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BACKGROUND Intracranial fusiform aneurysms are circumferential dilations of cerebral arteries that can lead to complications including ischemic stroke due to vessel occlusion, subarachnoid hemorrhage, or intracerebral hemorrhage. Treatment options for fusiform aneurysms have expanded significantly in recent years. Microsurgical treatment options include proximal and distal surgical occlusion and microsurgical trapping of the aneurysm, usually in association with high-flow bypass procedures. Endovascular treatment options include the placement of coils and/or flow diverters. OBSERVATIONS Here the authors report a case of aggressive surveillance and treatment of a man with multiple progressive, recurrent, and de novo fusiform aneurysms of the left anterior cerebral circulation over 16 years. Because the long-term course of his treatment coincided with the recent expansion of endovascular treatment options, he underwent every type of treatment listed above. LESSONS This case demonstrates the wide range of therapeutic options for fusiform aneurysms and how the treatment model for these lesions has evolved.

Background and purpose Acute ischemic stroke has increasingly become a procedural disease following the demonstrated benefit of mechanical thrombectomy (MT) for emergent large vessel occlusion (ELVO) on clinical outcomes and tissue salvage in randomized trials. Given these data and anecdotal experience of decreased numbers of decompressive hemicraniectomies (DHCs) performed for malignant cerebral edema, we sought to correlate the numbers of strokes, thrombectomies, and DHCs performed over the timeline of the 2013 failed thrombolysis/thrombectomy trials, to the 2015 modern randomized MT trials, to post-DAWN and DEFUSE 3. Materials and methods This is a multicenter retrospective compilation of patients who presented with ELVO in 11 US high-volume comprehensive stroke centers. Rates of tissue plasminogen activator (tPA), thrombectomy, and DHC were determined by current procedural terminology code, and specificity to acute ischemic stroke confirmed by each institution. Endpoints included the incidence of stroke, thrombectomy, and DHC and rates of change over time. Results Between 2013 and 2018, there were 55,247 stroke admissions across 11 participating centers. Of these, 6145 received tPA, 4122 underwent thrombectomy, and 662 patients underwent hemicraniectomy. The trajectories of procedure rates over time were modeled and there was a significant change in MT rate ( p = 0.002) without a concomitant change in the total number of stroke admissions, tPA administration rate, or rate of DHC. Conclusions This real-world study confirms an increase in thrombectomy performed for ELVO while demonstrating stable rates of stroke admission, tPA administration and DHC. Unlike prior studies, increasing thrombectomy rates were not associated with decreased utilization of hemicraniectomy.

Ventriculoperitoneal (VP) shunts are the most common treatment for hydrocephalus in both pediatric and adult patients. Complications resulting from the abdominal portion of shunts include tube disconnection, obstruction of the shunt tip, catheter migration, infection, abdominal pseudocysts, and bowel perforation. However, other less common complications can occur. The authors present a unique case of a patient with a longstanding VP shunt presenting with an acute abdomen secondary to knotting of the peritoneal portion of the catheter tubing. A 13-year-old male with past medical history significant for myelomeningocele, requiring ventriculoperitoneal shunt placement at 18 months of age, presented to an outside hospital with chief complaint of abdominal pain. Cross-sectional imaging revealed spontaneous knot formation within the shunt tubing around the base of the small bowel mesentery. He was then transferred to our facility for general and neurosurgical evaluation. His abdominal exam was notable for diffuse distension in addition to tenderness to palpation with guarding and rebound. Given his tenuous clinical status and peritonitis, he was emergently booked for abdominal exploration. He underwent bowel resection, externalization of his shunt, with later re-anastomosis and shunt internalization. He eventually made a full recovery. Given the potential for significant bowel loss with this and other shunt-related complications, this case serves as a reminder that even longstanding VP shunts should be considered in the differential diagnosis of abdominal pain in any patient with a shunt.

OBJECTIVE Loeys-Dietz syndrome (LDS) is a rare autosomal dominant condition characterized by aneurysms of the aorta, aortic branches, and intracranial arteries; skeletal and cutaneous abnormalities; and craniofacial malformations. Previous authors have reported that higher craniofacial severity index (CFI) scores, which indicate more severe craniofacial abnormalities, correlate with the severity of aortic aneurysm pathology. However, the association between syndromic features and the formation of intracranial aneurysms in LDS patients has yet to be determined. In this study, the authors evaluate the incidence of phenotypic abnormalities, craniofacial features, and Chiari malformation type I (CM-I) in a large LDS cohort and explore possible risk factors for the development of intracranial aneurysms. METHODS This was a retrospective cohort study of all patients with LDS who had been seen at the Marfan Syndrome and Aortopathy Center at Washington University School of Medicine in St. Louis in 2010–2022. Medical records were reviewed to obtain demographic, clinical, and radiographic data. The prevalence of craniofacial, skeletal, and cutaneous pathologies was determined. Bivariate logistic regression was performed to identify possible risk factors for the formation of an intracranial aneurysm. RESULTS Eighty-one patients with complete medical records and intracranial vascular imaging were included in the analysis, and 18 patients (22.2%) had at least 1 intracranial aneurysm. Patients frequently demonstrated the thin or translucent skin, doughy skin texture, hypertelorism, uvular abnormalities, and joint hypermobility typical of LDS. CM-I was common, occurring in 7.4% of the patients. Importantly, the patients with intracranial aneurysms were more likely to have CM-I (22.2%) than those without intracranial aneurysms (3.2%). The mean CFI score in the cohort with available data was 1.81, with higher means in the patients with the TGFBR1 or TGFBR2 disease-causing variants (2.05 and 3.30, respectively) and lower in the patients with the SMAD3, TGFB2, or TGFB3 pathogenic variants (CFI < 1). No significant CFI difference was observed in patients with or without intracranial aneurysms (2.06 vs 1.74, p = 0.61). CONCLUSIONS CM-I, and not the CFI, is significantly associated with the presence of intracranial aneurysms in patients with LDS. Surveillance for intracranial aneurysms is essential in all patients with LDS and should not be limited to those with severe phenotypes. Long-term monitoring studies will be necessary to determine whether a correlation between craniofacial abnormalities and adverse outcomes from intracranial aneurysms (growth, intervention, or rupture) exists.

OBJECTIVE The Neurosurgery Research and Education Foundation (NREF) provides diverse funding opportunities for in-training and early-career neurosurgeon-scientists. The authors analyzed the impact of NREF funding on the subsequent career success of neurosurgeons in obtaining research funding and academic achievements. METHODS The NREF database was queried to identify NREF winners from 2000 to 2015. The award recipients were surveyed to obtain information about their demographic characteristics, academic career, and research funding. Only subsequent research support with an annual funding amount of $50,000 or greater was included. The primary outcome was the NREF impact ratio, defined as the ratio between NREF award research dollars and subsequent grant funding dollars. The secondary outcomes were time to subsequent grant funding as principal investigator (PI), clinical practice settings, and final academic position achieved. RESULTS From 2000 to 2015, 158 neurosurgeons received 164 NREF awards totaling $8.3 million (M), with $1.7 M awarded to 46 Young Clinician Investigators (YCIs), $1.5 M to 18 Van Wagenen Fellows (VWFs), and $5.1 M to 100 resident Research Fellowship Grant (RFG) awardees. Of all awardees, 73% have current academic appointments, and the mean ± SD number of publications and H-index were 71 ± 82 and 20 ± 15, respectively. The overall response rate to our survey was 70%, and these respondents became the cohort for our analysis. In total, respondents cumulatively obtained $776 M in post–NREF award grant funding, with the most common sources of funding including the National Institutes of Health ($327 M) and foundational awards ($306 M). The NREF impact ratios for awardees were $1:$381 for YCI, $1:$113 for VWF, and $1:$41 for resident RFG. Awardees with NREF projects in functional neurosurgery, pediatric neurosurgery, and neuro-oncology had the highest NREF impact ratios of $1:$194, $1:$185, and $1:$162, respectively. Of respondents, 9% became department chairs, 26% became full professors, 82% received at least 1 subsequent research grant, and 66% served as PI on a subsequent research grant after receiving their NREF awards. CONCLUSIONS In-training and early-career neurosurgeons who were awarded NREF funding had significant success in acquiring subsequent grant support, research productivity, and achievements of academic rank. NREF grants provide a tremendous return on investment across various career stages and subspecialities. They also appeared to have a broader impact on trajectory of research and innovation within the field of neurosurgery.

BACKGROUND Ewing sarcoma is a neoplasm within the family of small round blue cell tumors and most frequently arises from skeletal bone. Primary involvement of the central nervous system in these lesions is extremely rare, with an incidence of 1%. OBSERVATIONS A case is presented of a 34-year-old man who presented with left facial numbness, multiple intracranial lesions, a lumbar intradural lesion, and diffuse spinal leptomeningeal involvement. A lumbar laminectomy and biopsy were performed, which revealed the diagnosis of extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. The patient had a rapidly progressive clinical decline despite total neuroaxis radiation and multiple lines of chemotherapeutic treatments, eventually dying from his disease and its sequelae 6 months after diagnosis. LESSONS The authors’ review of 40 cases in the literature revealed only 2 patients with isolated intraaxial cranial lesions, 4 patients with cranial and spine involvement, and an additional 34 patients with spine lesions. The unique characteristics of this patient’s case, including his presentation with diffuse disease and pathology that included a rare V600E BRAF mutation, are discussed in the context of the available literature.

Background Moyamoya disease and syndrome are progressive steno-occlusive cerebrovascular diseases that manifest clinically with ischemic episodes. There is evidence for the use of electroencephalography (EEG) in preoperative and long-term postoperative evaluation of these patients, as well as in the intraoperative period to monitor for changes correlated with perioperative ischemic events. However, the utility of EEG in the immediate postprocedure time period has not previously been described. Methods We review six patients who underwent pial synangiosis from 2017 to 2019. EEGs from the preoperative, intraoperative, and immediate postoperative period were evaluated, as well as clinical examination changes and subsequent interventions. Results Six patients with postoperative EEG monitoring following pial synangiosis were included. EEG data was collected preoperatively, intraoperatively, and continuously postoperatively. Preoperatively, five of six patients had normal background activity on EEG, whereas one of six had hemispheric asymmetry. Three patients had new or worsening hemispheric intracerebral asymmetry on EEG during the immediate postsurgical period. Two of these had no clinical manifestations of ischemia, and one had transient left facial weakness. All three underwent blood pressure augmentation with improvement in the asymmetry on EEG and clinical improvement in the symptomatic patient. Conclusions Although widely accepted as a useful tool during the preoperative and intraoperative periods of evaluation and management of moyamoya disease and syndrome, we propose that the use of continuous EEG in the immediate postoperative period may have potential as a useful adjunct by both detecting early clinical and subclinical intracranial ischemia.

Object Intraventricular hemorrhage (IVH) is the most common cause of hydrocephalus in the pediatric population and is particularly common in preterm infants. The decision to place a ventriculoperitoneal shunt or ventricular access device is based on physical examination findings and radiographic imaging. The authors undertook this study to determine if head circumference (HC) measurements correlated with the Evans ratio (ER) and if changes in ventricular size could be detected by HC measurements. Methods All cranial ultrasound (CUS) reports at the authors' institution between 2008 and 2011 were queried for terms related to hydrocephalus and IVH, from which a patient cohort was determined. A review of radiology reports, HC measurements, operative interventions, and significant clinical events was performed for each patient in the study. Additional radiographic measurements, such as an ER, were calculated by the authors. Significance was set at a statistical threshold of p < 0.05 for this study. Results One hundred forty-four patients were studied, of which 45 (31%) underwent CSF diversion. The mean gestational age and birth weight did not differ between patients who did and those who did not undergo CSF diversion. The CSF diversion procedures were reserved almost entirely for patients with IVH categorized as Grade III or IV. Both initial ER and HC were significantly larger for patients who underwent CSF diversion. The average ER and HC at presentation were 0.59 and 28.2 cm, respectively, for patients undergoing CSF diversion, and 0.34 and 25.2 cm for those who did not undergo CSF diversion. There was poor correlation between ER and HC measurements regardless of gestational age (r = 0.13). Additionally, increasing HC was not found to correlate with increasing ERs on consecutive CUSs (φ = −0.01, p = 0.90). Patients who underwent CSF diversion after being followed with multiple CUSs (10 of 45 patients) presented with smaller ERs and HC than those who underwent CSF diversion after a single CUS. Just prior to CSF diversion surgery, the patients who received multiple CUSs had ERs, but not HC measurements, that were similar to those in patients who underwent CSF diversion after a single CUS. Conclusions The HC measurement does not correlate with the ER or with changes in ER and therefore does not appear to be an adequate surrogate for serial CUSs. In patients who are followed for longer periods of time before CSF shunting procedures, the ER may play a larger role in the decision to proceed with surgery. Clinicians should be aware that the ER and HC are not surrogates for one another and may reflect different pathological processes. Future studies that take into account other physical examination findings and long-term clinical outcomes will aid in developing standardized protocols for evaluating preterm infants for ventriculoperitoneal shunt or ventricular access device placement.

Treatment with testosterone is neuroprotective/neurotherapeutic after a variety of motoneuron injuries. Here we assessed whether testosterone might have similar beneficial effects after spinal cord injury (SCI). Young adult female rats received either sham or T9 spinal cord contusion injuries and were implanted with blank or testosterone-filled Silastic capsules. Four weeks later, motoneurons innervating the vastus lateralis muscle of the quadriceps were labeled with cholera toxin-conjugated horseradish peroxidase, and dendritic arbors were reconstructed in three dimensions. Soma volume, motoneuron number, lesion volume, and tissue sparing were also assessed, as were muscle weight, fiber cross-sectional area, and motor endplate size and density. Contusion injury resulted in large lesions, with no significant differences in lesion volume, percent total volume of lesion, or spared white or gray matter between SCI groups. SCI with or without testosterone treatment also had no effect on the number or soma volume of quadriceps motoneurons. However, SCI resulted in a decrease in dendritic length of quadriceps motoneurons in untreated animals, and this decrease was completely prevented by treatment with testosterone. Similarly, the vastus lateralis muscle weights and fiber cross-sectional areas of untreated SCI animals were smaller than those of sham-surgery controls, and these reductions were both prevented by testosterone treatment. No effects on motor endplate area or density were observed across treatment groups. These findings suggest that regressive changes in motoneuron and muscle morphology seen after SCI can be prevented by testosterone treatment, further supporting a role for testosterone as a neurotherapeutic agent in the injured nervous system.

OBJECT Of the 1.7 million traumatic brain injuries (TBIs) in the US, a third occur in patients under 14 years of age. The rate of posttraumatic epilepsy (PTE) may be as high as 19% after severe pediatric TBI, but the risk for seizures after mild TBI is unknown. Although the rate of seizures after mild TBI may be low, current practice is often driven by high clinical concern for posttraumatic seizures. In this study, the authors evaluated electroencephalography (EEG) results and antiepileptic drug (AED) use in a large cohort of children with mild TBI to estimate the incidence of posttraumatic seizures in this population. METHODS Patients presenting to Children’s Hospital of Atlanta for mild TBI from 2010 to 2013 were evaluated. Five thousand one hundred forty-eight patients with mild TBI were studied and divided into 3 groups: 4168 who were discharged from the emergency department, 868 who were admitted without neurosurgical intervention, and 112 who underwent neurosurgical procedures (craniotomy for hematoma evacuation or elevation of depressed skull fractures) but were discharged without an extended stay. Demographic information, CT characteristics, EEG reports, and prescriptions for AEDs were analyzed. Long-term follow-up was sought for all patients who underwent EEG. Correlation between EEG result and AED use was also evaluated. RESULTS All patients underwent head CT, and admitted patients were more likely to have an abnormal study (p < 0.0001). EEG evaluations were performed for less than 1.0% of patients in all 3 categories, without significant differences between groups (p = 0.97). Clinicians prescribed AEDs in less than 2.0% of patients for all groups, without significant differences between groups (p = 0.094). Even fewer children continue to see a neurologist for long-term seizure management. The EEG result had good negative predictive value, but only an abnormal EEG reading that was diagnostic of seizures correlated significantly with AED prescription (p = 0.04). CONCLUSIONS EEG utilization and AED prescription was low in all 3 groups, indicating that seizures following mild TBI are likely rare events. EEG has good negative predictive value for patients who did not receive AEDs, but has poorer positive predictive value for AED use.

Intraventricular hemorrhage (IVH) is the most common cause of hydrocephalus in the pediatric population and is particularly common in preterm infants. The decision to place a ventriculoperitoneal shunt or ventricular access device is based on physical examination findings and radiographic imaging. The authors undertook this study to determine if head circumference (HC) measurements correlated with the Evans ratio (ER) and if changes in ventricular size could be detected by HC measurements.All cranial ultrasound (CUS) reports at the authors' institution between 2008 and 2011 were queried for terms related to hydrocephalus and IVH, from which a patient cohort was determined. A review of radiology reports, HC measurements, operative interventions, and significant clinical events was performed for each patient in the study. Additional radiographic measurements, such as an ER, were calculated by the authors. Significance was set at a statistical threshold of p0.05 for this study.One hundred forty-four patients were studied, of which 45 (31%) underwent CSF diversion. The mean gestational age and birth weight did not differ between patients who did and those who did not undergo CSF diversion. The CSF diversion procedures were reserved almost entirely for patients with IVH categorized as Grade III or IV. Both initial ER and HC were significantly larger for patients who underwent CSF diversion. The average ER and HC at presentation were 0.59 and 28.2 cm, respectively, for patients undergoing CSF diversion, and 0.34 and 25.2 cm for those who did not undergo CSF diversion. There was poor correlation between ER and HC measurements regardless of gestational age (r = 0.13). Additionally, increasing HC was not found to correlate with increasing ERs on consecutive CUSs (φ = -0.01, p = 0.90). Patients who underwent CSF diversion after being followed with multiple CUSs (10 of 45 patients) presented with smaller ERs and HC than those who underwent CSF diversion after a single CUS. Just prior to CSF diversion surgery, the patients who received multiple CUSs had ERs, but not HC measurements, that were similar to those in patients who underwent CSF diversion after a single CUS.The HC measurement does not correlate with the ER or with changes in ER and therefore does not appear to be an adequate surrogate for serial CUSs. In patients who are followed for longer periods of time before CSF shunting procedures, the ER may play a larger role in the decision to proceed with surgery. Clinicians should be aware that the ER and HC are not surrogates for one another and may reflect different pathological processes. Future studies that take into account other physical examination findings and long-term clinical outcomes will aid in developing standardized protocols for evaluating preterm infants for ventriculoperitoneal shunt or ventricular access device placement.

The dendritic arbors of spinal motoneurons are dynamically regulated by a variety of factors, and several lines of evidence indicate that trophic interactions with the target musculature are of central importance. In highly androgen-sensitive motoneuron populations, androgens are thought to regulate motoneuron dendrites through their action at the receptor-enriched target musculature. Using rats transgenically modified to overexpress androgen receptor (AR) in skeletal muscle, we directly tested the hypothesis that the enhanced expression of AR in the target musculature can underlie the androgenic regulation of motoneuron dendritic morphology. The morphology of motoneurons innervating the quadriceps muscle was examined in wild-type (WT) rats as well as in rats that had been transgenically modified to overexpress ARs in their skeletal musculature. Motoneurons innervating the vastus lateralis muscle of the quadriceps in gonadally intact male rats, and castrated males with or without androgen replacement, were labeled with cholera toxin-conjugated horseradish peroxidase, and dendritic arbors were reconstructed in three dimensions. In WT rats, quadriceps motoneuron dendrites were insensitive to hormonal manipulation. In contrast, quadriceps motoneuron dendrites in gonadally intact transgenic males were larger than those of WT males. Furthermore, overexpression of ARs in the quadriceps muscle resulted in androgen sensitivity in dendrites, with substantial reductions in dendritic length occurring after castration; this reduction was prevented with testosterone replacement. Thus, it appears that the androgen sensitivity of motoneuron dendrites is conferred indirectly via the enrichment of ARs in the musculature.