Anti‐aquaporin‐4 antibody‐positive optic neuritis

Purpose: It has recently been reported that the anti‐aquaporin‐4 antibody (AQP4‐Ab) can be a specific marker of neuromyelitis optica. We present three cases of optic neuritis (ON) where the patients tested positive for AQP4‐Ab, but showed no neurological signs. Methods: Sera were obtained from 32 Japanese patients with ON and no other neurological abnormalities (mean age 46 ± 20 years). AQP4‐Ab was detected by indirect immunofluorescence staining using human‐AQP4‐transfected HEK 293 cells. Results: AQP4‐Ab was positive in three female patients (aged 9, 64 and 82 years). Their illness was characterized by bilateral severe optic nerve involvement, insufficient visual recovery, and autoimmune abnormalities (such as positive antinuclear antibody). Two of these patients experienced recurrent episodes of ON. In at least two episodes, the intracranial portion of the optic nerve showed significant inflammation on magnetic resonance imaging. Conclusions: These cases indicate that some ON patients have an immunological pathogenesis similar to that seen in neuromyelitis optica. In addition, examination for AQP4‐Ab positivity in the initial phase of ON is important in predicting the prognosis, including the possibility of the development of transverse myelitis.     

Treatment options for atypical optic neuritis

Context:Optic neuritis (ON) is defined as inflammation of the optic nerve and can have various etiologies. The most common presentation in the US is demyelinating, or “typical” ON, usually associated with multiple sclerosis. This is in contrast to “atypical” causes of ON, which differ in their clinical presentation, management, and prognosis. These atypical cases are characterized by lack of eye pain, exudates, and hemorrhages on exam, very severe, bilateral or progressive visual loss, or with failure to recover vision.Aims:The aim was to describe the clinical presentations of atypical ON and their treatments.Results:Types of atypical ON identified include neuromyelitis optica, autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy, idiopathic recurrent neuroretinitis, and optic neuropathy associated with systemic diseases. Atypical ON usually requires corticosteroid treatment and often will require aggressive immunosuppression.Conclusions:Unlike demyelinating ON, atypical ON requires treatment to preserve vision.     

Bilateral monosymptomatic optic neuritis following Mycoplasma pneumoniae infection: A case report and literature review

Herein, we report the clinical findings, treatment choice, and clinical course of a rare case of Mycoplasma pneumoniae (M. pneumoniae) infection with the sole manifestation of optic neuritis (ON). To the best of our knowledge, this is the first case presenting monosymptomatic visual loss without papillitis, neurological symptoms, and abnormal findings on brain imaging. Related articles about ON after M. pneumoniae infection were reviewed to summarize the clinical presentation, possible mechanisms, clinical survey, treatment, and prognosis of this condition. We propose that a Mycoplasma profile is necessary in children who present with ON, especially when this condition is accompanied by prodromal symptoms of the respiratory tract infection.     

Multifocal visual evoked potential in optic neuritis,ischemic optic neuropathy and compressive optic neuropathy

Purpose:

To investigate the effect of optic neuritis (ON), ischemic optic neuropathy (ION) and compressive optic neuropathy (CON) on multifocal visual evoked potential (mfVEP) amplitudes and latencies, and to compare the parameters among three optic nerve disorders.

Materials and Methods:

mfVEP was recorded for 71 eyes of controls and 48 eyes of optic nerve disorders with subgroups of optic neuritis (ON, n = 21 eyes), ischemic optic neuropathy (ION, n = 14 eyes), and compressive optic neuropathy (CON, n = 13 eyes). The size of defect in mfVEP amplitude probability plots and relative latency plots were analyzed. The pattern of the defect in amplitude probability plot was classified according to the visual field profile of optic neuritis treatment trail (ONTT).

Results:

Median of mfVEP amplitude (log SNR) averaged across 60 sectors were reduced in ON (0.17 (0.13-0.33)), ION (0.14 (0.12-0.21)) and CON (0.21 (0.14-0.30)) when compared to controls. The median mfVEP relative latencies compared to controls were significantly prolonged in ON and CON group of 10.53 (2.62-15.50) ms and 5.73 (2.67-14.14) ms respectively compared to ION group (2.06 (-4.09-13.02)). The common mfVEP amplitude defects observed in probability plots were diffuse pattern in ON, inferior altitudinal defect in ION and temporal hemianopia in CON eyes.

Conclusions:

Optic nerve disorders cause reduction in mfVEP amplitudes. The extent of delayed latency noted in ischemic optic neuropathy was significantly lesser compared to subjects with optic neuritis and compressive optic neuropathy. mfVEP amplitudes can be used to objectively assess the topography of the visual field defect.     

Atypical optic neuritis: An overview

Optic neuritis (ON) refers to conditions that involve inflammation of the optic nerve. Various autoantibodies have been found, which are associated with central nervous system inflammatory disorders and have provided much information about the immune targets and mechanisms that impact the prognosis, treatment, and recurrence of atypical ON. Therefore, neurologists and ophthalmologists together should work to find out clinical, laboratory, and imaging findings that may provide important clues to the etiology of atypical ON and its management. Various biomarkers have been identified to confirm and distinguish atypical optic neuritis from others. The purpose of this review is to present the current scenario of atypical ON and its clinical management.     

COVID-19-associated optic neuritis – A case series and review of literature

Neuroophthalmic manifestations are very rare in corona virus disease-19 (COVID-19) infection. Only few reports have been published till date describing COVID-19-associated neuroophthalmic manifestations. We, hereby, present a series of three cases who developed optic neuritis during the recovery period from COVID-19 infection. Among the three patients, demyelinating lesions were identified in two cases, while another case was associated with serum antibodies against myelin oligodendrocyte glycoprotein. All three patients received intravenous methylprednisolone followed by oral steroids according to the Optic Neuritis Treatment Trail ptotocol. Vision recovery was noted in all three patients, which was maintained at 2 months of the last follow up visit.     

Fractal analysis of fluoroangiographic patterns in anterior ischaemic optic neuropathy and optic neuritis: a pilot study

Background:  To evaluate by means of fractal analysis the vascular pattern of the optic nerve head obtained by fluorescein angiogram, in non-arteritic anterior ischaemic optic neuropathy (NAION) and optic neuritis (ON).
Methods:  Twenty-nine patients at the Department of Ophthalmology of the University of Siena, diagnosed as having either NAION or ON by clinical and instrumental criteria, were prospectively subjected to fractal analysis: 11 patients with NAION and 18 patients with ON. In the ON group, 12 patients showed optic disc oedema, whereas six patients showed no optic disc oedema. The unaffected eyes of six patients with NAION and of seven patients with ON associated with optic disc oedema served as controls.
Results:  The mean fractal dimension D was 1.84 ± 0.09 in the NAION group, 1.92 ± 0.04 in the ON group with optic disc oedema, 1.86 ± 0.04 in the ON group without optic disc oedema and 1.63 ± 0.06 in the control group; all case groups showed significantly higher values than controls ( P  < 0.01). Among the case groups, the ON group with optic disc oedema showed a significantly higher mean fractal dimension value than the others ( P  < 0.01).
Conclusions:  Our data suggest that eyes with ON and NAION seem to have increased vascular complexity in the optic nerve head, manifested as an increase in fractal dimension.     

ICGA对NAION与视神经炎的鉴别诊断作用

目的:初步探讨ICGA对非动脉炎性前部缺血型视神经病变与视神经炎鉴别诊断中的作用。方法:临床资料完整的不易确诊视盘水肿患者27例27眼,经眼底照相后,采用视网膜血管和脉络膜血管双项造影检查。同期对年龄性别匹配20例20眼正常对照眼进行造影检查,对脉络膜血管造影的连续图像进行分析和总结。结果:对照眼20例ICGA检查结果显示早期视盘周围脉络膜血管区荧光充盈正常。27眼视盘水肿者接受ICGA检查后,从ICGA造影早期连续图片的表现特点将视盘水肿可明确分为缺血性和非缺血性;缺血性早期连续像表现为一过性的视盘缺血区与脉络膜区域性低荧光相对应,可直接判定缺血范围和缺血程度,随即血管荧光充盈正常,16眼考虑为对非动脉炎性前部缺血型视神经病变,11眼早期连续像表现为视盘周围脉络膜血管区荧光快速充盈,与正常对照眼表现相同,考虑为视神经炎。结论:对一些视盘水肿鉴别诊断存在困惑或疑难时,脉络膜血管造影有一过性视盘脉络膜区域性荧光充盈缺损为非动脉炎性前部缺血型视神经病变的特征性改变,该变化有助于视盘水肿性疾病的鉴别,值得重视。     

Critical review: Typical and atypical optic neuritis

Typical optic neuritis is an idiopathic demyelinating condition that is often associated with multiple sclerosis. This has been well characterized and has an excellent prognosis. Atypical optic neuritis can result from an inflammatory, infectious, or autoimmune disorder. Differentiating the two types of optic neuritis is paramount and may be challenging early on in the clinical course. This review describes the recent literature describing the pathophysiology, clinical presentation, neuroimaging, and management of these disorders.     

Etanercept associated optic neuropathy

We report the case of a 14-year-old boy who developed optic neuropathy subsequent to the use of etanercept. There have been 15 reported cases of anti-TNF-alpha-associated optic neuropathy to date and their characteristics are reviewed in this report, as well as possible pathophysiologic mechanisms behind such phenomenon. Such cases demonstrate the importance of prompt ophthalmologic evaluation of visual changes in patients being treated with anti-TNF-alpha antagonists.     

Optic neuritis — Etiology?

A 44-year-old otherwise healthy woman was referred to Washington University with previous diagnoses of pars planitis and retrobulbar neuritis, and with a current complaint of markedly decreased vision (light perception only) in the right eye. Among the findings at the time of this evaluation were posterior uveitis and evidence of optic neuropathy and of a disordered immune system. The patient responded to pulsed high-dose corticosteroid therapy. A subsequent similar episode in the left eye also was resolved with such treatment. Dr. Burde describes the case in detail and asks Drs. Keltner, Gittinger and Miller to offer diagnoses. Their answers vary considerably.     

Neuromyelitis optica and aquaporin‐4 (AQP4) autoantibodies in consecutive optic neuritis patients in Southern Finland

Purpose: To analyse the frequency of neuromyelitis optica (NMO) among consecutive optic neuritis (ON) patients in Southern Finland and the feasibility of Aquaporin‐4 (AQP4) autoantibody assay in the diagnosis of NMO. Methods: Consecutive patients with symptoms suggestive of acute ON and managed in the Helsinki University Central Hospital were evaluated critically screened for AQP4 autoantibody during a 47.5‐month period. The antibodies were determined using radioimmunoprecipitation method. AQP4 index >15 was considered positive, 10–15 borderline and <10 normal. Brain magnetic resonance imaging (MRI) was performed for all patients. Results: Of the 300 patients with suspected ON, 191 were eventually diagnosed as ON, and 66 (35%) of them had a previous diagnosis or were diagnosed with multiple sclerosis (MS). Of the 125 patients without MS diagnosis, 62 (50%) had demyelinative lesions in MRI, which is a risk factor for developing MS. Two patients (1.1%; 95% CI 0.3–4.5) fulfilled the criteria of NMO. Positive AQP4 antibodies were found in three patients (1.6% 95% CI 0.3–4.5), one of them had NMO, one had MS and one became diagnosed with MS a month later. Borderline autoantibody levels were found in 10 patients, 7 of whom had MS. Conclusions: NMO is rare among ON patients in the population of Southern Finland. In this small cohort, the sensitivity and positive predictive values of the AQP4 autoantibody index for NMO were low, 1/2 and 1/3 respectively, and do not support initiating routine screening.     

视神经炎和前段缺血性视神经病变及视乳头水肿的视野改变分析

目的:比较特发性脱髓鞘性视神经炎(idiopathic demyelinating optic neuritis,IDON)、非动脉炎性前段缺血性视神经病变(non-arteritic anterior ischemic optic neuropathy,NAION)和视乳头水肿(papilledema,PE)患者的视野变化特点,分析其发生机制。方法:回顾性病例研究。收集2011-03/2012-05期间在中国医科大学附属第四医院眼科诊治的IDON,NAION和PE患者的视野资料,记录患者的年龄、性别组成、最佳矫正视力(BCVA)、瞳孔大小、完成视野检测的时间、视野缺损的类型、部位、平均缺损(MD)和模式缺损(PSD)情况,SPSS 12.0统计软件包比较三组患者之间的差异。结果:IDON患者17例20眼,NAION患者21例26眼,PE患者11例22眼。三组患者平均年龄、性别比例、BCVA、瞳孔大小、完成视野检测的时间、视野MD和PSD的差异均具有统计学意义(P<0.05)。结论:IDON,NAION和PE患者的视野表现复杂多样,但又各有特点,这与三种疾病的发病机制密切相关,为视神经疾病的鉴别诊断提供依据。     

Systemic corticosteroids in nonarteritic ischemic optic neuropathy

Nonarteritic ischemic optic neuropathy (NAION) is one of the most prevalent optic nerve disorders seen in ophthalmic practice. The role of corticosteroid therapy in NAION remains a highly controversial area of debate in ophthalmology. This brief review will provide an overview of the current clinical evidence on this topic as well as some comment on the medical debate.     

Leber's hereditary optic neuropathy masquerading as optic neuritis with spontaneous visual recovery

We report a case of Leber's hereditary optic neuropathy (LHON) masquerading as optic neuritis with late visual recovery. A 28‐year‐old man had gradual visual loss in both eyes for two weeks. Visual acuity was 0.4 in the right eye and 0.7 in the left. Fundus examination revealed hyperaemic discs in each eye. Fluorescein angiography revealed dye leakage at both optic discs in the late phase. Static perimetry (Humphrey 30‐2) revealed bilateral relative central scotomata. Magnetic resonance imaging of the optic nerves was normal and his lumbar puncture showed normal opening pressure. He received steroid pulse therapy for three days. Nevertheless, vision in his right eye deteriorated to 0.1 one month later and left vision worsened to 0.05 six months later. Fifteen months after onset, his vision began to improve. At 21 months, his vision recovered to 0.9 R and 1.0 L. Peripheral blood DNA sequencing revealed 14484 mutation of mitochondrial DNA (mtDNA). Visual recovery can occur in patients with Leber's hereditary optic neuropathy with mtDNA 14484 mutation. LHON could be misdiagnosed as optic neuritis in some cases. Molecular examination of mtDNA mutation can confirm the diagnosis of LHON in clinically controversial patients. We should keep in mind the diagnosis of LHON when optic neuritis shows poor response to pulse therapy.     

RNFL thickness in MS‐associated acute optic neuritis using SD‐OCT: critical interpretation and limitations

Purpose: Axonal loss is considered a key prognostic factor in diagnosing and monitoring the progress of multiple sclerosis (MS). The purpose of our research was to determine whether the measurement of retinal nerve fibre layer thickness (RNFLT) as measured with high‐resolution spectral‐domain optical coherence tomography (SD‐OCT) differs between optic nerve injury following acute optic neuritis (ON) or following unregistered subclinical axonal damage in patients with MS. Methods: High‐resolution SD‐OCT measurements of RNFLT were initially carried out in the acute phase of ON and again after 3 months, in 25 patients with clinical definite MS and 25 sex‐ and age‐matched healthy controls, all at the University Eye Hospital, Vienna. Results: Conventional OCT‐based RNFLT analysis correctly identified all three patients with initial RNFL swelling. However, only two of three acute ON eyes with a history of ON were registered with RNFLT decrease in seven peripapillary sectors (PPs). The remaining have only been revealed using RNFLT symmetry comparison. Two of 22 (9%) first‐episode ON eyes were labelled as pathologic. The number and metric RNFL values of pathologically labelled PPs remained unchanged after 3 months. Our age‐ and sex‐match‐based measurement model, with patients with MS being plotted individually and towards the fellow eye, identified all acute ON eyes (with a history of prior ON) with RNFLT reduction in 11 PPs. A global RNFL loss was registered in 36.4% (eight of 22 eyes). However, in 72%, or 16 of 22 ON eyes presenting with first episode of acute ON, a segmental RNFL loss was initially registered in 39 PPs upon baseline examination. The number of PPs with identified axonal decrease increased to a total of 48 PPs within the observational period. Conclusions: Spectral‐domain optical coherence tomography imaging of identical scanning locations, combined with an optimized scan centring around the optic disc, offers the technological potential of detecting prior, subtle, clinically unregistered optic nerve injury within MS individuals. Significant discrepancy in RNFLT to the potential ON eye may be achieved by comparing OCT metrics with the fellow eye and a sufficient number of age and sex‐matched controls.     

Clinical characteristics of optic neuritis in Koreans greater than 50 years of age

Purpose

To report clinical characteristics of optic neuritis (ON) in Koreans >50 years of age.

Methods

A retrospective chart review was performed on patients with ON between January 2000 and December 2009. We obtained the best-corrected visual acuity (BCVA), Goldmann perimetry, relative afferent pupillary defect (RAPD), and color function tests as well as brain magnetic resonance imaging (MRI) findings in patients who were in the acute stage of the disorder.

Results

Nine eyes in eight patients were included. The mean age of patients at presentation was 60.5 years (range, 53 to 71 years). Six patients were female, and two were male. There was one patient with bilateral ON. The mean BCVA at presentation was 20 / 400 (no light perception-20 / 70). Eight eyes (89%) complained of pain with eye movement. Six eyes (66%) had disc edema. Central scotoma was the most common field defect. All eyes had color abnormalities. Five eyes in four patients showed abnormalities of the involved optic nerves on MRI. The patients were followed for a mean of 11.3 months (range, 2 to 34 months). All of the patients recovered to a BCVA of 20 / 40 or better within 2 months. On the last follow-up, the mean BCVA was 20 / 20 (20 / 40 to 20 / 16). Four eyes showed remnant central scotoma. One eye had remnant RAPD, and two eyes had mild color abnormalities.

Conclusions

Although ON is uncommon in elderly patients, it can develop in patients >50 years of age, and clinical features of optic neuritis in elderly patients are similar to those of younger patients.     

Diameter of the optic nerve in idiopathic optic neuritis and in anterior ischemic optic neuropathy

Purpose: There is considerable overlap in the clinical profile of patients with idiopathic optic neuritis(ON) and anterior ischemic optic neuropathy (AION). We tested the hypothesis that the retrobulbar diameter of the optic nerve may be a criterion for the differential diagnosis between ON and AION. Methods: The diameter of the optic nerve was measured by B-scan ultrasonography with the eye in an abducted position. Only patients with a unilateral optic neuropathy were included, 16 ON patients (mean age 24years, 5 with and 11 without disc swelling) and 9patients with AION (mean age 72 years). As controls for the ON patients 10 young normal subjects (mean age25 years) and as controls for the AION patients 10elderly subjects with eye problems not related to the optic nerve (mean age 76 years) were examined. Results: In the ON patients with disc swelling the diameter of the optic nerve was 5.4 ± 0.5 mm in the affected and 3.0 ± 0.3 mm in the unaffected side. This difference was significant (Wilcoxon-test, p = 0.043). In the ON patients without disc swelling the diameter of the optic nerve was 4.4 ± 0.4 mm in the affected and 3.0 ± 0.3 mm in the unaffected side. This difference was significant (Wilcoxon-test, p = 0.003). In the AION patients the diameter of the optic nerve was 3.0 ± 0.3 mm on the affected and2.8 ± 0.4 mm on the unaffected side. This difference was not significant (Wilcoxon-test, p =0.093). Comparing the optic nerves with ON and AION to those of the controls, the diameter was significantly enlarged in the nerves with ON and normal in the nerves with AION (one factor repeated ANOVA). Conclusion: The diameter of the optic nerve is increased in ON without disc swelling and even more so in ON with disc swelling. The enlargement is probably due to edema of the nerve itself, not the surrounding subarachnoidal space. In AION, the diameter of the optic nerve is normal. Measuring the diameter of the optic nerve by B-scan ultrasonography is particularly useful in the differential diagnosis between ON with disc swelling and AION. This revised version was published online in August 2006 with corrections to the Cover Date.