scholarly journals Seizure Semiology, EEG, and Imaging Findings in Epilepsy Secondary to Mitochondrial Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Anthony L. Fine ◽  
Greta Liebo ◽  
Ralitza H. Gavrilova ◽  
Jeffrey W. Britton
Keyword(s):  
Status Epilepticus ◽  
Phenotypic Variability ◽  
Mitochondrial Disorder ◽  
Case Series ◽  
Mitochondrial Disorders ◽  
Imaging Features ◽  
Retrospective Case ◽  
Tertiary Referral Center ◽  
Focal Status Epilepticus ◽  
Patients With Epilepsy

Background: Identification of an underlying mitochondrial disorder can be challenging due to the significant phenotypic variability between and within specific disorders. Epilepsy can be a presenting symptom with several mitochondrial disorders. In this study, we evaluated clinical, electrophysiologic, and imaging features in patients with epilepsy and mitochondrial disorders to identify common features, which could aid in earlier identification of a mitochondrial etiology.Methods: This is a retrospective case series from January 2011 to December 2019 at a tertiary referral center of patients with epilepsy and a genetically confirmed diagnosis of a mitochondrial disorder. A total of 164 patients were reviewed with 20 patients fulfilling inclusion criteria.Results: A total of 20 patients (14 females, 6 males) aged 0.5–61 years with epilepsy and genetically confirmed mitochondrial disorders were identified. Status epilepticus occurred in 15 patients, with focal status epilepticus in 13 patients, including 9 patients with visual features. Abnormalities over the posterior cerebral regions were seen in 66% of ictal recordings and 44% of imaging studies. All the patients were on nutraceutical supplementation with no significant change in disease progression seen. At last follow-up, eight patients were deceased and the remainder had moderate-to-severe disability.Discussion: In this series of patients with epilepsy and mitochondrial disorders, we found increased propensity for seizures arising from the posterior cerebral regions. Over time, electroencephalogram (EEG) and imaging abnormalities increasingly occurred over the posterior cerebral regions. Focal seizures and focal status epilepticus with visual symptoms were common. Additional study is needed on nutraceutical supplementation in mitochondrial disorders.

Transmastoid Hydroxyapatite Resurfacing for Sigmoid Sinus Wall Anomalies Causing Pulsatile Tinnitus

2021 ◽  
pp. 000348942098740
Author(s):  
Ian Newberry ◽  
Julie Highland ◽  
Alvin DeTorres ◽  
Richard Gurgel
Keyword(s):  
Case Series ◽  
Sigmoid Sinus ◽  
Pulsatile Tinnitus ◽  
Retrospective Case ◽  
Tertiary Referral Center ◽  
Operative Outcomes ◽  
Sinus Wall ◽  
Objective Tinnitus ◽  
Materials Used

Objective: Comprising 4% of tinnitus, pulsatile tinnitus (PT) can be particularly difficult for affected patients as well as surgeons looking to address their symptoms. Often the cause is not identified but can be secondary to turbulent flow in or near the sigmoid sinus, particularly if there is an identifiable sigmoid sinus dehiscence (SSDe) and/or diverticulum (SSDi). These sigmoid sinus wall anomalies (SSWA) may be treated with transmastoid sigmoid sinus resurfacing; however, this intervention remains relatively novel and its technique, materials used, resolution success, and complications need to be continuously reviewed. Methods: A retrospective case series of patients with PT due to SSWA at a tertiary referral center was reviewed. A total of 6 patients (7 ears) treated by transmastoid resurfacing using hydroxyapatite (HA) were retrospectively assessed. Pre-operative demographics and symptoms, pre- and post-operative hearing results, and post-operative outcomes were reviewed. Results: All patients were female with an average BMI of 32.9 (±5.4) and a mean age of 45.5 years (±15.3). Mean follow-up was 648 days. Objective tinnitus was noted in all ears with SSDi (100%); however, no objective tinnitus was noted with purely SSDe. In 100% of ears, PT was diminished with ipsilateral jugular compression and was amplified with contralateral head turn. Pre-operative symptoms of PT resolved in all patients, but delayed recurrence (>1 year) occurred in 1 ear (14%). No patient had pre- or post-operative hearing loss. No major complications were encountered. Conclusions: Transmastoid resurfacing for SSWA with HA bone cement is a safe, reliable intervention in properly identified PT patients.

scholarly journals Tratamento cirúrgico do ceratocisto odontogênico por meio de enucleação e osteotomia periférica: relato de caso

2020 ◽  
Vol 9 (6) ◽  
pp. 531-534
Author(s):  
Diogo Henrique Marques ◽  
Maylson Alves Nogueira Barros ◽  
Vitor Bruno Teslenco ◽  
Cláudio Marcio Santana Junior ◽  
Lucas Marques Meurer ◽  
...  
Keyword(s):  
Case Report ◽  
Meta Analysis ◽  
Benign Lesion ◽  
Dentigerous Cyst ◽  
Case Series ◽  
Odontogenic Keratocyst ◽  
Imaging Features ◽  
Retrospective Case ◽  
Odontogenic Keratocysts

Introdução: Os ceratocistos odontogênicos (CCA) são considerados raros cistos de desenvolvimento, derivados dos remanescentes da lâmina dentária, com atividade intraóssea benigna, porém localmente invasivo e agressivo. O tratamento para o ceratocisto odongênico é variado, podendo-se encontrar modalidades tais como:enucleação, isolada ou associada a curetagem, com osteotomia periférica, aplicação da solução de Carnoy ou crioterapia, descompressão, marsupialização e ressecções. Objetivo: O presente trabalho tem como objetivo relatar um caso de ceratocisto odontogênico, onde foi escolhida abordagem conservadora por curetagem e osteotomia periférica. Relato de caso: Paciente de 68 anos, leucoderma, referiu ao exame clínico dor espontânea em região retromolar esquerda e parestesia em lábio inferior. A paciente foi submetida a biopsia por aspiração e excisional, após confirmação histopatológica foi proposto uma enucleação associada a osteotomia periférica sob anestesia geral. A paciente permanece em acompanhamento clínico e radiográfico, sem sinais de recidiva da lesão. Conclusão: Embora apresentem um comportamento agressivo, os ceratocistos odontogêncios podem ser tratados com segurança, de forma conservadora, por meio de enucleação seguida de osteotomia periférica com mínimo de morbidade. Descritores: Osteotomia; Curetagem; Cistos Odontogênicos. Referências Borghesi A, Nardi C, Giannitto C, Tironi A, Maroldi R, Di Bartolomeo F, Preda L. Odontogenic keratocyst: imaging features of a benign lesion with an aggressive behaviour. Insights Imaging. 2018 Oct;9(5):883-897. Park JH, Kwak EJ, You KS, Jung YS, Jung HD. Volume change pattern of decompression of mandibular odontogenic keratocyst. Maxillofac Plast Reconstr Surg. 2019 Jan 7;41(1):2.  Karaca C, Dere KA, Er N, Aktas A, Tosun E, Koseoglu OT, Usubutun A. Recurrence rate of odontogenic keratocyst treated by enucleation and peripheral ostectomy: Retrospective case series with up to 12 years of follow-up. Med Oral Patol Oral Cir Bucal. 2018 Jul 1;23(4):e443-e448.  Guerra LAP, Silva PS, Dos Santos RLO, Silva AMF, Albuquerque DP. Tratamento conservador de múltiplos tumores odontogênicos ceratocístico em paciente não sindrômico. Rev cir traumatol. buco-maxilo-fac. 2013; 13(2):43-50. Sundaragiri KS, Saxena S, Sankhla B, Bhargava A. Non syndromic synchronous multiple odontogenic keratocysts in a western Indian population: A series of four cases. J Clin Exp Dent. 2018;10(8):e831-6. Freitas AD, Veloso DA, Santos ALF, Freitas VA. Maxillary odontogenic keratocyst: a clinical case report. RGO Rev Gaúch Odontol. 2015; 63(4):484-88. Madhireddy MR, Prakash AJ, Mahanthi V, Chalapathi KV. Large Follicular Odontogenic Keratocyst affecting Maxillary Sinus mimicking Dentigerous Cyst in an 8-year-old Boy: A Case Report and Review. Int J Clin Pediatr Dent. 2018 Jul-Aug;11(4):349-351.  Moura BS, Cavalcante MA, Hespanhol W. Tumor odontogênico ceratocistico. Rev Col Bras Cir., 2016;43(6):466-71. Valori FP, Costa E, Buscatti MY, Oliveira JX, Costa C. Tumor odontogênico queratocístico: características intrínsecas e elucidação da nova nomenclatura do queratocisto odontogênico. J Health Sci Inst. 2010;28(1):80-3. Slusarenko da Silva Y, Stoelinga PJW, Naclério-Homem MDG. The presentation of odontogenic keratocysts in the jaws with an emphasis on the tooth-bearing area: a systematic review and meta-analysis. Oral Maxillofac Surg. 2019;23(2):133-47.

scholarly journals Acute bacterial nephritis. A descriptive study of an underdiagnosed entity

2020 ◽  
Vol 80 (3) ◽  
pp. 1-10
Author(s):  
Pedro Antonio Madero-Morales ◽  
Rigoberto Pallares-Méndez ◽  
Rodrigo Romero-Mata ◽  
Guillermo Vizcarra-Mata ◽  
Andrés Guillén-Lozoya
Keyword(s):  
Acute Pyelonephritis ◽  
Clinical Characteristics ◽  
Clinical Course ◽  
Case Series ◽  
Therapy Response ◽  
Time Frame ◽  
Clinical Findings ◽  
Imaging Features ◽  
Imaging Studies ◽  
Retrospective Case

Background: Acute bacterial nephritis is an infectious process diagnosed through imaging studies. The clinical course of the disease has been shown to be more aggressive than acute pyelonephritis. It continues to be underdiagnosed, thus there are few studies on the entity in the literature. Objective: To describe the clinical characteristics and imaging features of acute bacterial nephritis, as well as its clinical course. Design: A descriptive, retrospective case series was conducted. Materials and methods: Thirty-two cases of acute bacterial nephritis in patients admitted to the hospital within the time frame of 2009 to 2016 were reviewed. The patients’ clinical characteristics upon admission were registered, as well as inpatient clinical progression, culture results, and antibiotic therapy response. The imaging studies were re-evaluated and the diagnostic consistency with either the focal or multifocal disease presentation was confirmed. Results: Cases predominated in women (n=29, 90.62%) and the most frequently associated comorbidities were diabetes (n=16, 50%) and obesity (n=9, 28.25%). The most important clinical findings upon admission were fever (n=15, 46.87%) and leukocytosis (n=27, 84.38%). Escherichia coli was the most commonly isolated bacterium (63.63%). Both acute focal bacterial nephritis and acute multifocal bacterial nephritis were observed in 46.87% (n=15) and 53.13% (n=17) of the patients, respectively. Imaging studies were required for all diagnoses. Conclusion: Fever and leukocytosis are the main findings in acute bacterial nephritis. Imaging studies are necessary for making the diagnosis, given that acute pyelonephritis and acute bacterial nephritis cannot be clinically differentiated.

scholarly journals P.101 Conservative management of meningiomas with a moderate to high peritumoral brain edema index: a single-institution report

2019 ◽  
Vol 46 (s1) ◽  
pp. S40
Author(s):  
H Yan ◽  
B Karmur ◽  
D Jeong ◽  
M Bernstein
Keyword(s):  
Brain Edema ◽  
Medical Management ◽  
Case Series ◽  
Imaging Features ◽  
Retrospective Case ◽  
Patient Morbidity ◽  
Large Spread ◽  
Peritumoral Brain Edema ◽  
Mri Scans

Background: Meningiomas are the most commonly occurring benign intracranial tumors. When presenting with peritumoral brain edema (PTBE), surgical treatment can lead to patient morbidity. This retrospective case series aims to describe the conservative medical management of moderate to large meningiomas with large PTBE. Methods: Patients with suspected meningiomas greater than 2.0cm and edema index greater than 2.0 were identified by screening 3345 MRI scans between 2012-2017. Imaging analysis included MR imaging features of suspected meningiomas and clinical data was gathered from the electronic patient record (patient age, sex, patient symptoms, follow-up duration, and follow-up symptoms). Results: We report on 31 patients who received conservative medical management. Presenting complaints included headache, seizure, weakness; many presented asymptomatically. The average follow-up time was 3.96 years. At the final follow-up appointment, 19 (61%) patients were asymptomatic. Among symptomatic patients, seizures were the most common complaint. There was no mortality reported in our cohort and the average tumor progression was 7.04cm3/year. Conclusions: In this retrospective report of meningioma patients with high edema index, we found that most patients remained asymptomatic or had stable symptoms after at least 1-yr follow up after medical treatment. This study provides insight around the surgical decision-making for meningiomas with large spread of edema.

Efficacy, Outcomes, and Alignment Following Isolated Fibular Sesamoidectomy via a Plantar Approach

2019 ◽  
Vol 40 (12) ◽  
pp. 1375-1381 ◽  
Author(s):  
Samuel E. Ford ◽  
Christopher R. Adair ◽  
Bruce E. Cohen ◽  
W. Hodges Davis ◽  
J. Kent Ellington ◽  
...  
Keyword(s):  
Pain Relief ◽  
Functional Outcome ◽  
Case Series ◽  
Office Visit ◽  
Administrative Database ◽  
Retrospective Case ◽  
Level Of Evidence ◽  
Tertiary Referral Center ◽  
Flexion Strength

Background: The purpose of this study was to evaluate patients for intermediate-term pain relief, functional outcome, and changes in hallux alignment following isolated, complete fibular sesamoidectomy via a plantar approach for sesamoid-related pain recalcitrant to conservative treatment. Methods: A retrospective query of a tertiary referral center administrative database was performed using the Current Procedural Terminology code 28135 for sesamoidectomy between 2005 and 2016. Patients who underwent an isolated fibular sesamoidectomy were identified and contacted to return for an office visit. The primary outcome measure was change in visual analog pain score at final follow-up. Secondary measures included satisfaction, hallux flexion strength, hallux alignment, pedobarographic assessment, and postoperative functional outcome scores. Patients who met the 2-year clinical or radiographic follow-up minimum were included. Ninety fibular sesamoidectomies were identified. Thirty-six sesamoidectomies met inclusion criteria (median 60-month follow-up). The average patient was 36 years old and underwent sesamoidectomy 1.1 years after initial diagnosis. Results: Median visual analog scale scores improved 5 (6 to 1) points at final follow-up ( P < .001). Final postoperative mean hallux valgus angle did not differ from preoperative values (10.5 degrees/8.5 degrees, P = .12); similarly, the intermetatarsal angle did not differ (8.0 degrees/7.9 degrees, P = .53). Eighty-eight percent of patients would have surgery again and 70% were “very satisfied” with their result. Hallux flexion strength (mean 14.7 pounds) did not differ relative to the contralateral foot (mean 16.1 pounds) ( P = .23). Among the full 92 case cohort, 3 patients underwent 4 known reoperations. Conclusion: Fibular sesamoidectomy effectively provided pain relief (median 5-year follow-up) for patients with sesamoid pathology without affecting hallux alignment. Level of Evidence: Level IV, retrospective case series.

scholarly journals Continuous Visual Focal Status Epilepticus as the Primary Presentation of NMDA-R and GAD65-R Autoimmune Epilepsy

2020 ◽  
Vol 11 ◽  
Author(s):  
Elma M. Paredes-Aragón ◽  
Héctor E. Valdéz-Ruvalcaba ◽  
Andrea Santos-Peyret ◽  
Marcela Cisneros-Otero ◽  
Raúl Medina-Rioja ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Early Identification ◽  
Acid Decarboxylase ◽  
Visual Aura ◽  
Epilepsia Partialis Continua ◽  
Autoimmune Epilepsy ◽  
Immune Mediated ◽  
Focal Status Epilepticus ◽  
Patients With Epilepsy ◽  
Primary Presentation

Epilepsia partialis continua (EPC) has changed in its clinical and pathophysiological definition throughout time. Several etiologies have been described in addition to classic causes of EPC. The following case depicts a young woman who had a peculiar onset of epilepsy with a continuous visual aura becoming a form of chronic recurrent and non-progressive EPC. The patient was initially misdiagnosed as a non-neurological entity (assumed psychiatric in origin), but finally, an immune-mediated epilepsy was diagnosed, and EEG showed focal status epilepticus during evolution. Once the diagnosis was achieved and immune treatment was established, the patient is seizure free. Early identification of an immune basis in patients with epilepsy is important because immunotherapy can reverse the epileptogenic process and reduce the risk of chronic epilepsy. To date, this is the only case reported with EPC manifesting as a continuous visual aura associated with antiglutamic acid decarboxylase 65 (anti-GAD65) and anti-N-methyl-d-aspartate (anti-NMDA) antibodies.

scholarly journals Clinical Characteristics of Severe COVID-19 in China: A Case Series and Meta-analysis

2021 ◽  
Author(s):  
Geling Teng ◽  
Hua Hu ◽  
Xia Wang ◽  
Junsheng Wang ◽  
Hongmei Zhang ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Coronary Heart Disease ◽  
Heart Disease ◽  
Bacterial Infections ◽  
Clinical Signs ◽  
Treatment Strategies ◽  
Case Series ◽  
Imaging Features ◽  
Chest Tightness ◽  
Retrospective Case

Abstract Background: There have been inconsistent reports regarding the unique manifestations of severe coronavirus disease 2019 (COVID-19) occurring in China. This study analyzed the clinical manifestation of 13 severe COVID-19 cases at a single institution and compared the data to previously reported characteristics of severe COVID-19 in China. Methods: This retrospective case study included patients with severe COVID-19 who were admitted to the isolation ward of the Shandong Chest Hospital from January 2020 to February 2020. The clinical signs and symptoms, laboratory examination results, imaging features, treatment strategies, and patient prognoses were summarized. A database search was then conducted for studies published through December 2020 documenting characteristics of severe COVID-19 cases in China. The pooled results for severe COVID-19 patients in China were calculated by using the random-effects model.Results: A total of 4 severe and 9 critical patients were included from Shandong Chest Hospital. The average patient age was 55.3 (range 23-88) years, and 61.5% of patients were male. Chest computed tomography for all patients showed multiple lesions as ground-glass shadows in both lungs. All patients presented bacterial infection and various degrees of liver and myocardial injury. The treatment strategies for patients included antibiotics, immunoglobulin, and glucocorticoids, and mechanical ventilation was used in all patients for respiratory failure. Two patients died, and 11 recovered. In the pooled data for severe COVID-19 patients, the most common comorbidities were hypertension, diabetes mellitus, and coronary heart disease. The common signs in these patients were fever, cough, fatigue, chest tightness, and a leukocyte count > 10. Conclusions: Older males with hypertension, diabetes mellitus, and coronary heart disease may be at higher risk of developing severe COVID-19. Patients should be assessed for concomitant bacterial infections. Cardiac and liver enzymes, fever, cough, fatigue, chest tightness, and leukocytosis should be monitored for signs of disease progression.

Dysphagia characteristics in Huntington’s disease patients: insights from the Fiberoptic Endoscopic Evaluation of Swallowing and the Swallowing Disturbances Questionnaire

CNS Spectrums ◽  
2018 ◽  
Vol 24 (04) ◽  
pp. 413-418 ◽  
Author(s):  
Yael Manor ◽  
Yael Oestreicher-Kedem ◽  
Alona Gad ◽  
Jennifer Zitser ◽  
Achinoam Faust-Socher ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Oropharyngeal Dysphagia ◽  
Case Series ◽  
Solid Food ◽  
Self Report ◽  
Endoscopic Evaluation ◽  
Retrospective Case ◽  
Tertiary Referral Center

BackgroundHuntington’s disease (HD) is a neurodegenerative disease characterized by increasing dysphagia as the disease progresses. Specific characteristics of the HD dysphagia are not well defined.ObjectiveTo characterize the swallowing disturbances of HD patients, to evaluate the feasibility of Fiberoptic Endoscopic Evaluation of Swallowing (FEES) in assessing dysphagia in HD patients, and to discern the relation between FEES findings and patients’ self-report on dysphagia symptoms and swallowing related quality of life (SWAL-QOL).MethodA retrospective case series in a tertiary referral center. All recruited HD patients underwent Bed Side Swallowing Evaluation (BSE), FEES, the Unified Huntington’s Disease Rating Scale (UHDRS), and the Montreal Cognitive Assessment (MoCA). All completed the Swallowing Disturbances Questionnaire (SDQ) and the SWAL-QOL questionnaire.ResultsFourteen HD patients were recruited. All were able to complete the FEES study. The FEES demonstrated delayed swallowing reflex, solid food residues, and pre/post swallowing spillage in most patients (50%, 53.5%, 83.3%, and 87.5%, respectively). The mean SDQ score was 13.2. Significant correlations were found between the SWAL-QOL fear of eating score; the SDQ oral, pharyngeal, and total scores; and the FEES parameters of pureed and solid food bolus flow time. Significant correlations were also found between the total UHDRS score, the volitional cough score, and the SWAL-QOL disease burden score.ConclusionHD patients exhibit prominent unique oropharyngeal dysphagia features that may serve as a marker of disease progression. The FEES and the SDQ are valuable tools for detecting these features in HD patients with swallowing disturbance.

scholarly journals Granulomatous Lymphocytic Interstitial Lung Disease (GL-ILD): CT Imaging Features and Clinical Findings to Expedite a Timely Diagnosis

2021 ◽  
Author(s):  
Avanthika Wynn ◽  
Eugene A. Berkowitz ◽  
Brent P. Little ◽  
Srihari Veeraraghavan ◽  
Frank Schneider ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Research Question ◽  
Case Series ◽  
Lower Lobe ◽  
Clinical Findings ◽  
Ct Imaging ◽  
Imaging Features ◽  
Retrospective Case ◽  
Case Series Study

Abstract Background: Establishing a diagnosis of Granulomatous Lymphocytic-Interstitial Lung Disease (GL-ILD) is difficult due to the overlapping CT imaging and histologic features, and are often confused with infection, sarcoidosis and/or follicular bronchiolitis. Research Question: Identify specific CT imaging features and clinical characteristics to suggest a confident diagnosis of Granulomatous Lymphocytic-Interstitial Lung Disease (GL-ILD).Study Design and Methods: IRB-approved retrospective case series study involving the review of the electronic medical record and CT chest imaging of eight patients with GL-ILD.Results: Bronchocentric airway-centered lower lobe predominant (part solid nodules more common than ground glass or solid nodules) ranging from 5 to 10 mm in size were found consistently in a majority of the eight patients with GL-ILD. Interpretation: In combination with the above pulmonary nodular pattern, mediastinal and hilar lymphadenopathy in conjunction, either splenomegaly or splenectomy and/or abdominal lymphadenopathy are excellent CT imaging features to prospectively suggest the diagnosis of GL-ILD in a patient with CVID.

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

2021 ◽  
Author(s):  
Aïsha Sooltangos ◽  
Istvan Bodi ◽  
Prajwal Ghimire ◽  
Konstantinos Barkas ◽  
Sinan Al-Barazi ◽  
...  
Keyword(s):  
Proton Beam ◽  
Case Series ◽  
Cell Tumor ◽  
Imaging Features ◽  
Csf Rhinorrhea ◽  
Proton Beam Radiotherapy ◽  
Tertiary Referral Center ◽  
Notochord Cell ◽  
Ecchordosis Physaliphora ◽  
Imaging Surveillance

Abstract Objectives Ecchordosis physaliphora (EP) is a benign notochord lesion of the clivus arising from the same cell line as chordoma, its malignant counterpart. Although usually asymptomatic, it can cause spontaneous cerebrospinal fluid (CSF) rhinorrhea. Benign notochordal cell tumor (BNCT) is considered another indolent, benign variant of chordoma. Although aggressive forms of chordoma require maximal safe resection followed by proton beam radiotherapy, BNCT and EP can be managed with close imaging surveillance without resection or radiotherapy. However, while BNCT and EP can be distinguished from more aggressive forms of chordoma, differentiating the two is challenging as they are radiologically and histopathologically identical. This case series aims to characterize the clinicopathological features of EP and to propose classifying EP and BNCT together for the purposes of clinical management. Design Case series. Setting Tertiary referral center, United Kingdom. Participants Patients with suspected EP from 2015 to 2019. Main Outcome Measures Diagnosis of EP. Results Seven patients with radiological suspicion of EP were identified. Five presented with CSF rhinorrhea and two were asymptomatic. Magnetic resonance imaging features consistently showed T1-hypointense, T2-hyperintense nonenhancing lesions. Diagnosis was made on biopsy for patients requiring repair and radiologically where no surgery was indicated. The histological features of EP included physaliphorous cells of notochordal origin (positive epithelial membrane antigen, S100, CD10, and/or MNF116) without mitotic activity. Conclusion EP is indistinguishable from BNCT. Both demonstrate markers of notochord cell lines without malignant features. Their management is also identical. We therefore propose grouping EP with BNCT. Close imaging surveillance is required for both as progression to chordoma remains an unquantified risk.

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