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Chordomas are centrally located, expansile soft tissue neoplasms that arise from the remnants of the embryological notochord. Hemorrhagic presentation is exceedingly rare and can resemble pituitary apoplexy. Moreover, a purely intrasellar location of a chordoma is extremely uncommon. We report a case of a hemorrhagic intrasellar chordoma in an adult male, which presented similarly to pituitary apoplexy and was resolved with surgical resection.A 69-year-old male presented with a 4 week history of acute onset headache and concurrent diplopia, with significantly reduced testosterone and slightly reduced cortisol. His left eye demonstrated a sixth cranial nerve palsy. Magnetic resonance imaging of the brain showed a large hemorrhagic mass in the pituitary region with significant compression of the left cavernous sinus and superior displacement of the pituitary gland. The patient underwent an endoscopic endonasal transsphenoidal approach for the resection of the lesion. Near total resection was achieved. Final pathology revealed chordoma with evidence of intratumoral hemorrhage, further confirmed by immunopositive stain for brachyury. Post-operatively, the patient had improved diplopia and was discharged home on low dose hydrocortisone. At 3-month follow-up, his diplopia was resolved and new MRI showed stable small residual disease.Apoplectic chordomas are uncommon given chordoma's characteristic lack of intralesional vascularity and represent a diagnostic challenge in the sellar region. Our unique case demonstrates that despite our initial impression of pituitary apoplexy, this was ultimately a case of apoplectic chordoma that responded well to endoscopic endonasal surgery.

Objective In this systematic review, preoperative educational interventions for patients undergoing neurosurgical treatment are identified and their impact on patient knowledge acquisition and satisfaction is assessed. Methods The review was conducted in accordance with the PRISMA guidelines and used PubMed, Google Scholar, and MEDLINE databases. Studies evaluating before and after cohort or control group comparison were identified between 2007 and 2019 and were independently scored and evaluated by 3 authors. Results Eighty-one articles were assessed for eligibility and 15 met the inclusion criteria. Patient educational interventions were text-based (2 studies), multimedia/video-based (3), mobile/tablet-based (5), or used virtual reality (2) or three-dimensional printing (3). Interventions were disease-specific for cerebrovascular lesions (5), degenerative spine disease (2), concussion/traumatic brain injury (2), movement disorders (1), brain tumor (1), adolescent epilepsy (1), and other cranial/spinal elective procedures (3). Eleven studies (n = 18–175) documented patient knowledge acquisition using self-reported knowledge questionnaires (5) or more objective assessments based on true/false or multiple-choice questions (6). Most studies (10/11) reported statistically significant increases in patient knowledge after implementation of the intervention. Ten studies (n = 14–600) documented patient satisfaction using validated satisfaction surveys (2), Likert scale surveys (6), or other questionnaires (2). Although all studies reported increases in patient satisfaction after the intervention, only 4 were statistically significant. Conclusions Patient educational interventions using various modalities are broadly applicable within neurosurgery and ubiquitously enhance patient knowledge and satisfaction. Interventions should be implemented when possible.

A 13-year-old female patient was diagnosed with a tectal glioma (TG), a subgroup of astrocytoma that can result in obstructive hydrocephalus secondary to aqueductal stenosis. Endoscopic third ventriculostomy (ETV) is used to treat this type of hydrocephalus with a good success rate. Our institution performs ETV and Ommaya reservoir (OR) placement in these cases. The OR allows measurement of intracranial pressure (ICP) and cerebrospinal fluid (CSF) access and a method for performing ventricular dye studies to evaluate third ventricular stoma (TVS) patency. In this case, a porencephalic cyst (PC) developed around the OR’s ventricular catheter (OVC) two and a half months after surgery. The PC is thought to have developed in association with TVS stoma closure and resolved after ETV revision.

BackgroundNeuroimaging studies have shown gray matter structural and functional alterations in patients with idiopathic blepharospasm (iBSP) but with variations. Here we aimed to investigate the specific and common neurostructural/functional abnormalities in patients with iBSP.MethodsA systematic literature search from PubMed, Web of Science and Embase was conducted to identify relevant publications. We conducted separate meta-analysis for whole-brain voxel-based morphometry (VBM) studies and for functional imaging studies, and a multimodal meta-analysis across VBM and functional studies in iBSP, using anisotropic effect size-based signed differential mapping.ResultsThe structural database comprised 129 patients with iBSP and 144 healthy controls whilst the functional database included 183 patients with iBSP and 253 healthy controls. The meta-analysis of VBM studies showed increased gray matter in bilateral precentral and postcentral gyri, right supplementary motor area and bilateral paracentral lobules, while decreased gray matter in right superior and inferior parietal gyri, left inferior parietal gyrus, left inferior temporal gyrus, left fusiform gyrus and parahippocampal gyrus. The meta-analysis of functional studies revealed hyperactivity in right dorsolateral superior frontal gyrus, left thalamus and right fusiform gyrus, while hypoactivity in left temporal pole, left insula, left precentral gyrus, bilateral precuneus and paracentral lobules, right supplementary motor area and middle frontal gyrus. The multimodal meta-analysis identified conjoint anatomic and functional changes in left precentral gyrus, bilateral supplementary motor areas and paracentral lobules, right inferior occipital gyrus and fusiform gyrus.ConclusionsThe patterns of conjoint and dissociated gray matter alterations identified in the meta-analysis may enhance our understanding of the pathophysiological mechanisms underlying iBSP.

Neuroimaging studies have identified both brain structural and functional abnormalities in patients with idiopathic cervical dystonia (iCD), but often yield diverse results. We aim to find the specific and common neurostructural/functional abnormalities in iCD by conducting separate and multimodal meta-analyses across structural and functional neuroimaging studies.A systematic literature search was conducted to identify relevant publications. Separate meta-analysis for whole-brain voxel-based morphometry (VBM) studies and for functional imaging studies, and a multimodal meta-analysis across VBM and functional studies in iCD were conducted using anisotropic effect size-based signed differential mapping.We included twenty-seven studies, including nine structural datasets comprising 152 iCD patients and 188 healthy controls, and seventeen functional datasets describing 352 iCD patients and 296 healthy controls. The multimodal analysis showed overlap between anatomic and functional changes in bilateral precentral and postcentral gyri, bilateral paracentral lobules, right supplementary motor area, bilateral median cingulate/paracingulate gyri, right caudate nucleus and thalamus, right cerebellum and lingual gyrus. We also found gray matter alterations alone in bilateral dorsolateral superior frontal gyri, left middle temporal gyrus, right inferior parietal gyrus, and left cerebellum; and altered functional activity alone in right fusiform gyrus, bilateral precuneus.The current meta-analyses revealed significant conjoint and dissociated brain structural and functional abnormalities in iCD, and emphasized the involvement of cortico-thalamo-basal ganglia circuit and cerebellum.

Sinus pericranii (SP) are abnormal vascular connections between extracranial scalp venous channels and intracranial dural sinuses. This vascular abnormality rarely results in significant sequelae, but in select cases, it can be symptomatic. We describe the case of a 7-year-old girl with an SP who experienced intermittent visual, motor, and sensory symptoms not previously described in the literature. Her symptoms resolved after surgical treatment of the SP. We propose a mechanism for her symptoms and the rationale for the role of neurosurgical intervention along with a review of the literature.

Background and purpose Historically, overall outcomes for patients with high-grade subarachnoid hemorrhage (SAH) have been poor. Generally, between physicians, either reluctance to treat, or selectivity in treating such patients has been the paradigm. Recent studies have shown that early and aggressive care leads to significant improvement in survival rates and favorable outcomes of grade V SAH patients. With advancements in both neurocritical care and end-of-life care, non-treatment or selective treatment of grade V SAH patients is rarely justified. Current paradigm shifts towards early and aggressive care in such cases may lead to improved outcomes for many more patients. Materials and methods We performed a detailed review of the current literature regarding neurointensive management strategies in high-grade SAH, discussing multiple aspects. We discussed the neurointensive care management protocols for grade V SAH patients. Results Acutely, intracranial pressure control is of utmost importance with external ventricular drain placement, sedation, optimization of cerebral perfusion pressure, osmotherapy and hyperventilation, as well as cardiopulmonary support through management of hypotension and hypertension. Conclusions Advancements of care in SAH patients make it unethical to deny treatment to poor Hunt and Hess grade patients. Early and aggressive treatment results in a significant improvement in survival rate and favorable outcome in such patients.

Cavernous malformations (CMs) account for approximately 5% to 10% of all CNS vascular malformations, and intraventricular CMs (IVCMs) are a rare subtype, accounting for 2.5% to 10.8% of all intracranial CMs. IVCMs can expand rapidly, leading to compression of adjacent structures, intraventricular hemorrhage, and obstructive hydrocephalus. Diagnosis is challenging because it can mimic a variety of other lesions.A 71-year-old man presented after a fall because of imbalance. MRI of the head showed a homogenously enhancing 2-cm mass in the posterior aspect of the right lateral ventricle, with blood layering in the right occipital horn and adjacent parietal edema and leptomeningeal enhancement, as well as a pituitary lesion.The patient underwent a right parietal craniotomy for resection of the mass. The ventricle was accessed through a transsulcal approach through the intraparietal sulcus using a tubular retractor system. The mass was arising from the choroid plexus and dissected free in a piecemeal fashion. Postoperative imaging confirmed gross total resection, and the patient had an uneventful recovery.Here, we present the first case of a choroid plexus IVCM removed using a tubular retractor system. We demonstrate that this is a safe and effective approach for this rare lesion given the minimal traction on brain parenchyma and enhanced visualization of a deep-seated cavernoma in the lateral ventricle.

Aneurysmal subarachnoid hemorrhage (aSAH) is an emergent neurosurgical condition associated with high morbidity and mortality. The prognostic significance of baseline frailty status in aSAH patients has not been previously evaluated in a large, nationally representative sample.Clinical outcomes data from the National Inpatient Sample from 2010-2018 were compared among sub-cohorts stratifying admissions by increasing frailty thresholds [(assessed using the 11-point modified frailty index (mFI-11)]. The previously validated NIS-SAH Severity Score (NIS-SSS) and NIS-SAH Outcome Measure (NIS-SOM) were utilized. Complex samples multivariable logistic regression and receiver operating characteristic (ROC) curve analyses were performed to assess adjusted associations and discrimination of frailty for endpoints.Among 64,102 aSAH hospitalizations (mean age 55.4 years), 20.4% of admissions were classified as robust (mFI=0), 43.4% as pre-frail (mFI = 1), 24.9% as frail (mFI = 2), and 11.2% as severely frail (mFI ≥ 3). Following multivariable analysis adjusting for age and aSAH severity, increasing frailty was independently associated with NIS-SOM (OR = 1.15, 95% CI 1.09-1.21; p0.001), extended length of hospital stay (eLOS) (OR = 1.08, 1.02-1.13; p = 0.008), neurological complications (OR = 1.08, 1.03-1.13; p0.001), and medical complications (OR = 1.14, 1.08-1.21; p0.001). Based on ROC curve analysis, frailty achieved an AUC of 0.59 (0.58-0.60) and 0.54 (0.53-0.55) for NIS-SOM and eLOS, respectively. Age and NIS-SSS demonstrated significantly greater discrimination for NIS-SOM [AUC 0.69 (0.68-0.70) and 0.79 (0.78-0.80), respectively), while NIS-SSS achieved significantly greater discrimination for eLOS [(AUC 0.74 (0.73-0.75)] in comparison to both age and frailty.This national database evaluation of frailty in aSAH patients demonstrates an independent association between increasing frailty and poor functional outcome. Age and aSAH severity, however, may be more robust prognostic factors.

False localizing signs (FLS) and other misleading neurological signs have long been an intractable aspect of neurocritical care. Because they suggest an incorrect location or etiology of the pathological lesion, they have often led to misdiagnosis and mismanagement of the patient. Here, we reviewed the existing literature to provide an updated, comprehensive descriptive review of these difficult to diagnose signs in neurocritical care. For each sign presented, we discuss the non-false localizing presentation of symptoms, the common FLS or misleading presentation, etiology/pathogenesis of the sign, and diagnosis, as well as any other clinically relevant considerations. Within cranial neuropathies, we cover cranial nerves III, IV, V, VI, VII, VIII, as well as multiple cranial nerve involvement of IX, X, and XII. FLS ophthalmologic symptoms indicate diagnostically challenging neurological deficits, and here we discuss downbeat nystagmus, ping-pong-gaze, one-and-a-half syndrome, and wall-eyed bilateral nuclear ophthalmoplegia (WEBINO). Cranial herniation syndromes are integral to any discussion of FLS and here we cover Kernohan's notch phenomenon, pseudo-Dandy Walker malformation, and uncal herniation. FLS in the spinal cord have also been relatively well documented, but in addition to compressive lesions, we also discuss newer findings in radiculopathy and disc herniation. Finally, pulmonary syndromes may sometimes be overlooked in discussions of neurological signs but are critically important to recognize and manage in neurocritical care, and here we discuss Cheyne-Stokes respiration, cluster breathing, central neurogenic hyperventilation, ataxic breathing, Ondine's curse, and hypercapnia. Though some of these signs may be rare, the framework for diagnosing and treating them must continue to evolve with our growing understanding of their etiology and varied presentations.

Aneurysmal subarachnoid hemorrhage (aSAH) is devastating, with delayed cerebral ischemia (DCI) significantly contributing to the high morbidity and mortality rates. Cholesterol has been studied as a measure of nutritional status in other neurological pathologies, but reports examining cholesterol's effects on aSAH outcomes are sparse. This study aimed to elucidate the effect of low total cholesterol (TC) and high density lipoprotein (HDL) on mortality and DCI following aSAH.We performed a retrospective cohort study at a quaternary academic medical center between June 2014 and July 2018. All patients had aSAH confirmed by digital subtraction angiography and had TC measured on admission. Primary outcomes were mortality and DCI. Secondary outcome was radiographic vasospasm. Univariate and multivariate logistic regressions were performed.There were 75 aSAH patients, with an average age of 58.7 ± 1.7 (range: 14-89) and HuntHess score of 2.8 ± 0.1, included for analysis. Those with a low TC 160 mg/dL had 3 times increased odds of DCI (OR = 3.4; 95 %CI: 1.3-9.0; p = 0.0175) and a nearly 5 times increased odds of death (OR = 4.9; 95 %CI: 1.1-18.3; p = 0.0339). Low HDL 40 mg/dL was associated with 12 times increased odds of DCI (OR = 12.3; 95 %CI: 2.7-56.4; p = 0.0003) but no significant differences in death (p = 0.2205). In multivariate analysis, low TC was an independent risk factor for increased mortality (OR = 5.6; 95 %CI: 1.2-27.6; p = 0.0335) while low HDL was associated with increased risk for DCI (OR = 17.9; 95 %CI: 3.1-104.4; p = 0.0013). There was no effect of TC or HDL on radiographic vasospasm.Low TC and HDL are independent predictors of increased mortality and DCI, respectively, following aSAH. Low TC and HDL may be markers of poor overall health, in addition to having some pathophysiological effect on cerebral vasculature. These results may have practical implications for the improvement of aSAH prognostication and management.

Background: Arachnoid cysts (AC) may cause hydrocephalus and neurological symptoms, necessitating surgical intervention. Cyst drainage may result in postoperative complications, however, these interventions are not normally associated with the subsequent development of acute hydrocephalus. Herein, we present two unique cases of AC drainage with postoperative development of acute communicating hydrocephalus. Case Description: Case 1. A 75-year-old female presented with progressive headaches, cognitive decline, and questionable seizures. Her neurological examination was non-focal, but a head computed tomography scan (CT) identified a large right frontal AC with mass effect. She subsequently underwent craniotomy and decompression of the cyst. Postoperatively, her neurological examination deteriorated, and a head CT demonstrated new communicating hydrocephalus. The opening pressure was elevated upon placement of an external ventricular drain. Her hydrocephalus improved on follow-up imaging, but her neurological examination failed to improve, and she ultimately expired. Case 2. A 61-year-old female presented with headache and seizures attributed to a left parietal AC. She underwent open craniotomy for fenestration of the cyst into the Sylvian fissure. Postoperatively, her neurologic examination deteriorated, and she developed acute communicating hydrocephalus. She was initially managed with external ventricular drainage (EVD). The hydrocephalus resolved after several days, and the EVD was subsequently removed. Late follow-up imaging at 2 years showed that the regression of the AC was maintained. Conclusion: Acute development of hydrocephalus is a potential complication of intracranial AC fenestration. A better understanding of intracranial cerebrospinal fluid flow dynamics may better inform as to the underlying cause of this complication.

Increasing age has been associated with worse outcomes following aneurysmal subarachnoid hemorrhage (aSAH), yet frailty's effect on aSAH outcomes has never been studied. The most common frailty measurement tool is the modified frailty index (mFI). The goal of this study is to compare the effect of frailty versus age as predictors of aSAH outcomes and mortality.Our institutional aSAH series were retrospectively identified and divided into non-frail (mFI = 0-1) and frail (mFI≥2) cohorts based on admission mFI scores. Primary outcomes were mortality and discharge location. Univariate and multivariate analysis were performed.There were 217 aSAH patients identified and 57 were frail (26.3%). Forty-one (18.9%) patients died and 74 (34%) were discharged home. Frail patients were significantly older (p 0.0001) and had higher HuntHess (HH) (p = 0.005) and Fisher (p = 0.0255) scores. Frail patients were less likely to receive an intervention (OR = 0.3; 95%CI:0.1-0.6); p = 0.0056), be discharged home (OR = 0.32; 95%CI:0.16-0.68; P = 0.0020), and were more likely to expire (OR = 2.4; 95%CI:1.2-5; P = 0.0183) and develop a complication (OR = 2.6; 95%CI:1.1-6.6; P = 0.0277). Multivariate regressions showed that the HH score (OR = 2.7; 95%CI: 1.9-3.0; P 0.0001) followed by age≥65 (OR = 2.7; 95%CI:1.2-6.0; p = 0.012) were the only independent predictors of mortality. Likewise, discharge home was best predicted by HH score (OR = 0.24; 95%CI:0.15-0.37; p 0.0001) and age (OR = 0.25; 95%CI:0.1-0.6; p = 0.003).Frailty is associated with worse aSAH grades, more complications, and increased mortality, however, increasing age and HH scores were the only independent predictors of aSAH outcomes. This study suggests that HH score and increasing patient age, and not the accumulated co-morbidities at the time of aSAH, better predict outcomes.