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Background and importance Dural arteriovenous fistulas (dAVFs) are vascular abnormalities of the central nervous system that can cause a wide array of neurological dysfunction depending on their location, flow, and propensity to rupture. Symptomatic dAVFs at the cranio-cervical junction usually result in hemorrhage or cervical myelopathy. Distantly located dAVFs of the foramen magnum are a rare cause of thoracic intrinsic myelopathy. Clinical presentation An 83-yr-old man presented with progressive lower extremity weakness, numbness, and difficulty walking along with episodes of bowel incontinence. Magnetic resonance imaging of the cervical spine demonstrated multilevel cervical disc disease with stenosis and longitudinal cervical cord signal change extending into the upper thoracic spinal cord. Cerebral and spinal angiography revealed a dAVF in the lateral foramen magnum region. Given the location, feeding vasculature, and morphology of the fistula, endovascular embolization was not attempted. Microsurgical resection with confirmative indocyanine green fluorescent imaging was performed with adequate obliteration of the fistula. The patient's neurological baseline was preserved postoperatively with improvement of lower extremity numbness. Conclusion We present a brief overview of this neuropathologic entity and demonstrate microsurgical resection of a foramen magnum dAVF through operative video. Craniocervical dAVFs should remain on the differential diagnosis of patients presenting with progressive thoracolumbar myelopathy.

Lumbar spinal fusion is a commonly performed procedure to stabilize the spine, and the frequency with which this operation is performed is increasing. Multiple factors are involved in achieving successful arthrodesis. Systemic factors include patient medical comorbidities—such as rheumatoid arthritis and osteoporosis—and smoking status. Surgical site factors include choice of bone graft material, number of fusion levels, location of fusion bed, adequate preparation of fusion site, and biomechanical properties of the fusion construct. Rates of successful fusion can vary from 65 to 100%, depending on the aforementioned factors. Diagnosis of pseudoarthrosis is confirmed by imaging studies, often a combination of static and dynamic radiographs and computed tomography. Once pseudoarthrosis is identified, patient factors should be optimized whenever possible and a surgical plan implemented to provide the best chance of successful revision arthrodesis with the least amount of surgical risk.

Background Patients with Klippel-Feil syndrome may present with neurologic complaints such as neck pain, radiculopathy and gait instability. Here we describe surgical management of a patient with congenital fusion of the occipital-cervical region and also block circumferential fusion of dens to T3 with spinal cord compression. This report is the first of its kind with such extensive fusion. Case Description Our patient was a 56 year-old female, who presented with neck pain and tingling in all extremities. On exam, she had a short neck, prominent jaw with extremely limited range of motion in neck and features of myelopathy. CT showed fusion of the dens to T3 vertebrae. Patient underwent sub-occipital craniectomy, C1 laminectomy and Occiput to T5 posterior fixation and fusion with neurologic improvement. Conclusion This is the first reported case of Klippel-Feil syndrome with fusion of all cervical vertebrae down to T3. We recommend surgery for advanced cases of myelopathy or radiculopathy due to stenosis and spinal instability.

Objective Cystic vestibular schwannomas (CVSs) are anecdotally believed to have worse clinical and tumor-control outcomes than solid vestibular schwannomas (SVSs); however, no data have been reported to support this belief. In this study, we characterize the clinical outcomes of patients with CVSs versus those with SVSs. Design This is a retrospective review of prospectively collected data. Setting This study is set at single high-volume neurosurgical institute. Participants We queried a database for details on all patients diagnosed with vestibular schwannomas between January 2009 and January 2014. Main Outcome Measures Records were retrospectively reviewed and analyzed using univariate and multivariate analyses to study the differences in clinical outcomes and tumor progression or recurrence. Results Of a total of 112 tumors, 24% (n = 27) were CVSs and 76% (n = 85) were SVSs. Univariate analysis identified the extent of resection, Koos grade, and tumor diameter as significant predictors of recurrence (p ≤ 0.005). However, tumor diameter was the only significant predictor of recurrence in the multivariate analysis (p = 0.007). Cystic change was not a predictor of recurrence in the univariate or multivariate analysis (p ≥ 0.40). Postoperative facial nerve and hearing outcomes were similar for both CVSs and SVSs (p ≥ 0.47). Conclusion Postoperative facial nerve outcome, hearing, tumor progression, and recurrence are similar for patients with CVSs and SVSs. As CVS growth patterns and responses to radiation are unpredictable, we favor microsurgical resection over radiosurgery as the initial treatment. Our data do not support the commonly held belief that cystic tumors behave more aggressively than solid tumors or are associated with increased postoperative facial nerve deficits.

OBJECTIVEMinimally invasive anterolateral retroperitoneal approaches for lumbar interbody arthrodesis have distinct advantages attractive to spine surgeons. Prepsoas or transpsoas trajectories can be employed with differing complication profiles because of the inherent anatomical differences encountered in each approach. The evidence comparing them remains limited because of poor quality data. Here, the authors sought to systematically review the available literature and perform a meta-analysis comparing the two techniques.METHODSA systematic review and meta-analysis was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A database search was used to identify eligible studies. Prepsoas and transpsoas studies were compiled, and each study was assessed for inclusion criteria. Complication rates were recorded and compared between approach groups. Studies incorporating an analysis of postoperative subsidence and pseudarthrosis rates were also assessed and compared.RESULTSFor the prepsoas studies, 20 studies for the complications analysis and 8 studies for the pseudarthrosis outcomes analysis were included. For the transpsoas studies, 39 studies for the complications analysis and 19 studies for the pseudarthrosis outcomes analysis were included. For the complications analysis, 1874 patients treated via the prepsoas approach and 4607 treated with the transpsoas approach were included. In the transpsoas group, there was a higher rate of transient sensory symptoms (21.7% vs 8.7%, p = 0.002), transient hip flexor weakness (19.7% vs 5.7%, p < 0.001), and permanent neurological weakness (2.8% vs 1.0%, p = 0.005). A higher rate of sympathetic nerve injury was seen in the prepsoas group (5.4% vs 0.0%, p = 0.03). Of the nonneurological complications, major vascular injury was significantly higher in the prepsoas approach (1.8% vs 0.4%, p = 0.01). There was no difference in urological or peritoneal/bowel injury, postoperative ileus, or hematomas (all p > 0.05). A higher infection rate was noted for the transpsoas group (3.1% vs 1.1%, p = 0.01). With regard to postoperative fusion outcomes, similar rates of subsidence (12.2% prepsoas vs 13.8% transpsoas, p = 0.78) and pseudarthrosis (9.9% vs 7.5%, respectively, p = 0.57) were seen between the groups at the last follow-up.CONCLUSIONSComplication rates vary for the prepsoas and transpsoas approaches owing to the variable retroperitoneal anatomy encountered during surgical dissection. While the risks of a lasting motor deficit and transient sensory disturbances are higher for the transpsoas approach, there is a reciprocal reduction in the risks of major vascular injury and sympathetic nerve injury. These results can facilitate informed decision-making and tailored surgical planning regarding the choice of minimally invasive anterolateral access to the spine.

Commonly used for Parkinson’s disease (PD), deep brain stimulation (DBS) produces marked clinical benefits when optimized. However, assessing the large number of possible stimulation settings (i.e., programming) requires numerous clinic visits. Here, we examine whether functional magnetic resonance imaging (fMRI) can be used to predict optimal stimulation settings for individual patients. We analyze 3 T fMRI data prospectively acquired as part of an observational trial in 67 PD patients using optimal and non-optimal stimulation settings. Clinically optimal stimulation produces a characteristic fMRI brain response pattern marked by preferential engagement of the motor circuit. Then, we build a machine learning model predicting optimal vs. non-optimal settings using the fMRI patterns of 39 PD patients with a priori clinically optimized DBS (88% accuracy). The model predicts optimal stimulation settings in unseen datasets: a priori clinically optimized and stimulation-naïve PD patients. We propose that fMRI brain responses to DBS stimulation in PD patients could represent an objective biomarker of clinical response. Upon further validation with additional studies, these findings may open the door to functional imaging-assisted DBS programming., Deep brain stimulation programming for Parkinson’s disease entails the assessment of a large number of possible simulation settings, requiring numerous clinic visits after surgery. Here, the authors show that patterns of functional MRI can predict the optimal stimulation settings.

Several vascularized bone grafts (VBGs) have been introduced for reconstruction and augmenting fusion of the spine. The expanding use of VBGs in the field of spinoplastic reconstruction, however, has highlighted the need to clarify the nomenclature for bony reconstruction as well as establish the position of VBGs on the bony reconstructive algorithm. In the current literature, the terms “flap” and “graft” are often applied inconsistently when describing vascularized bone transfer. Such inconsistency creates barriers in communication between physicians, confusion in interpreting the existing studies, and difficulty in comparing surgical techniques. VBGs are defined as bone segments transferred on their corresponding muscular attachments without a named major feeding vessel. The bone is directly vascularized by the muscle attachments and unnamed periosteal feeding vessels. VBGs are best positioned as a separate entity in the bony reconstruction algorithm between nonvascularized bone grafts (N-VBGs) and bone flaps. VBGs offer numerous advantages as they supply fully vascularized bone to the recipient site without the microsurgical techniques or pedicle dissection required for raising bone flaps. Multiple VBGs have been introduced in recent years to optimize these benefits for spinoplastic reconstruction.

Failed fusion in the cervical spine is a multifactorial problem stemming from a combination of patient and surgical factors. Patient-related risk factors such as steroid use, poor bone quality, and smoking can be optimized preoperatively. Age, prior radiation, prior surgery, and underlying genetics are nonmodifiable patient-centered risk factors. Surgical risks for failed fusion include the number of segments fused, anterior versus posterior approach for fusion, the type of bone graft, and the instrumentation utilized. Many symptomatic cases of failed fusion (pseudarthrosis) result in pain, neurological deficits, or loosened hardware necessitating a revision surgery consisting of extending the prior construct and utilizing additional allografts or autografts to augment the fusion. Given the relatively mobile nature of the cervical spine, pseudoarthrosis (either known or anticipated) must be recognized by the spine surgeon, and steps should be considered to optimize the likelihood of future fusion. This consists of both performing a rigid fixation and using appropriate bone graft to enhance the environment for arthrodesis. Vascularized bone grafts are a useful tool to augment fusion and provide added structural stability in cases at high risk of pseudoarthrosis.

Instrumented fixation and fusion of the thoracic spine present distinct challenges and complications including pseudarthrosis and junctional kyphosis. When complications arise, morbidity to the patient can be significant, involving neurologic injury, failure of instrumentation constructs, as well as iatrogenic spinal deformity. Causes of fusion failure are multifactorial, and incompletely understood. Most likely, a diverse set of biomechanical and biologic factors are at the heart of failures. Revision surgery for thoracic fusion failures is complex and often requires revision or extension of instrumentation, and frequently necessitates complex soft tissue manipulation to manage index level injury or to augment the changes of fusion.

Background Obtaining successful arthrodesis at the craniocervical junction and atlantoaxial joint can be more challenging than in other segments of the cervical spine. This challenge stems from the relatively hypermobile joints between the occipital condyles, the motion that occurs at C1 and C2, as well as the paucity of dorsal bony surfaces for posterior arthrodesis. While multiple different techniques for spinal fixation in this region have been well described, there has been little investigation into auxiliary methods to improve fusion rates. Objective To describe the use of an occipital bone graft to augment bony arthrodesis in the supraaxial cervical spine using a multidisciplinary approach. Methods We review the technique for harvesting and placing a vascularized occipital bone graft in 2 patients undergoing revision surgery at the craniocervical junction. Results The differentiation from nonvascularized bone graft, either allograft or autograft, to a bone graft using vascularized tissue is a key principle of this technique. It has been well established that vascularized bone heals and fuses in the spine better than structural autogenous grafts. However, the morbidity and added operative time of harvesting a vascularized flap, such as from the fibula or rib, precludes its utility in most degenerative spine surgeries. Conclusion By adapting the standard neurosurgical procedure for a suboccipital craniectomy and utilizing the tenets of flap-based reconstructive surgery to maintain the periosteal and muscular blood supply, we describe the feasibility of using a vascularized and pedicled occipital bone graft to augment instrumented upper cervical spinal fusion. The use of this vascularized bone graft may increase fusion rates in complex spine surgeries.

OBJECTIVE An advantage of lateral lumbar interbody fusion (LLIF) surgery is the indirect decompression of the neural elements that occurs because of the resulting disc height restoration, spinal realignment, and ligamentotaxis. The degree to which indirect decompression occurs varies; no method exists for effectively predicting which patients will respond. In this study, the authors identify preoperative predictive factors of indirect decompression of the central canal. METHODS The authors performed a retrospective evaluation of prospectively collected consecutive patients at a single institution who were treated with LLIF without direct decompression. Preoperative and postoperative MRI was used to grade central canal stenosis, and 3D volumetric reconstructions were used to measure changes in the central canal area (CCA). Multivariate regression was used to identify predictive variables correlated with radiographic increases in the CCA and clinically successful improvement in visual analog scale (VAS) leg pain scores. RESULTS One hundred seven levels were treated in 73 patients (mean age 68 years). The CCA increased 54% from a mean of 0.96 cm2 to a mean of 1.49 cm2 (p < 0.001). Increases in anterior disc height (74%), posterior disc height (81%), right (25%) and left (22%) foraminal heights, and right (12%) and left (15%) foraminal widths, and reduction of spondylolisthesis (67%) (all p < 0.001) were noted. Multivariate evaluation of predictive variables identified that preoperative spondylolisthesis (p < 0.001), reduced posterior disc height (p = 0.004), and lower body mass index (p = 0.042) were independently associated with radiographic increase in the CCA. Thirty-two patients were treated at a single level and had moderate or severe central stenosis preoperatively. Significant improvements in Oswestry Disability Index and VAS back and leg pain scores were seen in these patients (all p < 0.05). Twenty-five (78%) patients achieved the minimum clinically important difference in VAS leg pain scores, with only 2 (6%) patients requiring direct decompression postoperatively due to persistent symptoms and stenosis. Only increased anterior disc height was predictive of clinical failure to achieve the minimum clinically important difference. CONCLUSIONS LLIF successfully achieves indirect decompression of the CCA, even in patients with substantial central stenosis. Low body mass index, preoperative spondylolisthesis, and disc height collapse appear to be most predictive of successful indirect decompression. Patients with preserved disc height but severe preoperative stenosis are at higher risk of failure to improve clinically.

Magnetic resonance guided focused ultrasound (MRgFUS) thalamotomy is a novel and minimally invasive ablative treatment for essential tremor. The size and location of therapeutic lesions producing the optimal clinical benefits while minimizing adverse effects are not known. We examined these relationships in patients with essential tremor undergoing MRgFUS. We studied 66 patients with essential tremor who underwent MRgFUS between 2012 and 2017. We assessed the Clinical Rating Scale for Tremor (CRST) scores at 3 months after the procedure and tracked the adverse effects (sensory, motor, speech, gait, and dysmetria) 1 day (acute) and 3 months after the procedure. Clinical data associated with the postoperative Day 1 lesions were used to correlate the size and location of lesions with tremor benefit and acute adverse effects. Diffusion-weighted imaging was used to assess whether acute adverse effects were related to lesions encroaching on nearby major white matter tracts (medial lemniscus, pyramidal, and dentato-rubro-thalamic). The area of optimal tremor response at 3 months after the procedure was identified at the posterior portion of the ventral intermediate nucleus. Lesions extending beyond the posterior region of the ventral intermediate nucleus and lateral to the lateral thalamic border were associated with increased risk of acute adverse sensory and motor effects, respectively. Acute adverse effects on gait and dysmetria occurred with lesions inferolateral to the thalamus. Lesions inferolateral to the thalamus or medial to the ventral intermediate nucleus were also associated with acute adverse speech effects. Diffusion-weighted imaging revealed that lesions associated with adverse sensory and gait/dysmetria effects compromised the medial lemniscus and dentato-rubro-thalamic tracts, respectively. Lesions associated with adverse motor and speech effects encroached on the pyramidal tract. Lesions larger than 170 mm3 were associated with an increased risk of acute adverse effects. Tremor improvement and acute adverse effects of MRgFUS for essential tremor are highly dependent on the location and size of lesions. These novel findings could refine current MRgFUS treatment planning and targeting, thereby improving clinical outcomes in patients.

OBJECTIVESpinal osteotomies and anterior column realignment (ACR) are procedures that allow preservation or restoration of spine lordosis. Variations of these techniques enable different degrees of segmental, regional, and global sagittal realignment. The authors propose a comprehensive anatomical classification system for ACR and its variants based on the level of technical complexity and invasiveness. This serves as a common language and platform to standardize clinical and radiographic outcomes for the utilization of ACR.METHODSThe proposed classification is based on 6 anatomical grades of ACR, including anterior longitudinal ligament (ALL) release, with varying degrees of posterior column release or osteotomies. Additionally, a surgical approach (anterior, lateral, or posterior) was added. Reliability of the classification was evaluated by an analysis of 16 clinical cases, rated twice by 14 different spine surgeons, and calculation of Fleiss kappa coefficients.RESULTSThe 6 grades of ACR are as follows: grade A, ALL release with hyperlordotic cage, intact posterior elements; grade 1 (ACR + Schwab grade 1), additional resection of the inferior facet and joint capsule; grade 2 (ACR + Schwab grade 2), additional resection of both superior and inferior facets, interspinous ligament, ligamentum flavum, lamina, and spinous process; grade 3 (ACR + Schwab grade 3), additional adjacent-level 3-column osteotomy including pedicle subtraction osteotomy; grade 4 (ACR + Schwab grade 4), 2-level distal 3-column osteotomy including pedicle subtraction osteotomy and disc space resection; and grade 5 (ACR + Schwab grade 5), complete or partial removal of a vertebral body and both adjacent discs with or without posterior element resection. Intraobserver and interobserver reliability were 97% and 98%, respectively, across the 14-reviewer cohort.CONCLUSIONSThe proposed anatomical realignment classification provides a consistent description of the various posterior and anterior column release/osteotomies. This reliability study confirmed that the classification is consistent and reproducible across a diverse group of spine surgeons.

Background Context C2 pedicle and pars screws require accurate placement to avoid injury to nearby neurovascular structures. Freehand, fluoroscopically guided, and computed tomography (CT)–based navigation techniques have been described in the medical literature. Purpose The present study aims to compare the safety and accuracy of the freehand technique versus stereotactic navigation for the placement of C2 pedicle and pars screws. Study Design/Setting This study was a retrospective review of consecutive patients treated with posterior fixation constructs. Patient Sample A total of 220 consecutive patients were treated with posterior fixation constructs containing C2 pars or pedicle screws placed at our institution. Outcome Measures Computed tomography imaging was used to assess the accuracy of screw placement. Intraoperative complications and incidence of stroke or mortality within 30 days of the operation were analyzed. Methods A retrospective review was conducted of consecutive patients treated with posterior fixation constructs containing C2 pars or pedicle screws placed by spine surgeons between January 1, 2010, and August 31, 2016. Clinical and radiographic data were collected and analyzed. Screw accuracy was graded independently by two reviewers according to the following criteria: grade A (no breach), grades B–E (breach with transverse foramen obstruction of 1%–25%, 26%–50%, 51%–75%, or 76%–100%, respectively), and grade M (medial breach). Screws were divided into acceptable (grades A and B) and unacceptable (grades C–E and M). Results A total of 426 C2 pars or pedicle screws (312 freehand, 114 navigated) were placed in 220 patients (160 freehand, 60 navigated). Complications were similar between the groups: three vertebral artery injuries (two [1%] freehand, one [2%] navigated; p>.99), five deaths (four [3%] freehand, one [2%] navigated; p>.99), and one (2%) stroke in the navigated group (p=.61). Computed tomography imaging was available for accuracy grading of 182 screws (131 freehand, 51 navigated). No breaches (grade A) occurred in 113 of the freehand screws (86%) and in 34 of the navigated screws (67%) (p=.006). More screws had acceptable placement in the freehand group (123 of 131, 94%) than in the navigated group (42 of 51, 82%) (p=.02). Conclusions In patients with postoperative CT imaging (43%), the freehand technique was found to be more accurate than CT-based navigation for C2 pedicle or pars screw placement. Complication rates did not differ between the two techniques in this study.

Background Idiopathic spinal cord herniation (ISCH) is a rare pathology of the spine defined by herniation of the spinal cord through a dural defect. Objective To highlight the operative management of ISCH and the surgical nuances of ISCH repairs conducted at our institution. Methods This retrospective review examines consecutive patients with ISCH who were treated surgically between January 1, 2010, and July 31, 2017, at Barrow Neurological Institute, Phoenix, Arizona. Results Four patients with ISCH presented with thoracic myelopathy and lower extremity weakness during the study period. Treatment consisted of reduction of the herniated spinal cord and filling of the dural defect with a collagen-based dural regeneration matrix. In 3 patients the dural edges were covered with a collagen-matrix intradural sling, and in 1 patient they were repaired primarily with interrupted sutures. Three of the 4 patients experienced improvement in myelopathic symptoms; the fourth patient suffered neurological decline in the immediate postoperative period. Conclusion ISCH is a complex pathological condition likely to result in progressive myelopathy. Surgery offers patients the possibility of stabilizing the progression of the spinal cord dysfunction and perhaps restoring neurological function. However, extreme care must be taken during surgery to minimize manipulation of the fragile herniated cord.

OBJECTIVESeizures are the most common presenting symptom of newly diagnosed WHO Grade II gliomas (low-grade glioma [LGG]) and significantly impair quality of life. Although gross-total resection of LGG is associated with better seizure control, it remains unclear whether an extent of resection (EOR) “threshold” exists for long-term seizure control. Specifically, what proportion of FLAIR-positive tissue in patients with newly diagnosed LGG must be removed to achieve Engel Class I seizure freedom? To clarify the EOR threshold for long-term seizure control, the authors analyzed data from a consecutive series of patients with newly diagnosed LGG who presented with seizures and subsequently underwent microsurgical resection.METHODSThe authors identified consecutive patients with newly diagnosed LGG who presented with seizures and were treated at the Barrow Neurological Institute between 2002 and 2012. Patients were dichotomized into those who were seizure free postoperatively and those who were not. The EOR was calculated by quantitative comparison of pre- and postoperative MRI. Univariate analysis of these 2 groups included the chi-square test and the Mann-Whitney U-test, and a multivariate logistic regression was constructed to predict the impact of multiple independent variables on the likelihood of postoperative seizure freedom. To determine a threshold of EOR that optimizes seizure freedom, a receiver operating characteristic curve was plotted and the optimal point of discrimination was determined.RESULTSData from 128 patients were analyzed (male/female ratio 1.37:1; mean age 40.8 years). All 128 patients presented with seizures, usually generalized (n = 57, 44.5%) or simple partial (n = 57, 44.5%). The median EOR was 90.0%. Of 128 patients, 46 (35.9%) had 100% volumetric tumor resection, 64 (50.0%) had 90%–99% volumetric tumor resection, and 11 (8.6%) had 80%–89% volumetric tumor resection. Postoperatively, 105 (82%) patients were seizure free (Engel Class I); 23 (18%) were not (Engel Classes II–IV). The proportion of seizure-free patients increased in proportion to the EOR. Predictive variables included in the regression model were preoperative Karnofsky Performance Scale score, seizure type, time from diagnosis to surgery, preoperative number of antiepileptic drugs, and EOR. Only EOR significantly affected the likelihood of postoperative Engel Class I status (OR 11.5, 95% CI 2.4–55.6; p = 0.002). The receiver operating characteristic curve generated based on Engel Class I status showed a sensitivity of 0.65 and 1 – specificity of 0.175, corresponding to an EOR of 80%.CONCLUSIONSFor adult patients with LGG who suffer seizures, the results suggest that seizure freedom can be attained when EOR > 80% is achieved. Improvements in both the proportion of seizure-free patients and the durability of seizure freedom were observed beyond this 80% threshold. Interestingly, this putative EOR seizure-freedom threshold closely approximates that reported for the overall survival benefit in newly diagnosed hemispheric LGGs, suggesting that a minimum level of residual tumor burden is necessary for both disease and symptomatic progression.

OBJECTIVEFusiform dolichoectatic vertebrobasilar aneurysms are rare, challenging lesions. The natural history of these lesions and medium- and long-term patient outcomes are poorly understood. The authors sought to evaluate patient prognosis after diagnosis of fusiform dolichoectatic vertebrobasilar aneurysms and to identify clinical and radiographic predictors of neurological deterioration.METHODSThe authors reviewed multiple, prospectively maintained, single-provider databases at 3 large-volume cerebrovascular centers to obtain data on patients with unruptured, fusiform, basilar artery dolichoectatic aneurysms diagnosed between January 1, 2000, and January 1, 2015.RESULTSA total of 50 patients (33 men, 17 women) were identified; mean clinical follow-up was 50.1 months and mean radiographic follow-up was 32.4 months. At last follow-up, 42% (n = 21) of aneurysms had progressed and 44% (n = 22) of patients had deterioration of their modified Rankin Scale scores. When patients were dichotomized into 2 groups— those who worsened and those who did not—univariate analysis showed 5 variables to be statistically significantly different: sex (p = 0.007), radiographic brainstem compression (p = 0.03), clinical posterior fossa compression (p < 0.001), aneurysmal growth on subsequent imaging (p = 0.001), and surgical therapy (p = 0.006). A binary logistic regression was then created to evaluate these variables. The only variable found to be a statistically significant predictor of clinical worsening was clinical symptoms of posterior fossa compression at presentation (p = 0.01).CONCLUSIONSFusiform dolichoectatic vertebrobasilar aneurysms carry a poor prognosis, with approximately one-half of the patients deteriorating or experiencing progression of their aneurysm within 5 years. Despite being high risk, intervention—when carefully timed (before neurological decline)—may be beneficial in select patients.

Background The diagnosis and management of patients with idiopathic intracranial hypertension (IIH) frequently relies on lumbar puncture to ascertain intracranial pressure (ICP). However, ICP values derived this way may be spurious owing to patient body habitus and behavior. We recently incorporated direct continuous ICP monitoring into the work-up for IIH. Methods Through billing records, we identified all patients during a 3-year period who had a diagnosis of IIH and who underwent ICP monitoring before shunt placement or revision. Patient demographics and clinical data were reviewed. Results Of 30 patients who underwent ICP monitoring with an intraparenchymal wire, 17 had undergone lumbar puncture within the previous 6 months. Results from lumbar punctures showed an elevated opening pressure in all 17 patients, whereas only 2 patients (12%) were found to have consistently elevated ICP with direct ICP monitoring. Of 15 patients being evaluated for shunting, 4 (27%) were found to have elevated ICP. Of the 15 patients with existing shunts, 2 patients (13%) were found to have malfunctioning shunts after pressure monitoring, and 3 patients (20%) had shunts that were found to be unnecessary and were removed. No patient experienced any complication from invasive monitoring. Conclusions Direct ICP monitoring is the gold standard for determining ICP and can be safely and effectively applied to the work-up and treatment of patients with IIH to reduce the occurrence of misdiagnosis and unnecessary surgery.

High-frequency deep brain stimulation (DBS) was introduced in the late 1980s for the treatment of movement disorders. This reversible, adjustable, and non-ablative therapy has been used to treat more than 100,000 people worldwide. The surgical procedure used to implant the DBS system, as well as the effects of chronic electrical stimulation, have been shown to be safe and effective through many clinical trials. Given the ability to therapeutically modulate the motor circuits of the brain in this manner, clinicians have considered using DBS for other neurodegenerative and neuropsychiatric disorders involving non-motor circuits, including appetite, mood, and cognition. This article highlights several recent studies exploring the feasibility of using DBS to modulate memory, specifically in the context of memory disorders such as Alzheimer disease.

Objective Some aneurysms without a definable neck and associated parent vessel pathology are particularly difficult to treat and may require clipping with circumferential wrapping. We report the largest available contemporary series examining the techniques of Gore-Tex clip-wrapping of ruptured and unruptured intracranial aneurysms and patient outcomes. Methods The presentation, location, and shape of the aneurysm; wrapping technique; outcome at discharge and last follow-up; and any change in the aneurysm at last angiographic follow-up were reviewed retrospectively in 30 patients with Gore-Tex clip-wrapped aneurysms. Results Gore-Tex clip-wrapping was used in 8 patients with ruptured aneurysms and 22 patients with unruptured aneurysms. Aneurysms included 23 fusiform, 3 blister, and 4 otherwise complex, multilobed, or giant aneurysms. Of the 30 aneurysms, 63% were in the anterior circulation. The overall mean patient age was 52.5 years (range, 17–80 years). Postoperatively, there were no deaths or worsening of neurologic status and no parent vessel stenoses or strokes. The mean Glasgow Outcome Scale score at last follow-up was 4.7. The mean follow-up time was 42.3 months (median, 37.0 months; range, 3–96 months). There were 105.8 patient follow-up years. Aneurysms recurred in 2 patients with Gore-Tex clip-wrapping. No patients developed rehemorrhage. Overall risk of recurrence was 1.9% annually. Conclusions Gore-Tex has excellent material properties for circumferential wrapping of aneurysms and parent arteries. It is inert and does not cause a tissue reaction or granuloma formation. Gore-Tex clip-wrapping can be used safely for microsurgical management of ruptured and unruptured cerebral aneurysms with acceptable recurrence and rehemorrhage rates.

Lateral anterior column release (ACR) is a powerful extension of the minimally invasive lateral lumbar interbody fusion procedure that incorporates division of the anterior longitudinal ligament to allow manipulation of the anterior and middle spinal columns. The resulting surgical control permits restoration of significant segmental lordosis that, when combined with varying posterior column releases, can achieve global sagittal realignment on par with traditional 3-column osteotomies. As a result, ACR is a factor in the growth of minimally invasive strategies for the correction of spinal deformities.

Traditional open approaches to correct lumbar spine deformities include 3-column osteotomies, such as a pedicle subtraction osteotomy (PSO). Minimally invasive surgical (MIS) techniques have been developed for lateral transpsoas anterior column realignment (ACR). These 2 techniques have not previously been combined. Our objective was to investigate the cadaveric feasibility of a combined hybrid MIS ACR-PSO technique for deformity correction and to review early clinical experience.The feasibility of the combined ACR-PSO technique was evaluated in 4 fresh cadaveric specimens. The operative experience, complications, and early clinical outcomes in patients treated with the combined approach to correct global sagittal imbalance were reviewed.In the cadaveric study, the combined ACR-PSO resulted in a mean 46.0° increase in lordosis (P0.001). Nine patients treated with ACR-PSO were evaluated (mean age, 65.2 years; 33.3% female). Most patients (6/9; 66.7%) underwent ACR at L1/2 or L3/4; 8 (88.8%) had PSO at L3 or L4. On average, 10.6 ± 3.8 levels were fused. Significant decreases in mean pelvic tilt (P = 0.01), spinopelvic mismatch (P0.001), and T1 spinopelvic inclination (P = 0.03) were observed postoperatively; mean lumbar lordosis (P = 0.007), intradiscal angle (P = 0.001), and thoracic kyphosis (P = 0.04) significantly increased. The overall complication rate was 55.6% (5/9).Our early experience suggests that a combined ACR-PSO with posterior fixation allows for significant correction of segmental lordosis and global imbalance. This combined approach may maximize results attainable with hybrid MIS-open surgical techniques and represents a complementary option to PSO and other posterior approaches.

The indications for operative intervention after thoracolumbar spine trauma have been well described. Advances in minimally invasive techniques, including percutaneous pedicle screw fixation and mini-open anterolateral retractor-based approaches can improve surgical outcomes when appropriately applied by reducing blood loss, operative duration and post-operative pain. Moreover, they allow for theoretical advantages by preservation of muscular and skeletal blood supply and innervation that is typically lost during the muscular dissection of open approaches. For thoracolumbar spine fractures, percutaneous fixation allows for internal bracing of unstable fractures during healing while maintaining sagittal alignment. In instances of neurological compromise from fracture retropulsion, corpectomies may be required, and mini-open lateral approaches adopted from degenerative disease applications allow for a minimally invasive manner to treat the defect. These further allow for placement of wide rectangular-footprint expandable vertebral body replacement devices to provide anterior column support. We believe this allows for lower rates of subsidence and helps to maintain the biomechanical integrity necessary to prevent post-traumatic malalignment and kyphosis. Together, these minimally invasive techniques combined supply the spine surgeon with a minimally invasive armamentarium to treat nearly all thoracolumbar spine trauma. Surgeons should be comfortable with the strengths and shortcomings of these approaches in order to successfully apply them for this pathology.

Background The majority of Parkinson's disease patients with deep brain stimulation (DBS) use a monopolar configuration, which presents challenges for EEG and MRI studies. The literature reports algorithms to convert monopolar to bipolar settings. Purpose/hypothesis To assess brain responses of Parkinson's disease patients implanted with DBS during fMRI studies using their clinical and presumed equivalent settings using a published conversion recipe. Study type Prospective. Subjects Thirteen DBS patients. Field strength/sequence 1.5T and 3T, fMRI using gradient echo-planar imaging. Assessment Patients underwent 30/30sec ON/OFF DBS fMRI scans using monopolar and bipolar settings. To convert to a bipolar setting, the negative contact used for the monopolar configuration remained constant and the adjacent dorsal contact was rendered positive, while increasing the voltage by 30%. fMRI activation/deactivation maps and motor Unified Parkinson's Disease Rating Scale (UPDRS-III) scores were compared for patients in both configurations. Statistical tests T-tests were used to compare UPDRS scores and volumes of tissue activated (VTA) diameters in monopolar and bipolar configurations. Results The patterns of fMRI activation in the monopolar and bipolar configurations were generally different. The thalamus, pallidum, and visual cortices exhibited higher activation using the patient's clinical settings than the presumed equivalent settings. VTA diameters were lower (7 mm vs. 6.3 mm, P = 0.047) and UPDRS scores were generally higher in the bipolar (33.2 ± 16) than in the monopolar configuration (28.3 ± 17.4), without reaching statistical significance (P > 0.05). Data conclusion Monopolar and bipolar configurations result in different patterns of brain activation while using a previously published monopolar-bipolar conversion algorithm. Clinical benefits may be achieved with varying patterns of brain responses. Blind conversion from one to the other should be avoided for purposes of understanding the mechanisms of DBS. Level of evidence 1 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2018.

Lateral interbody fusion has distinct advantages over traditional posterior approaches. When adjunctive percutaneous pedicle screw fixation is required, placement from the lateral decubitus position theoretically increases safety and improves operative efficiency by obviating the need for repositioning. However, safe cannulation of the contralateral, down-side pedicles remains technically challenging and often prohibitive. In this video, we present the case of a 59-yr-old man with refractory back pain and bilateral lower extremity radiculopathy that was worse on the left than right side. The patient provided written informed consent before undergoing treatment. We performed minimally invasive single-position lateral interbody fusion with robotic (ExcelsiusGPS, Globus Medical Inc, Audubon, Pennsylvania) bilateral percutaneous pedicle screw fixation for the treatment of asymmetric disc degeneration, dynamic instability, and left paracentral disc herniation with corresponding stenosis at the L3-4 level. A left-sided minimally invasive transpsoas lateral interbody graft was placed with fluoroscopic guidance. Without changing the position of the patient or breaking the sterile field, an intraoperative cone-beam computed tomography image was obtained for navigational screw placement with stereotactic trackers in the iliac spine. Screw trajectories were planned using the robotic navigation software and were placed percutaneously in the bilateral L3 and L4 pedicles using the robotic arm. Concomitant lateral fluoroscopy may be used if desired to ensure the fidelity of the robotic guidance. The patient recovered well postoperatively and was discharged home within 36 h, without complication. Single-position lateral interbody fusion and percutaneous pedicle screw fixation can be accomplished using robotic-assisted navigation and pedicle screw placement. Used with permission from Barrow Neurological Institute.

OBJECTIVEMinimally invasive anterior and lateral approaches to the lumbar spine are increasingly used to treat and reduce grade I spondylolisthesis, but concerns still exist for their usage in the management of higher-grade lesions. The authors report their experience with this strategy for grade II spondylolisthesis in a single-surgeon case series and provide early clinical and radiographic outcomes.METHODSA retrospective review of a single surgeon’s cases between 2012 and 2016 identified all patients with a Meyerding grade II lumbar spondylolisthesis who underwent minimally invasive lateral lumbar interbody fusion (LLIF) or anterior lumbar interbody fusion (ALIF) targeting the slipped level. Demographic, clinical, and radiographic data were collected and analyzed. Changes in radiographic measurements, Oswestry Disability Index (ODI), and visual analog scale (VAS) scores were compared using the paired t-test and Wilcoxon signed rank test for continuous and ordinal variables, respectively.RESULTSThe average operative time was 199.1 minutes (with 60.6 ml of estimated blood loss) for LLIFs and 282.1 minutes (with 106.3 ml of estimated blood loss), for ALIFs. Three LLIF patients had transient unilateral anterior thigh numbness during the 1st week after surgery, and 1 ALIF patient had transient dorsiflexion weakness, which was resolved at postoperative week 1. The mean follow-up time was 17.6 months (SD 12.5 months) for LLIF patients and 10 months (SD 3.1 months) for ALIF patients. Complete reduction of the spondylolisthesis was achieved in 12 LLIF patients (75.0%) and 7 ALIF patients (87.5%). Across both procedures, there was an increase in both the segmental lordosis (LLIF 5.6°, p = 0.002; ALIF 15.0°, p = 0.002) and overall lumbar lordosis (LLIF 2.9°, p = 0.151; ALIF 5.1°, p = 0.006) after surgery. Statistically significant decreases in the mean VAS and the mean ODI measurements were seen in both treatment groups. The VAS and ODI scores fell by a mean value of 3.9 (p = 0.002) and 19.8 (p = 0.001), respectively, for LLIF patients and 3.8 (p = 0.02) and 21.0 (p = 0.03), respectively, for ALIF patients at last follow-up.CONCLUSIONSEarly clinical and radiographic results from using minimally invasive LLIF and ALIF approaches to treat grade II spondylolisthesis appear to be good, with low operative blood loss and no neurological deficits. Complete reduction of the spondylolisthesis is frequently possible with a statistically significant reduction in pain scores.

OBJECTIVEThe clinical results of deep brain stimulation (DBS) of the subthalamic nucleus (STN) are highly dependent on accurate targeting and target implantation. Several targeting tactics are in current use, including image-only and/or electrophysiologically guided approaches using microelectrode recordings (MERs). The purpose of the present study was to make an appraisal of imaging only–based versus imaging with the addition of intraoperative MER-guided STN electrode targeting.METHODSThe authors evaluated 100 consecutive patients undergoing STN DBS. The position of the STN target was estimated from preoperative MR images (direct target) or in relation to the position of the anterior and posterior commissures (indirect target). MERs were obtained for each trajectory. The authors tracked which targets were adjusted intraoperatively as a consequence of MER data. The final placement of 182 total STN electrodes was validated by intraoperative macrostimulation through the implanted DBS electrodes. The authors compared the image-based direct, indirect, MER-guided target adjustments and the final coordinates of the electrodes as seen on postoperative MRI.RESULTSIn approximately 80% of the trajectories, there was a good correspondence between the imaging-based and the MER-guided localization of the STN target. In approximately 20% of image-based targeting trajectories, however, the electrophysiological data revealed that the trajectory was suboptimal, missing the important anatomical structures to a significant extent. The greatest mismatch was in the superior-inferior axis, but this had little impact because it could be corrected without changing trajectories. Of more concern were mismatches of 2 mm or more in the mediolateral (x) or anteroposterior (y) planes, discrepancies that necessitated a new targeting trajectory to correct for the mis-targeting. The incidence of mis-targetting requiring a second MER trajectory on the first and second sides was similar (18% and 22%).CONCLUSIONSAccording to the present analysis, approximately 80% of electrodes were appropriately targeted using imaging alone. In the other 20%, imaging alone led to suboptimal targeting that could be corrected by a trajectory course correction guided by the acquired MER data. The authors’ results suggest that preoperative imaging is insufficient to obtain optimal results in all patients undergoing STN DBS.

BACKGROUND CONTEXT Postural stability (PS) is closely associated with quality of life and falls in the elderly. Recent work has demonstrated impaired gait and increased falls in patients with ASD, suggesting impaired postural stability due to biomechanical constraints of the spinal deformity. However, while previous studies have investigated the effect of spinal malalignment on static balance, no previous work has specifically focused on postural stability or dynamic balance in patients with ASD, nor the sensorimotor contribution to instability. Computerized dynamic posturography (CDP) is a widely used diagnostic and prognostic tool for assessment of postural instability in diseases of gait and imbalance such as Parkinson's disease, osteoarthritis, and vestibular dysfunction. PURPOSE The objective of our study was to assess postural stability of patients with adult spinal deformity (ASD) using cCDP and (1) compare with published normative controls, (2) determine the relationship between fall status and postural stability, and (3) determine the relationship between quality of life and postural stability in ASD STUDY DESIGN/SETTING Prospective analysis of postural stability using CDP. PATIENT SAMPLE Eleven consecutive patients with severe adult spinal deformity who were seen and evaluated in an outpatient clinic. OUTCOME MEASURES Equilibrium scores (ES) during Sensory Organization Testing (SOT) across 6 conditions with varying sensorimotor deprivation, motor latency during Motor Control Testing (MCT), and a history of falls in community setting. METHODS Eleven patients with ASD were prospectively enrolled. Inclusion criteria: coronal cobb (CC)≥20°, Pelvic incidence-lumbar lorodsis (PI-LL) >10°, sagittal vertical axis (SVA) ≥5 cm, pelvic tilt (PT)≥25°, and thoracic kyphosis (TK)≥60°. Exclusion criteria: inability to stand, presence of confounding neurological conditions. PS was determined using two randomly assigned tests with CDP: (1) SOT to evaluate the voluntary use of sensory information; and (2) motor control test (MCT) to assess involuntary postural reactions in response to external perturbations. Patient performance was compared to age-gender matched controls. QOL metrics included SRS-22 and SF-36 RESULTS Mean age was 65±4.6, and 6/13 (46%) were female. Mean PI-LL was 31.2±12.7, PT 32.8±8.0, SVA 11.8±6.3 cm, TK 27.3±16.6, and CC 14.5±16.7. Four patients reported falling in the previous 6 months (36%). Those patients who self reported falling in past 6 months demonstrated significantly lower SOT (Conditions 2–6, p CONCLUSIONS ASD patients demonstrate impaired postural stability and dynamic balance compared to normal controls. Further, postural stability and QOL metrics correlated with self-reported falls. These findings suggest that ASD patients have drastically altered balance, which predisposes these patients to elevated risk of falls and injury as well as decreased ambulatory confidence and quality of life.

Spinal arteriovenous malformations (AVMs) are a diverse population of vascular lesions associated with significant long-term morbidity and neurologic impairment. Spinal AVMs can be classified into three categories: intramedullary, extradural-intradural, and conus, depending on their angioarchitecture and anatomy. Extradural-intradural lesions are typically incurable due to their diffuse nature, but patients may benefit from targeted therapy of symptomatic components. Intramedullary and conus AVMs are amenable to safe surgical resection when combined with endovascular embolization and use of a pial dissection technique that spares entry into the neural parenchyma.

Background Hypothalamic hamartomas (HH) are rare lesions associated with treatment-resistant epilepsy. Open surgery results in modest seizure control (about 50%) but has a significant associated morbidity. Radiosurgery is limited to a subset of patients due to latent therapeutic effects. Magnetic resonance imaging-guided laser interstitial thermal therapy (LITT) offers a novel minimally invasive option. Objective To evaluate a single center's outcomes for the LITT treatment of HH. Methods We retrospectively reviewed our experience with LITT for the treatment of HH using our institution's prospectively maintained patient database. Results Eighteen patients (mean age, 21.1 yr; median age, 11 yr) underwent 21 total LITT treatments for HH. Mean follow-up was 17.4 mo. The length of stay was 1 night for 16 (89%) patients. At the end of follow-up, 11 of 18 patients (61%) had full disconnection of the HH, and 12 of 15 (80%) patients with gelastic seizures and 5 (56%) of 9 patients with nongelastic seizures were seizure free (International League Against Epilepsy Class 1). Immediate complications included a 39% (7/18) incidence of neurological deficits, including 1 case of hemiparesis. At the end of follow-up, 22% of patients (4/18) had persistent deficits. The hypothyroidism that occurred was delayed in 11% of patients (2/18), as was short-term memory loss (22%, 4/18) and weight gain (22%, 4/18). Conclusion LITT therapy for HH can achieve excellent rates of seizure control with low morbidity and a short postoperative stay in a majority of patients. Additional research is needed to assess the durability of results and the full spectrum of cognitive outcomes.

Object Astrocytomas and oligodendrogliomas are primary CNS tumors that remain a challenge to differentiate histologically because of their morphological variability and because there is a lack of reliable differential diagnostic markers. To identify proteins that are differentially expressed between astrocytomas and oligodendrogliomas, the authors analyzed the proteomic expression patterns and identified uniquely expressed proteins in these neoplasms. Methods Proteomes of astrocytomas and oligodendrogliomas were analyzed using 2D gel electrophoresis and subsequent computerized gel analysis to detect differentially expressed proteins. The proteins were identified using high-performance liquid chromatography accompanied by tandem mass spectrometry. To determine the role of the differentially expressed proteins in astrocytes, undifferentiated glial cell cultures were treated with dibutyryl–cyclic adenosine monophosphate (cAMP). Results Two-dimensional gel electrophoresis revealed that glutamine synthetase was differentially expressed in astrocytomas and oligodendrogliomas. Western blot and immunohistochemical analyses confirmed the increased expression of glutamine synthetase in astrocytomas compared with oligodendrogliomas. Whereas glutamine synthetase expression was demonstrated across all grades of astrocytomas (Grade II–IV [15 tumors]) and oligoastrocytomas (4 tumors), it was expressed in only 1 oligodendroglioma (6% [16 tumors]). Treatment of undifferentiated glial cell cultures with dibutyryl-cAMP resulted in astrocyte differentiation that was associated with increased levels of glial fibrillary acidic protein and glutamine synthetase. Conclusions These data indicate that glutamine synthetase expression can be used to distinguish astrocytic from oligodendroglial tumors and may play a role in the pathogenesis of astrocytomas.

BACKGROUND AND IMPORTANCE We present a unique case of an anterior cranial base von Hippel-Lindau disease (VHL)-associated microcystic neoplasm. To determine the lesion's relationship with VHL and its appropriate management, we discuss its salient clinical, pathological, and molecular features. CLINICAL PRESENTATION A 36-year-old woman with VHL presented with a 3-month history of phantosmia. Serial magnetic resonance imaging studies revealed a lesion within the ethmoid and frontal sinus region that was first evident 18 months before symptom development and demonstrated progressive growth over the interval period. The lesion was resected via a transbasal approach. Histopathological and immunohistochemical analysis revealed a microcystic lesion composed of bland clear cells and underlying endothelial cells consistent with a VHL-associated microcystic neoplasm that are not known to metastasize. Molecular testing demonstrated loss of heterozygosity of the VHL locus, verifying the tumor as a VHL-related neoplasm. CONCLUSION Because primary VHL-associated microcystic tumors in the anterior cranial base have not been described previously, the natural history of these tumors remains unclear. Based on the benign features of these lesions, they can be managed conservatively with close observation and surgical intervention reserved for those that produce symptoms.

Neurofibromatosis type 2 (NF2) is a multiple neoplasia syndrome and is caused by a mutation of the NF2 tumor suppressor gene that encodes for the tumor suppressor protein merlin. Biallelic NF2 gene inactivation results in the development of central nervous system tumors, including schwannomas, meningiomas, ependymomas, and astrocytomas. Although a wide variety of missense germline mutations in the coding sequences of the NF2 gene can cause loss of merlin function, the mechanism of this functional loss is unknown. To gain insight into the mechanisms underlying loss of merlin function in NF2, we investigated mutated merlin homeostasis and function in NF2-associated tumors and cell lines. Quantitative protein and RT-PCR analysis revealed that whereas merlin protein expression was significantly reduced in NF2-associated tumors, mRNA expression levels were unchanged. Transfection of genetic constructs of common NF2 missense mutations into NF2 gene-deficient meningioma cell lines revealed that merlin loss of function is due to a reduction in mutant protein half-life and increased protein degradation. Transfection analysis also demonstrated that recovery of tumor suppressor protein function is possible, indicating that these mutants maintain intrinsic functional capacity. Further, increased expression of mutant protein is possible after treatment with specific proteostasis regulators, implicating protein quality control systems in the degradative fate of mutant tumor suppressor proteins. These findings provide direct insight into protein function and tumorigenesis in NF2 and indicate a unique treatment paradigm for this disorder.

Background and importance Vein of Galen aneurysmal malformations (VGAMs) arise from persistent arteriovenous shunting from primitive choroidal vessels into the median prosencephalic vein of Markowski, the embryonic precursor of the vein of Galen. VGAMs rarely present past infancy, and their natural history in adults is unknown. We report the first case of a familial-associated VGAM in an asymptomatic adult female patient. The clinical features of this case are presented alongside a systematic review of the literature on adult VGAM cases to assess the natural history, clinical management, and genetic basis of this rare neurovascular lesion. Clinical presentation A previously healthy 44-year-old woman with a family history of a VGAM in a stillborn presented with an 8-week onset of dizziness and vertigo that spontaneously resolved. Time-resolved magnetic resonance angiography identified a choroidal VGAM. No intervention was undertaken at this time because of the patient's asymptomatic status after 9 months of follow-up. Conclusion Based on our review of the literature, this is the first case report of a familial-associated VGAM in an adult patient and suggests that VGAM development can be genetically linked. Of 15 adult VGAM cases previously reported, all patients were either symptomatic or treated, thus precluding determination of VGAM natural history in adults. Patient outcomes correlated with the severity of presenting symptoms, which ranged from asymptomatic to immediately life-threatening. We hypothesize that self-selection may render VGAMs to be more benign for them to persist past childhood. Further investigation of the molecular biology underlying VGAM development is warranted.

OBJECTIVEThe aim of this article is to review the authors’ and published experience with deep brain stimulation (DBS) therapy for the treatment of patients with Alzheimer’s disease (AD) and Parkinson’s disease dementia (PDD).METHODSTwo targets are current topics of investigation in the treatment of AD and PDD, the fornix and the nucleus basalis of Meynert. The authors reviewed the current published clinical experience with attention to patient selection, biological rationale of therapy, anatomical targeting, and clinical results and adverse events.RESULTSA total of 7 clinical studies treating 57 AD patients and 7 PDD patients have been reported. Serious adverse events were reported in 6 (9%) patients; none resulted in death or disability. Most studies were case reports or Phase 1/2 investigations and were not designed to assess treatment efficacy. Isolated patient experiences demonstrating improved clinical response after DBS have been reported, but no significant or consistent cognitive benefits associated with DBS treatment could be identified across larger patient populations.CONCLUSIONSPDD and AD are complex clinical entities, with investigation of DBS intervention still in an early phase. Recently published studies demonstrate acceptable surgical safety. For future studies to have adequate power to detect meaningful clinical changes, further refinement is needed in patient selection, metrics of clinical response, and optimal stimulation parameters.

Background The external carotid artery (ECA) anastomoses in many distal territories supplied by the internal carotid artery (ICA) and is an important source of collateral circulation to the brain. Stenosis of the ECA in ipsilateral ICA occlusion can produce ischemic sequelae. Objective To examine the effectiveness of ECA stenting in treating symptomatic ipsilateral ICA occlusion. Methods We retrospectively reviewed patient databases from 5 academic medical centers to identify all individuals who underwent ECA stenting after 1998. For all discovered cases, coinvestigators used a common submission form to harvest relevant demographic information, clinical data, procedural details, and follow-up results for further analysis. Results Twelve patients (median age, 66 years; range, 45-79 years) were identified for our cohort. Vessel disease involvement included severe ECA stenosis >or= 70% in 11 patients and ipsilateral ICA occlusion in all patients. Presenting symptoms included signs of transient ischemic attack, stroke, and amaurosis fugax. ECA stenting was associated with preservation of neurological status in 11 patients and resolution of symptoms in 5 patients at a median follow-up time of 26 months (range, 1-87 months; mean, 29 months). Symptomatic in-stent restenosis did not occur within any patient during the follow-up course. Conclusion We found ECA stenting in symptomatic ipsilateral ICA disease to be a potentially effective strategy to preserve neurological function and to relieve ischemic symptoms. Further investigation with larger studies and longer follow-up periods is warranted to elucidate the true indications of this management strategy.

Background The far-lateral transcondylar surgical approach is often used to clip vertebral artery (VA) and posterior inferior cerebellar artery (PICA) aneurysms. The role of condyle resection during this approach is controversial. Objective To evaluate patient outcomes in patients with VA-PICA aneurysms in whom drilling the occipital condyle was not necessary. Methods Between May 2005 and December 2012, a total of 56 consecutive patients with incidental or ruptured VA-PICA aneurysms underwent surgery with a far-lateral approach without condylar resection. Clinical presentation, surgical reports, presurgery and postsurgery radiological examinations, and clinical follow-up reports were assessed. Anatomic aneurysm location was analyzed through angiography or computed tomography angiography. We compared postsurgical Glasgow Outcome Scale scores, modified Rankin Scale scores, and morbidity in 2 groups: those with aneurysms in the anterior medullary segment and those with aneurysms in the lateral medullary segment. Results The predominant presentation was subarachnoid hemorrhage in 34 patients (60.7%). Most aneurysms (n = 27 [48.2%]) were located in the lateral medullary segment of the PICA, followed by the anterior medullary segment (n = 25 [44.6%]). Total aneurysm occlusion was achieved in 100% of patients, and bypass techniques were necessary in 3 patients (5.4%). Fifty-two patients (92.8%) had Glasgow Outcome Scale scores of 4 or 5 postsurgery. Conclusions A far-lateral approach that leaves the occipital condyle intact is adequate for treating most patients with VA-PICA aneurysms.

Background Intracranial hemangioblastomas are highly vascular tumors that account for 1% to 2% of all central nervous system tumors. Preoperative embolization has been proposed to limit the often significant intraoperative blood loss associated with resection and potentially make the tumor more soft/necrotic and thus more amenable to gross total resection. The safety and efficacy of preoperative embolization of intracranial hemangioblastomas, however, are not well characterized. Objective To evaluate the safety and efficacy of preoperative endovascular embolization of intracranial hemangioblastomas using a variety of embolic agents. Methods A retrospective review of all surgically resected intracranial hemangioblastomas treated with preoperative embolization between 1999 and 2014 at 2 high-volume centers was performed. Clinical and radiographic criteria, including von Hippel-Lindau status, magnetic resonance imaging tumor characteristics, embolization-related complications, degree of angiographic devascularization, intraoperative blood loss, ability to obtain gross total resection, transfusion requirements, and operative time, were analyzed. Results A total of 54 patients underwent surgery, with 24 undergoing preoperative embolization followed by surgical resection, and 30 patients undergoing surgical resection alone. Embolization-related neurological complications were seen in 6 patients (25%), including 3 hemorrhages when polyvinyl alcohol particles (P = .04) were used and 3 infarctions when liquid embolic agents were used (P = .27). Permanent neurological deficits were seen in 15%. Conclusion Preoperative embolization of intracranial hemangioblastomas should be performed with caution, given the potential for neurological morbidity. Further studies are needed to help guide patient and embolic agent selection.

Background/Objective: Decisions to use open surgery or radiotherapy in pediatric patients with familial neoplastic syndromes must consider not only the symptomatic benefits of treatment, but also future limitations these treatments may impose. Specifically, open surgical resection of noncurable tumors may preclude or encumber future lesion resections, while radiotherapy has detrimental effects on pediatric cognitive development and increases the risk of future malignancy development. We provide the first report of using a novel 3.0-mm diffusing laser tip with laser-induced thermal therapy (LiTT) to treat a pediatric patient with neurofibromatosis type 1 (NF-1). Methods: A 12-year-old boy with NF-1 presented with a progressively enlarging lesion in the right midbrain. A stereotactic biopsy was performed, followed by LiTT with a novel 3.0-mm laser applicator. Results: MRI 1 week after LiTT showed stable gross total ablation of the lesion with reduction in fluid-attenuated inversion recovery signal. The patient remained neurologically intact 6 months after his procedure, and follow-up MRI showed no evidence of recurrence. Conclusion: LiTT is a powerful adjunct to conventional open surgical and radiotherapy modalities in the treatment of patients with familial neoplastic syndromes or incurable lesions. The novel laser applicator tip described expands the treatment scope of this technique.

Glioblastoma multiforme is the most common and malignant primary brain tumor. Recent evidence indicates that a subset of glioblastoma tumor cells have a stem cell like phenotype that underlies chemotherapy resistance and tumor recurrence. We utilized a new "multidimensional" capillary isoelectric focusing nano-reversed-phase liquid chromatography platform with tandem mass spectrometry to compare the proteomes of isolated glioblastoma tumor stem cell and differentiated tumor cell populations. This proteomic analysis yielded new candidate proteins that were differentially expressed. Specifically, two isoforms of the membrane proteolipid neuronatin (NNAT) were expressed exclusively within the tumor stem cells. We surveyed the expression of NNAT across 10 WHO grade II and III gliomas and 23 glioblastoma (grade IV) human tumor samples and found NNAT was expressed in a subset of primary glioblastoma tumors. Through additional in vitro studies utilizing the U87 glioma cell line, we found that expression of NNAT is associated with significant increases in cellular proliferation. Paralleling the in vitro results, when NNAT levels were evaluated in tumor specimens from a consecutive cohort of 59 glioblastoma patients, the presence of increased levels of NNAT were found to be a an independent risk factor (P = 0.006) for decreased patient survival through Kaplan-Meier and multivariate analysis. These findings indicate that NNAT may have utility as a prognostic biomarker, as well as a cell-surface target for chemotherapeutic agents.

Mesenchymal stems cells (MSCs) are a population of multipotent cells residing in several readily available adult tissue compartments, thus allowing for their ex vivo expansion. To date, therapeutic applications of MSCs have focused on their ability to hone to and support the repair of damaged tissues. More recent evidence suggests that MSCs possess low immunogenicity and a diverse array of immunosuppressive properties. In thisarticle, we will review the basic biology of bone marrow-derived MSCs including their immunoregulatory effects and the putative mechanisms underlying them. We will then present some of the recent clinical applications of MSCs that have leveraged these effects for the treatment of immune-mediated dermatoses such as graft-versus-host disease and systemic lupus erythematosus. Although MSCs offer great therapeutic promise, we will also highlight a number of pertinent challenges that should be overcome before their successful clinical translation.

OBJECTIVE Detachable endovascular coils have become a common treatment strategy for carotid cavernous sinus aneurysms (CCAs), but previously unrecognized postprocedure complications may emerge as longer follow-up data are accumulated. In this report, the authors document the first known cases of delayed cranial neuropathy following CCA coiling in 3 patients, all of whom present at least a year postprocedure without aneurysm regrowth. The potential mechanisms underlying this syndrome are discussed as well as their implications on the selection and optimal endovascular management of CCA patients. CLINICAL PRESENTATION Three previously healthy females aged 50, 60, and 62 underwent CCA coiling at our institution and subsequently developed ipsilateral cranial nerve palsies at 56, 28, and 14 months, respectively, post-procedure. At presentation, all 3 patients had a new, recurrent area of flow in their CCA without changes in aneurysm size. INTERVENTION One patient declined further treatment. In the other 2 patients, a stent was placed across the aneurysm neck, and one patient underwent additional coiling. Unfortunately, all 3 patients remained symptomatic at their latest follow-up. CONCLUSION Because of the intimate anatomic environment of the cavernous sinus, neural elements within it may be particularly susceptible to persistent mass or dynamic effects exacerbated by remnant or recurrent flow across the neck of a coiled aneurysm. These 3 cases prompted the authors to advocate for more aggressive efforts to achieve and maintain CCA occlusion. Furthermore, when such efforts are unsuccessful, consideration of traditional carotid occlusion strategies with or without bypass is warranted.