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Michael Fana, Eleanor C. Smith, Jessica L. Gable, Nihal Manjila and Sunil Manjila

The Nazi regime held power for well over a decade in Germany and were steadfast in their anti-Semitic agenda. Among the massive cohort of immigrants to America were approximately 5056 Jewish physicians, including several highly esteemed neurologists and neuroscientists of the time. Emigrating to a new world proved difficult and provided new challenges by way of language barriers, roadblocks in medical careers, and problems integrating into an alien system of medical training and clinical practice. In this article, the authors examine the tumultuous and accomplished lives of three Jewish German and Austrian neurologists and neuroscientists during the time of the Third Reich who shaped the foundations of neuroanatomy and neuropsychology: Josef Gerstmann, Adolf Wallenberg, and Franz Josef Kallmann. The authors first examine the successful careers of these individuals in Germany and Austria prior to the Third Reich, followed by their journeys to and lives in the United States, to demonstrate the challenges an émigré physician faces for career opportunities and a chance at a new life. This account culminates in a description of these scientists’ eponymous syndromes.

Although their stories are a testimony to the struggles in Nazi Germany, there are intriguing and notable differences in their ages, ideologies, and religious beliefs, which highlight a spectrum of unique circumstances that impacted their success in the United States. Furthermore, in this account the authors bring to light the original syndromic descriptions: Gerstmann discovered contralateral agraphia and acalculia, right-left confusion, and finger agnosia in patients with dominant angular gyrus damage; Wallenberg described a constellation of symptoms in a patient with stenosis of the posterior inferior cerebellar artery; and Kallmann identified an association between hypogonadotropic hypogonadism and anosmia based on family studies. The article also highlights the unresolved confusions and international controversies about these syndromic descriptions. Still, these unique cerebral syndromes continue to fascinate neurologists and neurosurgeons across the world, from residents in training to practicing clinicians and neuroscientists alike.

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Sunil Manjila, Timothy Bazil, Matthew Kay, Unni K. Udayasankar and Maroun Semaan

OBJECTIVE

There is no definitive or consensus classification system for the jugular bulb position that can be uniformly communicated between a radiologist, neurootologist, and neurosurgeon. A high-riding jugular bulb (HRJB) has been variably defined as a jugular bulb that rises to or above the level of the basal turn of the cochlea, within 2 mm of the internal auditory canal (IAC), or to the level of the superior tympanic annulus. Overall, there is a seeming lack of consensus, especially when MRI and/or CT are used for jugular bulb evaluation without a dedicated imaging study of the venous anatomy such as digital subtraction angiography or CT or MR venography.

METHODS

A PubMed analysis of “jugular bulb” comprised of 1264 relevant articles were selected and analyzed specifically for an HRJB. A novel classification system based on preliminary skull base imaging using CT is proposed by the authors for conveying the anatomical location of the jugular bulb. This new classification includes the following types: type 1, no bulb; type 2, below the inferior margin of the posterior semicircular canal (SCC), subclassified as type 2a (without dehiscence into the middle ear) or type 2b (with dehiscence into the middle ear); type 3, between the inferior margin of the posterior SCC and the inferior margin of the IAC, subclassified as type 3a (without dehiscence into the middle ear) and type 3b (with dehiscence into the middle ear); type 4, above the inferior margin of the IAC, subclassified as type 4a (without dehiscence into the IAC) and type 4b (with dehiscence into the IAC); and type 5, combination of dehiscences. Appropriate CT and MR images of the skull base were selected to validate the criteria and further demonstrated using 3D reconstruction of DICOM files. The microsurgical significance of the proposed classification is evaluated with reference to specific skull base/posterior fossa pathologies.

RESULTS

The authors validated the role of a novel classification of jugular bulb location that can help effective communication between providers treating skull base lesions. Effective utilization of the above grading system can help plan surgical procedures and anticipate complications.

CONCLUSIONS

The authors have proposed a novel anatomical/radiological classification system for jugular bulb location with respect to surgical implications. This classification can help surgeons in complication avoidance and management when addressing HRJBs.

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Sunil Manjila, Timothy Bazil, Matthew Thomas, Sunithi Mani, Matthew Kay and Unni Udayasankar

This paper is a narrative review of extraaxial developmental venous anomalies (eDVAs) of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, enlarged emissary veins, and venous varices or aneurysmal malformations. The article highlights the natural history, anatomy, embryology, imaging, clinical implications, and neurosurgical significance of these lesions, which the authors believe represent a continuum, with different entities characterized by distinct embryopathologic features. The indications and surgical management options are discussed for these individual intracranial pathologies with relevant illustrations, and a novel classification is proposed for persistent falcine sinus (PFS). The role of neurointervention and/or microsurgery in specific cases such as sinus pericranii and enlarged emissary veins of the skull is highlighted.

A better understanding of the pathophysiology and developmental anatomy of these lesions can reduce treatment morbidity and mortality. Some patients, including those with vein of Galen malformations (VOGMs), can present with the added systemic morbidity of a high-output cardiac failure. Although VOGM is the most studied and classified of the above-mentioned eDVAs, the authors believe that grouping the former with the other venous anomalies/abnormalities listed above would enable the clinician to convey the exact morphophysiological configuration of these lesions, predict their natural history with respect to evolving venous hypertension or stroke, and extrapolate invaluable insights from VOGM treatment to the treatment of other eDVAs. In recent years, many of these symptomatic venous malformations have been treated with endovascular interventions, although these techniques are still being refined. The authors highlight the broad concept of eDVAs and hope that this work will serve as a basis for future studies investigating the role of evolving focal venous hypertension/global intracranial hypertension and possibilities of fetal surgical intervention in these cases.

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Ciro Ramos-Estebanez, Maryo Kohen, Jonathan Pace, Alireza Bozorgi, Sunil Manjila, Vilakshan Alambyan, Ifeyinwa Nwankwo, Michael DeGeorgia, Nicholas C. Bambakidis and Faruk Orge

OBJECTIVE

Approximately 10% of patients with subarachnoid hemorrhage (SAH) become permanently, legally blind. The average cost of lifetime support and unpaid taxes for each blind person amounts to approximately $900,000. This study evaluates the feasibility and potential role of bedside optical coherence tomography (OCT) in Terson’s syndrome (TS) in patients with acute SAH (aSAH) and its potential role in blindness prevention.

METHODS

The authors conducted an open-label pilot study, in which 31 patients with an angiographic diagnosis of aSAH were first screened for TS with dilated funduscopy and then with OCT in the acute phase and at 6-week follow-up visits. Outpatient mood assessments (Patient Health Questionnaire–depression module, Hamilton Depression Scale), and quality of life general (NIH Patient-Reported Outcomes Measurement Information System) and visual scales (25-item National Eye Institute Visual Functioning Questionnaire) were measured at 1 and 6 weeks after discharge. Exclusion criteria included current or previous history of severe cataracts, severe diabetic retinopathy, severe macular degeneration, or glaucoma.

RESULTS

OCT identified 7 patients with TS, i.e., a 22.6% incidence in our aSAH sample: 7 in the acute phase, including a large retinal detachment that was initially missed by funduscopy and diagnosed by OCT in follow-up clinic. Dilated retinal funduscopy significantly failed to detect TS in 4 (57.1%) of these 7 cases. Intraventricular hemorrhage was significantly more common in TS cases (85.7% vs 25%). None of the participants experienced any complications from OCT examinations. Neither decreased quality of life visual scale scores nor a depressed mood correlated with objective OCT pathological findings at the 6-week follow-up after discharge. There were no significant mood differences between TS cases and controls.

CONCLUSIONS

OCT is the gold standard in retinal disease diagnosis. This pilot study shows that bedside OCT examination is feasible in aSAH. In this series, OCT was a safe procedure that enhanced TS detection by decreasing false-negative/inconclusive funduscopic examinations. It allows early diagnosis of macular holes and severe retinal detachments, which require acute surgical therapy to prevent legal blindness. In addition, OCT aids in ruling out potential false-positive visual deficits in individuals with a depressed mood at follow-up.

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Sunil Manjila, Gagandeep Singh, Obinna Ndubuizu, Zoe Jones, Daniel P. Hsu and Alan R. Cohen

The authors demonstrate the use of an endovascular plug in securing a carotid artery pseudoaneurysm in an emergent setting requiring craniotomy for a concurrent subdural empyema.

They describe the case of a 14-year-old boy with sinusitis and bifrontal subdural empyema who underwent transsphenoidal exploration at an outside hospital. An injury to the right cavernous segment of the ICA caused torrential epistaxis. Bleeding was successfully controlled by inflating a Foley balloon catheter within the sphenoid sinus, and the patient was transferred to the authors’ institution. Emergent angiography showed a dissection of the right cavernous carotid artery, with a large pseudoaneurysm projecting into the sphenoid sinus at the site of arterial injury. The right internal carotid artery was obliterated using pushable coils distally and an endovascular plug proximally. The endovascular plug enabled the authors to successfully exclude the pseudoaneurysm from the circulation. The patient subsequently underwent an emergent bifrontal craniotomy for evacuation of a left frontotemporal subdural empyema and exenteration of both frontal sinuses. He made a complete neurological recovery.

Endovascular large-vessel sacrifice, obviating the need for numerous coils and antiplatelet therapy, has a role in the setting of selected acute neurosurgical emergencies necessitating craniotomy. The endovascular plug is a useful adjunct in such circumstances as the device can be deployed rapidly, safely, and effectively.

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Akshay Sharma, Gabrielle E. Rieth, Joseph E. Tanenbaum, James S. Williams, Nakao Ota, Srikant Chakravarthi, Sunil Manjila, Amin Kassam and Bulent Yapicilar

OBJECTIVE

The middle clinoid process (MCP) is a bony projection that extends from the sphenoid bone near the lateral margin of the sella turcica. The varied prevalence and morphological features of the MCP in populations stratified by age, race, and sex are unknown; however, the knowledge of its anatomy and preoperative recognition on CT scans can aid greatly in complication avoidance and management. The aim of this study was to further illustrate the surgical anatomy of the parasellar region and to quantify the incidence of MCP and caroticoclinoid rings (CCRs) in dried preserved human anatomical specimens.

METHODS

The presence, dimensions, morphological classification (incomplete, contact, and CCR), and intracranial relations of the MCP were measured in 2726 dried skull specimens at the Hamann-Todd Osteological Collection at the Cleveland Museum of Natural History. Specific morphometric data points were recorded from each of these hemiskulls, and categorized based on age, sex, and ethnicity. Linear and logistic regressions were used to determine associations between explanatory variables and MCP morphology. Computed tomography scans of the skull specimens were obtained to explore radiological landmarks for different types of MCPs. Illustrative intraoperative videos were also analyzed in the light of these crucial surgical landmarks.

RESULTS

The sample included 2250 specimens from males and 476 from females. Specimens were classified as either “white” (60.5%) or “black” (39.2%). An MCP was found in 42% of specimens, with 60% of those specimens presenting bilaterally. Fully ossified CCR comprised 27% of all MCPs, and contact (defined as contact without ossification between MCP and anterior clinoid process) comprised 4% of all MCPs. White race (relative to black race) and increasing age were significant predictors of MCP presence (p < 0.001). White race was significantly associated with greater average MCP height (p < 0.001). Among skulls with CCR, both male sex and older age (> 70 years relative to < 50 years) were associated with increased CCR diameter (p < 0.001). No other significant predictors or associations were observed. The CT scans of skulls replicated and validated the authors’ morphometric observations on incomplete, contact, and CCR patterns adequately. The surgical strategies of clinoid bone removal are validated, with appropriate video illustrations.

CONCLUSIONS

Variations in the patterns of bony MCPs can pose a significant risk for injury to the internal carotid artery during parasellar procedures, especially those involving clinoidectomy and optic strut drilling. Understanding parasellar anatomy, especially on skull-base CT imaging, may be integral to surgical planning and preoperative risk counseling in both transcranial and extended endonasal procedures, as well as to preparing for complications management perioperatively.

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Jason K. Hsieh, Christopher S. Hong, Sunil Manjila, Mark L. Cohen, Simon Lo, Lisa Rogers and Andrew E. Sloan

The authors present the case of a primary gliosarcoma with an isocitrate dehydrogenase-1 (IDH1) mutation. A 75-year-old man presented with a 3-day history of multiple focal seizures and was found on MRI to have a 2.2-cm left parietal enhancing mass lesion. Brain MRI for tremor performed 8 years prior to this presentation was normal. En bloc resection revealed a high-grade glioma with sarcomatous components that was immunoreactive for the R132H variant of IDH1 by antibody. Gliosarcoma is a rare variant of glioblastoma that arises most frequently as a primary tumor, and has equal or worse survival and an increased propensity for extracranial metastases compared with other Grade 4 gliomas. In contrast, isocitrate dehydrogenase-1 and -2 mutations are associated with low-grade gliomas with increased survival and less commonly with glioblastoma. To the authors' knowledge, there has been only 1 other published report of a primary gliosarcoma carrying an isocitrate dehydrogenase mutation. This rare genetic-histological combination highlights potential differences between glioblastoma and gliosarcoma and may warrant additional study.

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Berje Shammassian, Sunil Manjila, Efrem Cox, Kaine Onwuzulike, Dehua Wang, Mark Rodgers, Duncan Stearns and Warren R. Selman

Intracranial ectopic salivary gland rests within dural-based lesions are reported very infrequently in the literature. The authors report the unique case of a 12-year-old boy with a cerebellar medulloblastoma positive for sonic hedgehog (Shh) that contained intraaxial mature ectopic salivary gland rests. The patient underwent clinical and radiological monitoring postoperatively, until he died of disseminated disease. An autopsy showed no evidence of salivary glands within disseminated lesions. The intraaxial presence of salivary gland rests and concomitant Shh positivity of the described tumor point to a disorder in differentiation as opposed to ectopic developmental foci, which are uniformly dural based in the described literature. The authors demonstrate the characteristic “papilionaceous” appearance of the salivary glands with mucicarmine stain and highlight the role of Shh signaling in explaining the intraaxial presence of seromucous gland analogs. This article reports the first intraaxial posterior fossa tumor with heterotopic salivary gland rests, and it provides molecular and embryopathological insights into the development of these lesions.

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Anand Kaul, Ahmed Abbas, Gabriel Smith, Sunil Manjila, Jonathan Pace and Michael Steinmetz

Fatal craniovertebral junction (CVJ) injuries were the most common cause of death in high-speed motor sports prior to 2001. Following the death of a mutual friend and race car driver, Patrick Jacquemart (1946–1981), biomechanical engineer Dr. Robert Hubbard, along with race car driver and brother-in-law Jim Downing, developed the concept for the Head and Neck Support (HANS) device to prevent flexion-distraction injuries during high-velocity impact. Biomechanical testing showed that neck shear and loading forces experienced during collisions were 3 times the required amount for a catastrophic injury. Crash sled testing with and without the HANS device elucidated reductions in neck tension, neck compression, head acceleration, and chest acceleration experienced by dummies during high-energy crashes. Simultaneously, motor sports accidents such as Dale Earnhardt Sr.'s fatal crash in 2001 galvanized public opinion in favor of serious safety reform. Analysis of Earnhardt's accident demonstrated that his car's velocity parallel to the barrier was more than 150 miles per hour (mph), with deceleration upon impact of roughly 43 mph in a total of 0.08 seconds. After careful review, several major racing series such as the National Association for Stock Car Auto Racing (NASCAR) and Championship Auto Racing Team (CART) made major changes to ensure the safety of drivers at the turn of the 21st century. Since the rule requiring the HANS device in professional auto racing series was put in place, there has not been a single reported case of a fatal CVJ injury.

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Sunil Manjila, Margherita Mencattelli, Benoit Rosa, Karl Price, Georgios Fagogenis and Pierre E. Dupont

OBJECTIVE

Rigid endoscopes enable minimally invasive access to the ventricular system; however, the operative field is limited to the instrument tip, necessitating rotation of the entire instrument and causing consequent tissue compression while reaching around corners. Although flexible endoscopes offer tip steerability to address this limitation, they are more difficult to control and provide fewer and smaller working channels. A middle ground between these instruments—a rigid endoscope that possesses multiple instrument ports (for example, one at the tip and one on the side)—is proposed in this article, and a prototype device is evaluated in the context of a third ventricular colloid cyst resection combined with septostomy.

METHODS

A prototype neuroendoscope was designed and fabricated to include 2 optical ports, one located at the instrument tip and one located laterally. Each optical port includes its own complementary metal-oxide semiconductor (CMOS) chip camera, light-emitting diode (LED) illumination, and working channels. The tip port incorporates a clear silicone optical window that provides 2 additional features. First, for enhanced safety during tool insertion, instruments can be initially seen inside the window before they extend from the scope tip. Second, the compliant tip can be pressed against tissue to enable visualization even in a blood-filled field. These capabilities were tested in fresh porcine brains. The image quality of the multiport endoscope was evaluated using test targets positioned at clinically relevant distances from each imaging port, comparing it with those of clinical rigid and flexible neuroendoscopes. Human cadaver testing was used to demonstrate third ventricular colloid cyst phantom resection through the tip port and a septostomy performed through the lateral port. To extend its utility in the treatment of periventricular tumors using MR-guided laser therapy, the device was designed to be MR compatible. Its functionality and compatibility inside a 3-T clinical scanner were also tested in a brain from a freshly euthanized female pig.

RESULTS

Testing in porcine brains confirmed the multiport endoscope's ability to visualize tissue in a blood-filled field and to operate inside a 3-T MRI scanner. Cadaver testing confirmed the device's utility in operating through both of its ports and performing combined third ventricular colloid cyst resection and septostomy with an endoscope rotation of less than 5°.

CONCLUSIONS

The proposed design provides freedom in selecting both the number and orientation of imaging and instrument ports, which can be customized for each ventricular pathological entity. The lightweight, easily manipulated device can provide added steerability while reducing the potential for the serious brain distortion that happens with rigid endoscope navigation. This capability would be particularly valuable in treating hydrocephalus, both primary and secondary (due to tumors, cysts, and so forth). Magnetic resonance compatibility can aid in endoscope-assisted ventricular aqueductal plasty and stenting, the management of multiloculated complex hydrocephalus, and postinflammatory hydrocephalus in which scarring obscures the ventricular anatomy.