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OBJECTIVE Individuals with autism spectrum disorder (ASD) may display extreme behaviors such as self-injury or aggression that often become refractory to psychopharmacology or behavioral intervention. Deep brain stimulation (DBS) is a surgical alternative that modulates brain circuits that have yet to be clearly elucidated. In the current study the authors performed a connectomic analysis to identify brain circuitry engaged by DBS for extreme behaviors associated with ASD. METHODS A systematic review was performed to identify prior reports of DBS as a treatment for extreme behaviors in patients with ASD. Individual patients’ perioperative imaging was collected from corresponding authors. DBS electrode localization and volume of tissue activated modeling were performed. Volumes of tissue activated were used as seed points in high-resolution normative functional and structural imaging templates. The resulting individual functional and structural connectivity maps were pooled to identify networks and pathways that are commonly engaged by all targets. RESULTS Nine patients with ASD who were receiving DBS for symptoms of aggression or self-injurious behavior were identified. All patients had some clinical improvement with DBS. Connectomic analysis of 8 patients (from the systematic review and unpublished clinical data) demonstrated a common anatomical area of shared circuitry within the anterior limb of the internal capsule. Functional analysis of 4 patients identified a common network of distant brain areas including the amygdala, insula, and anterior cingulate engaged by DBS. CONCLUSIONS This study presents a comprehensive synopsis of the evidence for DBS in the treatment of extreme behaviors associated with ASD. Using network mapping, the authors identified key circuitry common to DBS targets.

BACKGROUND Intracranial epidermoid cysts are benign, slow-growing malformations that most commonly arise at the skull base. Maximizing resection of the cyst contents and the capsule reduces long-term recurrence but can be made difficult by cyst wall adherence to critical neurovascular structures. Expanded endonasal approaches (EEAs) offer an alternative to traditional open transcranial approaches for accessible epidermoid cysts. In this case report, the authors demonstrate a transclival EEA for a large, ventral brainstem epidermoid cyst. OBSERVATIONS A 41-year-old woman who presented with progressive headaches, diplopia, malaise, and fatigue was found to have a 4.7-cm midline, ventral brainstem epidermoid cyst. She underwent an expanded endonasal transclival approach that exposed the brainstem from the level of the dorsum sella to the tip of the basion. A near-total resection was completed with removal of all cyst contents and most of the capsular wall. Reconstruction was completed with Duragen, an autologous fat graft, and a nasoseptal flap. Postoperatively, she had a partial left cranial nerve VI palsy that remained stable 8 weeks after surgery. LESSONS The expanded endoscopic transclival approach can facilitate effective resection of midline, ventral epidermoid cysts.

MRI-guided focused ultrasound thalamotomy has been shown to be an effective treatment for medication refractory essential tremor. Here, we report a clinical-radiological analysis of 123 cases of MRI-guided focused ultrasound thalamotomy, and explore the relationships between treatment parameters, lesion characteristics and outcomes. All patients undergoing focused ultrasound thalamotomy by a single surgeon were included. The procedure was performed as previously described, and patients were followed for up to 1 year. MRI was performed 24 h post-treatment, and lesion locations and volumes were calculated. We retrospectively evaluated 118 essential tremor patients and five tremor-dominant Parkinson’s disease patients who underwent thalamotomy. At 24 h post-procedure, tremor abated completely in the treated hand in 81 essential tremor patients. Imbalance, sensory disturbances and dysarthria were the most frequent acute adverse events. Patients with any adverse event had significantly larger lesions, while inferolateral lesion margins were associated with a higher incidence of motor-related adverse events. Twenty-three lesions were identified with irregular tails, often extending into the internal capsule; 22 of these patients experienced at least one adverse event. Treatment parameters and lesion characteristics changed with increasing surgeon experience. In later cases, treatments used higher maximum power (normalized to skull density ratio), accelerated more quickly to high power, and delivered energy over fewer sonications. Larger lesions were correlated with a rapid rise in both power delivery and temperature, while increased oedema was associated with rapid rise in temperature and the maximum power delivered. Total energy and total power did not significantly affect lesion size. A support vector regression was trained to predict lesion size and confirmed the most valuable predictors of increased lesion size as higher maximum power, rapid rise to high-power delivery, and rapid rise to high tissue temperatures. These findings may relate to a decrease in the energy efficiency of the treatment, potentially due to changes in acoustic properties of skull and tissue at higher powers and temperatures. We report the largest single surgeon series of focused ultrasound thalamotomy to date, demonstrating tremor relief and adverse events consistent with reported literature. Lesion location and volume impacted adverse events, and an irregular lesion tail was strongly associated with adverse events. High-power delivery early in the treatment course, rapid temperature rise, and maximum power were dominant predictors of lesion volume, while total power, total energy, maximum energy and maximum temperature did not improve prediction of lesion volume. These findings have critical implications for treatment planning in future patients.

OBJECTIVE Laser interstitial thermal therapy (LITT) for mesial temporal lobe epilepsy (mTLE) is typically performed with one trajectory to target the medial temporal lobe (MTL). MTL structures such as piriform and entorhinal cortex are epileptogenic, but due to their relative geometry, they are difficult to target with one trajectory while simultaneously maintaining adequate ablation of the amygdala and hippocampus. We hypothesized that a two-trajectory approach could improve ablation of all relevant MTL structures. First, we created large-scale computer simulations to compare idealized one- vs two-trajectory approaches. A two-trajectory approach was then validated in an initial cohort of patients. METHODS We used magnetic resonance imaging (MRI) from the Human Connectome Project (HCP) to create subject-specific target structures consisting of hippocampus, amygdala, and piriform/entorhinal/perirhinal cortex. An algorithm searched for safe potential trajectories along the hippocampal axis (catheter one) and along the amygdala-piriform axis (catheter two) and compared this to a single trajectory optimized over all structures. The proportion of each structure ablated at various burn radii was evaluated. A cohort of 11 consecutive patients with mTLE received two-trajectory LITT; demographic, operative, and outcome data were collected. RESULTS The two-trajectory approach was superior to the one-trajectory approach at nearly all burn radii for all hippocampal subfields and amygdala nuclei (p

OBJECTIVE Country-by-country estimates of the macroeconomic disease burden of central nervous system (CNS) cancers are important when determining the allocation of resources related to neuro-oncology. Accordingly, in this study the authors investigated macroeconomic losses related to CNS cancer in 173 countries and identified pertinent epidemiological trends. METHODS Data for CNS cancer incidence, mortality, and disability-adjusted life years (DALYs) were collected from the Global Burden of Disease 2019 database. Gross domestic product data were combined with DALY data to estimate economic losses using a value of lost welfare approach. RESULTS The mortality-to-incidence ratio of CNS cancer in 2019 was 0.60 in high-income regions compared to 0.82 in Sub-Saharan Africa and 0.87 in Central Europe, Eastern Europe, and Central Asia. Welfare losses varied across both high- and low-income countries. Welfare losses attributable to CNS cancer in Japan represented 0.07% of the gross domestic product compared to 0.23% in Germany. In low- and middle-income countries, Iraq reported welfare losses of 0.20% compared to 0.04% in Angola. Globally, the DALY rate in 2019 was the same for CNS cancer as for prostate cancer at 112 per 100,000 person-years, despite a 75% lower incidence rate, equating to CNS cancer welfare losses of 182 billion US dollars. CONCLUSIONS Macroeconomic losses vary across high- and low-income settings and appear to be region specific. These differences may be explained by differences in regional access to screening and diagnosis, population-level genetic predispositions, and environmental risk factors. Mortality-to-incidence ratios are higher in low- and middle-income countries than in high-income countries, highlighting possible gaps in treatment access. Quantification of macroeconomic losses related to CNS cancer can help to justify the spending of finite resources to improve outcomes for neuro-oncological patients globally.

Background National medical student surveys amidst the coronavirus disease 2019 (COVID-19)–driven subinternship cancellations have demonstrated the need for supplemental, standardized subspecialty medical education, mentorship, and career planning nationally. We have presented the first live, cross-institutional virtual medical student subspecialty training camp to deliver standardized neurosurgical educational content to medical students during the COVID-19 pandemic, and its results on medical student anxiety and perceptions of neurosurgery. Methods The online training camp used a video conferencing platform that was open to all medical students. A post-training camp survey was administered. Results A total of 305 medical students registered for the event from 107 unique U.S. medical schools. Of the 305 medical students, 108 reported intending to apply to neurosurgery residency in 2021. The top medical student objectives for the training camp were program networking and mentorship. Of the 305 participants, 121 (39.7%) completed the post-training survey. Of the respondents, 65.0% reported improved neurosurgical knowledge, 79.8% reported decreased anxiety about subinternships and interviews, 82.5% reported increased enthusiasm about neurosurgery, and 100% desired a future annual virtual training camp because of the increased accessibility and decreased cost. This was especially important for students at institutions without home subspecialty programs and those with financial burdens. Conclusions COVID-19–driven innovations in medical education have accelerated changes that may have long been necessary. This virtual structure improved resource usage and scalability compared with in-person training, maintained social distancing, and democratized access to standardized, specialized content not often available through traditional medical curricula. Even as a supplement to in-person events, the virtual training camp model could be implemented by national medical societies, which might significantly increase medical students' preparedness for, and education in, neurosurgery and other subspecialties.

Background Hyperplasia of the choroid plexus represents a rare cause of communicating hydrocephalus in children. Recent work has associated such disease with genetic abnormalities (such as perturbations in chromosome 9). Given such extensive cerebrospinal fluid (CSF) overproduction, patients with choroid plexus hyperplasia often fail CSF diversion and therefore require adjuvant interventions. Case Description We present the case of a male infant with a ventriculoperitoneal shunt and radiographic choroid hyperplasia who presented to our institution with a massive abdominal hydrocele caused by an inability to absorb the significant amount of CSF drainage into the abdomen. Conclusion The child was treated with an endoscopic third ventriculostomy and choroid plexus coagulation; however, he still required CSF diversion via a ventriculoatrial shunt. A genetic workup showed tetraploidy of chromosome 9. We discuss criteria for selection of treatment strategies, including endoscopic third ventriculostomy with choroid plexus coagulation and/or CSF diversion, that may prevent the need for re-operation in select patients with hydrocephalus due to choroid plexus hyperplasia.

Soft tissue sarcoma (STS) of the sella is exceptionally rare. We conducted a case series, literature review, and nationwide analysis of primary and iatrogenic (radiation-associated) STS of the sella to define the clinical course of this entity. This study employed a multi-institutional retrospective case review, literature review, and nationwide analysis using the National Cancer Database (NCDB). We report five patients who were diagnosed at three institutions with malignant STS of the sella. All patients presented with symptoms related to mass effect in the sellar region. All tumors extended to the suprasellar space, with the majority displaying extension into the cavernous sinus. All patients underwent an operation via a transsphenoidal approach with a goal of maximal safe tumor resection in four patients and biopsy for 1 patient. Histopathologic evaluation demonstrated STS in all patients. Post-operative adjuvant radiotherapy and chemotherapy were given to 2 and 1 out of 4 patients with known post-operative clinical course, respectively. The 1-year and 5-year overall survival rates were 100% (5/5) and 25% (1/4). Twenty-two additional reports of primary, non-iatrogenic STS of the sella were identified in the literature. Including the three cases from our series, treatment included resection in all cases, and adjuvant radiotherapy and chemotherapy were utilized in 50% (12/24) and 17% (4/24) of cases, respectively. The national prevalence of malignant STS is estimated to be 0.01% among all pituitary and sellar tumors within the NCDB. We report the prevalence and survival rates of STS of the sella. Multimodal therapy, including maximal safe resection, chemotherapy, and radiotherapy are necessary to optimize outcomes for this uncommon pathology.

Background Spinal schwannomas are benign nerve sheath neoplasms that constitute about 30% of extramedullary spinal cord tumors. They are usually small, well-encapsulated tumors with low mitotic activity and concurrently carry a low risk of recurrence. Here, we report a case of atypical histologic variant of spinal schwannoma that had higher cellular density, nuclear atypia, and lack of encapsulation. To our best knowledge, no such cases of this atypical variant with regards to lumbar spine have been reported in the literature. Case Description A 66-year-old male had an incidental left-sided paraspinal mass discovered while undergoing workup for cholecystitis. On examination, the patient was neurologically intact. Imaging revealed the presence of a contrast-enhanced, partially cystic mass arising from the L3-4 intervertebral foramen and causing left psoas muscle displacement. A minimally invasive left L3-4 posterior extracavitary resection was done. Histopathologic examination revealed a partly unencapsulated tumor with higher than usual cellular density and nuclear atypia, resulting in a diagnosis of “atypical schwannoma.” Imaging at 6 months' follow-up showed stable postsurgical changes and residual tumor with no evidence of progression/recurrence. Conclusions Atypical schwannoma has higher cellular density and nuclear atypia and lacks encapsulation. A review of the literature suggests an increased risk of recurrence when compared with typical variants, and complete tumor removal should be attempted.

Background Lumbar puncture is a common procedure that can be safely performed in most patients. Certain populations may have increased risk for complications following lumbar puncture, but the significance of basilar invagination is often underappreciated. Case Description A 45-year-old woman with basilar invagination received multiple lumbar punctures in the workup of acute meningitis. Preprocedural computed tomography was obtained. Following lumbar puncture, the patient developed locked-in syndrome. Magnetic resonance imaging obtained several days later demonstrated severe compression and infarction of the medulla and inferior cerebellum by the odontoid process and ectopic cerebellar tonsils. The patient was transferred but at this point, surgical decompression was not possible. She did not regain significant neurologic function. Conclusions Basilar invagination is a risk factor for devastating neurologic complications following lumbar puncture. Awareness of this complication and prompt recognition of its occurrence may prevent future morbidity of lumbar puncture in patients with basilar invagination.

BACKGROUND Hypercoagulability with thrombosis and associated inflammation has been well-documented in COVID-19, and catastrophic cerebral venous sinus thromboses (CVSTs) have been described. Another COVID-19–related complication is bacterial superinfection, including sinusitis. Here, the authors reported three cases of COVID-19–associated sinusitis, meningitis, and CVST and summarized the literature about septic intracranial thrombotic events as a cause of headache and fever in COVID-19. OBSERVATIONS The authors described three adolescent patients with no pertinent past medical history and no prior COVID-19 vaccinations who presented with subacute headaches, photosensitivity, nausea, and vomiting after testing positive for COVID-19. Imaging showed subdural collections, CVST, cerebral edema, and severe sinus disease. Two patients had decline in mental status and progression of neurological symptoms. In all three, emergency cranial and sinonasal washouts uncovered pus that grew polymicrobial cultures. After receiving broad-spectrum antimicrobials and various additional treatments, including two of three patients receiving anticoagulation, all patients eventually became neurologically intact with varying ongoing sequelae. LESSONS These cases demonstrated similar original presentations among previously healthy adolescents with COVID-19 infections, concurrent sinusitis precipitating CVST, and subdural empyemas. Better recognition and understanding of the multisystem results of severe acute respiratory syndrome coronavirus 2 and the complicated sequelae allows for proper treatment.

IntroductionMRgFUS thalamotomy has gained popularity as an FDA approved, non-invasive treatment for patients with Essential Tremor and tremor predominant Parkinson's Disease. We present our initial clinical experience with 160 consecutive cases of MRgFUS thalamotomy and describe the clinical outcomes with long term follow-up.MethodsA retrospective chart review of all patients who underwent MRgFUS thalamotomy at our institution was performed. CRST Part A tremor scores were obtained pre-operatively and at each follow-up visit along with an assessment of side effects (SE). All patients had a post-operative MRI within 24 h to determine the location, size, and extent of the MRgFUS lesion.ResultsOne hundred and sixty unilateral MRgFUS Thalamotomies (Left, n = 128; Right, n = 32) were performed for medically refractory essential Tremor (n = 150) or tremor predominant Parkinson's disease (n = 10). Mean age at surgery was 75 Years (range: 48-93) and the mean skull density ratio (SDR) was 0.48 (range: 0.32-0.75; median: 0.46). In ET patients, both rest and postural tremor was abolished acutely and remained so at follow-up whereas intention tremor was reduced acutely by 93% below baseline, 87% at 3 months, 83.0% at 1-year, and 78% at 2 years. On post-operative day 1, the most common SE's included imbalance (57%), sensory disturbances (25%), and dysmetria (11%). All adverse events were rated as mild on the Clavien-Dindo Scale and improved over time. At 2-years follow-up, imbalance was seen in 18%, sensory disturbance in 10% and dysmetria in 8% patients. Mean clinical follow-up for all patients was 14 months (range: 1-48 months).ConclusionMRgFUS thalamotomy is a safe and effective procedure for long term improvement of unilateral tremor symptoms, with the most common side-effects being imbalance and sensory disturbance.

Background: Metastatic brain tumors typically arise from primary malignancies of the lung, kidney, breast, skin, and colorectum. Brain metastases originating from malignancies of the female genital tract are extremely rare. We present a case of fallopian tube brain metastasis and in so doing review the pertinent literature.Case Description: We describe a 59-year-old patient with a history of fallopian tube carcinoma who presented with an incidentally identified left frontal brain mass. MRI demonstrated an enhancing lesion in the left centrum semiovale with a second enhancing lesion noted in the cerebellar vermis. She underwent a left parietal craniotomy for resection of the dominant and clinically symptomatic lesion. Immunohistochemical stains were positive for PAX8 and p53, confirming fallopian tube origin.Conclusions: Fallopian tube cancer brain metastasis is extremely uncommon. We highlight the treatment and surgical resection of this patient's BRCA1 metastatic fallopian lesion and systematically review the literature regarding the pathogenesis, diagnosis, treatment, and histologic characteristics of the previously identified fallopian tube metastases to the central nervous system. The optimal course of treatment for brain metastasis of fallopian tube carcinoma has not been clearly defined due in part to the rarity of this condition. Consistent with BRCA1 neoplasms involving the breast and ovaries, the BRCA1 status of the patient's primary tumor likely increased the risk of central nervous system dissemination. This highlights a potential benefit of early screening of individuals with metastatic gynecologic malignancies associated with BRCA1 in the absence of any neurological symptoms.

OBJECTIVE Deep brain stimulation (DBS) is traditionally performed on an awake patient with intraoperative recordings and test stimulation. DBS performed under general anesthesia with intraoperative MRI (iMRI) has demonstrated high target accuracy, reduced operative time, direct confirmation of target placement, and the ability to place electrodes without cessation of medications. The authors describe their initial experience with using iMRI to perform asleep DBS and discuss the procedural and radiological outcomes of this procedure. METHODS All DBS electrodes were implanted under general anesthesia by a single surgeon by using a neuronavigation system with 3-T iMRI guidance. Clinical outcomes, operative duration, complications, and accuracy were retrospectively analyzed. RESULTS In total, 103 patients treated from 2015 to 2019 were included, and all but 1 patient underwent bilateral implantation. Indications included Parkinson’s disease (PD) (65% of patients), essential tremor (ET) (29%), dystonia (5%), and refractory epilepsy (1%). Targets included the globus pallidus pars internus (12.62% of patients), subthalamic nucleus (56.31%), ventral intermedius nucleus of the thalamus (30%), and anterior nucleus of the thalamus (1%). Technically accurate lead placement (radial error ≤ 1 mm) was obtained for 98% of leads, with a mean (95% CI) radial error of 0.50 (0.46–0.54) mm; all leads were placed with a single pass. Predicted radial error was an excellent predictor of real radial error, underestimating real error by only a mean (95% CI) of 0.16 (0.12–0.20) mm. Accuracy remained high irrespective of surgeon experience, but procedure time decreased significantly with increasing institutional and surgeon experience (p = 0.007), with a mean procedure duration of 3.65 hours. Complications included 1 case of intracranial hemorrhage (asymptomatic) and 1 case of venous infarction (symptomatic), and 2 patients had infection at the internal pulse generator site. The mean ± SD voltage was 2.92 ± 0.83 V bilaterally at 1-year follow-up. Analysis of long-term clinical efficacy demonstrated consistent postoperative improvement in clinical symptoms, as well as decreased drug doses across all indications and follow-up time points, including mean decrease in levodopa-equivalent daily dose by 53.57% (p < 0.0001) in PD patients and mean decrease in primidone dose by 61.33% (p < 0.032) in ET patients at 1-year follow-up. CONCLUSIONS A total of 205 leads were placed in 103 patients by a single surgeon under iMRI guidance with few operative complications. Operative time trended downward with increasing institutional experience, and technical accuracy of radiographic lead placement was consistently high. Asleep DBS implantation with iMRI appears to be a safe and effective alternative to standard awake procedures.

BACKGROUND AND PURPOSE: Cephaloceles are relatively rare conditions caused by a congenital and/or acquired skull defect. The incidence of associated venous brain anomalies with regard to cephaloceles remains to be fully elucidated. Accordingly, we sought to assess the prevalence of sigmoid sinus dehiscence and diverticula in patients with spontaneous skull base cephaloceles. MATERIALS AND METHODS: Our institutional data base was retrospectively queried from 2005 to 2018. Patients in whom spontaneous skull base cephaloceles were identified were ultimately included in the study cohort. These patients subsequently had their sigmoid sinuses re-evaluated with focused attention on the possible presence of dehiscence and/or diverticula. RESULTS: We identified 56 patients: 12 men and 44 women. After re-evaluation of the sigmoid sinuses, evidence of dehiscence and/or diverticula was noted in 21 patients. The right sigmoid sinus was involved in 11 patients, and the left sigmoid sinus was involved in 7 patients, including 3 cases of diverticulum. In 3 patients, evidence of bilateral sigmoid sinus dehiscence and diverticula was noted. Female sex was associated with sigmoid sinus dehiscence and diverticula by univariate analysis (P = .019). By linear regression, cephalocele volume was negatively associated with sigmoid sinus dehiscence and diverticula (coefficient, −2266, P value < .007, adjusted R(2) = 0.1077). By univariate logistic regression using average cephalocele volume as a cutoff, we demonstrate a statistically significant finding of lower volumes being associated with sigmoid sinus dehiscence and diverticula with an odds ratio of 3.58 (P = .05). CONCLUSIONS: The prevalence of sigmoid sinus dehiscence and diverticula in patients with cephalocele is high. Female sex is associated with sigmoid sinus dehiscence and diverticula. The cephalocele volume appears to be inversely proportional to sigmoid sinus dehiscence and diverticula.

Background Eccrine carcinoma involvement of the central nervous system (CNS) is exceedingly rare. The prognosis and response to treatment of this pathology remain poorly characterized. Methods A retrospective case series and literature review were conducted. Results CNS-invading eccrine carcinoma was diagnosed in 3 patients (2 male and 1 female; age range, 60–79 years), including 2 cases of brain metastases and 1 case of brain-invading skull metastasis with subsequent spinal metastasis. The interval from primary tumor to CNS invasion was 18–51 months. All patients received multimodal therapy following diagnosis of CNS involvement. One patient who harbored a NOTCH1 mutation demonstrated a durable oncologic response after treatment with the immune checkpoint inhibitor pembrolizumab and lived 39 months after CNS invasion. The other 2 patients were discharged to hospice care within 1 month after the diagnosis of eccrine carcinoma brain metastasis. Including this case series, 23 cases of eccrine carcinoma invasion or metastasis to the CNS have been reported, with survival after diagnosis of CNS involvement ranging from a few weeks to 4 years. Conclusions We present 3 cases of eccrine carcinoma metastatic to the CNS, including the first reported case to our knowledge of eccrine carcinoma treated with immunotherapy. This case, harboring a NOTCH1 mutation, demonstrated the longest durable oncologic response reported in this rare disease. Genomic and molecular testing may play increasingly important roles in the evaluation of these metastases.

Background Aicardi syndrome is a severe neurodevelopmental disorder that occurs primarily in females and is characterized by seizures, agenesis of the corpus callosum, and chorioretinal lacunae, which occur together in the majority of affected individuals. Seizures begin in infancy and tend to progress in intensity and are often refractory to standard multimodal medication treatments. Case Description We present here a unique case of a 12-year-old girl with partial agenesis of the corpus callosum who underwent a corpus callosotomy for treatment of medically refractory epilepsy. In so doing, we also review the literature with regard to the neurosurgical management of these unique patients. Conclusions For the subset of children who present with partial, rather than complete, agenesis of the corpus callosum, corpus callosotomy should be considered as a treatment option to reduce seizure burden.

Orbital asymmetry in unicoronal synostosis impacts craniofacial appearance and can potentiate functional visual disturbances, such as strabismus. Surgical treatment aims to normalize overall cranial morphology, including that of the orbits. The purpose of this study was to compare postoperative changes in orbital asymmetry following 2 common procedures.Preoperative and 1-year postoperative computed tomography scans for patients with isolated, nonsyndromic unicoronal synostosis treated from 2007 to 2012, at 2 academic institutions were analyzed. Only patients treated by endoscopic suturectomy and postoperative helmeting or bilateral fronto-orbital advancement were included. Orbital index, depth, and volume asymmetry were determined for each patient both pre- and 1-year postoperatively. Student's t-tests were used to compare pre- and postoperative asymmetries within each treatment group. Regression analyses were used to examine postoperative change in asymmetry between treatment groups.Scans from 12 patients treated by fronto-orbital advancement and 23 treated by endoscopic suturectomy were analyzed. Differences between synostotic and nonsynostotic orbital index, depth, and volume were statistically significant both pre- and postoperatively. Statistically significant postoperative improvements in asymmetry were observed for orbital index, depth, and volume following suturectomy. Regression analysis indicated that the amount of pre- to postoperative change in all measures of asymmetry did not depend on surgical technique. Residual asymmetry following both procedures was apparent at 1 year postoperatively.Orbital asymmetry is improved, but not resolved following both fronto-orbital advancement and endoscopic suturectomy. Degree of improvement in symmetry is independent of surgical technique used.

Background The predominant neurosurgical approach to medication-refractory essential tremor is thalamic deep brain stimulation (DBS). The emergence of magnetic resonance-guided focused ultrasound (MRgFUS) thalamotomy has reawakened the debate surrounding the use of DBS versus thalamotomy for this indication. Herein, we aimed to provide a contemporary comparison between DBS and MRgFUS. Methods Two controlled trials that evaluated DBS and MRgFUS for the unilateral treatment of refractory essential tremor were compared. Clinical outcomes extracted included postural tremor score in the treated upper extremity, quality of life (QoL), and incidence of adverse events (AE). Results Baseline patient characteristics were comparable in the 2 studies, except that DBS patients were younger and had more severe baseline tremor. Both DBS- and MRgFUS-treated patients had significant tremor improvement that was sustained for 1-year posttreatment, and significant improvement in QoL. The MRgFUS cohort had higher rates of persistent neurologic AE, whereas the DBS group had higher rates of surgery- and hardware-related AEs, including intracranial hemorrhage. Conclusions In context of prior literature, both DBS and MRgFUS significantly improve tremor control and QoL. The 2 approaches are predominantly differentiated by their AE-profile. Additional head-to-head comparison on matched clinical populations are required to more accurately compare clinical efficacy and long-term outcomes.

Focused ultrasound (FUS) has been under investigation for neurosurgical applications since the 1940s. Early experiments demonstrated ultrasound as an effective tool for the creation of intracranial lesions; however, they were limited by the need for craniotomy to avoid trajectory damage and wave distortion by the skull, and they also lacked effective techniques for monitoring. Since then, the development and hemispheric distribution of phased arrays has resolved the issue of the skull and allowed for a completely transcranial procedure. Similarly, advances in MR technology have allowed for the real-time guidance of FUS procedures using MR thermometry. MR-guided FUS (MRgFUS) has primarily been investigated for its thermal lesioning capabilities and was recently approved for use in essential tremor. In this capacity, the use of MRgFUS is being investigated for other ablative indications in functional neurosurgery and neurooncology. Other applications of MRgFUS that are under active investigation include opening of the blood-brain barrier to facilitate delivery of therapeutic agents, neuromodulation, and thrombolysis. These recent advances suggest a promising future for MRgFUS as a viable and noninvasive neurosurgical tool, with strong potential for yet-unrealized applications.

OBJECTIVETraumatic brain injury (TBI) remains a significant cause of neurological morbidity and mortality. Each year, more than 1.7 million patients present to the emergency department with TBI. The goal of this study was to evaluate the prognosis of traumatic cerebral intraparenchymal hemorrhage (tIPH), to develop subclassifications of these injuries that relate to prognosis, and to provide a more comprehensive assessment of hemorrhagic progression contusion (HPC) by analyzing the rate at which tIPH “blossom” (i.e., expansion), depending on a variety of intrinsic and modifiable factors.METHODSIn this retrospective study, 726 patients (age range 0–100 years) were admitted to a level 1 trauma center with tIPH during an 8-year period (2005–2013). Of these patients, 491 underwent both admission and follow-up head CT (HCT) within 72 hours. The change in tIPH volume over time, the expansion rate, was recorded for all 491 patients. Effects of prehospital and in-hospital variables were examined using ordinal response logistic regression analyses. These variables were further examined using multivariate linear regression analysis to accurately predict the extent to which a hemorrhage will progress.RESULTSOf the 491 (67.6%) patients who underwent both admission and follow-up HCT, 368 (74.9%) patients experienced HPC. These hemorrhages expanded on average by 61.6% (4.76 ml) with an average expansion rate of 0.71 ml per hour. On univariate analysis, certain patient characteristics were significantly (p < 0.05) related to HPC, including age (> 60 years), admission Glasgow Coma Scale score, blood alcohol level, international normalized ratio, absolute platelet count, transfusion of platelets, concomitant anticoagulation and antiplatelet medication, the initial tIPH volume on admission HCT, and ventriculostomy. Increased expansion rate was significantly associated with patient disposition to hospice or death (p < 0.001). To determine which factors most accurately predict overall patient disposition, an ordinal-response logistic regression identified systolic blood pressure, Injury Severity Score, admission Glasgow Coma Scale score, follow-up scan volume, transfusion of platelets, and ventriculostomy as predictors of patient discharge disposition following tIPH. A multivariate logistic regression identified several prehospital and in-hospital variables (age, Injury Severity Score, blood alcohol level, initial scan volume, concomitant epidural hematoma, presence of subarachnoid hemorrhage, transfusion of platelets, and ventriculostomy) that predicted the volumetric expansion of tIPH. Among these variables, the admission tIPH volume by HCT proved to be the factor most predictive of HPC.CONCLUSIONSSeveral factors contribute to the rate at which traumatic cerebral contusions blossom in the acute posttraumatic period. Identifying the intrinsic and modifiable aspects of cerebral contusions can help predict the rate of expansion and highlight potential therapeutic interventions to improve TBI-associated morbidity and mortality.

OBJECTIVEEarly radiographic findings in patients with traumatic brain injury (TBI) have been studied in hopes of better predicting injury severity and outcome. However, prior attempts have generally not considered the various types of intracranial hemorrhage in isolation and have typically not excluded patients with potentially confounding extracranial injuries. Therefore, the authors examined the associations of various radiographic findings with short-term outcome to assess the potential utility of these findings in future prognostic models.METHODSThe authors retrospectively identified 1716 patients who had experienced TBI without major extracranial injuries, and categorized them into the following TBI subtypes: subdural hematoma (SDH), traumatic subarachnoid hemorrhage, intraparenchymal hemorrhage (which included intraventricular hemorrhage), and epidural hematoma. They specifically considered isolated forms of hemorrhage, in which only 1 subtype was present.RESULTSIn general, the presence of an isolated SDH was more likely to result in worse outcomes than the presence of other isolated forms of traumatic intracranial hemorrhage. Discharge to home was less likely and perihospital mortality rates were generally higher in patients with SDH. These findings were not simply related to age and were not fully captured by the admission Glasgow Coma Scale (GCS) score. The presence of SDH had a much higher sensitivity for poor outcome than the presence of other TBI subtypes, and was more sensitive for these poor outcomes than having a low GCS score (3–8).CONCLUSIONSIn these ways, SDH was the most important finding associated with poor outcome, and the authors show that consideration of SDH, specifically, can augment age and GCS score in classification and prognostic models for TBI.

Deep brain stimulation (DBS) has been reported to have beneficial effects in severe, treatment-refractory cases of obsessive-compulsive disorder (OCD) and Tourette syndrome (TS). In this report, the authors present the first case in which DBS was used to treat the neuropsychiatric symptoms of Kleefstra syndrome, a rare genetic disorder characterized by childhood hypotonia, intellectual disability, distinctive facial features, and myriad psychiatric and behavioral disturbances. A 24-year-old female patient with childhood hypotonia, developmental delay, and diagnoses of autism spectrum disorder, OCD, and TS refractory to medical management underwent the placement of bilateral ventral capsule/ventral striatum (VC/VS) DBS leads, with clinical improvement. Medical providers and family observed gradual and progressive improvement in the patient's compulsive behaviors, coprolalia, speech, and social interaction. Symptoms recurred when both DBS electrodes failed because of lead fracture and dislodgement, although the clinical benefits were restored by lead replacement. The symptomatic and functional improvements observed in this case of VC/VS DBS for Kleefstra syndrome suggest a novel indication for DBS worthy of further investigation.