Acute bilateral vision loss after endovascular treatment of an intracranial aneurysm.

Acute bilateral vision loss (ABVL) is a rare and challenging diagnostic issue that is most often caused by a neurological disorder. Since it can be the presenting symptom of potentially life-threatening diseases, priority should be given to excluding such diagnoses. Special caution is required if ABVL symptoms result after an intracranial intervention. This article reports on a diagnostic approach for a patient suffering from ABVL due to vitreous hemorrhage related to a subarachnoid hemorrhage (SAH) after endovascular intracranial aneurysm treatment. This case study highlights the importance of imaging interpretation and its consequences.

Admission serum high mobility group box 1 (HMGB1) protein predicts delayed cerebral ischemia following aneurysmal subarachnoid hemorrhage.

High mobility group box 1 protein (HMGB1) is a prototypical damage associated particle and acts as a key player in aseptic inflammation. HMGB1 appears critical for the crosstalk of a prothrombotic and proinflammatory state that is implicated in mediating and exacerbating ischemic brain injury. The role of HMGB1 in aneurysmal subarachnoid hemorrhage (aSAH) remains to be elucidated. A prospective, single blinded observational study was designed to investigate the role of HMGB1 in aSAH. Serial serum HMGB1 level quantification on admission day 0, 4, 8, and 12 was performed. Primary outcome measures were delayed cerebral ischemia (DCI - new infarction on CT) and poor functional outcome (90-day modified Rankin Scale 4-6). The role of HMGB1 levels for DCI, functional outcome and radiological vasospasm prediction was analyzed. Collectively, 83 aSAH patients were enrolled. Five patients died within 48 h. In 29/78 patients (37.2%), DCI was identified. In multivariable analysis, radiological vasospasm and admission HMGB1 were independent predictors for DCI. Younger age and higher white blood cell count, but not insult burden (World Federation of Neurosurgical Societies scale, modified Fisher scale, intraparenchymal or intraventricular hematoma existence) correlated with admission HMGB1 levels. Serial HMGB1 levels did not differ between patients with or without DCI, poor functional outcome or radiological vasospasm development. Admission serum HMGB1 does not reflect initial insult burden but serves as an independent biomarker predictive of DCI. Further studies are warranted to disentangle the role of HMGB1 surrounding the sequelae of aSAH.

Neuro-ophthalmological evaluation including optical coherence tomography surrounding venous sinus stenting in idiopathic intracranial hypertension with papilledema: a case series.

Venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) has been demonstrated to achieve significant symptom improvement while harboring a low periinterventional morbidity profile. Comprehensive neuro-ophthalmological monitoring represents a cornerstone of disease monitoring. The value of optical coherence tomography (OCT) requires further exploration. Patients with IIH and papilledema who underwent VSS between 04/2018 and 02/2021 were retrospectively reviewed. Clinical and radiological were analyzed. Neuro-ophthalmological data included visual acuity, visual fields, fundoscopy categorized via Frisén scale, and OCT obtained retinal nerve fiber layer (RNFL) thickness were analyzed. Of 39 IIH patients who underwent cerebral angiography with transverse-sigmoid sinus pressure evaluation, 18 patients with IIH and papilledema underwent 21 transverse-sigmoid sinus stenting (TSST) procedures. After TSST, manometry showed a significant reduction of maximum transverse sinus pressures and trans-stenotic gradient pressures (p = 0.005 and p < 0.001, respectively). Chronic headaches, visual disturbance resolved and pulsatile tinnitus improved significantly. Visual fields remained similar, while papilledema Frisén scales and visual acuity significantly improved. The OCT calculated RNFL thickness significantly decreased in all patients. Stratification according to a minimal-low degree (Frisén 1-2) and moderate-marked degree (Frisén 3-4) papilledema demonstrated a significant reduction of RNFL thickness in both groups. Venous sinus stenting provides favorable clinical and neuro-ophthalmological outcomes. This study demonstrates that neuro-ophthalmologic testing augmented with OCT evaluation provides objective data that can be used as a biomarker for treatment success for managing patients with different extents of papilledema and may inform patient management.

Effects of White Matter Hyperintensities on 90-Day Functional Outcome after Large Vessel and Non-Large Vessel Stroke.

INTRODUCTION: White matter hyperintensity (WMH) burden is a critically important cerebrovascular phenotype related to the diagnosis and prognosis of acute ischemic stroke. The effect of WMH burden on functional outcome in large vessel occlusion (LVO) stroke has only been sparsely assessed, and direct LVO and non-LVO comparisons are currently lacking. MATERIAL AND METHODS: We reviewed acute ischemic stroke patients admitted between 2009 and 2017 at a large healthcare system in the USA. Patients with LVO were identified and clinical characteristics, including 90-day functional outcomes, were assessed. Clinical brain MRIs obtained at the time of the stroke underwent quantification of WMH using a fully automated algorithm. The pipeline incorporated automated brain extraction, intensity normalization, and WMH segmentation. RESULTS: A total of 1,601 acute ischemic strokes with documented 90-day mRS were identified, including 353 (22%) with LVO. Among those strokes, WMH volume was available in 1,285 (80.3%) who had a brain MRI suitable for WMH quantification. Increasing WMH volume from 0 to 4 mL, age, female gender, a number of stroke risk factors, presence of LVO, and higher NIHSS at presentation all decreased the odds for a favorable outcome. Increasing WMH above 4 mL, however, was not associated with decreasing odds of favorable outcome. While WMH volume was associated with functional outcome in non-LVO stroke (p = 0.0009), this association between WMH and functional status was not statistically significant in the complete case multivariable model of LVO stroke (p = 0.0637). CONCLUSION: The burden of WMH has effects on 90-day functional outcome after LVO and non-LVO strokes. Particularly, increases from no measurable WMH to 4 mL of WMH correlate strongly with the outcome. Whether this relationship of increasing WMH to worse outcome is more pronounced in non-LVO than LVO strokes deserves additional investigation.

Loss of consciousness at onset of aneurysmal subarachnoid hemorrhage in good-grade patients.

Loss of consciousness (LOC) at presentation with aneurysmal subarachnoid hemorrhage (aSAH) has been associated with early brain injury and poor functional outcome. The impact of LOC on the clinical course after aSAH deserves further exploration. A retrospective analysis of 149 aSAH patients who were prospectively enrolled in the Cerebral Aneurysm Renin Angiotensin Study (CARAS) between 2012 and 2015 was performed. The impact of LOC was analyzed with emphasis on patients presenting in excellent or good neurological condition (Hunt and Hess 1 and 2). A total of 50/149 aSAH patients (33.6%) experienced LOC at presentation. Loss of consciousness was associated with severity of neurological condition upon admission (Hunt and Hess, World Federation of Neurosurgical Societies (WFNS), Glasgow Coma Scale (GCS) grade), hemorrhage burden on initial head CT (Fisher CT grade), acute hydrocephalus, cardiac instability, and nosocomial infection. Of Hunt and Hess grade 1 and 2 patients, 21/84 (25.0%) suffered LOC at presentation. Cardiac instability and nosocomial infection were significantly more frequent in these patients. In multivariable analysis, LOC was the predominant predictor of cardiac instability and nosocomial infection. Loss of consciousness at presentation with aSAH is associated with an increased rate of complications, even in good-grade patients. The presence of LOC may identify good-grade patients at risk for complications such as cardiac instability and nosocomial infection.

Olfactory dysfunction in patients undergoing supraorbital keyhole craniotomy for clipping of unruptured aneurysms.

Olfactory performance has rarely been assessed in the perioperative phase of elective aneurysm surgery. Here, we assessed the risk for olfactory deterioration following surgical treatment of unruptured cerebral aneurysm via the supraorbital keyhole craniotomy. A retrospective review of patients with electively treated cerebral aneurysms who underwent perioperative assessment of olfactory function using a sniffin' sticks odor identification test between January 2015 and January 2016 was performed. A subgroup of patients without history of subarachnoid hemorrhage, without prior aneurysm treatment, and confirmed olfactory function underwent supraorbital keyhole craniotomy for aneurysm clipping. Microscopic and endoscopic videos were reviewed for this subgroup. Sixty-four patients who underwent elective aneurysm treatment either via surgical clipping or endovascular aneurysm obliteration were identified. Prior to treatment, 4/64 (6.3%) demonstrated bilateral anosmia. Collectively, 14 patients (21.9%) met subgroup criteria of supraorbital keyhole craniotomy for aneurysm clipping. Here, olfactory performance significantly decreased postoperatively on the side of craniotomy (ipsilateral, P = 0.007), whereas contralateral and bilateral olfactory function remained unaltered (P = 0.301 and P = 0.582, respectively). Consequently, 4/14 patients (28.6%) demonstrated ipsilateral anosmia 3 months after surgery. One patient (1/14, 7.1%) also experienced contralateral anosmia resulting in bilateral anosmia. Intraoperative visualization of the olfactory tract and surgical maneuvers do not facilitate prediction of olfactory outcome. The supraorbital keyhole craniotomy harbors a specific risk for unilateral olfactory deterioration. Lack of perioperative olfactory assessment likely results in underestimation of the risk for olfactory decline. Despite uneventful surgery, prediction of postoperative olfactory function and dysfunction remain challenging. Clin. Anat. 33:316-323, 2020. © 2019 Wiley Periodicals, Inc.

Risk Factors for Acute Ischemic Stroke Caused by Anterior Large Vessel Occlusion.

Background and Purpose- Accurate prediction of acute ischemic stroke (AIS) caused by anterior large vessel occlusion (LVO) that is amendable to mechanical thrombectomy remains a challenge. We developed and validated a prediction model for anterior circulation LVO stroke using past medical history elements present on admission and neurological examination. Methods- We retrospectively reviewed AIS patients admitted between 2009 and 2017 to 3 hospitals within a large healthcare system in the United States. Patients with occlusions of the internal carotid artery or M1 or M2 segments of the middle cerebral artery were randomly split into 2/3 derivation and 1/3 validation cohorts for development of an anterior circulation LVO prediction model and score that was further curtailed for potential use in the prehospital setting. Results- A total of 1654 AIS were reviewed, including 248 (15%) with proximal anterior circulation LVO AIS. In the derivation cohort, National Institutes of Health Stroke Scale score at the time of cerebrovascular imaging, current smoking status, type 2 diabetes mellitus, extracranial carotid, and intracranial atherosclerotic stenosis was significantly associated with anterior circulation LVO stroke. The prehospital score was curtailed to National Institutes of Health Stroke Scale score, current smoking status, and type 2 diabetes mellitus. The areas under the curve for the prediction model, prehospital score, and National Institutes of Health Stroke Scale score alone were 0.796, 0.757, and 0.725 for the derivation cohort and 0.770, 0.689, and 0.665 for the validation cohort, respectively. The Youden index J was 0.46 for a score of >6 with 84.7% sensitivity and 62.0% specificity for the prediction model. Conclusions- Previously reported LVO stroke prediction scores focus solely on elements of the neurological examination. In addition to stroke severity, smoking, diabetes mellitus, extracranial carotid, and intracranial atherosclerotic stenosis were associated with anterior circulation LVO AIS. Although atherosclerotic stenosis may not be known until imaging is obtained, smoking and diabetes mellitus history can be readily obtained in the field and represent important elements of the prehospital score supplementing National Institutes of Health Stroke Scale score.

Perioperative olfactory dysfunction in patients with meningiomas of the anteromedial skull base.

Olfactory dysfunction represents a main symptom in olfactory groove meningiomas (OGM). Besides this, olfactory function has been sparsely investigated in patients suffering from supratentorial meningiomas. Here, the authors explore pre- and postoperative variables associated with olfactory dysfunction in supratentorial meningioma patients. This is a retrospective study on supratentorial meningioma patients who underwent meningioma resection between January 2015 and January 2016. Preoperative and postoperative olfactory performance was quantified using a lateralized sniffin' stick odor identification test. Meningiomas affecting the olfactory system (n = 23) were compared to meningiomas in other locations among the control group (n = 40). Meningiomas that affected the olfactory system had odds of 3.6 and 3.7 in being associated with ipsilateral (lesional) and bilateral anosmia, respectively. Subgroup analysis revealed that meningiomas causing a midline shift across the frontal base and older age represented risk factors for preoperative anosmia. The odds of experiencing acquired postoperative ipsilateral anosmia were significantly increased in olfactory system affecting meningiomas (OR 11.1). Subgroup analysis highlighted OGMs to represent the predominant location associated with deterioration. General surgical complications predisposed patients to loss of contralateral (OR 12.3) and bilateral olfactory function (OR 27.8). Older age and meningiomas causing a midline shift across the frontal base predispose patients to preoperative olfactory dysfunction. Resection of OGMs and surgical complications are risk factors for postoperative olfactory deterioration to anosmia. Likely, olfactory dysfunction is underrecognized even in OGMs. In OGM surgery, however, preoperative lateralized testing might be critical to selecting an appropriate surgical route to preserve olfactory function. Clin. Anat. 32:524-533, 2019. © 2019 Wiley Periodicals, Inc.

Comparison of microscopic and endoscopic view of the internal acoustic meatus: A cadaveric study.

The endoscope is thought to provide an improved exposure of the internal acoustic meatus after retrosigmoid craniotomy for microsurgical resection of intrameatal tumors. The aim of this study is to quantify the differences in internal acoustic meatus (IAM) exposure comparing microscopic and endoscopic visualization. A retrosigmoid approach was performed on 5 cadaver heads. A millimeter gauge was introduced into the internal acoustic meatus, and examinations with a surgical microscope and 0°, 30° and 70° rigid endoscopes were performed. The extent of IAM depth visualized with the microscope and the different angled endoscopes were analyzed. The microscopic view allowed an average IAM depth visualization of 2.8 mm. The endoscope allowed an improved exposure of IAM in all cases. The 0°, 30° and 70° endoscopes permitted an exposure that was respectively 96% (5.5 mm), 139% (6.7 mm) and 200% (8.4 mm) more lateral than the microscopic view. Angled optics, however, provided an image distortion, specifically the 70° endoscope. The endoscope provides a superior visualization of the IAM compared to the microscope when using a retrosigmoid approach. The 30° endoscope represented an ideal compromise of superior visualization with marginal image distortion. Additional implementation of the endoscope into microsurgery of intrameatal tumors likely facilitates complete tumor removal and might spare facial and vestibulocochlear function. Clin. Anat. 31:398-403, 2018. © 2017 Wiley Periodicals, Inc.

The role of endothelial nitric oxide synthase -786 T/C polymorphism in cardiac instability following aneurysmal subarachnoid hemorrhage.

INTRODUCTION: Cardiac abnormalities are observed frequently after aneurysmal subarachnoid hemorrhage (aSAH). A subset of aSAH patients develops neurogenic cardiomyopathy, likely induced by catecholamine excess. Genetic polymorphisms of the endothelial nitric oxide synthase (eNOS) gene have been linked to decreased nitric oxide (NO) levels, coronary artery spasm, and myocardial infarction. The role of the eNOS single nucleotide polymorphism (SNP) -786 T/C in cardiac instability following aSAH has not been previously investigated. METHODS: From 2012 to 2015, aSAH patients were prospectively enrolled in the Cerebral Aneurysm Renin Angiotensin System (CARAS) study at two academic institutions. Blood samples were used to assess the eNOS SNP -786 T/C rs2070744 through 5'exonuclease (Taqman) genotyping assays. Associations between this polymorphism and cardiac instability following aSAH were analyzed. RESULTS: Multivariable analysis demonstrated a dominant effect of the C allele of eNOS SNP -786 T/C on cardiac instability in patients with aSAH. A lower Glasgow Coma Scale score and a history of ischemic vascular disease were also associated with cardiac instability. Furthermore, cardiac instability independently predicted poor functional outcome upon discharge from the hospital. CONCLUSIONS: The C allele of the eNOS SNP -786 T/C was independently associated with an increased risk for cardiac instability following aSAH. Cardiac instability itself was a risk factor for an unfavorable functional outcome upon discharge from the hospital.

Otto Mennicke (1876-) and the first description of skull base anomalies causing cerebellar tonsillar ectopia: one of the first mentions of the Chiari I malformation.

INTRODUCTION: Although Hans Chiari made significant and meaningful contributions to our understanding and classification of hindbrain herniations, others have also contributed to this knowledge. One figure who has been lost to history is Otto Mennicke. Herein, we discuss his role in our understanding of tonsillar ectopia and his life and connection to Hans Chiari. CONCLUSIONS: Our knowledge of what is now known as the Chiari malformations has been shaped by several clinicians including Otto Mennicke.

Preoperative navigated transcranial magnetic stimulation in patients with motor eloquent lesions with emphasis on metastasis.

Navigated transcranial magnetic stimulation (nTMS) is a frequently used, non-invasive method to map the motor cortex. It is of great value in the preoperative workup of patients that suffer from motor eloquent brain lesions. Here, we present a single-center experience using preoperative nTMS in cortical motor eloquent lesions with emphasis on metastasis. All patients that underwent preoperative nTMS between June 2013 and January 2016 were evaluated. A total of 61 patients underwent nTMS before undergoing surgery for a motor eloquent brain lesion. Patients suffered from cerebral metastasis (23), glioblastoma (16), high grade glioma WHO III (4), low grade glioma WHO II (4), lymphoma (2), meningioma (8), cavernous hemangioma (3), or arteriovenous malformation (1). Thirty patients (49.2%) presented with a preoperative motor deficit. One week after surgery, paresis had resolved or improved in 56.7% of the patients. Out of the patients with postoperative paresis, 89.5% experienced an improvement of motor status at follow-up. All metastatic lesions were completely resected compared to 78.9% of non-metastatic lesions (P = 0.02). Only 4.3% of patients with a metastatic lesion, but 26.3% of patients with a non-metastatic lesion experienced deterioration of motor function after surgery (P = 0.04). Preoperative nTMS is suitable for mapping of a variety of motor eloquent brain lesions resulting in favorable neurological outcome. Particularly in metastatic motor eloquent lesion, motor function appears to be preserved after surgery. Clin. Anat. 29:925-931, 2016. © 2016 Wiley Periodicals, Inc.

A family of nonclassical class I MHC genes contributes to ultrasensitive chemodetection by mouse vomeronasal sensory neurons.

The mouse vomeronasal organ (VNO) has a pivotal role in chemical communication. The vomeronasal sensory neuroepithelium consists of distinct populations of vomeronasal sensory neurons (VSNs). A subset of VSNs, with cell bodies in the basal part of the basal layer, coexpress Vmn2r G-protein-coupled receptor genes with H2-Mv genes, a family of nine nonclassical class I major histocompatibility complex genes. The in vivo, physiological roles of the H2-Mv gene family remain mysterious more than a decade after the discovery of combinatorial H2-Mv gene expression in VSNs. Here, we have taken a genetic approach and have deleted the 530 kb cluster of H2-Mv genes in the mouse germline by chromosome engineering. Homozygous mutant mice (ΔH2Mv mice) are viable and fertile. There are no major anatomical defects in their VNO and accessory olfactory bulb (AOB). Their VSNs can be stimulated with chemostimuli (peptides and proteins) to the same maximum responses as VSNs of wild-type mice, but require much higher concentrations. This physiological phenotype is displayed at the single-cell level and is cell autonomous: single V2rf2-expressing VSNs, which normally coexpress H2-Mv genes, display a decreased sensitivity to a peptide ligand in ΔH2Mv mice, whereas single V2r1b-expressing VSNs, which do not coexpress H2-Mv genes, show normal sensitivity to a peptide ligand in ΔH2Mv mice. Consistent with the greatly decreased VSN sensitivity, ΔH2Mv mice display pronounced deficits in aggressive and sexual behaviors. Thus, H2-Mv genes are not absolutely essential for the generation of physiological responses, but are required for ultrasensitive chemodetection by a subset of VSNs.

Relationship between pharyngitis and peri-odontoid pannus: A new etiology for some Chiari I malformations?

The pathophysiology underlying Chiari I malformations (CIMs) provides room for debate with several theories attempting to address this issue. We retrospectively reviewed many of our past patients with pediatric CIMs (specifically, those with peri-odontoid pannus), and present a hypothesis for the development of the malformation in some of said patients. Our experience with the pediatric CIM has shown that almost 1 in 20 patients who present with symptoms is found to have a peri-odontoid pannus. These masses ranged in size from 4 to 11 mm in diameter. Forty percent had a history of clinically significant pharyngitis or pharyngeal abscess. Pannus formation around the dens (odontoid) resulted in ventral compression of the craniocervical junction in each of these patients. Highlighting the hypermobility that causes such lesions, following fusion, the pannus and symptoms in several patients were diminished. Impairment of normal cerebrospinal fluid circulation out of the fourth ventricle and across the craniocervical junction appears to be a plausible endpoint in this discussion and a suitable explanation for some patients with CIM. Still, the mechanisms by which cerebrospinal fluid circulation is compromised may be variable and are not well understood. This is the first study dedicated to the evaluation of pannus formation in the CIM population. We hypothesize that pharyngeal inflammatory conditions contribute to the formation and progression of hindbrain herniation in a small subset of patients with CIMs.

Spinal diffusion tensor imaging: a comprehensive review with emphasis on spinal cord anatomy and clinical applications.

Magnetic resonance imaging technology allows for in vivo visualization of fiber tracts of the central nervous system using diffusion-weighted imaging sequences and data processing referred to as "diffusion tensor imaging" and "diffusion tensor tractography." While protocols for high-fidelity diffusion tensor imaging of the brain are well established, the spinal cord has proven a more difficult target for diffusion tensor methods. Here, we review the current literature on spinal diffusion tensor imaging and tractography with special emphasis on neuroanatomical correlations and clinical applications.

G protein G(alpha)o is essential for vomeronasal function and aggressive behavior in mice.

The rodent vomeronasal organ (VNO) mediates the regulation of species-specific and interspecies social behaviors. We have used gene targeting to examine the role of the G protein Gαo, encoded by the gene Gnao1, in vomeronasal function. We used the Cre-loxP system to delete Gαo in those cells that express olfactory marker protein, which includes all vomeronasal sensory neurons of the basal layer of the VNO sensory epithelium. Using electrophysiology and calcium imaging, we show that the conditional null mice exhibit strikingly reduced sensory responses in V2R receptor-expressing vomeronasal sensory neurons to specific molecular cues, including MHC1 antigens, major urinary proteins, and exocrine gland-secreting peptide. Gαo is also vital for vomeronasal sensing of two N-formylated mitochondrially encoded peptides derived from NADH dehydrogenase 1. Furthermore, we show that Gαo is an essential requirement for the display of male-male territorial aggression as well as maternal aggression in mice. Finally, we show that Gαo-dependent maternal aggression can be induced by major urinary proteins. These cellular and behavioral phenotypes identify Gαo as the primary G-protein α-subunit mediating the detection of peptide and protein pheromones by sensory neurons of the VNO.

Arterial supply of the upper cranial nerves: a comprehensive review.

The arterial supply to the upper cranial nerves is derived from a complex network of branches derived from the anterior and posterior cerebral circulations. We performed a comprehensive literature review of the arterial supply of the upper cranial nerves with an emphasis on clinical considerations. Arteries coursing in close proximity to the cranial nerves regularly give rise to small vessels that supply the nerve. Knowledge of the arteries supplying the cranial nerves is of particular importance during surgical approaches to the skull base.

Arterial supply of the lower cranial nerves: a comprehensive review.

The lower cranial nerves receive their arterial supply from an intricate network of tributaries derived from the external carotid, internal carotid, and vertebrobasilar territories. A contemporary, comprehensive literature review of the vascular supply of the lower cranial nerves was performed. The vascular supply to the trigeminal, facial, vestibulocochlear, glossopharyngeal, vagus, spinal accessory, and hypoglossal nerves are illustrated with a special emphasis on clinical issues. Frequently the external carotid, internal carotid, and vertebrobasilar territories all contribute to the vascular supply of an individual cranial nerve along its course. Understanding of the vasculature of the lower cranial nerves is of great relevance for skull base surgery.