treatment of the syncope.

BACKGROUND AND PURPOSE: Moyamoya disease is a progressive neurovascular pathology defined by steno-occlusive disease of the distal internal carotid artery and associated with the development of compensatory vascular collaterals. The etiology and exact anatomy of vascular collaterals have not been extensively studied. The aim of this study was to describe the anatomy of collaterals developed between the ophthalmic artery and the anterior cerebral artery in a Moyamoya population. MATERIALS AND METHODS: All patients treated for Moyamoya disease from 2004 to 2016 in 4 neurosurgical centers with available cerebral digital subtraction angiography were included. Sixty-three cases were evaluated, and only 38 met the inclusion criteria. Two patients had a unilateral cervical internal carotid occlusion that limited analysis of ophthalmic artery collaterals to one hemisphere. This study is consequently based on the analysis of 74 cerebral hemispheres. RESULTS: Thirty-eight patients fulfilled the inclusion criteria. The most frequently encountered anastomosis between the ophthalmic artery and cerebral artery was a branch of the anterior ethmoidal artery (31.1%, 23 hemispheres). In case of proximal stenosis of the anterior cerebral artery, a collateral from the posterior ethmoidal artery could be visualized (16 hemispheres, 21.6%). One case (1.4%) of anastomosis between the lacrimal artery and the middle meningeal artery that permitted the vascularization of a middle cerebral artery territory was also noted. CONCLUSIONS: Collaterals from the ophthalmic artery are frequent in Moyamoya disease. Their development depends on the perfusion needs of the anterior cerebral artery territories. Three other systems of compensation could be present (callosal circle, leptomeningeal anastomosis, and duro-pial anastomoses).

BACKGROUND Treatment of thoracic spinal dural arteriovenous fistulas (DAVFs) by microsurgery has recently been approached using minimally invasive spine surgery (MISS). The advantages of such an approach are offset by difficult maneuverability within the tubular retractor and by the creation of "tunnel vision" with reduced luminosity to a remote surgical target. OBJECTIVE To demonstrate how the pitfalls of MISS can be addressed by applying 3-D endoscopy to the minimally invasive approach of spinal DAVFs. METHODS We present 2 cases of symptomatic thoracic DAVFs that were not amenable to endovascular treatment. The DAVFs were excluded solely via a minimally invasive approach using a 3-D endoscope. RESULTS Two patients underwent exclusion of a DAVF following laminotomy, one through a midline 5-cm incision and the other through a paramedian 3-cm incision using minimally invasive nonexpandable tubular retractors. The dura opening, intradural exploration, fistula exclusion, and closure were performed solely under endoscopic 3-D magnification. No incidents were recorded and the postoperative course was marked by clinical improvement. Postoperative imaging confirmed the exclusion of the DAVFs. Anatomical details are exposed using intraoperative videos. CONCLUSION When approaching DAVFs via MISS, replacing the microscope with the endoscope remedies the limitations related to the "tunnel vision" created by the tubular retractor, but at the expense of losing binocular vision. We show that the 3-D endoscope resolves this latter limitation and provides an interesting option for the exclusion of spinal DAVFs.

The best management of brain arteriovenous malformation (bAVM) patients remains unknown. Randomized allocation may be more readily accepted when there is uncertainty and disagreement regarding the management of potential participants. In planning for a trial, we aimed to assess variability and agreement among physicians managing bAVM patients.A portfolio composed of 35 patients was sent to 47 clinicians of various specialties managing bAVM patients. For each patient, physicians were asked their best management decision (surgery/embolization/radiosurgery/conservative), their confidence level, and whether they would include the patient in a randomized trial comparing conservative and curative management. Seven physicians, who had access to all images of each patient, independently responded twice, to assess inter and intra-rater agreement using kappa statistics.The inter-rater agreement (30 raters, including 16 neuroradiologists) for best management decision was only "fair" (κ [95%CI]=0.210[0.157; 0.295]). Agreement remained below 'substantial' (κ.6) between physicians of the same specialty, and when no distinctions were made between various treatments (when responses were dichotomized as conservative versus curative). With access to all images the inter-rater agreement remained fair. The intra-rater agreement reached "substantial" only for the dichotomized decisions. Responding clinicians were willing to include 54.4% of patients (mainly unruptured bAVMs) in a randomized trial.There is a lack of agreement among clinicians involved in the management of bAVM patients. In this study a substantial proportion of clinicians were willing to offer randomized allocation of management options to a substantial number of patients.

OBJECTIVE The ARUBA study (A Randomized Trial of Unruptured Brain Arteriovenous Malformations [AVMs]) on unruptured brain AVMs has been the object of comments and editorials. In the present study the authors aim to systematically review critiques, discuss design issues, and propose a framework for future trials. METHODS The authors performed a systematic review of the French and English literature on the ARUBA study published between January 2006 and February 2015. The electronic search, including the Cochrane Library, MEDLINE (PubMed and Ovid), CINAHL, and EMBASE databases, was complemented by hand searching and cross-referencing. The comments were categorized as items related to the design, the conduct, and the analysis and interpretation of the trial. RESULTS Thirty-one articles or letters were identified. The pragmatic design, with heterogeneity of patients and lack of standardization of the treatment arm, were frequently stated concerns. The choice of outcome measures was repeatedly criticized. During the trial, low enrollment rates, selection bias, and premature interruption of enrollment were frequent comments. The short follow-up period, the lack of subgroup analyses, the lack of details on the results of the various treatments, and a contentious interpretation of results were noted at the analysis stage. A fundamental problem was the primary hypothesis testing conservative management. The authors believe that other trials are needed. Future trials could be pragmatic, test interventions stratified at the time of randomization, and look for long-term, hard clinical outcomes in a large number of patients. CONCLUSIONS In the authors' view, the ARUBA trial is a turning point in the history of brain AVM management; future trials should aim at integrating trial methodology and clinical care in the presence of uncertainty.

Introduction Les anevrismes du segment A1 de l’artere cerebrale anterieure (ACA) constituent environ 1 % de tous les anevrismes intracrâniens. Peu d’etudes ont analyse ces anevrismes. En effectuant une revue systematique de la litterature, l’objectif de ce travail est de mieux caracteriser la conduite therapeutique, les resultats du traitement et les facteurs pronostiques chez les patients chez qui l’on a diagnostique un tel anevrisme. Methodes Une recherche extensive de la litterature utilisant Pubmed a ete effectuee suivant les lignes directrices PRISMA. Nous avons inclus des publications de toutes les langues lorsque les resumes etaient en francais ou en anglais. Resultats Cent trente et un articles sont retenus totalisant 688 patients : 58 % sont des femmes et l’âge moyen est de 51 ans. La rupture de l’anevrisme rend compte de la presentation initiale chez 53 % des patients. Les variantes anatomiques associees les plus frequentes sont la fenestration du segment A1 et une hypoplasie/aplasie du segment A1 controlateral. On retrouve une proportion importante d’anevrismes dissequants (6 %) et d’anevrismes fusiformes (8 %) sur une majorite (86 %) d’anevrismes sacciformes. La plupart des anevrismes mesurent 5 mm ou moins. Le traitement est rapporte pour 646 anevrismes : chirurgie 374 (58 %), endovasculaire 272. Quelle que soit la modalite de traitement, l’evolution est favorable dans la majorite des cas. Les facteurs de mauvais pronostics sont : une presentation hemorragique, un mauvais grade clinique a l’arrivee, une complication ischemique ainsi qu’un anevrisme dissequant ou fusiforme. Conclusion Les anevrismes du segment A1 demeurent difficiles a caracteriser en raison de donnees manquantes ou non rapportees de facon systematique dans la litterature. Lorsque mentionnees, les echelles des resultats cliniques utilisees sont variables ; les suivis a long terme sont rarement rapportes ; les plus grandes series rapportent des donnees groupees. Des etudes prospectives avec des donnees standardisees sont necessaires.

Introduction La maladie de Moyamoya est une pathologie neuro-vasculaire steno-occlusive touchant l’artere carotide interne et ses branches principales, associee au developpement de reseaux collateraux compensatoires. La description detaillee de ces reseaux compensatoires est pauvre dans la litterature actuelle. Generalement, la circulation posterieure est peu touchee rendant possible son utilite en compensant le besoin de flux sanguin des arteres cerebrales anterieures (ACA). Le but de cette etude est de decrire precisement les anastomoses naturelles developpees entre les arteres cerebrales anterieures et posterieures pour compenser l’hypoperfusion des territoires des ACA chez les patients presentant une maladie de Moyamoya. Materiel et methodes Nous avons inclus tous les patients traites pour une maladie de Moyamoya entre 2004 et 2018 dans 4 differents centres neurochirurgicaux ayant une arteriographie cerebrale disponible. Quarante patients (80 hemispheres) avec le diagnostic de maladie ou syndrome de Moyamoya ont ete analyses angiographiquement. Resultats Trente et un hemispheres (38,75 %) presentaient une circulation collaterale entre les arteres cerebrales anterieures et posterieures. La circulation collaterale la plus frequemment retrouvee vient de l’artere calleuse posterieure (cercle calleux, 20 % des cas) et de l’artere choroidienne posterieure (20 % des cas). L’autre collaterale moins frequente (15 % des cas) est une anastomose pio-piale entre les branches corticales des arteres cerebrales anterieures et posterieures. L’observation des arteriographies nous permet aussi d’evaluer la competence de ces anastomoses pour re-perfuser les territoires des ACA que nous classifions en 4 stades. Conclusion Nous avons retrouve 3 differents types d’anastomoses entre les arteres cerebrales anterieures et posterieures, avec une abilite plus ou moins importante pour compenser l’hypoperfusion du territoire anterieur. Leur developpement depend du besoin perfusionnel des territoires de l’ACA.

The recurrent artery of Heubner (RAH) is a very fascinating artery for various reasons. The first one is its early development in the embryologic and phylogenic life. The second one is the discrepancy between its diameter and its functional importance and the last but not least reason is the many variations of origin and course of this artery. For more than a century, numerous studies furnished important information about variations in origin, course, and parenchymal territory of the RAH. The most important anomaly concerning the RAH is the accessory middle cerebral artery and well-illustrates the complexity of its embryologic development. For these reasons, authors provide a review of anatomical variations of this artery that could be encountered with a particular attention given to the history and embryological knowledge.

A 24-year-old woman was referred to us for an intracranial haemorrhage in the left temporal lobe caused by a ruptured cavernous malformation; the bleeding extended over the left Heschl’s gyrus and Wernicke area. On admission, the patient had global aphasia. A few days later, she spontaneously improved but remained with mild residual comprehensive dysphasia. She reported hearing, in her right ear, recently heard words, which is consistent with palinacousis. Auditory acuity testing was normal. EEG showed focal slowing in the left temporal region with no epileptiform activity. During awake surgery for resection of the cavernous malformation, stimulation of the superior temporal gyrus did not provoke palinacousis. The patient made good recovery with complete resolution of the aphasia and no recurrence of palinacousis. We aimed to review this phenomenon and to provide a systematic review of the current literature.

Objective The management of unruptured and ruptured brain arteriovenous malformations (AVMs) remains controversial. The Treatment of Brain AVM Study (TOBAS) was designed to assess curative treatments in the management of AVMs. The purpose of our study is to provide a care trial context to brain AVM patients. Methods TOBAS is a pragmatic, prospective study including 2 randomized controlled trials and a registry. All AVM patients can be recruited. The preferred management modality will be predetermined prior to randomization by the team based on clinical judgment. Patients eligible for both conservative and interventional management will be randomly allocated conservative or curative treatment. Randomization will be stratified by a treatment modality (surgery, radiosurgery or embolization) and minimized according to a history of previous rupture and Spetzler-Martin grade. A second randomization will allocate eligible patients to embolization/no embolization prior to surgery or radiosurgery. The primary outcome of the study is death (any cause) or disabling stroke (mRS > 2) at 10 years. All patients managed according to clinical judgment alone will be included in the registry. The study is registered under: wwwTrials.gov , ID: NCT02098252 . Expected results A minimum recruitment of 540 patients is required to show that treatment can reduce the primary outcome by 10 % (from 25 to 15 %); 440 patients will be needed to show a 10 % increase in angiographic occlusion for a good clinical outcome with pre-embolization. Conclusion The trial is designed to offer optimal and verifiable care to patients with brain AVMs in spite of the uncertainty. We are currently seeking the participation of multiple centers.

BACKGROUND The ascending pharyngeal artery (APA) may, in very rare cases, supply the posterior inferior cerebellar artery (PICA). In reported cases, when such is the case, the ipsilateral vertebral artery (VA) does not supply the PICA, and most of the time it is hypoplastic. OBJECTIVE To describe a unique cadaveric observation of a direct anastomosis between the posterior division (neuromeningeal) of the jugular branch of the APA and the PICA, where the PICA is also supplied by a normal-size VA. METHODS A direct connection between the APA and the PICA was examined in a cadaveric specimen using a 3-dimensional endoscope and a surgical microscope. RESULTS The enlarged jugular branch of the posterior division of the APA entered intracranially via the jugular foramen in its pars vascularis. It then connected directly with the lateral medullary segment of the PICA. The first segment of the PICA originated from a left vertebral artery of normal size and continued its normal course beyond the junction with the jugular branch of the APA. CONCLUSION Both the VA and the jugular branch of the APA may simultaneously supply the PICA territory. Recognition of this anatomic variant is relevant when planning surgical or endovascular treatments.

Introduction La rupture d’un anevrisme spinal est une cause rare d’hemorragie sous-arachnoidienne (HSA). Ces cas representent un defi diagnostic car l’angiographie spinale ne fait pas partie du bilan d’investigation standard de l’HSA. Plusieurs cas rapportes sont associes a des conditions liees a une augmentation du flot arteriel spinal (coarctation de l’aorte, malformation arterioveineuse) ou a des maladies compromettant l’integrite de la paroi vasculaire (maladies du collagene, vasculites, maladies auto-immunes). Toutefois dans environ la moitie cas rapportes, aucune condition sous-jacente ne peut etre identifiee : il s’agit d’anevrismes spinaux isoles. L’objectif de cette etude est de reviser notre experience recente d’anevrismes spinaux rompus isoles afin de faire ressortir des elements physiopathologiques. Materiel et methodes Analyse des dossiers cliniques et radiologiques d’une serie de 3 patients admis consecutivement pour anevrisme spinal isole rompu. Etude de l’histopathologie d’un patient de cette serie opere pour un tel anevrisme. Resultats Tous les patients de cette serie ont presente un tableau systematique de vomissements repetes (de causes variees), precedent de quelques semaines a quelques mois de dorsalgies sentinelles evoluant vers une cephalee en coup de tonnerre. L’un des patients fut opere. L’examen histologique a revele la presence d’un anevrisme fusiforme dissequant avec hematome intramural. Conclusion La presence systematique dans notre serie de vomissements repetes precedant l’HSA suggerent un role des efforts de vomissements repetes dans la formation d’anevrisme spinaux et leur rupture eventuelle. Basees sur les donnees de la litterature, nous proposons que des poussees de pression lors des efforts de vomissement, combinees a la flexion du tronc, modifient les forces hemodynamiques dans les arteres spinales, augmentant les contraintes de cisaillement initiant la formation de ces anevrismes (side wall aneurysm). Cette serie met en lumiere une cause possible d’anevrismes spinaux, non associe a une malformation vasculaire ou a une maladie inflammatoire.