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No physician can successfully deliver high-value patient care in the modern-day health care system in isolation. Delivery of effective patient care requires integrated and collaborative systems that depend on dynamic professional relationships among members of the health care team. An overview of the socioeconomic implications of professional relationships within modern care delivery systems and potential employment models is presented.

Background Intracranial aneurysm formation after Gamma Knife radiosurgery (GKRS) is a rare complication that has only recently been reported in the literature. We report the case of a fatal distal superior cerebellar artery (SCA) aneurysm rupture in a woman treated twice with GKRS for trigeminal neuralgia along with a review of the literature regarding radiation-induced aneurysms. Case Description A 77-year-old white woman with a history of refractory right-sided trigeminal neuralgia treated with GKRS in 2001, and again in 2006 after a relapse, presented to our emergency department with complaints of a sudden-onset severe headache associated with vomiting, right eye vision loss, left-sided facial droop, and left-sided weakness with no history of hypertension or smoking prior to presentation. Initial head computed tomography scan without contrast demonstrated an intraparenchymal hemorrhage centered in the right middle cerebellar peduncle with subarachnoid hemorrhage in the basal cisterns and extension into the fourth ventricle causing early hydrocephalus. Head computed tomography angiography (CTA) demonstrated a distal right SCA aneurysm adjacent to the hemorrhage. The patient's mental status deteriorated into coma after suspected rerupture during the CTA requiring immediate intubation, external ventricular drain placement, and emergent cerebral angiogram with coil embolization. Ultimately, the patient never recovered despite medical and surgical management; therefore, care was withdrawn in accordance with her known wishes. Conclusions The pathophysiologic association of aneurysm formation after GKRS remains to be elucidated, but given the potentially fatal consequences of aneurysm rupture, we advocate for further research and propose serial vascular imaging during the postradiosurgery follow-up period for iatrogenic aneurysm formation surveillance.

Background Colloid cysts of the third ventricle are rare, slow-growing lesions of neuroepithelial origin. Although histopathologically benign, third ventricular colloid cysts are a well-known cause of sudden, unexpected coma and death. Several theories have been proposed to describe the rare, sudden onset of severe symptomatology and rapid clinical decline due to colloid cysts. Moreover, there is currently no clear consensus regarding the most suitable neurosurgical technique or the most effective approach for resection, nor do guidelines or standardized clinical indications exist to recommend observation versus intervention and/or resection in the asymptomatic patient. To highlight this conundrum, we present a clinical case of a hemorrhagic colloid cyst that resulted in fatality and discuss the relevant literature. Case Description A 21-year-old male presented to an outside hospital with an acute onset of a severe headache, nausea, and vomiting, which started while jogging. A few hours later, he precipitously declined into a comatose state. Radiographic imaging demonstrated a hemorrhagic third ventricular colloid cyst causing acute obstructive hydrocephalus, severe increased intracranial pressure, and downward herniation. He was then transferred to our institution for neurosurgical intervention. Despite aggressive cerebrospinal fluid drainage and medical management, he was declared brain dead several days later. Conclusions To our knowledge, this is the only case report to describe sudden-onset coma and death due to a hemorrhagic third ventricular colloid cyst occurring after exercising. We review the literature on fatal colloid cysts emphasizing the proposed mechanisms of sudden death and current neurosurgical management in both asymptomatic and symptomatic patients.

Objective To investigate the association between immunologic counts on admission and acute symptomatic hydrocephalus after aneurysmal subarachnoid hemorrhage (aSAH). Methods We conducted a retrospective analysis of 143 consecutive patients with aSAH. Patient demographics, clinical parameters, laboratory values, and radiographic imaging were obtained. Univariate and multivariate logistic regression analyses were performed to investigate parameters independently associated with acute symptomatic hydrocephalus. Receiver operating characteristic (ROC) curve analysis determined the best threshold value of neutrophil count to differentiate patients with and without hydrocephalus. Results Overall, acute symptomatic hydrocephalus developed in 39.16% of patients. In an adjusted multivariate logistic regression model, Hunt and Hess grade 4–5 (odds ratio [OR]: 16.052, 95% confidence interval [CI]: 1.188–216.983; P = 0.037), modified Fisher score 3–4 (OR: 10.107, 95% CI: 1.715–59.572; P = 0.011), intraventricular hemorrhage (OR: 4.578, 95% CI: 1.417–14.788; P = 0.011), neutrophil count (OR: 1.183, 95% CI: 1.033–1.354; P = 0.015), and prior ischemic stroke (OR: 7.003, 95% CI: 1.293–37.929; P = 0.024) were significantly associated with hydrocephalus. ROC analysis for neutrophil count confirmed an acceptable area under the curve (AUC 0.780, 95% CI: 0.701–0.859; P Conclusions Neutrophil count ≥9.80 × 103/mL on admission predicts acute symptomatic hydrocephalus after aSAH in an adjusted multivariate logistic regression model. Moreover, shunt dependence was associated with higher neutrophil counts.

Focal traumatic brain injury (TBI) induces astrogliosis, a process essential to protecting uninjured brain areas from secondary damage. However, astrogliosis can cause loss of astrocyte homeostatic functions and possibly contributes to comorbidities such as posttraumatic epilepsy (PTE). Scar-forming astrocytes seal focal injuries off from healthy brain tissue. It is these glial scars that are associated with epilepsy originating in the cerebral cortex and hippocampus. However, the vast majority of human TBIs also present with diffuse brain injury caused by acceleration-deceleration forces leading to tissue shearing. The resulting diffuse tissue damage may be intrinsically different from focal lesions that would trigger glial scar formation. Here, we used mice of both sexes in a model of repetitive mild/concussive closed-head TBI, which only induced diffuse injury, to test the hypothesis that astrocytes respond uniquely to diffuse TBI and that diffuse TBI is sufficient to cause PTE. Astrocytes did not form scars and classic astrogliosis characterized by upregulation of glial fibrillary acidic protein was limited. Surprisingly, an unrelated population of atypical reactive astrocytes was characterized by the lack of glial fibrillary acidic protein expression, rapid and sustained downregulation of homeostatic proteins and impaired astrocyte coupling. After a latency period, a subset of mice developed spontaneous recurrent seizures reminiscent of PTE in human TBI patients. Seizing mice had larger areas of atypical astrocytes compared with nonseizing mice, suggesting that these atypical astrocytes might contribute to epileptogenesis after diffuse TBI.SIGNIFICANCE STATEMENTTraumatic brain injury (TBI) is a leading cause of acquired epilepsies. Reactive astrocytes have long been associated with seizures and epilepsy in patients, particularly after focal/lesional brain injury. However, most TBIs also include nonfocal, diffuse injuries. Here, we showed that repetitive diffuse TBI is sufficient for the development of spontaneous recurrent seizures in a subset of mice. We identified an atypical response of astrocytes induced by diffuse TBI characterized by the rapid loss of homeostatic proteins and lack of astrocyte coupling while reactive astrocyte markers or glial scar formation was absent. Areas with atypical astrocytes were larger in animals that later developed seizures suggesting that this response may be one root cause of epileptogenesis after diffuse TBI.

Background: Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. We conducted a literature review to provide insight into the history, clinical significance, and neurosurgical management of this recently described novel cerebral arteriopathy. Summary: We performed a literature search using PubMed with the key words “ACTA2 mutation,” “ACTA2 cerebral arteriopathy,” and “multisystemic smooth muscle dysfunction syndrome.” Case reports with confirmed ACTA2 mutations and cerebral arteriopathy were included in our review. Our literature search revealed 15 articles (58 cases) of confirmed ACTA2 cerebral arteriopathy. Distinctive features of this arteriopathy included an aberrant internal carotid circulation with dilatation of the proximal segments, occlusive disease at the distal segments, and dolichoectasia. As such, mutant ACTA2 predisposed patients to ischemic strokes as children. Direct and indirect cerebral revascularization procedures are the mainstay treatment options with varying degrees of success. Key Messages: ACTA2 cerebral arteriopathy is a recently described novel cerebrovascular disease seen in patients with MSMDS. Patients currently diagnosed with moyamoya disease who also have dysfunction of smooth muscle organs may benefit from reevaluation by a medical geneticist and ACTA2 genotyping.

Background Acute subdural hemorrhage often occurs in those ≥65 years of age after trauma and tends to yield poor clinical outcomes. Previous studies have demonstrated a propensity toward high in-hospital mortality rates in this population; however, postdischarge mortality data are limited. The objective of the present study was to analyze short- and long-term mortality data after acute traumatic subdural hemorrhage in the geriatric population as well as review the impact of associated clinical variables including mechanism of injury, pre-morbid antithrombotic use, and need for surgical decompression on mortality rates. Methods We retrospectively reviewed 455 patients who presented with an isolated traumatic acute subdural hemorrhage to our level-1 trauma center over a 5 year period using our data registry. Patients were then cross-referenced in the National Social Security Death Index for postdischarge mortality rates. United States life tables were used for peer-controlled actuarial comparisons. Results Acute traumatic subdural hemorrhage is often a fatal injury in the geriatric population, especially if taking antithrombotics or requiring surgical decompression. Specifically, they have greater in-hospital mortality rates than adults with similar injuries and have significantly lower survival rates for several years following discharge compared with their peer-matched controls. Conclusions Here, we found that age is a significant predictor of both short- and long-term survival after acute traumatic subdural hemorrhage. Moreover, the present study corroborates that the need for surgical decompression or the use of pre-morbid antithrombotic medications is associated with increased overall mortality.

Background: Fourth ventricular outlet obstruction is an infrequent but well-established cause of tetraventricular hydrocephalus characterized by marked dilatation of the ventricular system with ballooning of the foramina of Monro, Magendie, and Luschka. Multiple processes including inflammation, infection, hemorrhage, neoplasms, or congenital malformations are known to cause this pathological obstruction. However, true idiopathic fourth ventricular outlet obstruction is a rare phenomenon with only a limited number of cases reported in the literature. Case Description: A 61-year-old female presented with several months of unsteady gait, intermittent headaches, confusion, and episodes of urinary incontinence. Conventional magnetic resonance imaging demonstrated tetraventricular hydrocephalus without transependymal flow, but with ventral displacement of the brainstem and dorsal displacement of the cerebellum without an obvious obstructive lesion on pre- or post-contrast imaging prompting a diagnosis of normal pressure hydrocephalus. However, constructive interference in steady state (CISS) and half-Fourier acquisition single-shot turbo spin echo (HASTE) sequences followed by fluoroscopic dynamic cisternography suggested encystment of the fourth ventricle with thin margins of arachnoid membrane extending through the foramina of Luschka bilaterally into the pontocerebellar cistern. Operative intervention was pursued with resection of an identified arachnoid web. Postoperative imaging demonstrated marked reduction in the size of ventricular system, especially of the fourth ventricle. The patient’s symptomatology resolved a few days after the procedure. Conclusion: Here, we describe an idiopathic case initially misdiagnosed as normal pressure hydrocephalus. The present case emphasizes the necessity of CISS sequences and fluoroscopic dynamic cisternography for suspected cases of fourth ventricular outlet obstruction as these diagnostic tests may guide surgical management and lead to superior patient outcomes.

Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. We conducted a literature review to provide insight into the history, clinical significance, and neurosurgical management of this recently described novel cerebral arteriopathy.We performed a literature search using PubMed with the key words "ACTA2 mutation," "ACTA2 cerebral arteriopathy," and "multisystemic smooth muscle dysfunction syndrome." Case reports with confirmed ACTA2 mutations and cerebral arteriopathy were included in our review. Our literature search revealed 15 articles (58 cases) of confirmed ACTA2 cerebral arteriopathy. Distinctive features of this arteriopathy included an aberrant internal carotid circulation with dilatation of the proximal segments, occlusive disease at the distal segments, and dolichoectasia. As such, mutant ACTA2 predisposed patients to ischemic strokes as children. Direct and indirect cerebral revascularization procedures are the mainstay treatment options with varying degrees of success. Key Messages: ACTA2 cerebral arteriopathy is a recently described novel cerebrovascular disease seen in patients with MSMDS. Patients currently diagnosed with moyamoya disease who also have dysfunction of smooth muscle organs may benefit from reevaluation by a medical geneticist and ACTA2 genotyping.

Glioblastoma is a highly aggressive neoplasm with an extremely poor prognosis. Despite maximal gross resection and chemoradiotherapy, these grade IV astrocytomas consistently recur. Glioblastoma cells exhibit numerous pathogenic mechanisms to decrease tumor immunogenicity while promoting gliomagenesis, which manifests clinically as a median survival of less than 2 years and few long-term survivors. Recent clinical trials of vaccine-based immunotherapeutics against glioblastoma have demonstrated encouraging results in prolonging progression-free survival and overall survival. Several vaccine-based treatments have been trialed, such as peptide and heat-shock proteins, dendritic cell-based vaccines, and viral-based immunotherapy. In this literature review, we discuss the immunobiology of glioblastoma, significant current and completed vaccine-based immunotherapy clinical trials, and broad clinical challenges and future directions of glioblastoma vaccine-based immunotherapeutics.

Background: Pseudotumor cerebri (PTC), also known as idiopathic intracranial hypertension (IIH), is a condition associated with increased intracranial pressure (ICP) in the absence of radiographic findings such as mass lesions or cerebral edema. Case Description: We describe a case of progressive headache and visual disturbances attributed to PTC that resulted from subacute superior sagittal sinus (SSS) stenosis by a metastatic tumor. Conclusions: Venous outflow obstruction often presents with an acute symptomatology including infarcts, hemorrhages, and seizures, but only rarely does it cause the progressive development of raised ICP. The sinister presentation of our patient's pathology stemmed from local mass effect caused by a tumor that has hitherto not been reported to cause intracranial hypertension (IH) and was best elucidated using magnetic resonance venography (MRV).

Background:Cauda equinasyndrome (CES) is an uncommon entity that presents acutely with all or some of the following symptoms; urinary incontinence from retention, fecal incontinence from loss of sphincter tone, saddle area hypoesthesia or anesthesia, and acute or progressive weakness in one or both lower extremities. The protean symptomatology is often mixed and is vulnerable to confounding comorbidities making the accurate and timely diagnosis of this syndrome uniquely challenging. Here, we present the case of a man who developed isolated sacral nerve dysfunction from CES in the midst of a diabetic crisis.Case Description:A 53-year-old male with a long history of uncontrolled Type 2 diabetes presented with acute-onset urinary and fecal incontinence, scrotal anesthesia, and a 3-day history of lower back pain with intermittent bilateral leg pain. This patient displayed no objective changes in leg strength, sensation, or reflexes. In addition, the patient tested positive for cocaine and had a blood glucose level of 800 mg/dL which confounded his clinical picture. The patient underwent bilateral laminectomies from L4–S1 with the removal of a large sequestered disc fragment from the S1–S2 disc space within 8 h of presentation with reasonable recovery.Conclusion:Highly variable presentations often confound the accurate and timely diagnosis of CES with severe implications on quality of life. Despite the limited functional recovery seen after surgical decompression, urgent or emergent intervention is paramount for treatment. Our patient’s presenting symptomatology and comorbidities highlight the need for practitioners to maintain a high index of suspicion in anyone with incontinence and back pain, regardless of distractors and even in the absence of other anticipated motor or sensory findings.