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Diagnostic Dilemma in the Management of a Cervical Spinal Cord Lesion: A Case Report of Neuromyelitis Optica Spectrum Disorder

Neuromyelitis Optica spectrum disorder is a condition in which antibodies are elaborated against aquaporin water channels in the end foot processes of astrocytes due to autoimmune dysregulation thereby initiating a cascade of neuroinflammatory events that culminates in astrocytic injury, dysfunctional axonal blebs and demyelination of the central nervous system neurons. Its cervical spine symptom may sometimes mimic a similar presentation of a cervical spine lesion, this is further underscored by its core clinical magnetic resonance image feature of longitudinally extensive transverse myelitis bearing radiologic similarities with an intramedullary spinal cord lesion thereby resonating the need to maintain a high index of suspicion when evaluating patients with either conditions. Laboratory elucidation of increased titers of aquaporin -4 immunoglobulin and seronegativity for oligoclonal bands is the diagnostic hallmark of the condition and helps differentiate it from similar conditions such as multiple sclerosis and myelin oligodendrocyte glycoprotein disorder. While the mainstay of treatment involves the use of steroids, immunosuppressive therapy and plasmapheresis, it is pertinent to highlight that surgery may have a potential benefit in patients with acute exacerbations of symptoms attributable to cervical spine compressive myelopathy due to the disease. We report a case of a 50-year-old female who had clinical features initially suspected to be a spinal cord lesion but eventually proven with laboratory parameters and histological analysis of resected tissue specimen to be in keeping with Neuromyelitis Optica spectrum disorder.

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Sustained Effect of Posteroventral Pallidotomy for X-Linked Dystonia Parkinsonism

<i>Background</i>: X-linked dystonia parkinsonism (XDP, DYT3), a disease endemic to Panay Island in the Philippines, is a progressive adult-onset sex-linked neurodegenerative movement disorder with features of both dystonia and parkinsonism. Medical treatment and botulinum toxin injection (BTX) are of limited benefit. Deep brain stimulation (DBS) of the bilateral globus pallidus internus (GPi) is effective but is expensive and requires specialized equipment, training and after care. Posteroventral pallidotomy (PVP) is an older known ablative procedure which has successfully treated cervical and generalized dystonia, but has not been previously used for XDP. <i>Case presentation</i>: Two male adults with genetically-confirmed XDP, presented with progressive and severely disabling dystonias. The mean preoperative Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was 80.5. Both XDP patients underwent a unilateral posteroventral pallidotomy. In the first case, there was a 38.46% decrease in BFMDRS score at 2 weeks sustained to 41.66% decrease (compared to pre-op BFMDRS at 4 years post-surgery). In the second case, there was a 33.73% decrease in BFMDRS at 2 months post-op, and a decrease of 86.74% in BFMDRS score at 3 years post-surgery. <i>Conclusion</i>: While not as effective as bilateral pallidal DBS, the benefits of a unilateral PVP are still substantial and sustained while also being less costly and resource expensive.

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A Rare Case of External Ear Arteriovenous Malformation Treated with Linac Based Radiosurgery

<i>Background: </i>Arteriovenous malformation (AVM) is a congenital vascular anomaly in which there are abnormal connections between arteries and veins in the form of fistula or nidus without an intervening capillary bed. External ear AVMs can cause swelling, redness, bleeding, and pain. Timely diagnosis and treatment are crucial to prevent complications. According to literature, there are different techniques to treat auricular AVM which include surgical excision followed by ear reconstruction, sclerotherapy and embolization. Stereotactic radiosurgery (SRS) has an established role in treating intracranial AVM with excellent obliteration rates but limited literature exists on its efficacy in treating auricular AVM in terms of obliteration rates and cosmetic outcome. <i>Case Presentation</i>: We report a rare case of right external ear AVM which was post embolization and post excision followed by reconstruction but had persistent residual nidus in right pinna treated by stereotactic radiosurgery. He was treated to a dose of 21 Gy in 3 fractions at 7 Gy per fraction for 3 consecutive days. He had completed treatment without any complications. Follow up after 2 weeks of treatment, clinically, patient had redness, swelling and persistent pulsations but no episode of bleeding. At the six-month follow-up, redness, swelling, and pulsations had decreased. Follow up after 1 year post treatment, clinically, redness, swelling and pulsations were not present. Dynamic Brain MRI with angiography showed complete obliteration of residual AVM nidus in right pinna. Patient was satisfied with the cosmetic outcome. <i>Conclusion</i>: We conclude that stereotactic radiosurgery can be used as an effective treatment modality for auricular AVMs. In our case, radiosurgery provided an excellent control and obliteration of the nidus and good cosmetic result eliminating the need for surgical reconstruction at the site of the nidus.

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Impact of Postsurgical Disconnection in Aggression Disorder Treatment, Latin American Single Center Experience: A Neuronal Connectomics Analysis

Recent advancements in the study of human behavior, along with significant technological progress, have provided a more complete understanding of the neural circuits involved in aggressive responses to external stimuli. Notably, aggression as a voluntary behavior differs from reactive aggression in its neural connections and cerebral connectomics. Aggression is identified as part of a series of responses to stimuli that pose a potential threat to an individual’s physical integrity. By identifying the neural pathways involved in pathological aggression, we can modify this behavior by disrupting these pathways through functional neurosurgery. This study aims to demonstrate, through neuronal connectomics, the effects of postsurgical disconnection following functional neurosurgical procedures designed to treat aggression disorders. For many years, brain function was believed to result solely from the activity of specific cortical areas, which conditioned cognitive responses. However, recent advances in neurology, neuroimaging, neuropsychology, and neurosurgery have shifted our understanding of brain function, revealing a more complex network of connectivity. Despite we are now able to predict the location of primary cortical areas, patients may still experience unanticipated deficits in functions like judgment or memory after surgery. This suggests that traditionally silent regions of the brain may be more anatomically intricate and functionally redundant than previously understood. One of the significant advancements in neuroimaging is Diffusion Tensor Imaging (DTI), which has revolutionized psychiatric surgery, neuroendoscopy, and neuro-oncology. DTI enabled the formation of the Human Connectome Project (HCP), a large-scale initiative that provides detailed data on the brain's connectivity in healthy individuals. The most recent HCP findings have reclassified cortical regions previously described by Brodmann, based on functional connectivity, myelination, and cortical thickness. These advancements contribute to a much more detailed framework for studying brain function and its anatomical organization. This paper explores how these developments in connectomics and neuroimaging are being applied to the understanding and treatment of aggression disorders through functional neurosurgery.

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Subarachnoid Hemorrhage Revealing a Fistula Point Venous Aneurysm in a Dural Arteriovenous Fistula at the Craniocervical Junction: A Case Report

Dural arteriovenous fistulas represent approximately 10% of all intracranial vascular malformations, of which 1 to 2% are located at the craniocervical junction. Their location at the craniocervical junction is uncommon but potentially significant, given its proximity to and relationship with vital anatomical structures in this region. The association of a dural arteriovenous fistula with a venous aneurysm is rare. In this report, we present the case of a 49-year-old patient with no particular pathological history who was admitted for acute headaches. The neurological examination revealed a patient with grade I WFNS, intracranial hypertension syndrome. Radiological investigations including CT, CT angio and MRI revealed a fisher IV subarachnoid hemorrhage due to a ruptured venous aneurysm associated to an arteriovenous fistula at the craniocervical junction. He underwent clipping of the veinous aneurysm and microsurgical obliteration of the fistula. The postoperative course was uneventful. The clinical examination at 1-year post-op was unremarkable, and the follow-up MRI at 1-year post-op showed complete obliteration of the malformation. This report showed ruptured dural arteriovenous fistula should be considered in the etiology workup for posterior fossa subarachnoid hemorrhage. Effective and safe obliteration is possible without angiography.

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Knowledge and Practice About Traumatic Brain Injury in the Hospital Environment in the DRC

<i>Introduction</i>: Traumatic brain injury (TBI) is a common reason for admission in our setting. It is the cause of primary brain lesions, representing a major public health problem since they are responsible for significant morbidity and mortality. <i>Materials and Methods</i>: Observational study with survey, run from December 1, 2023, to January 31, 2024, involving 256 health workers to verify the level of knowledge about traumatic brain injury. <i>Results</i>: The age group between 21 and 40 years constitutes 64.05% of cases (n=164), The average age is 29.38, with extremes between 24 and 65 years, We note a male predominance in 53.12% of cases (n= 136), Medical Doctors constitute 75% of the medical personnel interviewed (n=192), we note a clear predominance of 0 to 5 years old with 33.59% of cases (n=86), The faculty predominates as the place of learning about head injuries in 53.65% of cases (n=132), The neurosurgeon takes care of the management of craniocerebral trauma in 58.13% of cases (n=143), According to this staff, the biggest problem in the management of head trauma in our environment is the cost of treatment with 62.89% of cases (n=161) and in most cases (54.29% of cases or 139 patients), health personnel consider themselves to have a mean level of understanding of craniocerebral trauma. <i>Conclusion</i>: The management of TBI is currently a diagnostic, therapeutic, and prognostic challenge linked to several factors in our environment. A consensual approach standardized to adapt to our practical reality proves helpful in reducing morbidity and mortality.

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Surgical Treatment of Craniocervical Junction Tumors: Neurosurgery Department Experience of “Hôpital Principal De Dakar”

Introduction: Craniocervical tumors are uncommon but represent a medical emergency for diagnosis and treatment. Their approaches require of course surgical expertise in addition to adapted technical platform. In light of the limited resources available in our practice, we consequently made the decision to share our experience with the surgical treatment of this pathology. Patients and method: We performed a retrospective analysis on eight observations of patients treated in the neurosurgery department of “Hôpital Principal de Dakar” between July 2015 and December 2022 for progressive tumor spinal cord compression at the craniocervical junction. Results: We observed a 10% frequency, a mean age of 39.25 years, and extremes between 8 and 62 years. There was a 0.6 sex ratio. The most common risk factor was type I neurofibromatosis, and one case of Von Hippel-Lindau disease. All of our patients had pyramidal syndrome. Overall, there were 2 tumors with posterolateral extradural site and 4 tumors with extramedullary intradural location, including 2 posterolateral and 2 anterolateral. The location was intramedullary and cerebella-medullary in one case each. The posteromedial occipitocervical approach, which was employed on six patients, was the most utilized method. Five patients had total tumor resection, whereas three had partial resection. Two of our patients had postoperative complications: a worsening of motor deficit and a death following dependence on mechanical ventilation. We only received four histological confirmations for the anatomopathological samples. Our patients' evolution was positive over an average follow-up of 21 months, with neurological improvement and walking autonomy. Conclusion: Tumors affecting the craniocervical junction are still treated surgically. Despite the need for improvement in our working conditions, our results appear to be satisfactory.

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Patterns of Microbial Infections in Post-Infective Hydrocephalus and Treatment Methods at Bugando Medical Center

Background: Post Infective Hydrocephalus is among the complicated types of hydrocephalus and poses a challenge in management of these patients, globally. Antibiotics susceptibility patterns of common bacteria implicating in pyogenic ventriculitis complicated with hydrocephalus were not clearly known at our setting. Therefore, we determined antibiotics susceptibility patterns of common bacteria implicating in pyogenic ventriculitis complicated with hydrocephalus to recommend empirical therapy while waiting for culture and sensitivity results at Bugando Medical Centre (BMC) in Mwanza, Tanzania. Methods: Thirty-five patients with hydrocephalus who had undergone cerebrospinal fluid (CSF) tapping and analysis were selected randomly retrospectively and their bacteriological culture and sensitivity results were analyzed to determine the common microbial patterns. Patients were rationally prescribed antibiotics depending on laboratory results for 7 days and were later done control CSF cultures until negative cultures were achieved. Results: A total of 35 patients with median (interquartile range [IQR]) age was 5.5 [2-8] months were reviewed. The majority of participants were males, 62.9% (22/35). 20% (7/35) and 37.1% (13/35) had turbid CSF-shunt sample and first culture positive results, respectively, whereas one patient had culture positive with two pathogens resulting to 14 isolates. Gram positive bacteria were predominantly isolated (66.7%, 8/14), whereby S. aureus was frequently encountered (35.7%, 5/14). Overall, gram positive and negative bacteria exhibited least resistance against tetracycline (16.7% and 25%) and ciprofloxacin (16.7% and 25%) respectively. Three (8.6%) patients died from respiratory complications. Conclusion: Post infective hydrocephalus is a clinically challenging but treatable form of hydrocephalus and Staphylococcus aurous is the most common cause. This study found that the use of a seven-day regimen as cost effective and associated with a shorter hospital stay with the focus on CSF analysis with subsequent microbial targeted antibiotic therapy to achieve sterile CSF culture. Treatment strategy should be multidisciplinary approach with collaboration from microbiologists, radiologists, pediatricians and neurosurgeons.

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400 Gramme on Skull Base and Face: Complete Removal and Reconstruction

<i>Background</i>: Huge craniofacial tumors represent a surgical challenge not only for high risk of intraoperative damage of the brain and sense organs of the face, but also for esthetical issues which may occur postoperatively. This surgery is riskier when these processes are represented by massive compact bone such as osteomas. <i>Case presentation</i>: A young patient with huge disfiguring osteoma of anterior skull base extended to sphenoidal, frontal, maxillary sinuses as well as in orbits benefit for a complete removal with craniofacial reconstruction through a surgical teamwork including neurosurgeon, maxillofacial surgeon and ophthalmologist; with an overt satisfactory cosmetic result. Preoperative planning is reported as well as the intraoperative procedure and the postoperative reconstruction and follow up. <i>Conclusion</i>: Craniofacial osteoma represents a surgical and cometic challenge for their resection. This resection is tailored through sophisticated devices such as preoperative 3D (3 dimension) printed model, intraoperative neuronavigation, and the use on custom-made bone. In place where this reported patient is managed, these tools are unavailable. Thus, priority should be given to teamwork with accurate preoperative planning and manually confectioned cranioplasty. Endoscopic approach for these processes gained more and more indication in the resection of these craniofacial and skull base osteomas. Since this device require a learning curve, laboratory training for is proper using as well as in microneurosurgery techniques is highly recommended. A hope is encountered though the humanitarian implication of some organization and universities.

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