Publications by authors named "Erik H Middlebrooks"

76 Publications

Encephalocele-Associated Drug Resistant Epilepsy of Adult-Onset: Diagnosis, Management, and Outcomes.

World Neurosurg 2021 May 5. Epub 2021 May 5.

Epileptogenic encephaloceles, most frequently located in the temporal lobe, are a known lesional cause of focal epilepsy. Data are limited regarding diagnosis, management, and outcomes of patients with epilepsy in the setting of an encephalocele, as the literature is mostly comprised of case reports, case series, and retrospective studies. We conducted a broad literature review for manuscripts related to encephaloceles and epilepsy regardless of level of evidence. Hence, this manuscript provides a summary of all available literature related to the topic. Thirty-six scientific reports that fulfilled our inclusion criteria were reviewed. The majority of reported cases presented with focal impaired awareness seizures and/or generalized tonic-clonic seizures. Although most of the encephaloceles were located in the temporal lobe, we found five cases of extra-temporal encephaloceles causing epilepsy. A greater proportion of cases that underwent either lesionectomy or lobectomy were seizure free at time of follow-up. In the temporal lobe, there is no clear consensus on the appropriate management for epileptic encephaloceles and further studies are warranted to understand the associated factors and long-term outcomes associated to epilepsy secondary to encephaloceles. Published data suggests that these cases could be potentially be manageable with surgical procedures including lesionectomy or lobectomy. Additionally, due to data suggesting similar results between procedures, a more conservative surgery with lesionectomy and defect repair rather than a lobectomy may potentially have lower surgical risks while having similar seizure freedom.
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http://dx.doi.org/10.1016/j.wneu.2021.04.121DOI Listing
May 2021

ANCA-associated vasculitis and severe proximal muscle weakness.

Proc (Bayl Univ Med Cent) 2021 Jan 14;34(3):384-386. Epub 2021 Jan 14.

Department of Internal Medicine, Mayo Clinic, Jacksonville, Florida.

ANCA-associated vasculitis is a multiorgan autoimmune inflammatory disease that has a heterogeneous clinical presentation. Our case report provides additional evidence supporting the association between granulomatosis with polyangiitis and myositis. In our patient with proximal muscle weakness and pain, a normal creatine kinase and lack of antibodies to muscular fiber units ruled out primary myositis. Distinct magnetic resonance imaging of the brain within the deep gray matter in addition to positive serologies were consistent with a diagnosis of granulomatosis with polyangiitis. ANCA-associated vasculitis, specifically granulomatosis with polyangiitis, may be overlooked if musculoskeletal manifestations are the presenting symptoms. Prompt and aggressive treatment prevented this patient from experiencing multiorgan failure.
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http://dx.doi.org/10.1080/08998280.2020.1871177DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8059901PMC
January 2021

Comparative connectivity correlates of dystonic and essential tremor deep brain stimulation.

Brain 2021 Apr 23. Epub 2021 Apr 23.

Department of Radiology, Mayo Clinic, Jacksonville, FL, USA.

The pathophysiology of dystonic tremor and essential tremor remains partially understood. In patients with medication-refractory dystonic tremor or essential tremor, deep brain stimulation (DBS) targeting the thalamus or posterior subthalamic area has evolved into a promising treatment option. However, the optimal DBS targets for these disorders remains unknown. This retrospective study explored the optimal targets for DBS in essential tremor and dystonic tremor using a combination of volumes of tissue activated estimation and functional and structural connectivity analyses. We included 20 patients with dystonic tremor who underwent unilateral thalamic DBS, along with a matched cohort of 20 patients with essential tremor DBS. Tremor severity was assessed preoperatively and approximately 6 months after DBS implantation using the Fahn-Tolosa-Marin Tremor Rating Scale. The tremor-suppressing effects of DBS were estimated using the percentage improvement in the unilateral tremor-rating scale score contralateral to the side of implantation. The optimal stimulation region, based on the cluster centre of gravity for peak contralateral motor score improvement, for essential tremor was located in the ventral intermediate nucleus region and for dystonic tremor in the ventralis oralis posterior nucleus region along the ventral intermediate nucleus/ventralis oralis posterior nucleus border (4 mm anterior and 3 mm superior to that for essential tremor). Both disorders showed similar functional connectivity patterns: a positive correlation between tremor improvement and involvement of the primary sensorimotor, secondary motor and associative prefrontal regions. Tremor improvement, however, was tightly correlated with the primary sensorimotor regions in essential tremor, whereas in dystonic tremor, the correlation was tighter with the premotor and prefrontal regions. The dentato-rubro-thalamic tract, comprising the decussating and non-decussating fibres, significantly correlated with tremor improvement in both dystonic and essential tremor. In contrast, the pallidothalamic tracts, which primarily project to the ventralis oralis posterior nucleus region, significantly correlated with tremor improvement only in dystonic tremor. Our findings support the hypothesis that the pathophysiology underpinning dystonic tremor involves both the cerebello-thalamo-cortical network and the basal ganglia-thalamo-cortical network. Further our data suggest that the pathophysiology of essential tremor is primarily attributable to the abnormalities within the cerebello-thalamo-cortical network. We conclude that the ventral intermediate nucleus/ventralis oralis posterior nucleus border and ventral intermediate nucleus region may be a reasonable DBS target for patients with medication-refractory dystonic tremor and essential tremor, respectively. Uncovering the pathophysiology of these disorders may in the future aid in further improving DBS outcomes.
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http://dx.doi.org/10.1093/brain/awab074DOI Listing
April 2021

Intraoperative Seizure Detection During Active Resection of Glioblastoma Through a Novel Hollow Circular Electrocorticography Array.

Oper Neurosurg (Hagerstown) 2021 Apr 22. Epub 2021 Apr 22.

Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA.

Background And Importance: Data supporting the use of electrocorticography (ECoG) monitoring during electrical stimulation in awake craniotomies for resection of supratentorial neoplasms is robust, but its applicability during active resection is often limited by the inability to keep the array in place. Given the known survival benefit of gross total resection in glioma surgery, novel approaches to surgical monitoring are warranted to maximize safe resection and optimize surgical outcomes in patients with glioblastoma.

Clinical Presentation: A 68-yr-old right-handed woman presented to the emergency department with confusion. Imaging studies revealed a bifrontal intra-axial brain lesion. She underwent a left-sided awake craniotomy procedure with cortical and subcortical mapping. During surgical resection, multiple electrographic seizures were detected on continuous ECoG monitoring with a customized 22-channel high-density hollow circular array. She remained without clinical evidence of seizures at 3 mo after surgery.

Conclusion: We report a unique case of serial electrographic seizures detected during continuous intraoperative ECoG monitoring during active surgical resection of a glioblastoma using a novel circular hollow array during an awake craniotomy. The use of continuous ECoG monitoring during active resection may provide additional data, with potential influence in outcomes for patients undergoing resection of high-grade glial neoplasms.
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http://dx.doi.org/10.1093/ons/opab110DOI Listing
April 2021

The benefits of inferolateral transtubercular route on intradural surgical exposure using the endoscopic endonasal transclival approach.

Acta Neurochir (Wien) 2021 Apr 13. Epub 2021 Apr 13.

Department of Neurosurgery, Microsurgical Neuroanatomy Laboratory, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Background: Surgical access to the ventral pontomedullary junction (PMJ) can be achieved through various corridors depending on the location and extension of the lesion. The jugular tubercle (JT), a surgically challenging obstacle to access the PMJ, typically needs to be addressed in transcranial exposures. We describe the endoscopic endonasal transclival approach (EETCA) and its inferolateral transtubercular extension to assess the intradural surgical field gained through JT removal. We also complement the dissections with an illustrative case.

Methods: EETCA was surgically simulated, and the anatomical landmarks were assessed in eight cadaveric heads. Microsurgical dissections were additionally performed along the endoscopic surgical path. Lastly, we present an intraoperative video of the trans-JT approach in a patient with lower clival chordoma.

Results: The EETCA allowed adequate extracranial visualization and removal of the JT. The surgical bony window-obtained along the clivus and centered at the JT via the EETCA-measured 11 × 9 × 7 mm. Removal of the JT provided an improved intradural field within the lower third of the cerebellopontine cistern to expose an area bordered by the cranial nerves VII/VIII and flocculus superior and anterior margin of the lateral recess of the fourth ventricle and cranial nerves IX-XI inferiorly, centered on the foramen of Luschka.

Conclusions: Removal of the JT via EETCA improves exposure along the lower third of the cerebellopontine and upper cerebellomedullary cisterns. The inferolateral transtubercular extension of the EETCA provides access to the lateral recess of the fourth ventricle, in combination with the ventral midline pontomedullary region.
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http://dx.doi.org/10.1007/s00701-021-04835-xDOI Listing
April 2021

Crying with depressed affect induced by electrical stimulation of the anterior insula: A stereo EEG case study.

Epilepsy Behav Rep 2021 31;15:100421. Epub 2020 Dec 31.

Department of Neurology, Mayo Clinic, Jacksonville, FL, USA.

Stereo-EEG (sEEG) is an invasive recording technique used to localize the seizure-onset zone for epilepsy surgery in people with drug-resistant focal seizures. Pathological crying reflects disordered emotional expression and the anterior insula is known to play a role in empathy and socio-emotional processing. We describe a patient where electrical stimulation mapping (ESM) of the anterior insula during sEEG generated pathological crying and profound sadness that was time-locked to the electrical stimulus. We evaluated a 35-year-old left-handed female for repeat epilepsy surgery. The patient had drug resistant focal impaired awareness seizures despite a previous left temporal neocortical resection informed by an invasive study using subdural grid and strip electrodes seven years earlier. She was studied invasively with 10 sEEG electrodes sampling temporal, occipital, and insular targets. In the process of functional mapping, stimulation of the anterior insular cortex provoked tearful crying with sad affect, reproducible upon repeat stimulation. Our case is unique in demonstrating transitory pathological crying with profound sadness provoked by ESM of the left anterior insula. Furthermore we demonstrate repeated time-synched crying from electrical stimulation, which supports the hypothesis that the anterior insula in the brain plays an important role in the biology of emotion, as implicated by previous studies.
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http://dx.doi.org/10.1016/j.ebr.2020.100421DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7817500PMC
December 2020

Creutzfeldt-Jakob Disease: An Unusual Presentation of Corticobasal Syndrome.

Cureus 2020 Nov 9;12(11):e11393. Epub 2020 Nov 9.

Radiology, Mayo Clinic, Jacksonville, USA.

Corticobasal syndrome is an atypical parkinsonian syndrome consisting of a constellation of clinical findings that can be the result of various etiologies. While most cases are a result of a tauopathy, such as corticobasal degeneration, other etiologies must be considered in the evaluation of patients presenting with corticobasal syndrome. We present a case of a patient presenting with clinical features of corticobasal syndrome due to a prion disease, Creutzfeldt-Jakob disease (CJD), who was initially misdiagnosed due to known pitfalls in the CJD diagnostic criteria. We further discuss this unusual manifestation of CJD presenting as corticobasal syndrome and relevant diagnostic consideration in the evaluation of this entity.
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http://dx.doi.org/10.7759/cureus.11393DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7725196PMC
November 2020

A Rare Congenital Cause of Epilepsy.

Cureus 2020 Oct 27;12(10):e11204. Epub 2020 Oct 27.

Neuroradiology, Mayo Clinic, Jacksonville, USA.

Enlarged parietal foramina (PFM) are congenital calvarial defects characterized by bilateral parietal bone defects (>5 mm), occurring on each side of the sagittal suture along its posterior aspect. While often lacking underlying intracranial malformations, there has been increasing recognition of coexisting brain malformations in certain subtypes. We present a case of a 12-year-old girl presenting with new-onset grand mal seizure with developmental delay and a known family history of epilepsy. Brain MRI revealed large, bilateral parietal bone defects with underlying cortical malformation (polymicrogyria and ulegyria) and vascular abnormalities (persistent falcine sinus), related to PFM. This case report describes the genetic basis for recognized subtypes of PFM and the rare association of brain malformations associated with PFM due to mutations in the ALX4 homeobox gene.
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http://dx.doi.org/10.7759/cureus.11204DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7704017PMC
October 2020

Intraoperative Direct Stimulation Identification and Preservation of Critical White Matter Tracts During Brain Surgery.

World Neurosurg 2021 Feb 24;146:64-74. Epub 2020 Oct 24.

Department of Neurological Surgery, Mayo Clinic, Jacksonville, Florida, USA.

The study of brain connectomics has led to a rapid evolution in the understanding of human brain function. Traditional localizationist theories are being replaced by more accurate network, or hodologic, approaches that model brain function as widespread processes dependent on cortical and subcortical structures, as well as the white matter tracts (WMTs) that link these areas. Recent surgical literature suggests that WMTs may be more critical to preserve than cortical structures because of the comparably lower capacity of recovery of the former when damaged. Given the relevance of eloquent WMTs to neurologic function and thus quality of life, neurosurgical interventions must be tailored to maximize their preservation. Direct electric stimulation remains a vital tool for identification and avoidance of these critical tracts. Neurosurgeons therefore require proper understanding of the anatomy and function of WMTs, as well as the reported contemporary tasks used during intraoperative stimulation. We review the relevant tracts involved in language, visuospatial, and motor networks and the updated direct electric stimulation-based mapping tasks that aid in their preservation. The dominant-hemisphere language WMTs have been mapped using picture naming, semantic association, word repetition, reading, and writing tasks. For monitoring of vision and spatial functions, the modified picture naming and line bisection tasks, as well as the recording of visual evoked potentials, have been used. Repetitive movements and monitoring of motor evoked potentials and involuntary movements have been applied for preservation of the motor networks.
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http://dx.doi.org/10.1016/j.wneu.2020.10.100DOI Listing
February 2021

A movement disorder specialist gets a taste of his own medicine.

Parkinsonism Relat Disord 2020 12 6;81:18-19. Epub 2020 Oct 6.

Department of Neurology, Mayo Clinic, Rochester, MN, USA. Electronic address:

We present a case in which a movement disorder neurologist developed two different hyperkinetic movements due to corticospinal tract hyperexcitability, which resolved completely with surgical removal of an epidural spinal canal tumor. The first-hand description of these movements creates a unique opportunity for enhanced insight into these complex movement phenomena.
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http://dx.doi.org/10.1016/j.parkreldis.2020.10.007DOI Listing
December 2020

Improved detection of focal cortical dysplasia using a novel 3D imaging sequence: Edge-Enhancing Gradient Echo (3D-EDGE) MRI.

Neuroimage Clin 2020 28;28:102449. Epub 2020 Sep 28.

Department of Radiology, Mayo Clinic, Jacksonville, FL, USA.

Epilepsy is a common neurological disorder with focal cortical dysplasia (FCD) being one of the most common lesional causes. Detection of FCD by MRI is a major determinant of surgical outcome. Evolution of MRI sequences and hardware has greatly increased the detection rate of FCD, but these gains have largely been related to the more visible Type IIb FCD, with Type I and IIa remaining elusive. While most sequence improvements have relied on increasing contrast between gray and white matter, we propose a novel imaging approach, 3D Edge-Enhancing Gradient Echo (3D-EDGE), to directly image the gray-white boundary. By acquiring images at an inversion time where gray and white matter have equal signal but opposite phases, voxels with a mixture of gray and white matter (e.g., at the gray-white boundary) will have cancellation of longitudinal magnetization producing a thin area of signal void at the normal boundary. By creating greater sensitivity for minor changes in T1 relaxation, microarchitectural abnormalities present in FCD produce greater contrast than on other common MRI sequences. 3D-EDGE had a significantly greater contrast ratio between lesion and white matter for FCD compared to MP2RAGE (98% vs 17%; p = 0.0006) and FLAIR (98% vs 19%; p = 0.0006), which highlights its potential to improve outcomes in epilepsy. We present a discussion of the framework for 3D-EDGE, optimization strategies, and analysis of a series of FCDs to highlight the benefit of 3D-EDGE in FCD detection compared to commonly used sequences in epilepsy.
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http://dx.doi.org/10.1016/j.nicl.2020.102449DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7552096PMC
September 2020

Pallidal Connectivity Profiling of Stimulation-Induced Dyskinesia in Parkinson's Disease.

Mov Disord 2021 02 1;36(2):380-388. Epub 2020 Oct 1.

Department of Radiology, Mayo Clinic, Jacksonville, Florida, USA.

Objectives: The aim of this study is to identify anatomical regions related to stimulation-induced dyskinesia (SID) after pallidal deep brain stimulation (DBS) in Parkinson's disease (PD) patients and to analyze connectivity associated with SID.

Methods: This retrospective study analyzed the clinical and imaging data of PD patients who experienced SID during the monopolar review after pallidal DBS. We analyzed structural and functional connectivity using normative connectivity data with the volume of tissue activated (VTA) modeling. Each contact was assigned to either that producing SID (SID VTA) or that without SID (non-SID VTA). Structural and functional connectivity was compared between SID and non-SID VTAs. "Optimized VTAs" were also estimated using the DBS settings at 6 months after implantation.

Results: Of the 68 consecutive PD patients who underwent pallidal implantation, 20 patients (29%) experienced SID. SID VTAs were located more dorsally and anteriorly compared with non-SID and optimized VTAs and were primarily in the dorsal globus pallidus internus (GPi) and dorsal globus pallidus externus (GPe). SID VTAs showed significantly higher structural connectivity than non-SID VTAs to the associative cortex and supplementary motor area/premotor cortex (P < 0.0001). Simultaneously, non-SID VTAs showed greater connectivity to the primary sensory cortex, cerebellum, subthalamic nucleus, and motor thalamus (all P < 0.0004). Functional connectivity analysis showed significant differences between SID and non-SID VTAs in multiple regions, including the primary motor, premotor, and prefrontal cortices and cerebellum.

Conclusion: SID VTAs were primarily in the dorsal GPi/GPe. The connectivity difference between the motor-related cortices and subcortical regions may explain the presence and absence of SID. © 2020 International Parkinson and Movement Disorder Society.
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http://dx.doi.org/10.1002/mds.28324DOI Listing
February 2021

Mapping angiography and transarterial technetium macroaggregated albumin particle simulation of recurrent atypical intracranial meningioma: feasibility for potential vascular brachytherapy.

Radiol Case Rep 2020 Nov 11;15(11):2278-2281. Epub 2020 Sep 11.

Department of Neurological Surgery, Mayo Clinic, 4500 San Pablo Rd, Jacksonville, FL 32224, USA.

Atypical meningioma (AM) (WHO-II) has a recurrence rate of 28% after gross total resection (GTR) with limited salvage options. Transarterial therapies may provide treatment opportunities in AM patients who exhausted standard-of-care therapy. In cases where favorable tumor vasculature and particle simulation demonstrate acceptable target dose, Yttrium-90 trans-arterial radioemobilization (TARE) could theoretically provide salvage therapy. A 67-year-old man presented with recurrent AM post gross total resection with adjuvant radiotherapy in 2012, 2014, and 2016. The patient was deemed a poor candidate for additional therapies. Tumor vasculature mapping was performed to determine TARE candidacy. Super-selective angiography and contrast-enhanced cone-beam computed tomography angiosomes demonstrated predominant pial collaterals and minor supply from a middle meningeal artery branch. Particle simulation was performed by infusing 0.3 mCi of 99mTc-macroaggregated albumin (99mTc-MAA). SPECT/CT-MRI fusion demonstrated conformal activity solely within the tumor volume perfused by the middle meningeal artery branch with a lung shunt fraction of 54.7%. The patient subsequently received off-label Nivolumab (PD-1 inhibitor). Mapping angiography for AM using 99mTc-MAA is feasible. It may identify candidates for TARE and potential AM patients with favorable blood supply. The potential for conformal intracranial vascular brachytherapy is intriguing, however, altered arterial supply in recurrent tumors is challenging.
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http://dx.doi.org/10.1016/j.radcr.2020.07.051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7494606PMC
November 2020

Postmortem Dissections of the Papez Circuit and Nonmotor Targets for Functional Neurosurgery.

World Neurosurg 2020 Dec 22;144:e866-e875. Epub 2020 Sep 22.

Laboratory of Microneurosurgical Anatomy, BP-A Beneficência Portuguesa de São Paulo, São Paulo, SP, Brazil; Center of Neurology and Neurosurgery Associates (CENNA), BP-A Beneficência Portuguesa de São Paulo, São Paulo, SP, Brazil; Department of Neurosurgery, Mayo Clinic, Jacksonville, Florida, USA.

Background: The Papez circuit was first described as the anatomic basis of emotion. Subsequent studies consolidated recognition of its limbic activities but showed a more important role in memory. Anatomic dissections and advanced neuroimaging have deepened our understanding of the various interconnections and white matter tracts present in this circuit. The aim of our study is to describe the anatomy of the Papez circuit through cadaveric dissection with correlation to ultrahigh-field magnetic resonance imaging (MRI) and MRI tractography.

Methods: White fiber dissection was performed on 5 cadaveric human brain hemispheres. The Papez circuit was dissected mediolaterally to show its anatomy and relation to other nuclei and tracts. Open-source MRI tractography data from the Human Connectome Project is combined with ultrahigh resolution, 7T structural MRI, and 17.6T diffusion tractography to further show the anatomy.

Results: The network connecting the anterior and posterior cingulate, entorhinal cortex, hippocampus, fimbria, dentate gyrus, fornix, mammillary bodies, and anterior thalamus was described using white matter fiber dissection and compared with MRI tractography and ultrahigh-field structural and diffusion MRI. We showed for the first time (through portmortem dissection) fibers directly connecting the anterior thalamic nucleus and the subgenual cingulate via the septal area.

Conclusions: The description of the anatomy of the Papez circuit through cadaveric dissection and comparisons with advanced neuroimaging studies allow a better understanding of its three-dimensional spatial layout, in addition to showing new areas of connectivity with adjacent structures and possibilities for surgical approaches or stimulation.
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http://dx.doi.org/10.1016/j.wneu.2020.09.088DOI Listing
December 2020

Multiple reader comparison of 2D TOF, 3D TOF, and CEMRA in screening of the carotid bifurcations: Time to reconsider routine contrast use?

PLoS One 2020 2;15(9):e0237856. Epub 2020 Sep 2.

Department of Radiology, Mayo Clinic, Rochester, Minnesota, United States of America.

Background And Purpose: MR contrast-enhanced techniques are undergoing increased scrutiny since the FDA applied a warning for gadolinium-based MR contrast agents due to gadolinium deposition within multiple organ systems. While CE-MRA provides excellent image quality, is it required in a screening carotid study? This study compares 2D TOF and 3D TOF MRA vs. CE-MRA in defining carotid stenosis in a large clinical patient population, and with multiple readers with varying experience.

Materials And Methods: 200 consecutive patients had their carotid bifurcations evaluated with 2D TOF, 3D TOF and CE-MRA sequences by 6 board-certified neuroradiologists. Stenosis and quality of examinations were defined for each study. Inter-rater reliability was assessed using two-way random effects intraclass correlation coefficients. Intra-reader reliability was computed via weighted Cohen's κ. Weighted Cohen's κ were also computed to assess agreement in stenosis ratings between enhanced images and unenhanced images.

Results: Agreement between unenhanced and enhanced ratings was substantial with a pooled weighted κ of 0.733 (0.628-0.811). For 5 of the 6 readers, the combination of unenhanced 2D TOF and 3D TOF showed better agreement with contrast-enhanced than either 2D TOF or 3D TOF alone. Intra-reader reliability was substantial.

Conclusions: The combination of 2D TOF and 3D TOF MRA showed substantial agreement with CE-MRA regarding degree of carotid stenosis in this large outpatient population across multiple readers of varying experience. Given the scrutiny that GBCA are undergoing due to concerns regarding CNS and soft tissue deposition, it seems prudent to reserve CE-MRA for cases which are not satisfactorily answered by the nonenhanced study or other noninvasive examinations.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0237856PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7467222PMC
October 2020

Syrinx Secondary to Chiari-like Tonsillar Herniation in Spontaneous Intracranial Hypotension.

World Neurosurg 2020 11 23;143:e268-e274. Epub 2020 Jul 23.

Department of Radiology, Mayo Clinic, Jacksonville, Florida, USA. Electronic address:

Objective: Syrinx development in patients with spontaneous intracranial hypotension (SIH) has rarely been described. To better understand this entity, we compared the clinical and radiographic findings in a series of patients with SIH and acquired Chiari-like tonsillar herniation with and without syrinx formation.

Methods: Six patients with syrinx in the setting of SIH and Chiari-like tonsillar herniation were retrospectively identified. The clinical and radiographic findings and outcomes were compared with those from a control group of patients with SIH and Chiari-like tonsillar herniation without syrinx.

Results: The patients with SIH and syrinx had had a higher opening pressure than had the control group (mean, 14.0 cm HO vs. 7.4 cm HO; P = 0.02) and a higher body mass index (mean, 33 kg/m vs. 26 kg/m; P = 0.01). The patients with syrinx had had an average obex displacement of 3.7 ± 2.2 mm below the plane of the foramen magnum compared with a position of 1.9 ± 3.1 mm above the plane of the foramen magnum in the control group (P = 0.004). The mean tonsillar descent was 12.7 ± 4.7 mm below the foramen magnum in those with syrinx compared with 5.9 ± 2.5 in the control group (P = 0.009). The clinical symptoms had improved in 83.3% of the patients with syrinx and 75% of the control patients after spinal cerebrospinal fluid leak closure. Three patients (50%) also had radiographic syrinx reduction.

Conclusion: Our results have shown that SIH can be an underrecognized cause of syrinx with key differences in body habitus and obex displacement compared with SIH without syrinx. In patients with tonsillar herniation into the foramen magnum associated with syrinx, the presence of SIH should be considered to avoid unnecessary foramen magnum decompression, even in those with a normal opening pressure.
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http://dx.doi.org/10.1016/j.wneu.2020.07.108DOI Listing
November 2020

Teaching NeuroImages: Vessel wall imaging of carotid web.

Neurology 2020 10 10;95(15):e2174-e2175. Epub 2020 Jul 10.

From the Departments of Neurology (N.G.) and Radiology (V.G., A.A.B., E.H.M.), Mayo Clinic, Jacksonville, FL.

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http://dx.doi.org/10.1212/WNL.0000000000010350DOI Listing
October 2020

Awake Craniotomy with Cortical and Subcortical Speech Mapping for Supramarginal Cavernoma Resection.

World Neurosurg 2020 09 22;141:260. Epub 2020 Jun 22.

Department of Neurologic Surgery, Mayo Clinic, Jacksonville, Florida, USA. Electronic address:

Awake craniotomy allows mapping of eloquent brain regions and monitoring neurocognitive functioning intraoperatively to maximize extent of resection and minimize cognitive morbidity. During resection of cavernous malformations in eloquent areas, intraoperative cognitive monitoring can also allow for safer maximal excision of the hemosiderin ring, which is correlated with improved seizure-free outcome. We present the case of a 33-year-old right-handed male with a new-onset seizure who presented to his local emergency department after experiencing visual hallucinations before losing consciousness. Computed tomography scan of the head revealed a calcified lesion in the left temporal/parietal area. Presurgical workup revealed left hemispheric language dominance and language activation within the overlying supramarginal gyrus representing phonologic working memory on functional magnetic resonance imaging. Diffusion tensor imaging identified the arcuate fasciculus and lateral portion of the superior longitudinal fasciculus to be intimately associated with the deep margin of the lesion. After consent was obtained, we performed an awake craniotomy and resection of the lesion through a transsulcal approach, with eloquent cortical mapping using a novel high-density circular grid, as well as subcortical stimulation/mapping and continuous intraoperative cognitive monitoring using multiple language paradigms; the patient was baselined on these paradigms preoperatively (Video 1). Several phonologic/paraphasic errors were made during resection of the hemosiderin ring, likely related to mechanical manipulation. The patient was discharged to home on postoperative day 4 with outpatient speech therapy for speech hesitancy. At 1-week postoperative testing, language skills were considered within normal limits.
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http://dx.doi.org/10.1016/j.wneu.2020.06.094DOI Listing
September 2020

Potential differences between monolingual and bilingual patients in approach and outcome after awake brain surgery.

J Neurooncol 2020 Jul 10;148(3):587-598. Epub 2020 Jun 10.

Department of Neurologic Surgery, Mayo Clinic, Jacksonville, FL, USA.

Introduction: 20.8% of the United States population and 67% of the European population speak two or more languages. Intraoperative different languages, mapping, and localization are crucial. This investigation aims to address three questions between BL and ML patients: (1) Are there differences in complications (i.e. seizures) and DECS techniques during intra-operative brain mapping? (2) Is EOR different? and (3) Are there differences in the recovery pattern post-surgery?

Methods: Data from 56 patients that underwent left-sided awake craniotomy for tumors infiltrating possible dominant hemisphere language areas from September 2016 to June 2019 were identified and analyzed in this study; 14 BL and 42 ML control patients. Patient demographics, education level, and the age of language acquisition were documented and evaluated. fMRI was performed on all participants.

Results: 0 (0%) BL and 3 (7%) ML experienced intraoperative seizures (P = 0.73). BL patients received a higher direct DECS current in comparison to the ML patients (average = 4.7, 3.8, respectively, P = 0.03). The extent of resection was higher in ML patients in comparison to the BL patients (80.9 vs. 64.8, respectively, P = 0.04). The post-operative KPS scores were higher in BL patients in comparison to ML patients (84.3, 77.4, respectively, P = 0.03). BL showed lower drop in post-operative KPS in comparison to ML patients (- 4.3, - 8.7, respectively, P = 0.03).

Conclusion: We show that BL patients have a lower incidence of intra-operative seizures, lower EOR, higher post-operative KPS and tolerate higher DECS current, in comparison to ML patients.
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http://dx.doi.org/10.1007/s11060-020-03554-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7968692PMC
July 2020

Examining the function of the visual word form area with stereo EEG electrical stimulation: A case report of pure alexia.

Cortex 2020 08 28;129:112-118. Epub 2020 Apr 28.

Department of Neurological Surgery, Mayo Clinic, Jacksonville, FL, USA; Department of Neurology, Mayo Clinic, Jacksonville, FL, USA.

Functional imaging studies have implicated an area in the left lateral fusiform gyrus, known as the visual word form area (VWFA), in pre-lexical orthographic processing. There are very few studies that have examined the functional specificity of this area in patients with discrete lesions limited to this region. Here we describe a rare opportunity to examine the functional specificity of the VWFA in a patient with stereo EEG (sEEG) electrodes implanted for localization of seizures prior to epilepsy surgery. sEEG offers the opportunity to create a transient and highly localized electrophysiological lesion to examine brain behavior correlates during functional mapping. In this case, word reading and writing as well as a variety of non-orthographic language functions (e.g., picture and face naming, auditory naming, and non-word repetition), were tested during electrical stimulation at a series of different electrode contact sites in the ventral temporal region. Pure alexia resulted from stimulation of the lateral fusiform gyrus at coordinates nearly identical to those published for the VWFA in the functional imaging literature.
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http://dx.doi.org/10.1016/j.cortex.2020.04.012DOI Listing
August 2020

Minimally invasive surgery and transsulcal parafascicular approach in the evacuation of intracerebral haemorrhage.

Stroke Vasc Neurol 2020 26;5(1):40-49. Epub 2019 Sep 26.

Neurological Surgery, Mayo Clinic, Jacksonville, Florida, USA.

Intracerebral haemorrhage (ICH) describes haemorrhage into the brain parenchyma that may result in a decline of the patient's neurological function. ICH is a common cause of morbidity and mortality worldwide. Aggressive surgical treatment for ICH has remained controversial as clinical trials have failed to demonstrate substantial improvement in patient outcome and mortality. Recently, promising mechanical and pharmacological minimally invasive surgery (MIS) techniques for the treatment of ICH have been described. MIS was designed with the objective of reducing morbidity due to complications of surgical manipulation. Mechanical MIS includes the use of tubular retractors and small diameter instruments for ICH removal. Pharmacological methods consist of catheter placement inside the haematoma cavity for the passive drainage of the haematoma over the course of several days. One of the most favourable approaches for MIS is the use of natural corridors for reaching the lesion, such as the transsulcal parafascicular approach. This approach provides an anatomical dissection of the subjacent white matter tracts, causing the least amount of damage while evacuating the haematoma. A detailed description of the currently known MIS techniques and devices is presented in this review. Special attention is given to the transsulcal parafascicular approach, which has particular benefits to provide a less traumatic MIS with promising overall patient outcome.
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http://dx.doi.org/10.1136/svn-2019-000264DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213514PMC
September 2020

Awake vs. asleep motor mapping for glioma resection: a systematic review and meta-analysis.

Acta Neurochir (Wien) 2020 07 9;162(7):1709-1720. Epub 2020 May 9.

Department of Neurological Surgery, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.

Background: Intraoperative stimulation (IS) mapping has become the preferred standard treatment for eloquent tumors as it permits a more accurate identification of functional areas, allowing surgeons to achieve higher extents of resection (EOR) and decrease postoperative morbidity. For lesions adjacent to the perirolandic area and descending motor tracts, mapping can be done with both awake craniotomy (AC) and under general anesthesia (GA).

Objective: We aimed to determine which anesthetic protocol-AC vs. GA-provides better patient outcomes by comparing EOR and postoperative morbidity for surgeries using IS mapping in gliomas located near or in motor areas of the brain.

Methods: A systematic literature search was carried out to identify relevant studies from 1983 to 2019. Seven databases were screened. A total of 2351 glioma patients from 17 studies were analyzed.

Results: A random-effects meta-analysis revealed a trend towards a higher mean EOR in AC [90.1% (95% C.I. 85.8-93.8)] than with GA [81.7% (95% C.I. 72.4-89.7)] (p = 0.06). Neurological deficits were divided by timing and severity for analysis. There was no significant difference in early neurological deficits [20.9% (95% C.I. 4.1-45.0) vs. 25.4% (95% C.I. 13.6-39.2)] (p = 0.74), late neurological deficits [17.1% (95% C.I. 0.0-50.0) vs. 3.8% (95% C.I. 1.1-7.6)] (p = 0.06), or in non-severe [28.4% (95% C.I. 0.0-88.5) vs. 20.1% (95% C.I. 7.1-32.2)] (p = 0.72), and severe morbidity [2.6% (95% C.I. 0.0-15.5) vs. 4.5% (95% C.I. 1.1-9.6)] (p = 0.89) between patients who underwent AC versus GA, respectively.

Conclusion: Mapping during resection of gliomas located in or near the perirolandic area and descending motor tracts can be safely carried out with both AC and GA.
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http://dx.doi.org/10.1007/s00701-020-04357-yDOI Listing
July 2020

Variability Between Direct and Indirect Targeting of the Anterior Nucleus of the Thalamus.

World Neurosurg 2020 07 14;139:e70-e77. Epub 2020 Apr 14.

Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA. Electronic address:

Background: Preoperative thalamic targeting methods have historically relied on indirect targeting techniques that do not fully account for variances in anatomy or for thalamic atrophy in epilepsy. We aimed to address variability noted between traditional indirect targeting and direct targeting methods for the anterior nucleus of the thalamus (ANT).

Methods: Fifteen consecutive patients undergoing ANT deep brain stimulator placement were evaluated (30 thalamic nuclei). Direct ANT targeting was performed using a fast gray matter acquisition T1 inversion recovery sequence and compared with standard stereotactic coordinates. Thalamic volumes were calculated for each patient, and degree of thalamic volume loss was assessed compared with matched control subjects. Vertex analysis was performed to assess shape changes in the thalamus compared with age- and sex-matched subjects.

Results: There was significant variation between direct and indirect targets in the y-axis and z-axis on both sides. On the left, the direct target was located at y = 2 ± 1.3 mm and z = 9.3 ± 1.8 mm (both P = 0.02). On the right, the direct target was located at y = 2.9 ± 1.8 mm and z = 9.2 ± 2 mm (both P ≤ 0.0003). There was no significant difference in the x-coordinate on either side (P > 0.5). Additionally, there was a correlation between thalamic volume and difference between direct and indirect targets in the y-axis and the z-axis.

Conclusions: We showed a significant difference in direct and indirect targeting in the y-axis and z-axis when targeting the ANT for deep brain stimulation for epilepsy. This difference is correlated to thalamic volume, with a larger difference noted in patients with thalamic atrophy.
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http://dx.doi.org/10.1016/j.wneu.2020.03.107DOI Listing
July 2020

A Comprehensive Review of Brain Connectomics and Imaging to Improve Deep Brain Stimulation Outcomes.

Mov Disord 2020 05 12;35(5):741-751. Epub 2020 Apr 12.

Fixel Institute for Neurological Diseases, University of Florida, Gainesville, Florida, USA.

DBS is an effective neuromodulatory therapy that has been applied in various conditions, including PD, essential tremor, dystonia, Tourette syndrome, and other movement disorders. There have also been recent examples of applications in epilepsy, chronic pain, and neuropsychiatric conditions. Innovations in neuroimaging technology have been driving connectomics, an emerging whole-brain network approach to neuroscience. Two rising techniques are functional connectivity profiling and structural connectivity profiling. Functional connectivity profiling explores the operational relationships between multiple regions of the brain with respect to time and stimuli. Structural connectivity profiling approximates physical connections between different brain regions through reconstruction of axonal fibers. Through these techniques, complex relationships can be described in various disease states, such as PD, as well as in response to therapy, such as DBS. These advances have expanded our understanding of human brain function and have provided a partial in vivo glimpse into the underlying brain circuits underpinning movement and other disorders. This comprehensive review will highlight the contemporary concepts in brain connectivity as applied to DBS, as well as introduce emerging considerations in movement disorders. © 2020 International Parkinson and Movement Disorder Society.
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http://dx.doi.org/10.1002/mds.28045DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7556988PMC
May 2020

Comparison Between Transcortical and Interhemispheric Approaches to the Atrium of Lateral Ventricle Using Combined White Matter Fiber Dissections and Magnetic Resonance Tractography.

World Neurosurg 2020 06 6;138:e478-e485. Epub 2020 Mar 6.

Institute of Neurological Sciences (ICNE), São Paulo, Bela Vista, São Paulo, Brazil; Evandro de Oliveira Microneurosurgery Laboratory, Beneficência Portuguesa Hospital, São Paulo, Brazil; Department of Neurosurgery, Mayo Clinic, Jacksonville, Florida, USA.

Objective: The objective of this study was to compare transcortical and posterior interhemispheric approaches to the atrium using a combined approach of white matter fiber dissections and magnetic resonance (MR) tractography.

Methods: Ten cerebral hemispheres were examined and dissected from the lateral-to-medial surface and from the medial-to-lateral surface, with special attention to the white matter tracts related to the atrium. MR tractography was used to show the relationship of three-dimensional white matter fibers with the atrium of the lateral ventricle and to compare with cadaveric dissection results.

Results: The atrium was related laterally to the superior longitudinal fasciculus II and III, middle longitudinal fasciculus, arcuate fasciculus, vertical occipital fasciculus, and sagittal stratum. Medially, it is related to the superior longitudinal fasciculus I, cingulum, sledge runner, and forceps major.

Conclusions: A combined approach of cadaveric white matter fiber dissections and MR tractography were used to describe the main white matter tracts related to the posterior interhemispheric approach and the transcortical approach, providing an in-depth understanding of the three-dimensional anatomy of white matter fibers and the atrium. In the present study, among approaches examined, the posterior interhemispheric parasplenial transprecuneus approach placed fewer eloquent tracts at risk; however, traversing the sledge runner and the forceps major is unavoidable by this approach.
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http://dx.doi.org/10.1016/j.wneu.2020.02.161DOI Listing
June 2020

Deep brain stimulation in essential tremor: targets, technology, and a comprehensive review of clinical outcomes.

Expert Rev Neurother 2020 04 2;20(4):319-331. Epub 2020 Mar 2.

Fixel Institute for Neurological Diseases, Department of Neurology, University of Florida, Gainesville, FL, USA.

: Essential tremor (ET) is a common movement disorder with an estimated prevalence of 0.9% worldwide. Deep brain stimulation (DBS) is an established therapy for medication refractory and debilitating tremor. With the arrival of next generation technology, the implementation and delivery of DBS has been rapidly evolving. This review will highlight the current applications and constraints for DBS in ET.: The mechanism of action, targets for neuromodulation, next generation guidance techniques, symptom-specific applications, and long-term efficacy will be reviewed.: The posterior subthalamic area and zona incerta are alternative targets to thalamic DBS in ET. However, they may be associated with additional stimulation-induced side effects. Novel stimulation paradigms and segmented electrodes provide innovative approaches to DBS programming and stimulation-induced side effects.
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http://dx.doi.org/10.1080/14737175.2020.1737017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7174089PMC
April 2020

Endoscopic endonasal transclival approach to the ventral brainstem: Radiologic, anatomic feasibility and nuances, surgical limitations and future directions.

J Clin Neurosci 2020 Mar 28;73:264-279. Epub 2020 Jan 28.

Department of Neurosurgery, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.

The endoscopic endonasal transclival approach (EETA) is less invasive than traditional open approaches; however, there is currently limited data on the pre-operative features that may predict success of the EETA. Surgical landmarks and exposure of the EETA and expanded EETA are assessed. We retrospectively measured multiple anatomic features in 154 adult dry skulls, 22 C1 and C2 dry bone vertebrae, and 500 paranasal sinus computed tomography (CT) scans. We also dissected 13 formalin-fixed, silicone-injected adult cadaveric heads. Relevant qualitative and quantitative data were obtained with CT (n = 10) and cadaveric dissection (n = 13). They are expressed as mean (SD), as appropriate and compared with unpaired t tests. Categorical variables were compared with χ tests. We determined the prevalence of clival and sphenoid sinus anatomic variations and assessed potential exposure of the craniovertebral region, based on the relative position of the nasal bone tip, posterior point of the hard palate, and their relation to the position of C1 and C2. Depending on anatomic features, EETA should allow visualization of the ventral brainstem. The mean (SD) dimensions of the surgical window for EETA were 43.2 (5.1) × 18.3 (1.1) mm, in the craniocaudad and horizontal dimensions, respectively. The craniocaudad dimension enlarged to 60.2 (4.7) mm with expanded EETA. The EETA allowed satisfactory exposure for odontoid and C1 anterior arch resection (expanded EETA) in all specimens, regardless of the orientation of the palate. The combination of preoperative radiographic assessment and intraoperative considerations allows safe and effective application and facilitate selection of the most appropriate approach.
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http://dx.doi.org/10.1016/j.jocn.2020.01.012DOI Listing
March 2020

Functional Activation Patterns of Deep Brain Stimulation of the Anterior Nucleus of the Thalamus.

World Neurosurg 2020 Apr 27;136:357-363.e2. Epub 2020 Jan 27.

Department of Neurosurgery, Mayo Clinic, Jacksonville, Florida, USA.

Background: Deep brain stimulation (DBS) of the anterior nucleus of the thalamus (ANT) is a recently approved therapy for patients with drug-resistant epilepsy. To date, there is a poor understanding of the mechanism of action and lack of in vivo biomarkers. We propose a method for investigating the in vivo stimulation effects using blood-oxygen-level-dependent (BOLD) magnetic resonance imaging (MRI) and present the brain activation pattern associated with ANT DBS.

Methods: Two patients undergoing ANT DBS for epilepsy underwent BOLD MRI using a block design after the DBS was programmed to alternate ON/OFF in 30-second blocks. The scanner was triggered using surface electrophysiologic recordings to detect the DBS cycle. Nine total runs were obtained and were analyzed using a general linear model.

Results: Active ANT stimulation produced activation within several areas of the brain, including the thalamus, bilateral anterior cingulate and posterior cingulate cortex, precuneus, medial prefrontal cortex, amygdala, ventral tegmental area, hippocampus, striatum, and right angular gyrus.

Conclusions: Using block-design BOLD MRI, we were able to show widespread activation resulting from ANT DBS. Overlap with multiple areas of both the default mode and limbic networks was shown, suggesting that these nodes may modulate the effect of seizure control with ANT DBS.
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http://dx.doi.org/10.1016/j.wneu.2020.01.108DOI Listing
April 2020

T10-L3 Cystic Lesion of the Ventriculus Terminalis Presenting as Conus Medullaris Syndrome.

World Neurosurg 2020 Apr 16;136:146-149. Epub 2020 Jan 16.

Department of Neurologic Surgery, Mayo Clinic, Jacksonville, Florida, USA. Electronic address:

Background: Intramedullary spinal cord cysts are benign, rare, fluid-filled lesions that can present anywhere along the craniospinal axis. However, when present at the level of the ventriculus terminalis, conus medullaris syndrome may occur. Radical resection of the cyst wall and evacuation of the cyst content are the 2 surgical procedures of choice.

Case Description: We present the case of a 54-year-old woman with a long-lasting history of left lower-extremity weakness and recent onset of bladder dysfunction. On further assessment, magnetic resonance imaging of the thoracic and lumbar spine showed a T10-L3 intramedullary cystic lesion. Surgical fenestration of the cyst was rendered, but no biopsy was taken due to the highly functional tissue along the full extension of lesion.

Conclusions: Cystic lesions of the ventriculus terminalis are rare entities with a common presentation of severe, progressive neurologic impairment. Our case matches the classic presentation of conus medullaris syndrome. We describe and demonstrate through an operative video novel surgical techniques used to achieve successful fenestration of the cyst wall with remarkable neurologic symptom improvement. Moreover, the present case does not correlate with the classic radiographic characteristics available in current literature, such as involvement of 5 spinal segments.
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http://dx.doi.org/10.1016/j.wneu.2020.01.049DOI Listing
April 2020