今日の臨床サポート

視神経乳頭腫脹

著者: 柏井聡 愛知淑徳大学 健康医療科学部医療貢献学科視覚科学専攻

監修: 沖波聡 倉敷中央病院眼科

著者校正/監修レビュー済:2022/03/30
参考ガイドライン:
  1. 日本神経学会:多発性硬化症・視神経脊髄炎診療ガイドライン2017
  1. 日本眼科学会:抗アクアポリン 4 抗体陽性視神経炎診療ガイドライン2014
  1. 日本神経治療学会:標準的神経治療:視神経脊髄炎(NMO)2013
患者向け説明資料

概要・推奨   

  1. うっ血乳頭が疑われる場合、脳の画像検査が正常でも、必ず、脳脊髄圧を測定する(推奨度1)
  1. 前部視神経炎では、初診時に眼窩部(視神経から視交叉)の脂肪抑制造影MRIおよび頭部MRIの緊急検査を行い中枢神経系グリア自己抗体介在疾患および多発性硬化症について評価する(推奨度1)
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、 著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
柏井聡 : 未申告[2022年]
監修:沖波聡 : 特に申告事項無し[2022年]

改訂のポイント:
  1. 光干渉断層法(OCT)および光干渉断層血管撮影法(OCTA)の長足な進歩で非侵襲的に視神経乳頭の精緻な3次元形状解析、容積測定が可能となり、「視神経乳頭腫脹の3次元眼底画像解析」について改訂を行った。
  1. アクアポリン4、ミエリンオリゴデンドロサイト糖蛋白の自己抗体が同定され中枢神経系グリア自己抗体介在疾患の病態解析が進み、それに伴う視神経炎の治療指針の改訂を行った。

病態・疫学・診察

疫学情報・病態・注意事項  
  1. 検眼鏡的に視神経乳頭が腫脹している状態を乳頭腫脹optic disc swellingという。網膜神経節細胞の軸索である視神経は、その神経線維の中に順行性の速い軸索流と遅い軸索流、そして、逆行性の3種類が流れている。眼球内から出て有髄化した視神経は、それを取り巻くクモ膜下腔圧(脳圧)と眼球内圧の圧差を越えて行き来している。虚血やATPの枯渇は速い軸索流を止め、脳圧が亢進しても、低眼圧となっても、遅い軸索流が止まり、原因の如何にかかわらず、軸索流の渋滞は篩状板の前で起こり、乳頭は盛り上がる。乳頭が隆起するのは、せき止められた軸索流によって増大した軸索の容積増大に原因があって間質の浮腫性水分ではない。したがって、用語上「乳頭浮腫 optic disc “edema”」は不適切で、日本眼科学会用語集第6版では、乳頭浮腫は旧用語とし、乳頭腫脹を標準主要用語として推奨している。乳頭隆起(disc elevation)、混濁を伴い隆起した乳頭腫脹(disc swelling)は、検眼鏡的な他覚的所見を記述しているだけで、病因に基づく診断名ではない。注意が必要な用語にうっ血乳頭(papilledema)がある。うっ血乳頭は頭蓋内圧亢進に基づく視神経乳頭の腫脹を表す診断名で、脳圧亢進の裏付けがない限り、不用意に用いない。
  1. 視神経乳頭が隆起している場合、先天性に構造上隆起している乳頭隆起と、後天性に視神経乳頭が腫脹して隆起する乳頭腫脹に分かれる。
  1. 乳頭腫脹は、さらに、頭蓋内圧亢進に基づくうっ血乳頭、視神経の圧迫性病変による圧迫性視神経症、および、乳頭の局所的病因による乳頭腫脹の3つに分類できる。
問診・診察のポイント  
 
  1. 視神経乳頭の異常は脳の異常を反映することがあるため、患者の全身的予後と直接関係する。鑑別すべき疾患の順位は、年齢によって変わり、成人と乳幼児、小児では対応がまったく異なる。

これより先の閲覧には個人契約のトライアルまたはお申込みが必要です。

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文献 

A Miller, M Green, D Robinson
Simple rule for calculating normal erythrocyte sedimentation rate.
Br Med J (Clin Res Ed). 1983 Jan 22;286(6361):266. doi: 10.1136/bmj.286.6361.266.
Abstract/Text
PMID 6402065
Ramon L Font, Venkatesh C Prabhakaran
Histological parameters helpful in recognising steroid-treated temporal arteritis: an analysis of 35 cases.
Br J Ophthalmol. 2007 Feb;91(2):204-9. doi: 10.1136/bjo.2006.101725. Epub 2006 Sep 20.
Abstract/Text AIM: To establish the histological and immunohistochemical parameters that are helpful in recognising temporal arteritis in patients who have been treated with steroids before biopsy, and to analyse the clinical features and correlate them with the histological findings.
METHODS: A retrospective review of charts of 35 patients treated with steroids before obtaining temporal artery biopsy specimens, spanning a 11-year period from 1995 to 2005. The study was conducted at the Ophthalmic Pathology Laboratory, Cullen Eye Institute, Houston, Texas, USA. The clinical features were evaluated and correlated with the histopathological findings. Each case was evaluated with respect to age, sex, race, clinical findings, erythrocyte sedimentation rate, corticosteroid dosage (oral versus intravenous) and the duration of treatment. The time interval between obtaining the biopsy specimen and the onset of steroid treatment was carefully recorded for each patient. In selected cases, histiocytic markers (CD-68 and HAM-56) were used to identify the presence of epithelioid histiocytes, which characterises a granulomatous inflammation. Other immunohistochemical studies (CD3, CD20, CD4, CD8, CD45RO, CD45RA and S-100 protein) were performed in selected cases to characterise the inflammatory cells.
RESULTS: The three most reliable histopathological parameters of corticosteroid-treated temporal arteritis are the following: (1) complete or incomplete mantle of lymphocytes and epithelioid histiocytes located between the outer muscular layer and the adventitia; (2) large circumferential defects in the elastic lamina (best seen with the Movat's pentachrome); and (3) absent or few small multinucleated giant cells. In some cases the main artery appears normal, whereas the primary branches show evidence of a healing arteritis. The histological findings vary according to the duration of treatment before obtaining the biopsy specimen.
CONCLUSION: Striking histological differences can be recognised objectively between patients with active (untreated) giant cell arteritis and patients who have been treated with corticosteroids. The earliest histopathological changes were detected by the end of the first week after steroid treatment (usually after day 4 to the end of the first week). The histological findings were more difficult to recognise at 2-3 months after steroid treatment. Ophthalmic and general pathologists should be able to recognise this entity on the basis of the histological findings including the special stains and results of immunohistochemical studies (CD-68 and HAM-56).

PMID 16987903
Danielle Bury, Jacob Joseph, Timothy P Dawson
Does preoperative steroid treatment affect the histology in giant cell (cranial) arteritis?
J Clin Pathol. 2012 Dec;65(12):1138-40. doi: 10.1136/jclinpath-2012-200870. Epub 2012 Jul 26.
Abstract/Text INTRODUCTION: Giant cell arteritis (GCA) has been successfully treated with steroids for many years and temporal artery biopsy (TAB) is regarded as the gold standard diagnostic test. The primary aim of this study was to determine whether steroid pretreatment abrogates histological features of GCA reducing diagnostic return, as suspected on the basis of anecdotal evidence. This impacts upon patients suspected of having GCA and the need for prompt treatment balanced with the diagnostic need for TAB.
METHODS: A 6-year single-centre retrospective study of biopsies (2005-2011) was performed with interrogation of the medical notes for information regarding steroid use. The null hypothesis considered there was no association between steroid use and biopsy outcome.
RESULTS: No significant difference was found between steroid use and biopsy outcome, with biopsies still producing positive results after weeks of steroid treatment.
CONCLUSIONS: TAB is still useful in the diagnosis of GCA, even after commencing steroid treatment.

PMID 22844066
N Ray-Chaudhuri, D Ah Kiné, S O Tijani, D V Parums, N Cartlidge, N P Strong, M R Dayan
Effect of prior steroid treatment on temporal artery biopsy findings in giant cell arteritis.
Br J Ophthalmol. 2002 May;86(5):530-2.
Abstract/Text AIM: To examine the effect of up to 6 weeks of corticosteroid treatment on the positive temporal artery biopsy rate in giant cell arteritis (GCA).
METHODS: Prospective comparative clinical study of 11 patients meeting the American College of Rheumatology criteria for diagnosis of GCA. Patients underwent temporal artery biopsy within 1 week, at 2-3 weeks, or after 4 weeks of corticosteroid treatment.
RESULTS: Overall, nine of 11 (82%) patients had positive temporal artery biopsies. Six of seven (86%) biopsies performed after 4 or more weeks of steroid treatment were positive.
CONCLUSION: Temporal artery biopsy is useful several weeks after institution of steroids.

PMID 11973248
Hitoshi Ishikawa, Takeshi Kezuka, Keigo Shikishima, Akiko Yamagami, Miki Hiraoka, Hideki Chuman, Makoto Nakamura, Keika Hoshi, Toshiaki Goseki, Kimiyo Mashimo, Osamu Mimura, Takeshi Yoshitomi, Keiko Tanaka, Working Group on Diagnostic Criteria for Refractory Optic Neuritis Based on Neuroimmunological Perspective
Epidemiologic and Clinical Characteristics of Optic Neuritis in Japan.
Ophthalmology. 2019 Oct;126(10):1385-1398. doi: 10.1016/j.ophtha.2019.04.042. Epub 2019 May 6.
Abstract/Text PURPOSE: To elucidate the clinical and epidemiologic characteristics of optic neuritis in Japan.
DESIGN: Multicenter cross-sectional, observational cohort study.
PARTICIPANTS: A total of 531 cases of unilateral or bilateral noninfectious optic neuritis identified in 33 institutions nationwide in Japan.
METHODS: Serum samples from patients with optic neuritis were tested for anti-aquaporin-4 antibodies (AQP4-Abs) and anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) using a cell-based assay and were correlated with the clinical findings.
MAIN OUTCOME MEASURES: Antibody positivity, clinical and radiologic characteristics, and visual outcome.
RESULTS: Among 531 cases of optic neuritis, 12% were AQP4-Ab positive, 10% were MOG-Ab positive, 77% were negative for both antibodies (double-negative), and 1 case was positive for both antibodies. Pretreatment visual acuity (VA) worsened to more than a median 1.0 logarithm of the minimum angle of resolution (logMAR) in all groups. After steroid pulse therapy (combined with plasmapheresis in 32% of patients in AQP4-Ab-positive group), median VA improved to 0.4 logMAR in the AQP4-Ab-positive group, 0 logMAR in the MOG-Ab-positive group, and 0.1 logMAR in the double-negative group. The AQP4-Ab-positive group showed a high proportion of females, exhibited diverse visual field abnormalities, and demonstrated concurrent spinal cord lesions on magnetic resonance imaging (MRI) in 22% of the patients. In the MOG-Ab-positive group, although posttreatment visual outcome was good, the rates of optic disc swelling and pain with eye movement were significantly higher than those in the AQP4-Ab-positive and double-negative groups. However, most cases showed isolated optic neuritis lesions on MRI. In the double-negative group, 4% of the patients had multiple sclerosis. Multivariate logistic regression analysis of all participants identified age and presence of antibodies (MOG-Ab and AQP4-Ab) as significant factors affecting visual outcome.
CONCLUSIONS: The present large-scale cohort study revealed the clinicoepidemiologic features of noninfectious optic neuritis in Japan. Anti-aquaporin-4 antibody-positive optic neuritis has poor visual outcome. In contrast, MOG-Ab positive cases manifested severe clinical findings of optic neuritis before treatment, but few showed concurrent lesions in sites other than the optic nerve and generally showed good treatment response with favorable visual outcome. These findings indicate that autoantibody measurement is useful for prompt diagnosis and proper management of optic neuritis that tends to become refractory.

Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
PMID 31196727
Tetsuya Akaishi, Takayuki Takeshita, Noriko Himori, Toshiyuki Takahashi, Tatsuro Misu, Ryo Ogawa, Kimihiko Kaneko, Juichi Fujimori, Michiaki Abe, Tadashi Ishii, Kazuo Fujihara, Masashi Aoki, Toru Nakazawa, Ichiro Nakashima
Rapid Administration of High-Dose Intravenous Methylprednisolone Improves Visual Outcomes After Optic Neuritis in Patients With AQP4-IgG-Positive NMOSD.
Front Neurol. 2020;11:932. doi: 10.3389/fneur.2020.00932. Epub 2020 Sep 2.
Abstract/Text Objective: The purpose of this study was to elucidate the rapid impact of high-dose intravenous methylprednisolone pulse therapy (1,000 mg/day for 3 days) on the eventual visual prognosis in patients with serum anti-aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorders (NMOSDs) who had an attack of optic neuritis (ON). Methods: Data from 32 consecutive NMOSD patients (1 male and 31 female) with at least one ON attack, involving a total of 36 ON-involved eyes, were evaluated. The following variables at ON onset were evaluated: sex, age at the first ON episode, visual acuity at nadir, visual acuity after 1 year, duration from ON onset to treatment for an acute ON attack, cycles of high-dose intravenous methylprednisolone pulse therapy for the ON attack, and cycles of plasmapheresis for the ON attack. Among the 36 ON-involved eyes, 27 eyes were studied using orbital MRI with a short-T1 inversion recovery sequence and gadolinium-enhanced fat-suppressed T1 imaging before starting treatment in the acute phase. Results: In univariate analyses, a shorter duration from ON onset to the initiation of high-dose intravenous methylprednisolone pulse therapy favorably affected the eventual visual prognosis 1 year later (Spearman's rho = 0.50, p = 0.0018). The lesion length on orbital MRI was also correlated with the eventual visual prognosis (rho = 0.68, p < 0.0001). Meanwhile, the days to steroid pulse therapy and lesion length on orbital MRI did not show a significant correlation. These findings suggest that the rapidness of steroid pulse therapy administration affects the eventual visual prognosis independent of the severity of ON. In multivariate analysis, a shorter time from ON onset to the start of acute treatment (p = 0.0004) and a younger age at onset (p = 0.0071) were significantly associated with better visual outcomes. Conclusions: Rapid initiation of high-dose intravenous methylprednisolone pulse therapy is essential to preserve the eventual visual acuity in patients with serum AQP4-IgG-positive NMOSD. Once clinicians suspect acute ON with serum AQP4-IgG, swift administration of steroid pulse therapy before confirming the positivity of serum AQP4-IgG would be beneficial for preserving visual function.

Copyright © 2020 Akaishi, Takeshita, Himori, Takahashi, Misu, Ogawa, Kaneko, Fujimori, Abe, Ishii, Fujihara, Aoki, Nakazawa and Nakashima.
PMID 33013632
Jean-Baptiste Ducloyer, Angelique Caignard, Ramzi Aidaoui, Yolaine Ollivier, Guillaume Plubeau, Sonia Santos-Moskalyk, Lindsay Porphyre, Caroline Le Jeune, Lionel Bihl, Samy Alamine, Romain Marignier, Romain Bourcier, Mathilde Ducloyer, Michel Weber, Guylène Le Meur, Sandrine Wiertlewski, Pierre Lebranchu
MOG-Ab prevalence in optic neuritis and clinical predictive factors for diagnosis.
Br J Ophthalmol. 2020 Jun;104(6):842-845. doi: 10.1136/bjophthalmol-2019-314845. Epub 2019 Oct 3.
Abstract/Text OBJECTIVE: What is the proportion of antibodies to myelin oligodendrocyte glycoprotein (MOG-Ab) in optic neuritis (ON) in adults and what would be the ON presentation for which MOG-Ab should be tested?
METHODS: Multicentric prospective study conducted during 1 year on all patients diagnosed with acute ON in all ophthalmological units in hospitals in a region in western France.
RESULTS: Sixty-five patients were included. MOG-Ab prevalence was 14% (9/65) during an acute ON and 13% (7/55) after exclusion of patients already diagnosed with multiple sclerosis (MS) (8) or MOG+ON (2). Compared with MS and clinically isolated syndrome, MOG+ON had no female preponderance (67% of men in case of MOG+ON and 22% of men in case of MS and clinically isolated syndrome, p<0.05) were more often bilateral (44% vs 3%, p<0.005) and associated with optic disc swelling (ODS) (78% vs 14%, p<0.001). To predict MOG+ON, the positive predictive values (PPVs) of male sex, ODS and bilateral involvement were 29% (95% CI 9% to 48%), 41% (95% CI 18% to 65%) and 40% (95% CI 10% to 70%), respectively, while the negative predictive values (NPV) were 93% (95% CI 86% to 100%), 96% (95% CI 90% to 100%) and 91% (95% CI 83% to 99%), respectively. The combined factor 'ODS or bilateral or recurrent ON' was the best compromise between PPV (31% (95% CI 14% to 48%)) and NPV (100% (95% CI 100% to 100%)).
CONCLUSION: Among ON episodes, MOG-Ab were found in 14% of cases. MOG+ON occurred without female preponderance and was significantly associated with ODS and/or bilateral ON. Testing MOG-Ab only in patients presenting with ODS or bilateral or recurrent ON would limit MOG-Ab tests to fewer than half of all patients without the risk of missing any MOG+ON cases.

© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.
PMID 31582363
Valerie Purvin, Seema Sundaram, Aki Kawasaki
Neuroretinitis: review of the literature and new observations.
J Neuroophthalmol. 2011 Mar;31(1):58-68. doi: 10.1097/WNO.0b013e31820cf78a.
Abstract/Text Neuroretinitis (NR) is an inflammatory disorder characterized by optic disc edema and subsequent formation of a macular star figure. The underlying pathophysiology involves increased permeability of disc vasculature, but the etiology is not fully defined. In some cases, NR is probably due to an infectious process involving the disc; in others, a postviral or autoimmune mechanism is more likely. Cases can be divided into those in which a specific infectious agent has been identified, those considered idiopathic, and those with recurrent attacks. Some reports have not distinguished among these subgroups, and it is unclear if their clinical features vary. We reviewed the literature and our own patients looking particularly at features that might better distinguish these subtypes. Features common to all 3 groups included age, absence of pain, and fundus appearance. Preceding systemic symptoms were more common in patients with cat scratch disease (CSD) and uncommon in those with recurrence. The pattern and magnitude of visual field loss differed, more commonly confined to the central field in CSD cases and more severe in recurrent cases. Recovery of visual acuity and field was less substantial in recurrent cases even after the initial episode. MRI was usually normal in all 3 groups. Enhancement confined to the optic disc was found in all 3 groups, but enhancement of the retrobulbar optic nerve was seen only in recurrent cases. Findings that are strongly suggestive of CSD include very young age, preceding systemic symptoms, and poor visual acuity but with a small or absent relative afferent pupil defect (RAPD). In contrast, the following are suggestive of idiopathic NR with a high risk of recurrence: absence of systemic symptoms, visual field defect outside the central field, preserved visual acuity with a large RAPD, and poor recovery of vision. Decisions regarding evaluation and treatment should be made with these features in mind.

PMID 21317731
Sohan Singh Hayreh, M Bridget Zimmerman
Non-arteritic anterior ischemic optic neuropathy: role of systemic corticosteroid therapy.
Graefes Arch Clin Exp Ophthalmol. 2008 Jul;246(7):1029-46. doi: 10.1007/s00417-008-0805-8. Epub 2008 Apr 11.
Abstract/Text OBJECTIVE: To investigate systematically the role of systemic corticosteroid therapy in non-arteritic anterior ischemic optic neuropathy (NA-AION).
METHODS: The study consists of a cohort of 613 consecutive patients (696 eyes), first seen in our clinic from 1973 to 2000. Of this cohort, 312 patients (364 eyes) voluntarily opted for systemic steroid therapy, and 301 (332 eyes) for no treatment. At first visit, all patients in both groups had a detailed ophthalmic and medical history, and comprehensive ophthalmic evaluation. Visual evaluation was done by recording Snellen visual acuity, and visual fields with a Goldmann perimeter. The same ophthalmic evaluation was performed at each follow-up visit. Patients in the steroid-treated group were initially given 80 mg Prednisone daily for 2 weeks, and then tapered down to 70 mg for 5 days, 60 mg for 5 days, and then cutting down by 5 mg every 5 days. Visual outcome in the two groups was compared
RESULTS: Median follow-up was 3.8 years. At 6 months from onset of NA-AION, of the eyes with initial visual acuity 20/70 or worse and seen within 2 weeks of onset, there was visual acuity improvement in 69.8% (95% confidence interval (CI): 57.3%, 79.9%) in the treated group, compared to 40.5% (95% CI: 29.2%, 52.9%) in the untreated group (odds ratio of improvement: 3.39; 95% CI:1.62, 7.11; p = 0.001). Comparison of visual field defect at 6 months from onset of NA-AION, among those seen within 2 weeks of NA-AION onset with moderate to severe initial visual field defect, there was improvement in 40.1% (95% CI: 33.1%, 47.5%) of the treated group, and 24.5% (95% CI: 17.7%, 32.9%) of the untreated group (odds ratio: 2.06, 95% CI: 1.24, 3.40; p = 0.005). In both treated and untreated groups, the visual acuity and visual fields kept improving up to about 6 months from onset of NA-AION, and very little thereafter.
CONCLUSION: This study suggested that NA-AION eyes treated during the acute phase with systemic corticosteroids resulted in a significantly higher probability of improvement in visual acuity (p = 0.001) and visual field (p = 0.005) than in the untreated group. Both visual acuity and visual fields improved up to 6 months after onset of NA-AION.

PMID 18404273
Gema Rebolleda, Marta Pérez-López, Pilar Casas-LLera, Inés Contreras, Francisco José Muñoz-Negrete
Visual and anatomical outcomes of non-arteritic anterior ischemic optic neuropathy with high-dose systemic corticosteroids.
Graefes Arch Clin Exp Ophthalmol. 2013 Jan;251(1):255-60. doi: 10.1007/s00417-012-1995-7. Epub 2012 Mar 24.
Abstract/Text BACKGROUND: To evaluate the visual and anatomic outcomes after systemic steroid treatment in non-arteritic anterior ischemic optic neuropathy (NAION).
METHODS: Ten eyes from ten patients diagnosed with NAION and treated during the acute phase with 80 mg daily, tapering-down dose of corticosteroids were compared with a non-contemporary cohort of 27 patients that received no treatment. The visual outcomes of treated and untreated group were compared. Patients underwent complete ophthalmic examination including determination of Snellen visual acuity (VA), visual fields (VFs) (standard automated perimetry, Swedish Interactive Testing Algorithm 24-2 strategy), and optical coherence tomography (OCT) scanning of the optic nerve head at diagnosis, 6-8 weeks and 6 months after presentation.
RESULTS: No statistical differences were found between steroid-treated and untreated NAION for the median change in VA (Mann-Whitney P = 0.28), median change in VF mean deviation (MD) and median change in VF pattern standard deviation (PSD) (Mann-Whitney P = 0.213 and P = 0.07 respectively). Statistical analysis showed no differences when comparing average RNFL loss (P = 0.871) and RNFL loss for superior, nasal, inferior and temporal optic disc quadrants between both groups. Complications occurred in three of the ten treated patients (30%); in one of them, steroid therapy had to be discontinued. Another two patients developed a NAION in their fellow eye after 2 and 3 months while on low-dose prednisone. No complications developed in the control group. The study was interrupted early due to a significantly higher rate of complications observed in the treated group (P = 0.002)
CONCLUSION: High-dose systemic steroid treatment did not show any beneficial effect in visual and anatomic outcomes when given during the acute phase of NAION. Furthermore, it caused serious complications in a third of the patients treated.

PMID 22441810
Sohan Singh Hayreh
Management of ischemic optic neuropathies.
Indian J Ophthalmol. 2011 Mar-Apr;59(2):123-36. doi: 10.4103/0301-4738.77024.
Abstract/Text Ischemic optic neuropathies (IONs) consist primarily of two types: anterior ischemic optic neuropathy (AION) and posterior ischemic optic neuropathy (PION). AION comprises arteritic AION (A-AION: due to giant cell arteritis) and non-arteritic AION (NA-AION: due to other causes). PION consists of arteritic PION (A-PION: due to giant cell arteritis), non-arteritic PION (NA-PION: due to other causes), and surgical PION (a complication of several systemic surgical procedures). These five types of ION are distinct clinical entities etiologically, pathogenetically, clinically and from the management point of view. In the management of AION, the first crucial step with patients aged 50 and over is to identify immediately whether it is arteritic or not because A-AION is an ophthalmic emergency and requires urgent treatment with high-dose steroid therapy to prevent any further visual loss in one or both eyes. Patients with NA-AION, when treated with systemic corticosteroid therapy within first 2 weeks of onset, had significantly better visual outcome than untreated ones. Systemic risk factors, particularly nocturnal arterial hypotension, play major roles in the development of NA-AION; management of them is essential in its prevention and management. NA-PION patients, when treated with high-dose systemic steroid therapy during the very early stages of the disease, showed significant improvement in visual acuity and visual fields, compared to untreated eyes. A-PION, like A-AION, requires urgent treatment with high-dose steroid therapy to prevent any further visual loss in one or both eyes. There is no satisfactory treatment for surgical PION, except to take prophylactic measures to prevent its development.

PMID 21350282
Edward J Atkins
Nonarteritic anterior ischemic optic neuropathy.
Curr Treat Options Neurol. 2011 Feb;13(1):92-100. doi: 10.1007/s11940-010-0099-0.
Abstract/Text OPINION STATEMENT: Currently there is no generally accepted, well-proven treatment for nonarteritic anterior ischemic optic neuropathy (NAION). Most proposed treatments are empirical and include antithrombotics, vasodynamic agents, treatments aimed at reducing optic disc edema, and various neuroprotective strategies. Most potential treatments have been inadequately studied, prematurely embraced, or prematurely discarded. Evidence for antithrombotic agents is lacking, and small vessel arterial occlusion has never been demonstrated in NAION. Antiplatelet agents have not been studied in acute NAION, but they are often prescribed for acute treatment because of their proven role in stroke prevention. Because NAION is an ischemic disorder occurring more often after the age of 50 in patients with vascular risk factors, I recommend aggressive risk-factor management and antiplatelet therapy. The evidence that aspirin can help to prevent NAION in the fellow eye is divided. I recommend aspirin for secondary prevention, mostly for its proven role in stroke prevention. NAION occurs in patients with physiologically crowded optic nerves and small cup-to-disc ratios. Disc edema may contribute to a "compartment syndrome," which compresses the fine capillary blood supply of the optic nerve head, resulting in ischemia and axonal damage. There is some limited and debatable evidence that oral steroids may shorten the duration of disc edema and improve visual outcome in NAION. I discuss this evidence with patients who present acutely with NAION, and although I consider prescribing oral steroids on a case-by-case basis, I will not routinely recommend oral steroids until a properly randomized clinical trial is performed. Some neuroprotective strategies have been studied, but none have proven to be helpful. Although some (eg, brimonidine) are probably not harmful, I do not recommend these treatments. Early referral to low vision services may help to improve functional visual outcome.

PMID 21063919
Optic Neuritis Study Group
Visual function 15 years after optic neuritis: a final follow-up report from the Optic Neuritis Treatment Trial.
Ophthalmology. 2008 Jun;115(6):1079-1082.e5. doi: 10.1016/j.ophtha.2007.08.004. Epub 2007 Nov 5.
Abstract/Text OBJECTIVE: To assess visual function 15 years after acute unilateral optic neuritis.
DESIGN: Longitudinal follow-up of a randomized clinical trial.
PARTICIPANTS: Two hundred ninety-four patients who were randomized in the Optic Neuritis Treatment Trial between 1988 and 1991 and underwent examination in 2006.
TESTING: A neuro-ophthalmic examination included measurements of visual acuity, contrast sensitivity, and visual field. Quality of life was assessed with the National Eye Institute Visual Function Questionnaire and Neuro-ophthalmic Supplement.
MAIN OUTCOME MEASURES: Abnormal vision and health-related quality-of-life scores.
RESULTS: Seventy-two percent of the eyes affected with optic neuritis at study entry had visual acuity of > or = 20/20 and 66% of patients had > or = 20/20 acuity in both eyes. On average, visual function was slightly worse among patients with multiple sclerosis (MS) than among with those without MS. As expected, quality-of-life scores were lower when acuity was reduced and when neurologic disability from MS was present.
CONCLUSIONS: Long-term visual outcome is favorable for the majority of patients who experience optic neuritis even when MS is present.

PMID 17976727
D Pau, N Al Zubidi, S Yalamanchili, G T Plant, A G Lee
Optic neuritis.
Eye (Lond). 2011 Jul;25(7):833-42. doi: 10.1038/eye.2011.81. Epub 2011 Apr 29.
Abstract/Text AIMS: The aim of this study is to provide a clinical update on optic neuritis (ON), its association with multiple sclerosis (MS), and neuromyelitis optica (NMO).
METHODS: This study included a PubMed review of the literature written in the English language.
RESULTS: ON in adults is typically idiopathic or demyelinating, and is characterised by unilateral, subacute, painful loss of vision that is not associated with any systemic or other neurological symptoms. Demyelinating ON is associated with MS, and we review the key studies of ON including the ON treatment trial and several other MS treatment trials and NMO.
CONCLUSION: Acute demyelinating ON can occur in isolation or be associated with MS. Typical ON does not require additional evaluation other than cranial magnetic resonance imaging. NMO is likely a separate disorder from MS and the ON in NMO has a different treatment and prognosis.
METHODOLOGY: The authors conducted an English language search using Pubmed from the years 1964 to 2010 using the search terms 'ON', 'MS' and 'NMO'. The authors included original articles, review articles, and case reports, which revealed new aspects as far as epidemiology, histopathology, clinical manifestations, imaging, genetics, and treatment of ON. Titles were reviewed for topicality and full references were obtained. Letters to the editor, unpublished work, and abstracts were not included in this review.

PMID 21527960
Robin L Gal, Satyanarayana S Vedula, Roy Beck
Corticosteroids for treating optic neuritis.
Cochrane Database Syst Rev. 2012 Apr 18;4:CD001430. doi: 10.1002/14651858.CD001430.pub3. Epub 2012 Apr 18.
Abstract/Text BACKGROUND: Optic neuritis is an inflammatory disease of the optic nerve. It occurs more commonly in women than in men. Usually presenting with an abrupt loss of vision, recovery of vision is almost never complete. Closely linked in pathogenesis to multiple sclerosis, it may be the initial manifestation for this condition. In certain patients, no underlying cause can be found.
OBJECTIVES: To assess the effects of corticosteroids on visual recovery of patients with acute optic neuritis.
SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2012, Issue 1), MEDLINE (January 1950 to February 2012), EMBASE (January 1980 to February 2012), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to February 2012), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (www.clinicaltrials.gov) and the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). There were no date or language restrictions in the electronic searches for trials. The electronic databases were last searched on 21 February 2012. We also searched reference lists of identified trial reports to find additional trials.
SELECTION CRITERIA: We included randomized trials that evaluated corticosteroids, in any form, dose or route of administration, in people with acute optic neuritis.
DATA COLLECTION AND ANALYSIS: Two authors independently extracted the data on methodological quality and outcomes for analysis.
MAIN RESULTS: We included six randomized trials which included a total of 750 participants. Two trials evaluated low dose oral corticosteroids while one trial evaluated low dose intravenous corticosteroids across two treatment arms and two trials evaluated a higher dose of intravenous corticosteroids. One three-arm trial evaluated low-dose oral corticosteroids and high-dose intravenous corticosteroids against placebo. Trials evaluating oral corticosteroids compared varying doses of corticosteroids with placebo. Hence, we did not conduct a meta-analysis of such trials. In a meta-analysis of trials evaluating corticosteroids with total dose greater than 3000 mg administered intravenously, the relative risk of normal visual acuity with intravenous corticosteroids compared with placebo was 1.06 (95% confidence interval (CI) 0.89 to 1.27) at six months and 1.06 (95% CI 0.92 to 1.22) at one year. The risk ratio of normal contrast sensitivity for the same comparison was 1.10 (95% CI 0.92 to 1.32) at six months follow up. We did not conduct a meta-analysis for this outcome at one year follow up since there was substantial statistical heterogeneity. The risk ratio of normal visual field for this comparison was 1.08 (95% CI 0.96 to 1.22) at six months and 1.02 (95% CI 0.86 to 1.20) at one year. Quality of life was assessed and reported in one trial.
AUTHORS' CONCLUSIONS: There is no conclusive evidence of benefit in terms of recovery to normal visual acuity, visual field or contrast sensitivity with either intravenous or oral corticosteroids at the doses evaluated in trials included in this review.

PMID 22513900
Sidney M Gospe, John J Chen, M Tariq Bhatti
Neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein associated disorder-optic neuritis: a comprehensive review of diagnosis and treatment.
Eye (Lond). 2021 Mar;35(3):753-768. doi: 10.1038/s41433-020-01334-8. Epub 2020 Dec 15.
Abstract/Text Optic neuritis (ON) is the most common cause of acute optic neuropathy in patients younger than 50 years of age and is most frequently idiopathic or associated with multiple sclerosis. However, the discovery of aquaporin-4 immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein (MOG)-IgG as biomarkers for two separate central nervous system inflammatory demyelinating diseases has revealed that neuromyelitis optica spectrum disorder (NMSOD) and MOG-IgG-associated disease (MOGAD) are responsible for clinically distinct subsets of ON. NMOSD-ON and MOGAD-ON both demonstrate tendencies for bilateral optic nerve involvement and often exhibit a relapsing course with the potential for devastating long-term visual outcomes. Early and accurate diagnosis is therefore essential. This review will summarize the current understanding of the clinical spectra of NMOSD and MOGAD, the radiographic and serological findings which support their diagnoses, and the current evidence behind various acute and long-term therapeutic strategies for ON related to these conditions. A particular emphasis is placed on a number of recent multi-centre randomized placebo-controlled trials, which provide the first level I evidence for long-term treatment of NMOSD.

PMID 33323985
Marilou Caron-Cantin, Dean M Cestari, Elizabeth Fortin
Clinical and radiologic approach to 'typical' versus antibody-related optic neuritis.
Curr Opin Ophthalmol. 2019 Nov;30(6):412-417. doi: 10.1097/ICU.0000000000000614.
Abstract/Text PURPOSE OF REVIEW: Optic neuritis is an autoimmune optic neuropathy that has been associated with multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and more recently antimyelin oligodendrocyte glycoprotein (anti-MOG)-positive disorder. At initial presentation, it is often difficult to differentiate these entities given their significant overlap in clinical presentation and MRI findings. This review summarizes the distinguishing clinical and radiological features of MS, NMOSD, and anti-MOG disorders to help clinicians accurately diagnose and manage patients affected by these conditions.
RECENT FINDINGS: Antiaquaporin-4 (AQP4) and more recently anti-MOG antibodies are both associated with central nervous system demyelinating diseases that often initially present with optic neuritis. Serologic testing now allows for a new classification of these overlapping conditions that can help to differentiate 'typical' optic neuritis that is often associated with MS from 'atypical' optic neuritis associated with NMOSD and anti-MOG-positive disorder.
SUMMARY: Optic neuritis associated with MS, NMOSD, and anti-MOG-positive disease can have a similar clinical presentation. However, some clinical and radiologic findings can help clinicians to differentiate these entities so that they can be properly managed to optimize visual prognosis.

PMID 31503075
Sui Hsien Wong, Richard Patrick White
The clinical validity of the spontaneous retinal venous pulsation.
J Neuroophthalmol. 2013 Mar;33(1):17-20. doi: 10.1097/WNO.0b013e3182622207.
Abstract/Text BACKGROUND: The validity of the clinical dictum "the presence of spontaneous retinal venous pulsation (SVP) excludes raised intracranial pressure" has not been previously tested. We set out to determine the specificity and positive predictive value (PPV) of the presence of SVP to indicate normal intracranial pressure (ICP) in a routine clinical setting.
METHODS: We prospectively recruited patients undergoing lumbar puncture (LP), and 2 clinicians were blinded to the indications for LP and cerebrospinal fluid opening pressure (OP). Interobserver reliability was assessed.
RESULTS: There were 106 patients in our cohort with a median age of 44 years (range, 18-79 years) and median body mass index of 27.5 kg/m (range, 18-48 kg/m). SVP was present in 94 of 106 patients (88.7%). Thirteen of 106 (12.3%) patients had high OP (≥30 cmH2O), and SVP was present in 11 of 13 patients (86%) with high OP. The sensitivity (95% confidence interval) of the presence of SVP to exclude raised ICP was 0.89 (0.88-0.92), specificity of 0.15 (0.05-0.37), PPV of 0.88 (0.87-0.9), and negative predictive value of 0.17 (0.05-0.4). Interobserver agreement was moderate for SVP (kappa = 0.42).
CONCLUSIONS: Although the sensitivity and PPV of the presence of SVP to exclude raised ICP is high, it is not absolute. SVP can be seen in some patients with high ICP. Relying on the presence of SVP to exclude raised ICP may give a false sense of reassurance.

PMID 22801353
Abstract/Text A search for spontaneous retinal venous pulsations was carried out in 218 subjects. Spontaneous venous pulsations were present in 87.6% of 146 unselected subjects 20 to 90 years of age and absent in 100% of 33 patients with raised intracranial pressure without papilledema and ten patients with papilledema. Lumbar puncture in nine patients with raised intracranial pressure established the upper level at which spontaneous pulsations disappear as 190 mm H2O, and no pressure above 180 mm H2O was found in 29 patients with venous pulsations present prior to lumbar puncture. There was no correlation between the presence or absence of venous pulsations and blood pressure. Some normal subjects with absent pulsations showed definite pulsations on subsequent examinations. These findings confirm that the presence of spontaneous venous pulsations is a reliable indicator of an intracranial pressure below 180 to 190 mm H2O, while the absence of pulsations may be found with normal intracranial pressure and is therefore not a reliable guide to raised intracranial pressure.

PMID 619871
A S Jacks, N R Miller
Spontaneous retinal venous pulsation: aetiology and significance.
J Neurol Neurosurg Psychiatry. 2003 Jan;74(1):7-9.
Abstract/Text Spontaneous retinal venous pulsation is seen as a subtle variation in the calibre of the retinal vein(s) as they cross the optic disc. The physical principles behind the venous pulsations has been the point of much debate. Initial theories suggested that the pulsation occurred because of the rise in intraocular pressure in the eye with the pulse pressure. This article presents an argument that this is not the case. The pulsations are in fact caused by variation in the pressure gradient along the retinal vein as it traverses the lamina cribrosa. The pressure gradient varies because of the difference in the pulse pressure between the intraocular space and the cerebrospinal fluid. The importance of this is that as the intracranial pressure rises the intracranial pulse pressure rises to equal the intraocular pulse pressure and the spontaneous venous pulsations cease. Thus it is shown that cessation of the spontaneous venous pulsation is a sensitive marker of raised intracranial pressure. The article discusses the specificity of the absence of spontaneous venous pulsation and describes how the patient should be examined to best elicit this important sign.

PMID 12486256
James A McHugh, Linda D'Antona, Ahmed K Toma, Fion D Bremner
Spontaneous Venous Pulsations Detected With Infrared Videography.
J Neuroophthalmol. 2020 Jun;40(2):174-177. doi: 10.1097/WNO.0000000000000815.
Abstract/Text BACKGROUND: Assessment of spontaneous venous pulsation (SVP) is commonly undertaken to help determine whether intracranial pressure (ICP) is elevated. Previous studies using direct ophthalmoscopy or slit-lamp assessments have found that SVP is not observed in 67%-81% of subjects with normal ICP, and that interobserver agreement when grading SVP is poor.
METHODS: Patients (n = 105) undergoing clinically indicated retinal OCT scans, who were all believed to have normal ICP, had 10-second infrared video recordings performed with the Heidelberg Spectralis OCT system (Heidelberg Engineering GmbH, Heidelberg, Germany). The presence and amplitude of SVP in each video was independently graded by 2 neuro-ophthalmologists.
RESULTS: The 2 observers found SVP present in 97% and 98% of right eyes and in one or both eyes in 99% and 100% of subjects. Interobserver agreement was high (Cohen's kappa 0.82 for right eyes). Optic discs with a smaller cup had a significantly lower SVP amplitude (Spearman's rho = 0.22, P = 0.02).
CONCLUSIONS: Infrared video is widely available in eye clinics by the use of OCT imaging systems and is substantially more sensitive in detecting SVP than traditional assessments using ophthalmoscopy. SVP is absent in as few as 1% of people with presumed normal ICP.

PMID 31464805
Linda D'Antona, James A McHugh, Federico Ricciardi, Lewis W Thorne, Manjit S Matharu, Laurence D Watkins, Ahmed K Toma, Fion D Bremner
Association of Intracranial Pressure and Spontaneous Retinal Venous Pulsation.
JAMA Neurol. 2019 Dec 1;76(12):1502-1505. doi: 10.1001/jamaneurol.2019.2935.
Abstract/Text Importance: A convenient and reliable method for noninvasive intracranial pressure assessments is desirable to reduce the need for invasive procedures (eg, intracranial pressure monitoring and lumbar punctures) and allow clinicians to identify and treat patients with intracranial hypertension in a timely manner.
Objective: To determine whether infrared video assessment of spontaneous retinal venous pulsation is associated with intracranial pressure and is a valid tool to indicate the presence or absence of raised intracranial pressure in patients without papilledema.
Design, Setting, and Participants: A single-center prospective study was conducted at a tertiary referral center between January 2017 and May 2018. Patients consecutively admitted for clinically indicated elective 24-hour invasive intracranial pressure monitoring had ophthalmic review including infrared video recording of their spontaneous venous pulsation. Two neuro-ophthalmologists, who were masked to the intracranial pressure monitoring results, independently graded the spontaneous venous pulsation (grade 0 to 3). Analysis began in June 2018.
Main Outcomes and Measures: The association between simultaneously recorded intracranial pressure and spontaneous venous pulsation (binary variable: present/absent) assessed through retinal infrared video recordings was evaluated using a multiple linear regression model.
Results: Of 105 patients, the mean (SD) age was 39 (14) years, and 79 (75%) were women. The mean (SD) simultaneous intracranial pressure was 1 (5) mm Hg for 91 patients (86.7%) with spontaneous venous pulsations and 13 (14) mm Hg for 14 patients (13.3%) without spontaneous venous pulsations. A multiple linear regression model adjusted for 7 potential confounders confirmed a statistically significant association between intracranial pressure and spontaneous venous pulsation (β = -9.1; 95% CI, -13.7 to -4.6; P < .001; adjusted R2 = 0.42).
Conclusions and Relevance: The absence of spontaneous venous pulsation on retinal infrared video recordings is significantly associated with higher levels of intracranial pressure and should raise the suspicion of intracranial hypertension.

PMID 31498376
Meira Neudorfer, Maytal Siegman Ben-Haim, Igal Leibovitch, Anat Kesler
The efficacy of optic nerve ultrasonography for differentiating papilloedema from pseudopapilloedema in eyes with swollen optic discs.
Acta Ophthalmol. 2013 Jun;91(4):376-80. doi: 10.1111/j.1755-3768.2011.02253.x. Epub 2011 Sep 22.
Abstract/Text PURPOSE: To evaluate the diagnostic yield of optic nerve ultrasonography (US) in distinguishing between papilloedema (swollen discs owing to raised intracranial pressure) and pseudopapilloedema.
METHODS: We prospectively evaluated all patients with bilateral optic disc swelling who underwent a complete neuro-ophthalmological examination. Suitable patients were referred for neuroimaging (computerized tomography or magnetic resonance imaging) and lumbar puncture. They underwent optic nerve US (A-mode and B-mode), and the findings were compared with the final clinical assessment. Sensitivity, specificity and predictive values for US distinction between true papilloedema and pseudopapilloedema were calculated and compared with those of the other imaging tests.
RESULTS: Forty-four patients were enrolled. Ultrasonography detected papilloedema with a high degree of sensitivity (85%) when the normal optic nerve width (ONW) was set at ≤3.3 mm, and with an even higher degree of sensitivity (95%) when the normal ONW was set at ≤3.0 mm. Ultrasonography had a high negative predictive value for detecting papilloedema: 83% when the normal ONW was set at ≤3.3 mm and 93% when it was set at ≤3.0 mm. There was a significant correlation between the US findings and the final diagnosis (p < 0.001) when the upper limit of the normal ONW was set at 3.0 mm.
CONCLUSIONS: Ultrasonography findings of the ONW correlated well with the final diagnosis of papilloedema or pseudopapilloedema, especially when the upper limit of the normal ONW was set at 3.0 mm. Ultrasonography could be a useful non-invasive technique for differentiating papilloedema from other causes for swollen discs, such as pseudopapilloedema.

© 2011 The Authors. Acta Ophthalmologica © 2011 Acta Ophthalmologica Scandinavica Foundation.
PMID 21951833
Jochen Bäuerle, Max Nedelmann
Sonographic assessment of the optic nerve sheath in idiopathic intracranial hypertension.
J Neurol. 2011 Nov;258(11):2014-9. doi: 10.1007/s00415-011-6059-0. Epub 2011 Apr 28.
Abstract/Text The aim of this work was to investigate the potential of ultrasound-based optic nerve sheath diameter (ONSD) measurements in detecting raised intracranial pressure in patients with idiopathic intracranial hypertension (IIH) and to describe ONSD response to lumbar puncture. In ten patients with newly diagnosed IIH, transorbital sonography was carried out to assess ONSD, OND (optic nerve diameter), and optic disc elevation before and after lumbar puncture. Twenty-five patients with other neurological disorders served as controls. Subjects with IIH showed a significantly enlarged ONSD on both sides (6.4 ± 0.6 mm vs. 5.4 ± 0.5 mm in controls; p < 0.001). The best cut-off value of ONSD for detecting raised ICP was 5.8 mm with a sensitivity of 90% and a specificity of 84%. After lumbar puncture, ONSD decreased bilaterally (right 5.8 ± 0.7 mm, p < 0.004; left 5.9 ± 0.7 mm, p < 0.043). No post-puncture changes could be observed with regard to OND and optic disc elevation. Sonographic ONSD evaluation may be useful as an additional tool to identify patients with raised intracranial pressure, as in IIH. Furthermore, our data suggest a potential usefulness of this method for monitoring of treatment effects. The degree of ONSD response to lumbar puncture differs in subjects with IIH, which may possibly be related to findings of a defective CSF circulation in the optic nerve sheath in this disorder, a state that is referred to as optic nerve compartment syndrome.

PMID 21523461
Venkatakrishna Rajajee, Monique Vanaman, Jeffrey James Fletcher, Teresa Lee Jacobs
Optic nerve ultrasound for the detection of raised intracranial pressure.
Neurocrit Care. 2011 Dec;15(3):506-15. doi: 10.1007/s12028-011-9606-8.
Abstract/Text BACKGROUND: Optic nerve ultrasonography (ONUS) may help identify raised intracranial pressure (ICP). The optimal optic nerve sheath diameter (ONSD) cut-off for the identification of intracranial hypertension has not been established, with some clinical studies suggesting a higher cut-off than may be expected on the basis of prior laboratory investigation.
OBJECTIVE: To validate ONUS performed by neurointensivists as a technique for the detection of intracranial hypertension and identify the optimal ONSD criterion for the detection of ICP > 20 mmHg.
METHODS: Prospective blinded observational study. Patients in the ICU with either external ventricular drains or intraparenchymal ICP monitors at risk for intracranial hypertension were enrolled. The ONSD was measured by neurointensivists at the bedside with simultaneous invasive ICP measurement. An ROC curve was constructed to determine the optimal ONSD for the detection of ICP >  20 mmHg.
MEASUREMENTS AND RESULTS: A total of 536 ONSD measurements were performed on 65 patients. Diagnoses included subarachnoid hemorrhage, traumatic brain injury, intracerebral hemorrhage, ischemic stroke and brain tumor. ROC curve analysis revealed area under the curve (AUC) = 0.98 (95% CI 0.96-0.99; P < 0.0001 for AUC = 0.5). Optimal ONSD for detection of ICP > 20 mmHg was ≥0.48 cm sensitivity 96% (95% CI 91-99%); specificity 94% (92-96%). Sensitivity of the higher cutoff of ≥0.52 cm proposed by some authors was only 67% (58-75%), with specificity 98% (97-99%).
CONCLUSIONS: Bedside ONSD measurement, performed by neurointensivists, is an accurate, non-invasive method to identify ICP > 20 mmHg in a heterogeneous group of patients with acute brain injury. ONSD ≥0.48 cm has the greatest accuracy, however, internal validation of ONSD criteria may be required.

PMID 21769456
W D Newman, A S Hollman, G N Dutton, R Carachi
Measurement of optic nerve sheath diameter by ultrasound: a means of detecting acute raised intracranial pressure in hydrocephalus.
Br J Ophthalmol. 2002 Oct;86(10):1109-13.
Abstract/Text AIM: To evaluate the utility of measuring the optic nerve sheath diameter in children with shunted hydrocephalus, suspected of having raised intracranial pressure.
METHODS: 23 children with shunted hydrocephalus were examined, six had well controlled ICP, 17 however manifested symptoms suggestive of intracranial hypertension. A clinical history was taken from all patients and their parents or carers. The shunt valve was examined clinically, and signs of raised intracranial pressure were sought. Ultrasound examination was performed in both eyes to measure the optic nerve sheath diameters 3 mm behind the globe. These measurements were compared with control data obtained from 102 children who attended the radiology department for unrelated renal ultrasound examination.
RESULTS: Control data suggested that the upper limit of normal for optic nerve sheath diameter is 4.5 mm (measured 3 mm behind the globe) in patients over 1 year of age, and 4.0 mm in children less than 1 year of age. Those patients with functioning ventriculoperitoneal shunts had a mean optic nerve sheath diameter of 2.9 (SD 0.5) mm; those with raised intracranial pressure had a mean optic nerve sheath diameter of 5.6 (0.6) mm (p<0.0001). These results confirm that optic nerve sheath diameters in excess of the control data are strongly suggestive of raised intracranial pressure.
CONCLUSION: The evaluation of the optic nerve sheath diameter is a simple non-invasive procedure, which is a potentially useful tool in the assessment and monitoring of children with hydrocephalus suspected of having raised intracranial pressure.

PMID 12234888
Vivek Vijay, Susan P Mollan, James L Mitchell, Edward Bilton, Zerin Alimajstorovic, Keira A Markey, Anthony Fong, Jessica K Walker, Hannah S Lyons, Andreas Yiangou, Georgios Tsermoulas, Kristian Brock, Alexandra J Sinclair
Using Optical Coherence Tomography as a Surrogate of Measurements of Intracranial Pressure in Idiopathic Intracranial Hypertension.
JAMA Ophthalmol. 2020 Dec 1;138(12):1264-1271. doi: 10.1001/jamaophthalmol.2020.4242.
Abstract/Text Importance: There is an unmet need for noninvasive biomarkers of intracranial pressure (ICP), which manifests as papilledema that can be quantified by optical coherence tomography (OCT) imaging.
Objective: To determine whether OCT of the optic nerve head in papilledema could act as a surrogate measure of ICP.
Design, Setting, and Participants: This longitudinal cohort study used data collected from 3 randomized clinical trials that were conducted between April 1, 2014, and August 1, 2019. Participants who were female and had active idiopathic intracranial hypertension were enrolled from 5 National Health Service hospitals in the UK. Automated perimetry and OCT imaging were followed immediately by ICP measurement on the same day. Cohort 1 used continuous sitting telemetric ICP monitoring (Raumedic Neurovent P-tel device) on 1 visit. Cohort 2 was evaluated at baseline and after 3, 12, and 24 months and underwent lumbar puncture assessment of ICP.
Main Outcomes and Measures: Optical coherence tomography measures of the optic nerve head and macula were correlated with ICP levels, Frisén grading, and perimetric mean deviation. The OCT protocol included peripapillary retinal nerve fiber layer, optic nerve head, and macular volume scans (Spectralis [Heidelberg Engineering]). All scans were validated for quality and resegmented manually when required.
Results: A total of 104 women were recruited. Among cohort 1 (n = 15; mean [SD] age, 28.2 [9.4] years), the range of OCT protocols was evaluated, and optic nerve head central thickness was found to be most closely associated with ICP (right eye: r = 0.60; P = .02; left eye: r = 0.73; P = .002). Subsequently, findings from cohort 2 (n = 89; mean [SD] age, 31.8 [7.5] years) confirmed the correlation between central thickness and ICP longitudinally (12 and 24 months). Finally, bootstrap surrogacy analysis noted a positive association between central thickness and change in ICP at all points (eg, at 12 months, a decrease in central thickness of 50 μm was associated with a decrease in ICP of 5 cm H2O).
Conclusions and Relevance: In this study, optic nerve head volume measures on OCT (particularly central thickness) reproducibly correlated with ICP and surrogacy analysis demonstrated its ability to inform ICP changes. These data suggest that OCT has the utility to not only monitor papilledema but also noninvasively prognosticate ICP levels in idiopathic intracranial hypertension.

PMID 33090189
Tiziana Carandini, Luca Sacchi, Francesca Bovis, Matteo Azzimonti, Marco Bozzali, Daniela Galimberti, Elio Scarpini, Anna Margherita Pietroboni
Distinct patterns of MRI lesions in MOG antibody disease and AQP4 NMOSD: a systematic review and meta-analysis.
Mult Scler Relat Disord. 2021 Sep;54:103118. doi: 10.1016/j.msard.2021.103118. Epub 2021 Jun 30.
Abstract/Text BACKGROUND: the distinct MRI features of MOG-antibody disease (MOG-AD) and AQP4-NMOSD are still poorly defined. We performed a systematic review and meta-analysis to identify specific patterns of MRI abnormalities able to discriminate between MOG-AD and AQP4-NMOSD.
METHODS: fourteen case-series (1028 patients) were included. Outcomes were MRI lesion patterns in optic nerve (ON), brain and spinal cord (SC) that were selected after a systematic literature review and analysed separately as the event rate for individual MRI lesions in MOG-AD (experimental group) and AQP4-NMOSD (control group) by using a random effect model.
RESULTS: MOG-AD showed a higher number of MRI lesions than AQP4-NMOSD patients in the retrobulbar ON (OR=5.67; 95%CI=2.11-15.24; p=0.0006) with ON head swelling (OR=8.20; 95%CI=4.13-16.28; p<0.00001), corpus callosum (OR=2.30; 95%CI=1.11-4.76; p=0.02), pons (OR=2.87; 95%CI=1.45-5.67; p=0.002), and lumbar/conus SC (OR=3.47; 95%CI=1.66-7.24; p=0.0009). Conversely, lesions in the canalicular (OR=0.42; 95%CI=0.18-0.98; p=0.05) and intracranial ON (OR=0.30; 95%CI=0.11=0.84; p=0.02), area postrema (OR=0.12; 95%CI=0.02-0.61; p=0.01), medulla (OR=0.40; 95%CI=0.20-0.78; p=0.007), and cervical SC (OR=0.29; 95%CI=0.09-0.92; p=0.04) were prominent in patients with AQP4-NMOSD. Participants' age was found to be a source of heterogeneity across studies.
CONCLUSION: our study provides further evidence that MOG-AD and AQP4-NMOSD have distinct MRI features that may help clinicians for an early differential diagnosis.

Copyright © 2021. Published by Elsevier B.V.
PMID 34246019
Arlette L Bruijstens, Christian Lechner, Lorraine Flet-Berliac, Kumaran Deiva, Rinze F Neuteboom, Cheryl Hemingway, Evangeline Wassmer, E.U. paediatric MOG consortium, M Baumann, F Bartels, C Finke, C Adamsbaum, Y Hacohen, K Rostasy
E.U. paediatric MOG consortium consensus: Part 1 - Classification of clinical phenotypes of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders.
Eur J Paediatr Neurol. 2020 Nov;29:2-13. doi: 10.1016/j.ejpn.2020.10.006. Epub 2020 Nov 4.
Abstract/Text Over the past few years, increasing interest in the role of autoantibodies against myelin oligodendrocyte glycoprotein (MOG-abs) as a new candidate biomarker in demyelinating central nervous system diseases has arisen. MOG-abs have now consistently been identified in a variety of demyelinating syndromes, with a predominance in paediatric patients. The clinical spectrum of these MOG-ab-associated disorders (MOGAD) is still expanding and differs between paediatric and adult patients. This first part of the Paediatric European Collaborative Consensus emphasises the diversity in clinical phenotypes associated with MOG-abs in paediatric patients and discusses these associated clinical phenotypes in detail. Typical MOGAD presentations consist of demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM) in younger, and optic neuritis (ON) and/or transverse myelitis (TM) in older children. A proportion of patients experience a relapsing disease course, presenting as ADEM followed by one or multiple episode(s) of ON (ADEM-ON), multiphasic disseminated encephalomyelitis (MDEM), relapsing ON (RON) or relapsing neuromyelitis optica spectrum disorders (NMOSD)-like syndromes. More recently, the disease spectrum has been expanded with clinical and radiological phenotypes including encephalitis-like, leukodystrophy-like, and other non-classifiable presentations. This review concludes with recommendations following expert consensus on serologic testing for MOG-abs in paediatric patients, the presence of which has consequences for long-term monitoring, relapse risk, treatments, and for counselling of patient and families. Furthermore, we propose a clinical classification of paediatric MOGAD with clinical definitions and key features. These are operational and need to be tested, however essential for future paediatric MOGAD studies.

Copyright © 2020 The Authors. Published by Elsevier Ltd.. All rights reserved.
PMID 33162302
Jian Li, Dao-bin Zhou
New advances in the diagnosis and treatment of POEMS syndrome.
Br J Haematol. 2013 May;161(3):303-15. doi: 10.1111/bjh.12236. Epub 2013 Feb 8.
Abstract/Text POEMS syndrome is a clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Significant advances have been made in the diagnosis and treatment of POEMS syndrome over the last decade. Herein, the diagnostic criteria and characteristic features are reviewed, focusing the role of characteristic features in early diagnoses. Autologous peripheral blood stem cell transplantation has become the first-line treatment for younger patients with normal organ function. Autologous transplantation has resulted in a high response rate and durable remission. Moreover, transplantation-related morbidity and mortality has been significantly reduced over the past 5 years. Induction therapy before transplantation may improve the harvest of stem cells and decrease transplantation-related morbidity. Melphalan and dexamethasone is an effective and well-tolerated treatment for older patients or those with organ dysfunction. Novel agents may also offer benefits to patients with a poor performance status or renal dysfunction, and transform transplantation eligibility.

© 2013 Blackwell Publishing Ltd.
PMID 23398538
Bruno Royer, Lavinia Merlusca, Julie Abraham, Lucile Musset, Julien Haroche, Sylvain Choquet, Xavier Leleu, Catherine Sebban, Olivier Decaux, Lionel Galicier, Muriel Roussel, Christian Recher, Anne Banos, Isabelle Guichard, Jean-Marie Brisseau, Pascal Godmer, Olivier Hermine, Gaël Deplanque, Thierry Facon, Bouchra Asli, Véronique Leblond, Jean-Paul Fermand, Jean Pierre Marolleau, Arnaud Jaccard
Efficacy of lenalidomide in POEMS syndrome: a retrospective study of 20 patients.
Am J Hematol. 2013 Mar;88(3):207-12. doi: 10.1002/ajh.23374. Epub 2013 Jan 18.
Abstract/Text POEMS syndrome is a rare disorder characterized by polyneuropathy, monoclonal gammopathy, multiorgan involvement, and elevated vascular endothelial growth factor levels. Localized bone lesions require irradiation, whereas young patients with disseminated disease receive intensive treatment with stem cell support. Treatment of older and non responding patients is not yet standardized. We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. Four patients were newly diagnosed, and 16 had relapsed or progressed after treatment. All but one of the patients responded: clinical improvements were noted in neuropathies (16/20) organomegaly (13/13), peripheral edema (14/15), and pulmonary hypertension (5/5). At least a very good partial response was noted in 68% of patients, with partial responses in 26%. Serum VEGF levels fell markedly in all 17 patients with available values. Twelve patients had 18-FDG-PET/CT at diagnosis (11 with positive findings), and nine patients during follow-up. The number of lesions fell markedly in five cases and remained stable in two cases, while two patients became negative. During a median follow-up of 22 months, four patients relapsed. Toxicity, predominantly hematological, was mild and manageable. Lenalidomide thus appears to be effective in POEMS syndrome, inducing high rate of clinical and biological responses.

Copyright © 2012 Wiley Periodicals, Inc.
PMID 23335406

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