Childhood pseudotumor cerebri syndrome: demographics, clinical features and optical coherence tomography utilization as a new method for diagnosis, treatment efficiency and follow up

Childhood pseudotumor cerebri syndrome: demographics, clinical features and optical coherence tomography utilization as a new method for diagnosis, treatment efficiency and follow up | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Childhood pseudotumor cerebri syndrome: demographics, clinical features and optical coherence tomography utilization as a new method for diagnosis, treatment efficiency and follow up Burcu Daldaban Çiftçi, Hakan Gümüş, Duygu Gülmez Sevim, Mehmet Canpolat, and 4 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3947805/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Aim The aim of this study is to evaluate the demographic data, treatment efficacy, optical coherence tomography (OCT) results used for the diagnosis and follow-up of patients with pseudotumor cerebri syndrome (PCS) followed up in our pediatric neurology clinic between 2014 and 2019. Methods: We included children aged 26 cm/H2O in lumbar puncture. Patient data were obtained retrospectively from hospital records. Results: Retinal nerve fiber layer (RNFL) thickness increased significantly in OCT as CSF pressure increased. Further, RNFL thickness measured before and after treatment differed significantly. When the pretreatment body mass index and RNFL thickness of primary and secondary PCS cases were compared, it was found that RNFL thickness of obese patients was lower than that of non-obese patients. In conclusion, we suggest that OCT, which is an easy-to-apply and reliable test that has recently become widely used in pediatric PCS, should be used in the diagnosis of PCS, evaluation of treatment efficacy, and follow-up. Pediatrics pseudotumor cerebri optical coherence tomography Figures Figure 1 Figure 2 Introduction Pseudotumor cerebri syndrome (PCS), otherwise known as idiopathic intracranial hypertension syndrome, is a disease characterized by increased intracranial pressure with normal cerebrospinal fluid (CSF) composition, without intracranial space-occupying lesion, infection, or hydrocephalus. It does not cause any localized neurological deficits except abducens nerve palsy. Symptoms such as headache, double vision, vision loss, and pulsatile tinnitus may develop owing to the increased intracranial pressure [ 1 , 2 ]. The diagnosis of the disease is made by measuring the CSF opening pressure via lumbar puncture (LP) and cranial imaging methods. Definitive diagnosis is made by a CSF opening pressure of > 26 cm/H 2 O. However LP is invasive and painful method, especially for the pediatric population. Thus, noninvasive methods are more applicable for this group of age. Nowadays optical coherence tomography (OCT) utilization becomes more common for pediatric PSC diagnosis. Optical coherence tomography can be used in the pediatric population for evaluating papilledema and following up the disease progression[ 3 , 4 ]. OCT is a noninvasive imaging method that provides high-resolution images of the retina and the optic nerve structures. With the increase in intracranial pressure, the CSF pressure on the optic sheath increases the thickness of the peripapillary retinal nerve fiber layer (RNFL) and the optic nerve head becomes prominent[ 4 ]. OCT reveals that increased RNFL thickness is significantly higher in cases with increased intracranial pressure compared with that in cases with pseudopapilledema[ 4 , 5 ]. The aim of this study was to evaluate demographical data of PCS and the results of OCT utilized for PCS diagnosis, follow up and treatment efficacy in our pediatric neurology clinic between 2014 and 2019. Materials and Methods Ninety-eight patients aged 26 cm/H 2 O in the LP performed for determining the etiology were included in this study. Those with space-occupying lesions in cranial images, those with CSF opening pressure of ≤ 26 cm/H 2 O in the LP performed under sedation due to headache, those with a determined biochemical or microbiological pathology in CSF, those with a drusen body identified in fundoscopy (6 cases), and those aged > 18 years were excluded. The Revised Modified Dandy Criteria are used for the diagnosis of the PCS [ 2 ]. On examination, those with tanner stage 1, were considered as in prepubertal period. However those with tanner stage ≥ 2 were considered as in pubertal period. Those with body mass index (BMI) > 30 were considered obese. In addition to fundoscopy, nasal and temporal RNFL thicknesses were measured via Spectral Domain (SD)-OCT (Spectralis; Heidelberg Engineering, Heidelberg, Germany) at a wavelength of ~ 840 nm at the time of diagnosis, after treatment, and the follow-up and treatment efficacy of the cases were evaluated. Only SD-OCT images with a signal-to-noise value higher than 25 dB were analyzed. Scans with misalignment, decentration of the measurement circle, or poor illumination and those that were out of focus were excluded from the analysis. To assess the RNFL, a circular scan with a diameter of approximately 3.4 mm was performed after manually positioning the center on the middle of the optic disc. The RNFL spectralis protocol generated a map showing the average thickness as well as maps with 6 sector thicknesses (superonasal, nasal, inferonasal, inferotemporal, temporal, and superotemporal) [ 6 ]. Statistical analysis In summarizing data from the study, descriptive statistics were presented as mean ± standard deviation or median (interquartile range) for continuous variables. Statistical analyses were performed using Jamovi (Version 1.0.7) and JASP (version 0.11.1) programs, and the level of significance in the statistical analyses was accepted as 0.05 (p-value) Ethics This study was approved by the Erciyes University School of Medicine Ethics Committee (approval number/date 96681246/12.06.2019). Written informed consent was obtained from the patients and their parents before their participation in this study. Results Among the patients who presented to our outpatient clinic, headache (85.7%) and vision defect (32.7%) were the most prevalent symptoms (Table 1 ). As we observed the cranial magnetic resonance imaging (MRI) findings, the most common findings were sinus vein thrombosis (SVT) (7.1%), posterior optic glob flattening (7.1%) and partial empty sella (4.1%) (Fig. 1 ). When there is no underlying sinus anomaly or etiology such as SVT in PCS, it is referred to as primary PCS; in contrast, when an identifiable cause is indicated, it is referred to as secondary PCS [ 7 ]. In our study there were 35 patients in primary PCS group, 63 patients in secondary PCS group Table 1 Comparison of gender, etiology (primary and secondary), BMI, and CSF opening pressure with respect to the reason for starting additional medication (topiramate) p Resistance to acetazolamide monotherapy Gender Male 18 (48.7) 0.196 (x) Female 19 (51.4) BMI 27 [21–30] 0.008 (xxx) CSF opening pressure (cm/H 2 O) 38 [29–50] 0.325 (xxx) Etiology Secondary 31 (83.8) 0.999 (xx) Primary 6 (16.2) (x) Pearson’s chi-Squared test was used. Descriptive statistics are presented as numbers (%). (xx) Fisher's exact test was used. Descriptive statistics are presented as numbers (%). (xxx) Mann–Whitney U test was used. Descriptive statistics are presented as median [interquartile range]. P-values indicated in bold were statistically significant (p < 0.05). BMI, body mass index; CSF, cerebrospinal fluid The relation between SVT presence and mean CSF opening pressure (cm/H 2 O) in the subjects included in the study was statistically significant (p = 0.011). The CSF opening pressure was higher in cases with SVT (47 cm/H 2 O) than cases without SVT (31 cm/H 2 O). Further, although the CSF mean opening pressure was higher in secondary PCS cases than in primary PCS cases, the difference between two groups was not statistically significant (Table 2 ). Table 2 Measurement results of RNFL thicknesses according to CSF opening pressures before and after treatment Pearson's r p CSF opening pressure - Nasal RNFL (pretreatment) 0.282 0.025 CSF opening pressure - Nasal RNFL (posttreatment) -0.101 0.480 CSF opening pressure - Temporal RNFL (pretreatment) 0.261 0.039 CSF opening pressure - Temporal RNFL (posttreatment) -0.173 0.225 Pearson’s correlation coefficient was used. CSF, cerebrospinal fluid; RNFL, retinal nerve fiber layer There was a statistically significant, linear, unidirectional, and weak correlation between CSF opening pressure and the number of unloading LPs. In other words, as the CSF opening pressure increased, the need for repetitive unloading LPs also increased. As we know up to 15% of weight loss associated with PCS remission [ 8 ], first of all obese patients are referred to a hospital-based weight management programme. As the first line medical therapy, acetazolamide dose was 10–20 mg/kg/day. Treatment efficiency was evaluated by repetitive (every 3 months) OCT and visual field examinations. We administered topiramate (1–3 mg/kg/day) as a second line medical therapy to patients who had acetazolamide side effect (intolerable metabolic acidosis, 4 male, 10 female patients) or had resistance to acetazolamide monotherapy. Monotherapy resistance was observed in patients (18 male, 19 female patients) with high body mass index (BMI). A statistically significant relation was found between high BMI and the requirement for additional medication (topiramate) owing to drug resistance developed against the first-line acetazolamide therapy (It was not significant in the group who ceased acetozalamide treatment due to acidosis side effect (4 male, 10 female) and switched to topiramate treatment). When other comparisons were examined, no statistically significant differences were noted (Table 3 ). During the treatment, mean headache relief time was 30 days, mean vision defect relief time was 60 days. On follow up, mean drug withdrawal time was 6 months and mean follow up time was 1.8 years. Table 3 Comparison of RNFL thicknesses before and after treatment Pretreatment Posttreatment p Nasal RNFL 521.59 ± 157.97 425.88 ± 107.02 < 0.001 Temporal RNFL 738.41 ± 165.27 625.57 ± 118.33 < 0.001 Dependent samples t test was used. RNFL, retinal nerve fiber layer Objective and quantitative diagnostic and follow-up criteria are needed because patient compliance is not always optimal in the evaluation of visual acuity and visual field in the pediatric population, possible refractive errors may affect visual acuity, and the pediatric population gets bored and easily distracted during the examination. Although disc appearance gives an idea about the differential diagnosis of papilledema in fundoscopy, OCT is an objective, rapid, quantitative, and reproducible imaging method in the differential diagnosis of pseudopapilledema causes such as small optic disc appearance or optic disc drusen and in monitoring treatment response. The results of our study further prove OCT as a useful method (Fig. 2 ). When the CSF opening pressure measured by LP performed for diagnosis was compared with the nasal and temporal RNFL values obtained via OCT, a statistically significant increase was found in RNFL values as the CSF opening pressure increased. However, the difference in posttreatment values was not significant (Table 4). As we compared the pre- and posttreatment OCT values, the difference between the mean nasal and temporal RNFL values before and after the treatment was statistically significant (Table 5). The mean pre- and posttreatment nasal and temporal RNFL values were evaluated according to the etiology of the cases (primary and secondary cases). Pretreatment RNFL thickness was relatively greater in secondary cases compared with that in primary cases, but the difference between the mean values was not statistically significant. When the pre- and posttreatment BMI and nasal and temporal RNFL values were compared, no statistically significant differences were noted (p > 0.05 for each). In fact, RNFL values were lower in obese patients. Discussion We evaluated the demographical data of PCS and discussed the results of OCT which is a new and non-invasive method for diagnosis and follow up. Patients those with high BMI developed resistence to acetazolamide monotherapy. Thus additional topiramate therapy was required for adequate symptom control. We concluded that the relation between OCT results and the CSF pressure were statistically significant. Ozkale et al. found that the mean age of the patients was 11.15 ± 3.32 years and 40% of them were in the prepubertal period[ 9 ]. In our study, the mean age at presentation was 10.99 ± 3.28 years; further, 36.1% of the patients were in the prepubertal period and 63.9% were in the pubertal period, which was consistent with the existing literature[ 9 , 10 ]. In the present study, obesity was not observed in the prepubertal period, and all obese patients were in the pubertal period. This was attributed to an increasing tendency to develop obesity with age[ 11 , 12 ]. Consistent with the literature, the rate of obesity was lower in the prepubertal period than in the pubertal period in our study. When the CSF opening pressures of the primary and secondary cases were compared, the mean CSF opening pressure in those with primary PCS was 30 cm/H 2 O, whereas it was 36 cm/H 2 O in those with secondary PCS; however, the difference between the two was not statistically significant (p = 0.094) as similar to the results of Shawn et al.[ 13 ]. Although no statistical significance was obtained, secondary PCS cases showed a tendency of higher CSF opening pressures than primary cases. In the present study, there was no statistically significant relationship between BMI values and OCT measurements, but a statistically significant relationship was noted between CSF opening pressures and OCT measurements. Pollak et al. found a significant relationship between CSF opening pressure and the degree of papilledema, which was consistent with our findings[ 14 ]. Varied results have been reported in studies evaluating the response to medical treatment. While it was reported that 76.6% of the patients responded to acetazolamide treatment in one study, on the other hand the response to acetazolamide treatment was 37.8% in another study[ 15 , 16 ]. In our study, the rate of patients who responded to acetazolamide treatment was 62%. According to the results of the present study, a significant decrease was observed in both nasal and temporal RNFL thickness after papilledema treatment compared with the pretreatment values. However, although RNFL thickness was correlated with CSF opening pressure before treatment, the absence of this correlation after treatment may be attributed to the fact that the decrease in RNFL thickness takes longer than the improvement of CSF opening pressure, or to the increase in glial tissue formed after the improvement of papilledema. Therefore, the results of the present study are particularly important as they demonstrate that OCT is a useful method in the diagnosis and treatment follow up of PCS, but RNFL thickness may not completely return to normal in OCT during complete recovery of the papilledema. Further, OCT should be used together with the examination of clinical disc appearance in the follow-up period. In conclusion , PCS is a disease associated with increased CSF pressure, and its etiology is still not fully elucidated. The use of OCT has increased in the pediatric population in recent years, and it is an effective and reliable method in the diagnosis and follow-up of PCS and the evaluation of treatment efficacy. Declarations The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. Author Contribution Material preparation, data collection and analysis: A. C. F. Conceptualization: A. B. C. H.Methodology: A.B.C.H.Formal analysis and investigation: A.B.C.Writing - original draft preparation: A. C. F.Writing - review and editing:A. B. C. H. Supervision: B. C. H.Review: All authors reviewed the manuscript. References Standridge SM. Idiopathic intracranial hypertension in children: A review and algorithm. Pediatr Neurol 2010. https://doi.org/10.1016/j.pediatrneurol.2010.08.001. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013. https://doi.org/10.1212/WNL.0b013e3182a55f17. Gilbert AL, Heidary G. Update on the evaluation of pediatric idiopathic intracranial hypertension. Curr Opin Ophthalmol 2016. https://doi.org/10.1097/ICU.0000000000000317. Lee YA, Tomsak RL, Sadikovic Z, Bahl R, Sivaswamy L. Use of Ocular Coherence Tomography in Children With Idiopathic Intracranial Hypertension—A Single-Center Experience. Pediatr Neurol 2016. https://doi.org/10.1016/j.pediatrneurol.2015.10.022. Martinez MR, Ophir A. Optical coherence tomography as an adjunctive tool for diagnosing papilledema in young patients. J Pediatr Ophthalmol Strabismus 2011. https://doi.org/10.3928/01913913-20100719-05. González-López JJ, Rebolleda G, Leal M, Oblanca N, Muñoz-Negrete FJ, Costa-Frossard L, et al. Comparative diagnostic accuracy of ganglion cell-inner plexiform and retinal nerve fiber layer thickness measures by Cirrus and spectralis optical coherence tomography in relapsing-remitting multiple sclerosis. Biomed Res Int 2014;2014. https://doi.org/10.1155/2014/128517. Phillips PH, Sheldon CA. Pediatric Pseudotumor Cerebri Syndrome. J Neuroophthalmol 2017. https://doi.org/10.1097/WNO.0000000000000548. Sinclair AJ, Burdon MA, Nightingale PG, Ball AK, Good P, Matthews TD, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: Prospective cohort study. BMJ 2010;341:138. https://doi.org/10.1136/bmj.c2701. Özkale Y, Erol İ, Çoban Karataş M, Özkale M, Saygı S, Alkan Ö, et al. Pseudotumor Cerebri; Single Center Experience. Turkish J Pediatr Dis 2018:193–202. https://doi.org/10.12956/tjpd.2018.364. Suzuki H, Aoki K, Chiba K, Sato Y, Shiozawa Y, Shiraishi Y, et al. Mutational landscape and clonal architecture in grade II and III gliomas. Nat Genet 2015. https://doi.org/10.1038/ng.3273. Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 2002. https://doi.org/10.1177/08830738020170101401. Masri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH. Intracranial hypertension in children: Etiologies, clinical features, and outcome. J Child Neurol 2015. https://doi.org/10.1177/0883073815574332. Aylward SC, Waslo CS, Au JN, Tanne E. Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry. Pediatr Neurol 2016;61:76–82. https://doi.org/10.1016/j.pediatrneurol.2016.04.007. Pollak L, Zohar E, Glovinsky Y, Huna-Baron R. Reevaluation of presentation and course of idiopathic intracranial hypertension - a large cohort comprehensive study. Acta Neurol Scand 2013;127:406–12. https://doi.org/10.1111/ane.12060. Per H, Canpolat M, Gümüş H, Poyrazoĝlu HG, Yikilmaz A, Karaküçük S, et al. Clinical spectrum of the pseudotumor cerebri in children: Etiological, clinical features, treatment and prognosis. Brain Dev 2013. https://doi.org/10.1016/j.braindev.2012.08.008. Tovia E, Reif S, Oren A, Mitelpunkt A, Fattal-Valevski A. Treatment response in pediatric patients with pseudotumor cerebri syndrome. J Neuro-Ophthalmology 2017. https://doi.org/10.1097/WNO.0000000000000516. Table 4 and 5 Table 4 and 5 are not available with this version Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3947805","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":272596893,"identity":"777e1abb-b09b-4701-9708-207e3600b418","order_by":0,"name":"Burcu Daldaban Çiftçi","email":"","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Burcu","middleName":"Daldaban","lastName":"Çiftçi","suffix":""},{"id":272596894,"identity":"43f27ed2-5185-4569-a564-2c3a8e01fa02","order_by":1,"name":"Hakan Gümüş","email":"","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Hakan","middleName":"","lastName":"Gümüş","suffix":""},{"id":272596895,"identity":"05e6f12c-7a08-450f-9790-ead5e9efd4cd","order_by":2,"name":"Duygu Gülmez Sevim","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABFUlEQVRIiWNgGAWjYDCCAxBKBogZHwAJHgifjbAWkEpmAxQtPERoYZNACOPRwncjx/g1z6/DPLrt7c8qPu6xkzE43v6A4UPZYQZ76QNYtUjeyDGz5u07zGN25kDazRnPknkMzpwxYJxx7jADD18CVi0GQC3GvD1ALTcSjt3mOXCAByjCwMzbBtSCw2UILfcfthX/AWm5//wB81/8Wowf8/wA2cLMxswAtoXBgJkRjxbJM8/KGOc2pAP9ksYs2XMgmUfyTI7BwZ5z6Tw8Z3CE2PHkzR/e/LGWMzt+/OGHHwfs7PmAjAc/yqzl2Huwa2Fg4DCTYGxD4iscgEQWzphkYGB//IHhDxJfvgG32lEwCkbBKBiZAAC1/2QoH59QCwAAAABJRU5ErkJggg==","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Duygu","middleName":"Gülmez","lastName":"Sevim","suffix":""},{"id":272596896,"identity":"8f8810ef-e0c2-47af-a285-a0d6293c9d5a","order_by":3,"name":"Mehmet Canpolat","email":"","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Mehmet","middleName":"","lastName":"Canpolat","suffix":""},{"id":272596897,"identity":"865af26b-1d54-451b-9579-7294ca7c3856","order_by":4,"name":"Ümmügülsüm Özgül Gümüş","email":"","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Ümmügülsüm","middleName":"Özgül","lastName":"Gümüş","suffix":""},{"id":272596898,"identity":"a1aa3113-4284-4c46-81db-038f241556df","order_by":5,"name":"Murat Çiftçi","email":"","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Murat","middleName":"","lastName":"Çiftçi","suffix":""},{"id":272596899,"identity":"6ead0e8e-87b6-4d2a-a8cf-0d47effdbc5f","order_by":6,"name":"Sefer Kumandaş","email":"","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Sefer","middleName":"","lastName":"Kumandaş","suffix":""},{"id":272596900,"identity":"2228bff9-5516-4b08-978d-af47d27e1fe8","order_by":7,"name":"Hüseyin Per","email":"","orcid":"","institution":"Erciyes Üniversitesi Tıp Fakültesi Hastaneleri","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Hüseyin","middleName":"","lastName":"Per","suffix":""}],"badges":[],"createdAt":"2024-02-11 07:04:16","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3947805/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3947805/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":51140784,"identity":"7bfa9376-5bb6-427f-bab8-21a04ed28262","added_by":"auto","created_at":"2024-02-14 20:08:02","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":260508,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003e(a) \u003c/strong\u003eMagnetic resonance imaging findings of the included cases. 11-year-old girl, on T2-weighted MRI, posterior optic globe flattening (thick black arrow) and optic sheath enlargement. Cerebrospinal fluid space becomes prominent (thin black arrow). \u003cstrong\u003e(b)\u003c/strong\u003e 5-year-old boy, on T1-weighted MRI, partial empty sella sign (white arrow). \u003cstrong\u003e(c) \u003c/strong\u003e7-year-old boy, on MR venography, posterior sagittal sinus vein thrombosis (white arrow).\u003c/p\u003e","description":"","filename":"Fig1.Tiff.png","url":"https://assets-eu.researchsquare.com/files/rs-3947805/v1/0255aa73df45d1fb675506d1.png"},{"id":51140785,"identity":"8c561405-14d3-416a-94aa-1f3ed26107c2","added_by":"auto","created_at":"2024-02-14 20:08:02","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":478206,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003e(a-b) \u003c/strong\u003eA\u003cstrong\u003e \u003c/strong\u003e14-year-old girl with pseudotumor cerebri and papilledema. Optic nerve head optic coherence tomography taken at 3-month intervals reveals that the loss in the optic disc cupping improved and the peripapillary nerve fiber layer edema regressed over time. \u003cstrong\u003e(c)\u003c/strong\u003e However, the slight thickness increase still seen in the last follow-up, \u003cstrong\u003e(d)\u003c/strong\u003e especially in the nasal region, on clinical examination appears to be due to peripapillary gliosis, not edema.\u003c/p\u003e","description":"","filename":"Fig2.Tiff.png","url":"https://assets-eu.researchsquare.com/files/rs-3947805/v1/f933a16411dbff81cc1ba256.png"},{"id":53332327,"identity":"7a6399d4-65fc-48d2-aed1-d501ec611fdf","added_by":"auto","created_at":"2024-03-24 11:30:03","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":970720,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3947805/v1/5bcb97a9-b6bf-4b54-aee6-661981e4fc2f.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003eChildhood pseudotumor cerebri syndrome: demographics, clinical features and optical coherence tomography utilization as a new method for diagnosis, treatment efficiency and follow up\u003c/p\u003e","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePseudotumor cerebri syndrome (PCS), otherwise known as idiopathic intracranial hypertension syndrome, is a disease characterized by increased intracranial pressure with normal cerebrospinal fluid (CSF) composition, without intracranial space-occupying lesion, infection, or hydrocephalus. It does not cause any localized neurological deficits except abducens nerve palsy. Symptoms such as headache, double vision, vision loss, and pulsatile tinnitus may develop owing to the increased intracranial pressure [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe diagnosis of the disease is made by measuring the CSF opening pressure via lumbar puncture (LP) and cranial imaging methods. Definitive diagnosis is made by a CSF opening pressure of \u0026gt;\u0026thinsp;26 cm/H\u003csub\u003e2\u003c/sub\u003eO. However LP is invasive and painful method, especially for the pediatric population. Thus, noninvasive methods are more applicable for this group of age. Nowadays optical coherence tomography (OCT) utilization becomes more common for pediatric PSC diagnosis.\u003c/p\u003e \u003cp\u003eOptical coherence tomography can be used in the pediatric population for evaluating papilledema and following up the disease progression[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. OCT is a noninvasive imaging method that provides high-resolution images of the retina and the optic nerve structures. With the increase in intracranial pressure, the CSF pressure on the optic sheath increases the thickness of the peripapillary retinal nerve fiber layer (RNFL) and the optic nerve head becomes prominent[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. OCT reveals that increased RNFL thickness is significantly higher in cases with increased intracranial pressure compared with that in cases with pseudopapilledema[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe aim of this study was to evaluate demographical data of PCS and the results of OCT utilized for PCS diagnosis, follow up and treatment efficacy in our pediatric neurology clinic between 2014 and 2019.\u003c/p\u003e"},{"header":"Materials and Methods","content":"\u003cp\u003eNinety-eight patients aged\u0026thinsp;\u0026lt;\u0026thinsp;18 years who presented to our pediatric neurology outpatient clinic between 2014 and 2019, with or without bilateral papilledema detected on initial examination and CSF opening pressure of \u0026gt;\u0026thinsp;26 cm/H\u003csub\u003e2\u003c/sub\u003eO in the LP performed for determining the etiology were included in this study. Those with space-occupying lesions in cranial images, those with CSF opening pressure of \u0026le;\u0026thinsp;26 cm/H\u003csub\u003e2\u003c/sub\u003eO in the LP performed under sedation due to headache, those with a determined biochemical or microbiological pathology in CSF, those with a drusen body identified in fundoscopy (6 cases), and those aged\u0026thinsp;\u0026gt;\u0026thinsp;18 years were excluded. The Revised Modified Dandy Criteria are used for the diagnosis of the PCS [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eOn examination, those with tanner stage 1, were considered as in prepubertal period. However those with tanner stage\u0026thinsp;\u0026ge;\u0026thinsp;2 were considered as in pubertal period. Those with body mass index (BMI)\u0026thinsp;\u0026gt;\u0026thinsp;30 were considered obese.\u003c/p\u003e \u003cp\u003eIn addition to fundoscopy, nasal and temporal RNFL thicknesses were measured via Spectral Domain (SD)-OCT (Spectralis; Heidelberg Engineering, Heidelberg, Germany) at a wavelength of ~\u0026thinsp;840 nm at the time of diagnosis, after treatment, and the follow-up and treatment efficacy of the cases were evaluated. Only SD-OCT images with a signal-to-noise value higher than 25 dB were analyzed. Scans with misalignment, decentration of the measurement circle, or poor illumination and those that were out of focus were excluded from the analysis. To assess the RNFL, a circular scan with a diameter of approximately 3.4 mm was performed after manually positioning the center on the middle of the optic disc. The RNFL spectralis protocol generated a map showing the average thickness as well as maps with 6 sector thicknesses (superonasal, nasal, inferonasal, inferotemporal, temporal, and superotemporal) [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eStatistical analysis\u003c/h2\u003e \u003cp\u003eIn summarizing data from the study, descriptive statistics were presented as mean\u0026thinsp;\u0026plusmn;\u0026thinsp;standard deviation or median (interquartile range) for continuous variables. Statistical analyses were performed using Jamovi (Version 1.0.7) and JASP (version 0.11.1) programs, and the level of significance in the statistical analyses was accepted as 0.05 (p-value)\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003eEthics\u003c/h2\u003e \u003cp\u003e This study was approved by the Erciyes University School of Medicine Ethics Committee (approval number/date 96681246/12.06.2019). Written informed consent was obtained from the patients and their parents before their participation in this study.\u003c/p\u003e \u003c/div\u003e"},{"header":"Results","content":"\u003cp\u003eAmong the patients who presented to our outpatient clinic, headache (85.7%) and vision defect (32.7%) were the most prevalent symptoms (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). As we observed the cranial magnetic resonance imaging (MRI) findings, the most common findings were sinus vein thrombosis (SVT) (7.1%), posterior optic glob flattening (7.1%) and partial empty sella (4.1%) (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). When there is no underlying sinus anomaly or etiology such as SVT in PCS, it is referred to as primary PCS; in contrast, when an identifiable cause is indicated, it is referred to as secondary PCS [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. In our study there were 35 patients in primary PCS group, 63 patients in secondary PCS group\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eComparison of gender, etiology (primary and secondary), BMI, and CSF opening pressure with respect to the reason for starting additional medication (topiramate)\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colspan=\"2\" morerows=\"1\" nameend=\"c2\" namest=\"c1\" rowspan=\"2\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c4\" namest=\"c3\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c5\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003ep\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eResistance to acetazolamide monotherapy\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e\u003cb\u003eGender\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e18 (48.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.196\u003csup\u003e\u003cb\u003e(x)\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eFemale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e19 (51.4)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e \u003cp\u003e\u003cb\u003eBMI\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e27 [21\u0026ndash;30]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e\u003cb\u003e0.008\u003c/b\u003e \u003csup\u003e\u003cb\u003e(xxx)\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e \u003cp\u003e\u003cb\u003eCSF opening pressure (cm/H\u003c/b\u003e\u003csub\u003e\u003cb\u003e2\u003c/b\u003e\u003c/sub\u003e\u003cb\u003eO)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e38 [29\u0026ndash;50]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0.325\u003csup\u003e\u003cb\u003e(xxx)\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e\u003cb\u003eEtiology\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSecondary\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e31 (83.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\" morerows=\"1\" rowspan=\"2\"\u003e \u003cp\u003e0.999\u003csup\u003e\u003cb\u003e(xx)\u003c/b\u003e\u003c/sup\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePrimary\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e6 (16.2)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003e\u003csup\u003e\u003cb\u003e(x)\u003c/b\u003e\u003c/sup\u003e \u003cem\u003ePearson\u0026rsquo;s chi-Squared test was used. Descriptive statistics are presented as numbers (%).\u003c/em\u003e\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003e\u003csup\u003e\u003cb\u003e(xx)\u003c/b\u003e\u003c/sup\u003e \u003cem\u003eFisher's exact test was used. Descriptive statistics are presented as numbers (%).\u003c/em\u003e\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003e\u003csup\u003e\u003cb\u003e(xxx)\u003c/b\u003e\u003c/sup\u003e\u003cem\u003eMann\u0026ndash;Whitney U test was used. Descriptive statistics are presented as median [interquartile range].\u003c/em\u003e\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003e\u003cem\u003eP-values indicated in bold were statistically significant (p\u0026thinsp;\u0026lt;\u0026thinsp;0.05).\u003c/em\u003e\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003eBMI, body mass index; CSF, cerebrospinal fluid\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe relation between SVT presence and mean CSF opening pressure (cm/H\u003csub\u003e2\u003c/sub\u003eO) in the subjects included in the study was statistically significant \u003cb\u003e(p\u0026thinsp;=\u0026thinsp;0.011).\u003c/b\u003e The CSF opening pressure was higher in cases with SVT (47 cm/H\u003csub\u003e2\u003c/sub\u003eO) than cases without SVT (31 cm/H\u003csub\u003e2\u003c/sub\u003eO). Further, although the CSF mean opening pressure was higher in secondary PCS cases than in primary PCS cases, the difference between two groups was not statistically significant (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eMeasurement results of RNFL thicknesses according to CSF opening pressures before and after treatment\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"9\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c8\" colnum=\"8\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c9\" colnum=\"9\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c2\" namest=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c4\" namest=\"c3\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c6\" namest=\"c5\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colspan=\"2\" nameend=\"c8\" namest=\"c7\"\u003e \u003cp\u003ePearson's r\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c9\"\u003e \u003cp\u003ep\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eCSF opening pressure\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c5\" namest=\"c4\"\u003e \u003cp\u003e\u003cb\u003eNasal RNFL (pretreatment)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c7\" namest=\"c6\"\u003e \u003cp\u003e0.282\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c9\" namest=\"c8\"\u003e \u003cp\u003e\u003cb\u003e0.025\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eCSF opening pressure\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c5\" namest=\"c4\"\u003e \u003cp\u003e\u003cb\u003eNasal RNFL (posttreatment)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c7\" namest=\"c6\"\u003e \u003cp\u003e-0.101\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c9\" namest=\"c8\"\u003e \u003cp\u003e0.480\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eCSF opening pressure\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c5\" namest=\"c4\"\u003e \u003cp\u003e\u003cb\u003eTemporal RNFL (pretreatment)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c7\" namest=\"c6\"\u003e \u003cp\u003e0.261\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c9\" namest=\"c8\"\u003e \u003cp\u003e\u003cb\u003e0.039\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eCSF opening pressure\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c5\" namest=\"c4\"\u003e \u003cp\u003e\u003cb\u003eTemporal RNFL (posttreatment)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c7\" namest=\"c6\"\u003e \u003cp\u003e-0.173\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colspan=\"2\" nameend=\"c9\" namest=\"c8\"\u003e \u003cp\u003e0.225\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"9\"\u003e\u003cem\u003ePearson\u0026rsquo;s correlation coefficient was used.\u003c/em\u003e\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"9\"\u003eCSF, cerebrospinal fluid; RNFL, retinal nerve fiber layer\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eThere was a statistically significant, linear, unidirectional, and weak correlation between CSF opening pressure and the number of unloading LPs. In other words, as the CSF opening pressure increased, the need for repetitive unloading LPs also increased.\u003c/p\u003e \u003cp\u003eAs we know up to 15% of weight loss associated with PCS remission [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e], first of all obese patients are referred to a hospital-based weight management programme. As the first line medical therapy, acetazolamide dose was 10\u0026ndash;20 mg/kg/day. Treatment efficiency was evaluated by repetitive (every 3 months) OCT and visual field examinations. We administered topiramate (1\u0026ndash;3 mg/kg/day) as a second line medical therapy to patients who had acetazolamide side effect (intolerable metabolic acidosis, 4 male, 10 female patients) or had resistance to acetazolamide monotherapy.\u003c/p\u003e \u003cp\u003eMonotherapy resistance was observed in patients (18 male, 19 female patients) with high body mass index (BMI). A statistically significant relation was found between high BMI and the requirement for additional medication (topiramate) owing to drug resistance developed against the first-line acetazolamide therapy (It was not significant in the group who ceased acetozalamide treatment due to acidosis side effect (4 male, 10 female) and switched to topiramate treatment). When other comparisons were examined, no statistically significant differences were noted (Table\u0026nbsp;\u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). During the treatment, mean headache relief time was 30 days, mean vision defect relief time was 60 days. On follow up, mean drug withdrawal time was 6 months and mean follow up time was 1.8 years.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab3\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eComparison of RNFL thicknesses before and after treatment\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"4\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePretreatment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003ePosttreatment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003ep\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eNasal RNFL\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e521.59\u0026thinsp;\u0026plusmn;\u0026thinsp;157.97\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e425.88\u0026thinsp;\u0026plusmn;\u0026thinsp;107.02\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cb\u003e\u0026lt;\u0026thinsp;0.001\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eTemporal RNFL\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e738.41\u0026thinsp;\u0026plusmn;\u0026thinsp;165.27\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e625.57\u0026thinsp;\u0026plusmn;\u0026thinsp;118.33\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cb\u003e\u0026lt;\u0026thinsp;0.001\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"4\"\u003e\u003cem\u003eDependent samples t test was used.\u003c/em\u003e\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"4\"\u003eRNFL, retinal nerve fiber layer\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eObjective and quantitative diagnostic and follow-up criteria are needed because patient compliance is not always optimal in the evaluation of visual acuity and visual field in the pediatric population, possible refractive errors may affect visual acuity, and the pediatric population gets bored and easily distracted during the examination. Although disc appearance gives an idea about the differential diagnosis of papilledema in fundoscopy, OCT is an objective, rapid, quantitative, and reproducible imaging method in the differential diagnosis of pseudopapilledema causes such as small optic disc appearance or optic disc drusen and in monitoring treatment response. The results of our study further prove OCT as a useful method (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eWhen the CSF opening pressure measured by LP performed for diagnosis was compared with the nasal and temporal RNFL values obtained via OCT, a statistically significant increase was found in RNFL values as the CSF opening pressure increased. However, the difference in posttreatment values was not significant (Table\u0026nbsp;4).\u003c/p\u003e \u003cp\u003eAs we compared the pre- and posttreatment OCT values, the difference between the mean nasal and temporal RNFL values before and after the treatment was statistically significant (Table\u0026nbsp;5).\u003c/p\u003e \u003cp\u003eThe mean pre- and posttreatment nasal and temporal RNFL values were evaluated according to the etiology of the cases (primary and secondary cases). Pretreatment RNFL thickness was relatively greater in secondary cases compared with that in primary cases, but the difference between the mean values was not statistically significant.\u003c/p\u003e \u003cp\u003eWhen the pre- and posttreatment BMI and nasal and temporal RNFL values were compared, no statistically significant differences were noted (p\u0026thinsp;\u0026gt;\u0026thinsp;0.05 for each). In fact, RNFL values were lower in obese patients.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eWe evaluated the demographical data of PCS and discussed the results of OCT which is a new and non-invasive method for diagnosis and follow up. Patients those with high BMI developed resistence to acetazolamide monotherapy. Thus additional topiramate therapy was required for adequate symptom control. We concluded that the relation between OCT results and the CSF pressure were statistically significant.\u003c/p\u003e \u003cp\u003eOzkale et al. found that the mean age of the patients was 11.15\u0026thinsp;\u0026plusmn;\u0026thinsp;3.32 years and 40% of them were in the prepubertal period[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. In our study, the mean age at presentation was 10.99\u0026thinsp;\u0026plusmn;\u0026thinsp;3.28 years; further, 36.1% of the patients were in the prepubertal period and 63.9% were in the pubertal period, which was consistent with the existing literature[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. In the present study, obesity was not observed in the prepubertal period, and all obese patients were in the pubertal period. This was attributed to an increasing tendency to develop obesity with age[\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e, \u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. Consistent with the literature, the rate of obesity was lower in the prepubertal period than in the pubertal period in our study.\u003c/p\u003e \u003cp\u003eWhen the CSF opening pressures of the primary and secondary cases were compared, the mean CSF opening pressure in those with primary PCS was 30 cm/H\u003csub\u003e2\u003c/sub\u003eO, whereas it was 36 cm/H\u003csub\u003e2\u003c/sub\u003eO in those with secondary PCS; however, the difference between the two was not statistically significant (p\u0026thinsp;=\u0026thinsp;0.094) as similar to the results of Shawn et al.[\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Although no statistical significance was obtained, secondary PCS cases showed a tendency of higher CSF opening pressures than primary cases. In the present study, there was no statistically significant relationship between BMI values and OCT measurements, but a statistically significant relationship was noted between CSF opening pressures and OCT measurements. Pollak et al. found a significant relationship between CSF opening pressure and the degree of papilledema, which was consistent with our findings[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eVaried results have been reported in studies evaluating the response to medical treatment. While it was reported that 76.6% of the patients responded to acetazolamide treatment in one study, on the other hand the response to acetazolamide treatment was 37.8% in another study[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. In our study, the rate of patients who responded to acetazolamide treatment was 62%.\u003c/p\u003e \u003cp\u003eAccording to the results of the present study, a significant decrease was observed in both nasal and temporal RNFL thickness after papilledema treatment compared with the pretreatment values. However, although RNFL thickness was correlated with CSF opening pressure before treatment, the absence of this correlation after treatment may be attributed to the fact that the decrease in RNFL thickness takes longer than the improvement of CSF opening pressure, or to the increase in glial tissue formed after the improvement of papilledema. Therefore, the results of the present study are particularly important as they demonstrate that OCT is a useful method in the diagnosis and treatment follow up of PCS, but RNFL thickness may not completely return to normal in OCT during complete recovery of the papilledema. Further, OCT should be used together with the examination of clinical disc appearance in the follow-up period.\u003c/p\u003e \u003cp\u003e \u003cb\u003eIn conclusion\u003c/b\u003e, PCS is a disease associated with increased CSF pressure, and its etiology is still not fully elucidated. The use of OCT has increased in the pediatric population in recent years, and it is an effective and reliable method in the diagnosis and follow-up of PCS and the evaluation of treatment efficacy.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eThe authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.\u003c/p\u003e\n\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eMaterial preparation, data collection and analysis: A. C. F. Conceptualization: A. B. C. H.Methodology: A.B.C.H.Formal analysis and investigation: A.B.C.Writing - original draft preparation: A. C. F.Writing - review and editing:A. B. C. H. Supervision: B. C. H.Review: All authors reviewed the manuscript.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eStandridge SM. Idiopathic intracranial hypertension in children: A review and algorithm. Pediatr Neurol 2010. https://doi.org/10.1016/j.pediatrneurol.2010.08.001.\u003c/li\u003e\n\u003cli\u003eFriedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013. https://doi.org/10.1212/WNL.0b013e3182a55f17.\u003c/li\u003e\n\u003cli\u003eGilbert AL, Heidary G. Update on the evaluation of pediatric idiopathic intracranial hypertension. Curr Opin Ophthalmol 2016. https://doi.org/10.1097/ICU.0000000000000317.\u003c/li\u003e\n\u003cli\u003eLee YA, Tomsak RL, Sadikovic Z, Bahl R, Sivaswamy L. Use of Ocular Coherence Tomography in Children With Idiopathic Intracranial Hypertension\u0026mdash;A Single-Center Experience. Pediatr Neurol 2016. https://doi.org/10.1016/j.pediatrneurol.2015.10.022.\u003c/li\u003e\n\u003cli\u003eMartinez MR, Ophir A. Optical coherence tomography as an adjunctive tool for diagnosing papilledema in young patients. J Pediatr Ophthalmol Strabismus 2011. https://doi.org/10.3928/01913913-20100719-05.\u003c/li\u003e\n\u003cli\u003eGonz\u0026aacute;lez-L\u0026oacute;pez JJ, Rebolleda G, Leal M, Oblanca N, Mu\u0026ntilde;oz-Negrete FJ, Costa-Frossard L, et al. Comparative diagnostic accuracy of ganglion cell-inner plexiform and retinal nerve fiber layer thickness measures by Cirrus and spectralis optical coherence tomography in relapsing-remitting multiple sclerosis. Biomed Res Int 2014;2014. https://doi.org/10.1155/2014/128517.\u003c/li\u003e\n\u003cli\u003ePhillips PH, Sheldon CA. Pediatric Pseudotumor Cerebri Syndrome. J Neuroophthalmol 2017. https://doi.org/10.1097/WNO.0000000000000548.\u003c/li\u003e\n\u003cli\u003eSinclair AJ, Burdon MA, Nightingale PG, Ball AK, Good P, Matthews TD, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: Prospective cohort study. BMJ 2010;341:138. https://doi.org/10.1136/bmj.c2701.\u003c/li\u003e\n\u003cli\u003e\u0026Ouml;zkale Y, Erol İ, \u0026Ccedil;oban Karataş M, \u0026Ouml;zkale M, Saygı S, Alkan \u0026Ouml;, et al. Pseudotumor Cerebri; Single Center Experience. Turkish J Pediatr Dis 2018:193\u0026ndash;202. https://doi.org/10.12956/tjpd.2018.364.\u003c/li\u003e\n\u003cli\u003eSuzuki H, Aoki K, Chiba K, Sato Y, Shiozawa Y, Shiraishi Y, et al. Mutational landscape and clonal architecture in grade II and III gliomas. Nat Genet 2015. https://doi.org/10.1038/ng.3273.\u003c/li\u003e\n\u003cli\u003eKesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 2002. https://doi.org/10.1177/08830738020170101401.\u003c/li\u003e\n\u003cli\u003eMasri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH. Intracranial hypertension in children: Etiologies, clinical features, and outcome. J Child Neurol 2015. https://doi.org/10.1177/0883073815574332.\u003c/li\u003e\n\u003cli\u003eAylward SC, Waslo CS, Au JN, Tanne E. Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry. Pediatr Neurol 2016;61:76\u0026ndash;82. https://doi.org/10.1016/j.pediatrneurol.2016.04.007.\u003c/li\u003e\n\u003cli\u003ePollak L, Zohar E, Glovinsky Y, Huna-Baron R. Reevaluation of presentation and course of idiopathic intracranial hypertension - a large cohort comprehensive study. Acta Neurol Scand 2013;127:406\u0026ndash;12. https://doi.org/10.1111/ane.12060.\u003c/li\u003e\n\u003cli\u003ePer H, Canpolat M, G\u0026uuml;m\u0026uuml;ş H, Poyrazoĝlu HG, Yikilmaz A, Karak\u0026uuml;\u0026ccedil;\u0026uuml;k S, et al. Clinical spectrum of the pseudotumor cerebri in children: Etiological, clinical features, treatment and prognosis. Brain Dev 2013. https://doi.org/10.1016/j.braindev.2012.08.008.\u003c/li\u003e\n\u003cli\u003eTovia E, Reif S, Oren A, Mitelpunkt A, Fattal-Valevski A. Treatment response in pediatric patients with pseudotumor cerebri syndrome. J Neuro-Ophthalmology 2017. https://doi.org/10.1097/WNO.0000000000000516.\u003c/li\u003e\n\u003c/ol\u003e"},{"header":"Table 4 and 5","content":"\u003cp\u003eTable 4 and 5 are not available with this version\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Pediatrics, pseudotumor cerebri, optical coherence tomography","lastPublishedDoi":"10.21203/rs.3.rs-3947805/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3947805/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eAim\u0026nbsp; The aim of this study is to evaluate the demographic data, treatment efficacy, optical coherence tomography (OCT) results used for the diagnosis and follow-up of patients with pseudotumor cerebri syndrome (PCS) followed up in our pediatric neurology clinic between 2014 and 2019.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eMethods: We included children aged \u0026lt;18 years with or without bilateral papilledema detected on initial examination and cerebrospinal fluid (CSF) opening pressure of \u0026gt;26 cm/H2O in lumbar puncture. Patient data were obtained retrospectively from hospital records.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eResults: Retinal nerve fiber layer (RNFL) thickness increased significantly in OCT as CSF pressure increased. Further, RNFL thickness measured before and after treatment differed significantly. When the pretreatment body mass index and RNFL thickness of primary and secondary PCS cases were compared, it was found that RNFL thickness of obese patients was lower than that of non-obese patients. \u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eIn conclusion, we suggest that OCT, which is an easy-to-apply and reliable test that has recently become widely used in pediatric PCS, should be used in the diagnosis of PCS, evaluation of treatment efficacy, and follow-up.\u003c/p\u003e","manuscriptTitle":"Childhood pseudotumor cerebri syndrome: demographics, clinical features and optical coherence tomography utilization as a new method for diagnosis, treatment efficiency and follow up","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-02-14 20:07:57","doi":"10.21203/rs.3.rs-3947805/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"d7518f96-10f8-45d8-b494-3a0abf69355a","owner":[],"postedDate":"February 14th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-03-24T11:29:49+00:00","versionOfRecord":[],"versionCreatedAt":"2024-02-14 20:07:57","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-3947805","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3947805","identity":"rs-3947805","version":["v1"]},"buildId":"rHA-KDH7Qsr4HCuvH75dn","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}