Optic Neuropathy

Background and AimsAcute optic neuritis (ON) is an inflammatory condition affecting the optic nerve. Clinicians should suspect optic neuritis in cases of painful and rapidly progressive loss of central visual field. This condi-tion may be associated with a multitude of diseases, and mostly with multiple sclerosis (MS) where may present as an initial symptom. The literature reports that optic neuritis and MS occur in patients with inflammatory bowel disease (IBD) before and after the era of anti-tumor necrosis factor-α (TNF-α) drugs. At the present moment, there is little consensus for managing this complication, currently treated with corticosteroids and discontinuation of the causative agents.MethodsWe collected cases through a retrospective multicenter European Crohn's and Colitis Organization CONFER (Collaborative Network For Exceptionally Rare case reports) project. We also performed a comprehensive retrospective search of the available litterature on this topic.ResultWe rep...

Thyroid-associated orbitopathy (TAO) is usually diagnosed clinically. Early presentation of TAO such as ocular irritation, lid puffiness, and mild retraction may be overlooked and misdiagnosed. Careful clinical evaluation, laboratory investigations, and orbital imaging studies are needed for diagnosing early TAO. Knowing the pathogenesis will open the door for obtaining directed and effective treatment for the inflammatory process in TAO. Most patients with mild to moderate active TAO are treated with observation alone as the available treatment modalities effective in controlling the disease have many potential side effects. Severe active TAO, compressive optic neuropathy, and severe exposure keratopathy are the main indications for treatment with immunosuppressant agents, orbital radiotherapy, or orbital decompression. Surgery remains the final rehabilitation in TAO, which should be done during cicatricial (inactive) TAO when reliable and stable results can be obtained.

Orbital apex syndrome is characterized by vision loss from optic neuropathy and ophthalmoplegia due to the involvement of ocular motor nerves in the anatomical region of the orbital apex. Patients could present with signs and symptoms deriving from the involvement of structures within the orbital apex, the superior orbital fissure or the cavernous sinus. The primary focus of the ophthalmologist should be to locate the lesion and then identify its etiology. Clinical evaluation holds key to diagnosis which is aided then by certain serological and lab investigations and neuro-imaging modalities including brain and orbital MRI (Magnetic Resonance Imaging) with contrast, CT (Computed Tomography) scans. In rare instances, a biopsy may be needed to aid in diagnosis. Treatment depends on what the nature of the lesion is with inflammatory conditions usually responding to steroids and infections to antimicrobial agents. Through this review, the authors attempt to decode the approach to localizing the lesion, the etiopathology and the management of cases of orbital apex syndrome Keywords: orbital apex syndrome, Tolosa Hunt syndrome, orbital mucormycosis, orbital imaging • Optic nerve (II C.N) • Oculomotor nerve (III C.N) • Trochlear nerve (IV C.N) • Abducens nerve (VI C.N) • The first division of the trigeminal nerve (ophthalmic division of V C.N) Due to anatomical proximity, two other syndromes that can have overlapping features are the Superior orbital fissure syndrome and the cavernous sinus syndrome.

To assess quality of life (QoL) measures in Chinese Leber hereditary optic neuropathy (LHON) patients with the G11778A mutation. Chinese LHON patients with the G11778A mutation were prospectively evaluated using the Visual Function Index (VF-14) at 6 months, 1 year, and 3 years after the involvement of the second eye. Patients who completed the VF-14 at all 3 follow-up time designations were included in the analysis. Fifty-five patients met the inclusion criteria. The median age of patients when their vision was bilaterally affected was 16.3 years old (interquartile range: 13.9-18.3 years). In our patient cohort, 89.1% (n = 49) were male, and the ratio of males to females was 8.2:1. The median logMAR visual acuity was 2.0 (interquartile range: 1.0-2.6). The mean VF-14 scores of patients with LHON at 6-month, 1-year, and 3-year follow-ups were 18.0 ± 19.2 (range 0-85.4), 19.9 ± 20.0 (range 0-85.4), and 20.7 ± 20.2 (range 0-85.4), and the differences were statistically significant (P ...

Leber's hereditary optic neuropathy (LHON) is a fairly prevalent mitochondrial disorder (1:50,000) arising from the dysfunction of the mitochondrial respiratory chain, which eventually leads to apoptosis of retinal ganglion cells. The usual presentation is that of a young male with a sequential reduction in visual acuity. OCT has been used to study the pattern of optic nerve involvement in LHON, showing early thickening of the inferior and superior retinal nerve fibre layer and ganglion cell layer thinning corresponding with the onset of symptoms. Of the three primary mutations for LHON, the m.14484T>C mutation has the best visual prognosis. Recent emerging therapeutic options for LHON include idebenone and the introduction of genetic vector therapy, which is currently in phase III clinical trials. Screening of family members and adequate advice to avoid environmental triggers, such as smoking and alcohol consumption, are also cornerstones in the management of LHON.

Non-arteritic ischemic optic neuropathy (NAION) is the most common acute optic neuropathy over the age of 50 years. NAION is commonly associated with systemic vascular risk factors (diabetes, hypertension, hyperlipidemia) and small cup-to-disc-ratio. We have assessed the prevalence risk factors of NAION and the visual outcome in patients referred to a tertiary ophthalmology center in Kuwait.

Optic neuritis is a common presentation of demyelinating disorders such as multiple sclerosis. It typically presents with acute painful monocular vision loss, whereas chronic optic neuropathy can be caused by compressive lesions along the anterior visual pathway, genetic, toxic, or nutritional causes. We report an unusual presentation mimicking optic neuritis, which was subsequently diagnosed as optic nerve sheath meningioma (ONSM). Misinterpretation of white matter lesions on MRI of brain and the failure to image the optic nerves at the time of acute loss of vision led to the misdiagnosis of optic neuritis in this case. A comprehensive accurate history and ordering the appropriate imaging modality remain paramount in diagnosing progressive visual deterioration.

Leber’s hereditary optic neuropathy (LHON) is a fairly prevalent mitochondrial disorder (1:50,000) arising from the dysfunction of the mitochondrial respiratory chain, which eventually leads to apoptosis of retinal ganglion cells. The usual presentation is that of a young male with a sequential reduction in visual acuity. OCT has been used to study the pattern of optic nerve involvement in LHON, showing early thickening of the inferior and superior retinal nerve fibre layer and ganglion cell layer thinning corresponding with the onset of symptoms. Of the three primary mutations for LHON, the m.14484T>C mutation has the best visual prognosis. Recent emerging therapeutic options for LHON include idebenone and the introduction of genetic vector therapy, which is currently in phase III clinical trials. Screening of family members and adequate advice to avoid environmental triggers, such as smoking and alcohol consumption, are also cornerstones in the management of LHON.

Background Non-arteritic ischemic optic neuropathy (NAION) is the most common acute optic neuropathy over the age of 50 years. NAION is commonly associated with systemic vascular risk factors (diabetes, hypertension, hyperlipidemia) and small cup-to-disc-ratio. We have assessed the prevalence risk factors of NAION and the visual outcome in patients referred to a tertiary ophthalmology center in Kuwait. Materials and methods A retrospective review of new cases of NAION presenting within 2 weeks of onset were included and baseline clinical and demographics characteristic were determined. The prevalence of risk factors and the visual outcome (change in logMAR visual acuity, mean deviation of visual field) was compared between young NAION patients (below 50 years of age) and older NAION patients (over 50 years of age). The odds ratio of a final favorable visual outcome (visual acuity 20/40 or better) by age category was determined. Results Seventy-eight eyes of 78 patients with recent o...

Invasive fungal sinusitis can lead to dramatic complications in immunocompromised patients and requires prompt diagnosis. Here we report three cases with ophthalmological complications secondary to invasive fungal sinusitis in immunocompromised patients. From an ophthalmological point of view, these cases illustrate different clinical presentations, evolutions, complications, treatments, prognoses, and highlight different pathophysiological mechanisms. Diagnoses were delayed in all cases. In none of the cases did patients recover better vision than counting fingers at 24 months follow up, and two patients died. This case series highlights key points useful for quickly recognising this highly morbid infection in immunocompromised patients.

Au cours de la sarcoïdose, l'atteinte ophtalmologique touche 25 à 60 % des patients, mais l'oedème papillaire est une manifestation rare de la maladie. Exégèse. -Un homme de 43 ans a présenté une baisse brutale de l'acuité visuelle de l'oeil gauche en rapport avec un oedème papillaire. Malgré un traitement corticoïde à fortes doses, le déficit visuel s'est rapidement devenu bilatéral. Le scanner thoracique a mis en évidence une atteinte médiastinopulmonaire. L'examen histologique des biopsies pulmonaire, bronchique et ganglionnaire montra un granulome gigantocellulaire sans nécrose caséeuse. Le diagnostic de neuropathie optique sarcoïdosique fut retenu. Un traitement associant prednisone, méthotrexate et infliximab fut instauré, mais n'a pas permis d'amélioration notable de l'acuité visuelle. Conclusion. -Cette observation souligne la gravité potentielle des atteintes du nerf optique au cours de la sarcoïdose.

Geographic atrophy (GA) affects around 5 million individuals worldwide. Genome-wide, histopathologic, in vitro and animal studies have implicated the activation of the complement system and chronic local inflammation in the pathogenesis of GA. Recently, clinical trials have demonstrated that an intravitreal injection of pegcetacoplan, a C3 inhibitor, and avacincaptad pegol, a C5 inhibitor, both statistically significantly reduce the growth of GA up to 20% in a dose-dependent fashion. Furthermore, the protective effect of both pegcetacoplan and avacincaptad appear to increase with time. However, despite these anatomic outcomes, visual function has not improved as these drugs appear to only slow down the degenerative process. Unexpected adverse events included conversion to exudative NV-AMD with both drugs. Occlusive retinal vasculitis and anterior ischemic optic neuropathy have been reported in pegcetacoplan-treated eyes.

Leber hereditary optical neuropathy (LHON) is part of the class of optic neuropathies in which the mitochondrial function is impaired and is characterized by a painless, subacute, bilateral decrease of the central vision. We shall present the case of two brothers AM aged 31 and AT aged 40 who were diagnosed with LHON and whom we initiated treatment with idebenone 900 mg / day with monitoring at one month and 6 months. The mitochondrial DNA analysis demonstrated the existence of mutations 11778G>A for the mtND4 gene in both patients. Idebenone is a synthetic benzoquinone, analogue of ubiquinone. We found a slight but significant improvement in the visual field in patient AM at one month of treatment. We have not found another case in the literature with an improvement in vision so fast after this treatment, and this has led us to write this article. Keywords: Leber hereditary optical neuropathy (LHON), idebenone, mutations 11778G>A, mtND4 gene

The photopic negative response (PhNR) is a slow negative component of a flash photopic full-field ERG that has been shown to be specific for retinal ganglion cell (RGC) activity. Direct evaluation of RGC function is desirable in patients with Leber's hereditary optic neuropathy (LHON) in which the loss of central acuity can make it difficult to monitor patients with standard metrics. The purpose of this study was to evaluate the use of PhNR as an objective noninvasive clinical metric in LHON. Full-field photopic ERG recordings were collected in subjects with the mt.11778G>A/ND4 LHON mutation using a red on blue stimulus. The PhNR was identified using a computer-based automated detection system, and data were manually examined to remove movement artifacts. The PhNR amplitude was compared between controls (n = 13), carriers (n = 17), and affected (n = 6). Mean PhNR amplitude decreased significantly across groups (P < 0.0001). Post hoc Tukey's test revealed a significant ...

Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease, associated with mitochondrial DNA (mtDNA) point mutations and characterized by bilateral, usually sequential, rapid loss of central vision. The purpose of this study was to investigate electrophysiologically a small cohort of members from an extensive Brazilian family affected by LHON. Pattern-reversal visual evoked potentials (PVEP), and full-field electroretinograms (ERG) were performed on the four index members, all carrying the 11778 homoplasmic mtDNA mutation. They were a 14-year-old recently affected male, his unaffected mother, and her two affected brothers. The three affected members all had bilateral profound visual loss with visual acuities that ranged from 20/250 to CF, cecocentral defects, and severe dyschromatopsia (by FM-100). The unaffected (carrier) female had normal visual acuities, visual fields and color discrimination. Severely prolonged P100 latencies and decreased N75-P100 peak amplitudes were found in pattern-reversal VEPs for three affected members. Normal PVEP responses were found in the carrier female. Rod and cone ERG responses were normal in two affected members, but both the carrier mother and her affected son showed reduced peak-to-peak amplitude for single-flash cone response and 30 Hz flicker, with normal b-wave implicit times. Thus, optic nerve function, evaluated by PVEP, was severely reduced in LHON affected members and normal in the carrier female. However, reduced ERG cone responses suggest that LHON can also affect retinal elements, even in the absence of fundus and other clinical changes that constitute the full and classical expression of LHON.

Glaucoma is a leading cause of blindness worldwide, characterised by specific visual field defects due to the degeneration of retinal ganglion cells and damage to the optic nerve head (ONH). Elevated intraocular pressure (IOP) is the most important risk factor for glaucoma development. One of the clinical hallmarks of glaucomatous optic neuropathy is the excavation of the ONH, which consists of a progressive posterior displacement of the ONH surface and excavation of the pre-laminar tissues beneath the anterior-most aspect of the scleral canal, known as the anterior scleral ring. Radial optic neurotomy (RON) is a surgical technique that has been proposed for treating central retinal vein occlusion. While the original rationale of RON was the relief of increased tissue pressure within the optic nerve that results from occlusion of the central retinal vein, recent results are discussed here which suggest that by relaxing of the scleral ring of the prelaminar and laminar regions of the ONH, RON may alleviate the IOP-related connective tissue stress, and in turn, prevent the onset and reduce the progression of glaucomatous neuropathy.

Objective: To determine management patterns among clinicians who treat patients with Graves’ orbitopathy (GO) in Europe. Design and methods: Questionnaire survey including a case scenario of members of professional organisations representing endocrinologists, ophthalmologists and nuclear medicine physicians. Results: A multidisciplinary approach to manage GO was valued by 96.3% of responders, although 31.5% did not participate or refer to a multidisciplinary team and 21.5% of patients with GO treated by responders were not managed in a multidisciplinary setting. Access to surgery for sight-threatening GO was available only within weeks or months according to 59.5% of responders. Reluctance to refer urgently to an ophthalmologist was noted by 32.7% of responders despite the presence of suspected optic neuropathy. The use of steroids was not influenced by the age of the patient, but fewer responders chose to use steroids in a diabetic patient (72.1 vs 90.5%, P < 0.001). Development...

Background: This study was performed to determine clinical features of dysthyroid optic neuropathy (DON) across Europe. Methods: Forty seven patients with DON presented to seven European centres during one year. Local protocols for thyroid status, ophthalmic examination and further investigation were used. Each eye was classified as having definite, equivocal, or no DON. Results: Graves' hyperthyroidism occurred in the majority; 20% had received radioiodine. Of 94 eyes, 55 had definite and 17 equivocal DON. Median Clinical Activity Score was 4/7 but 25% scored 3 or less, indicating severe inflammation was not essential. Best corrected visual acuity was 6/9 (Snellen) or worse in 75% of DON eyes. Colour vision was reduced in 33 eyes, of which all but one had DON. Half of the DON eyes had normal optic disc appearance. In DON eyes proptosis was . 21 mm (significant) in 66% and visual fields abnormal in 71%. Orbital imaging showed apical muscle crowding in 88% of DON patients. Optic nerve stretch and fat prolapse were infrequently reported. Conclusion: Patients with DON may not have severe proptosis and orbital inflammation. Optic disc swelling, impaired colour vision and radiological evidence of apical optic nerve compression are the most useful clinical features in this series.

The objective of this study was to investigate the elasticity characteristics of the optic nerve using strain and shear wave elastography in patients with Behc ¸et's disease and to compare the results with those of healthy volunteers. Forty-six optic nerves from patients with Behc ¸et's disease and 54 optic nerves from healthy volunteers were investigated prospectively in this study using strain and shear wave elastography. There was a statistically significant difference in terms of elasticity patterns between patients and healthy volunteers (p , 0.001). Elastographic images of healthy volunteers revealed most optic nerves to be type 3 (51.8%); however, type 2 (40.7%) and type 1 (7.5%) were also observed. Elastographic examination of Behc ¸et's disease patients revealed type 2 in 52.2%, type 1 in 43.5% and type 3 in 4.3% of patients. Statistically significant differences were observed between patients and healthy volunteers in the analysis of shear wave elastography values (p , 0.001). Receiver operating characteristic curve analysis was perfect (0.933) (95% CI 5 0.885-0.980), and a cutoff value of 16.5 kPa shear had very high sensitivity and specificity for the patient group. Strain and shear wave elastography findings for the optic nerves of patients with Behc ¸et's disease were significantly different from those for healthy volunteers.

Weread with interest the article by Yamamoto(1) and appreciate the commentsconcerning our previous case report (2). Weagree that a large dose of thiamine (300 mg/day parenteral) should be started immediately, whenever Wernicke's encephalopathy (WE) is suspected. Werecently encountered a thiamine-deficient patient, who suffered from optic neuropathy (ON) in addition to nystagmus and 6th nerve palsy.

Craniosynostosis (CS) or the premature fusion of one or more cranial sutures in utero, or during the first years of life, can present in isolation or as a multisystem clinical disorder with a particular impact on visual function. Among ophthalmic complications, optic neuropathy is a significant cause of irreversible vision loss in these patients. Children with CS are at higher risk of developing elevated intracranial pressure which can lead to papilledema and, ultimately, optic atrophy. In addition, sometimes associated obstructive sleep apnea, abnormalities in central nervous system venous development, and Chiari malformation may contribute to optic neuropathy. Ophthalmologists have an important role in managing a number of coexistent ophthalmologic complications such as strabismus, anisometropia, amblyopia, ptosis, and exposure keratopathy in addition to maintaining surveillance for early signs of optic neuropathy; they play a critical consultative role contributing to the decision for primary or repeat decompressive surgery. In this article, we aim to review the etiology, diagnostic approach, and management of optic neuropathies in patients with craniosynostosis.

To report the long-term outcome of patients with indirect traumatic optic neuropathy (TON) which showed useful vision for a short period after trauma. METHODS. A cohort of 12 TON patients treated with steroids megadose immediately after trauma was followed every 6 months for an overall period of 5 years. Other than a full neuro-ophthalmologic examination, each visit included quantitative Goldmann perimetry and pattern reversal visual evoked potentials. The results of each examination were compared with the visual function at baseline. The main outcome measures were visual acuity and visual field. Data were analyzed using the Wilcoxon signed-rank test. A p value of less than 0.05 was considered statistically significant. RESULTS. All patients showed a stable visual function 5 years after optic nerve trauma. There was no difference in visual acuity levels (p=0.65) and no visual field surface area between the visit at baseline and the last follow-up. However, a significant improvement in visual field extension (p=0.036) was observed after perimetry evaluation. CONCLUSIONS. This cohort of patients clearly demonstrates that the residual visual function found in the short term after TON is maintained for at least 5 years. These findings add further important clinical information for patients with TON. Furthermore, these data may be helpful to better quantify morbidity related to optic nerve trauma and its permanent sequelae.

To describe the MR characteristics of optic neuropathy caused by vasculitis. Nine cases of optic neuropathy with diagnosis of vasculitis (six with systemic lupus erythematosis and one each with rheumatoid arthritis, Sjögren disease, and radiation vasculitis) were reviewed retrospectively. Patients were 31 to 62 years old, and all but one were women. All patients had MR imaging through the orbits and anterior visual pathways, five with fat suppression, with and without gadopentetate dimeglumine. Five patients also had MR imaging of the entire brain. The size and enhancement of various segments of the optic nerve and anterior visual pathways were studied. MR imaging with contrast material showed enhancement and enlargement of segments of the optic nerves and/or chiasm in six of the nine patients (all but three with systemic lupus erythematosis). Enlargement of a segment of the anterior visual pathway never occurred without enhancement, but enhancement alone did occur in three cases. O...

Optic neuritis frequently occurs in multiple sclerosis (MS), and shares several similarities with the optic neuritis of Leber's hereditary optic neuropathy (LHON), which is mainly due to maternally transmitted mitochondrial DNA (mtDNA) mutations. Our report shows for the first time that a mitochondrial DNA background could influence the clinical expression of MS. One European mtDNA haplogroup was found only in MS patients with optic neuritis but not in MS patients without visual symptoms. Therefore, we hypothesize that mtDNA haplogroup J might constitute a risk factor for optic neuritis occurrence when it is coincidentally associated with MS, but not be a risk factor for developing MS per se as suggested previously.

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Background: Wolfram Syndrome type 2 (WFS2) is considered a phenotypic and genotypic variant of WFS, whose minimal criteria for diagnosis are diabetes mellitus and optic atrophy. The disease gene for WFS2 is CISD2. The clinical phenotype of WFS2 differs from WFS1 for the absence of diabetes insipidus and psychiatric disorders, and for the presence of bleeding upper intestinal ulcers and defective platelet aggregation. After the first report of consanguineous Jordanian patients, no further cases of WFS2 have been reported worldwide. We describe the first Caucasian patient affected by WFS2. The proband was a 17 year-old girl. She presented diabetes mellitus, optic neuropathy, intestinal ulcers, sensorineural hearing loss, and defective platelet aggregation to ADP. Genetic testing showed a novel homozygous intragenic deletion of CISD2 in the proband. Her brother and parents carried the heterozygous mutation and were apparently healthy, although they showed subclinical defective platelet aggregation. Long runs of homozygosity analysis from SNP-array data did not show any degree of parental relationship, but the microsatellite analysis confirmed the hypothesis of a common ancestor. Our patient does not show optic atrophy, one of the main diagnostic criteria for WFS, but optic neuropathy. Since the "asymptomatic" optic atrophy described in Jordanian patients is not completely supported, we could suppose that the ocular pathology in Jordanian patients was probably optic neuropathy and not optic atrophy. Therefore, as optic atrophy is required as main diagnostic criteria of WFS, it might be that the so-called WFS2 could not be a subtype of WFS. In addition, we found an impaired aggregation to ADP and not to collagen as previously reported, thus it is possible that different experimental conditions or inter-patient variability can explain different results in platelet aggregation. Further clinical reports are necessary to better define the clinical spectrum of this syndrome and to re-evaluate its classification.

Three prevalent mitochondrial DNA pathogenic mutations at positions 11778, 3460, and 14484, which affect different subunits of Complex I, cause retinal ganglion cell death and optic nerve atrophy in Leber's hereditary optic neuropathy (LHON). The cell death is painless and without inflammation, consistent with an apoptotic mechanism. We have investigated the possibility that the LHON mutation confers a pro-apoptotic stimulus and have tested the sensitivity of osteosarcoma-derived cybrid cells carrying the most common and severe mutations (11778 and 3460) to cell death induced by Fas. We observed that LHON cybrids were sensitized to Fas-dependent death. Control cells that bear the same mitochondrial genetic background (the J haplogroup) without the pathogenic 11778 mutation are no more sensitive than other controls, indicating that increased Fas-dependent death in LHON cybrids was induced by the LHON pathogenic mutations. The type of death was apoptotic by several criteria, including induction by Fas, inhibition by the caspase inhibitor zVAD-fmk (zVal-Ala-Asp-fluoro-methyl ketone), activation of DEVDase activity (Asp-Glu-Val-Asp protease), specific cleavage of caspase-3, DNA fragmentation, and increased Annexin-V labeling. These data indicate that the most common and severe LHON pathogenic mutations 11778 and 3460 predispose cells to apoptosis, which may be relevant for the pathophysiology of cell death in LHON, and potential therapy.

To establish the clinical profile of simultaneous bilateral optic neuritis in adults, the efficacy of steroid therapy, extent of visual recovery, and neurological outcome. Methods: The authors performed a retrospective review of records of patients referred to a neuro-ophthalmology service with acute bilateral optic neuritis from 2000-4. Exclusion criteria included previous multiple sclerosis or myelopathy, known systemic disorders or medications associated with optic neuropathy, uveitis, or neoplasm. Patients received intravenous methylprednisolone followed by oral prednisone. Visual acuity (logMAR conversion), mean deviation (dB) for visual fields, percentage of Ishihara plates seen, ophthalmoscopy, and neurological evaluation were recorded at baseline and at 6 months or 12 months. Owing to strong correlation for visual loss between eyes, the results for the worse eye in each patient were analysed. Results: 11 men and four women, with an age range of 18-64 years, had bilateral decreased vision, 12 with pain on eye movement. Except for one patient, no aetiology was found. All patients had normal neurological evaluations, average visual acuity 1.71 (SD 0.55), colour vision 2.7% (SD 9.9%), and mean deviation 225.35 dB (SD 27.95 dB). Both optic nerves showed abnormal signal on magnetic resonance imaging. 14 patients improved and their last average visual acuity, colour vision, and mean deviation were 0.36 (SD 0.54), 69% (SD 46%), and 27.05 dB (SD 8.40 dB), respectively. No patient developed a neurological problem during the follow up with a mean of 11 months. Conclusion: Idiopathic acute bilateral optic neuritis without myelopathy occasionally occurs in adults. Vision recovers with corticosteroid therapy and during the first year neurological dysfunction will frequently not occur.

To establish the clinical profile of simultaneous bilateral optic neuritis in adults, the efficacy of steroid therapy, extent of visual recovery, and neurological outcome. Methods: The authors performed a retrospective review of records of patients referred to a neuro-ophthalmology service with acute bilateral optic neuritis from 2000-4. Exclusion criteria included previous multiple sclerosis or myelopathy, known systemic disorders or medications associated with optic neuropathy, uveitis, or neoplasm. Patients received intravenous methylprednisolone followed by oral prednisone. Visual acuity (logMAR conversion), mean deviation (dB) for visual fields, percentage of Ishihara plates seen, ophthalmoscopy, and neurological evaluation were recorded at baseline and at 6 months or 12 months. Owing to strong correlation for visual loss between eyes, the results for the worse eye in each patient were analysed. Results: 11 men and four women, with an age range of 18-64 years, had bilateral decreased vision, 12 with pain on eye movement. Except for one patient, no aetiology was found. All patients had normal neurological evaluations, average visual acuity 1.71 (SD 0.55), colour vision 2.7% (SD 9.9%), and mean deviation 225.35 dB (SD 27.95 dB). Both optic nerves showed abnormal signal on magnetic resonance imaging. 14 patients improved and their last average visual acuity, colour vision, and mean deviation were 0.36 (SD 0.54), 69% (SD 46%), and 27.05 dB (SD 8.40 dB), respectively. No patient developed a neurological problem during the follow up with a mean of 11 months. Conclusion: Idiopathic acute bilateral optic neuritis without myelopathy occasionally occurs in adults. Vision recovers with corticosteroid therapy and during the first year neurological dysfunction will frequently not occur.

Purpose: While the literature supports the use of radiation therapy for thyroid eye disease, it does not sufficiently describe in detail the results of radiation therapy for optic neuropathy associated with thyroid eye disease. The objective of this study is to quantify the changes in parameters of optic neuropathy after orbital irradiation for thyroid eye disease. Methods and Materials: Twelve consecutive patients with optic neuropathy from thyroid eye disease were followed by a single neuro-ophthalmology practice and treated by one radiation oncologist with radiation therapy from 1991 through 1995. All cases were prospectively followed for visual acuity, color vision, mean deviation, and/or foveal sensitivity and afferent pupillary defect. All patients received 2000 cGy in 10 fractions with megavoltage irradiation to the orbits. Results: Ten of 12 patients were evaluated for follow-up (one moved out of this country and one had a stroke, which confounded interpretation of examination results). An analysis was performed retrospectively while treatment and evaluation remained uniform. Five men and five women formed the basis of this study with a median age of 60 years (35-76 years). Nineteen eyes were evaluated for thyroid optic neuropathy. Improvement in optic nerve function occurred in eight of ten patients. Improvement was seen either during radiotherapy or within 2 weeks of completion. No long-term adverse effects were noted. Conclusion: This study objectively demonstrates improvement in optic neuropathy from radiation therapy for thyroid eye disease.

Objective-Previous studies have identified a higher prevalence of diabetes mellitus (DM) among patient cohorts with non-arteritic anterior ischemic optic neuropathy (NAION). We sought to determine the development of incident NAION among a group of newly diagnosed patients with DM and to estimate the incidence of NAION among the elderly. Design-Medicare 5% database study. Participants-25,515 patients with DM and an equal number of age-and gender-matched nondiabetics. Methods-Query of Medicare 5% claims files identified patients with new diagnosis of DM in 1994. A randomly selected control group was created using one-to-one propensity score matching. Patients with a diagnosis of giant cell arteritis, pre-existing DM, and age < 68 years or > 95 years were excluded. Patients with DM and controls were followed for the development of NAION over the following 4,745 days. Results-Each group was 85% White, 11% Black, and 4% other race, aged 76.4 years, and 40% male with a mean followup time of 7.6 years. In the diabetes group, 188 individuals developed AION (0.7%) compared to 131 individuals (0.5%; p<0.01) in the control group. In unadjusted Cox regression analysis, having diabetes mellitus was associated with a 43% increased risk (Hazard ratio [HR]: 1.431; 95% confidence interval [CI]: 1.145,1.789) of developing AION. After adjusting for other covariates, the risk of developing AION among individuals with DM was reduced to 40% (HR: 1.397; 95% CI: 1.115,1.750). Male gender increased an individual's risk of developing AION by 32% (HR: 1.319; 95% CI: 1.052,1.654). No other covariate was statistically

Ethambutol is a commonly used anti-tuberculous medications. Ocular toxicity of ethambutol is well established. It needs to be detected early and medication stopped promptly to avoid irreversible damage. A 64-year-old lady was referred for gradual deterioration of her vision of both eyes. Examination and investigations confirmed bilateral toxic optic neuropathy secondary to ethambutol. The drug was stopped with immediate effect. Three months later, her distance vision and colour vision improved significantly. The course and outcome of ethambutol induced toxic optic neuropathy is unpredictable. Optic nerve head remains normal on Fundoscopy until late in the disease. Improvement of vision after discontinuation of ethambutol occurs only in a minority of patients. This case highlights the importance of ocular screening to detect toxic neuropathies early to possibly prevent permanent visual disability.

Background Noninvasive repetitive transorbital alternating current stimulation (rtACS) can improve visual field size in patients with optic nerve damage, but it is not known if this is of subjective relevance. We now assessed patient reported outcomes to determine the association between visual field changes and vision-related quality of life (QoL). Methods Patients having visual field impairments long after optic nerve damage (mean lesion age 5.5 years) were randomly assigned to a rtACS (n 5 24) or sham stimulation group (n 5 18). Visual fields and patient reported outcome measures (vision-related QoL: National Eye Institute Visual Function Questionnaire, NEI-VFQ and health-related QoL: Short Form Health Survey, SF-36) were collected before and after a 10-day treatment course with daily sessions of 20 to 40 minutes. The primary outcome measure was the percent change from baseline of detection ability (DA) in defective visual field sectors as defined by computer-based high resolution perimetry (HRP). Secondary outcome parameters included further HRP parameters as well as static and kinetic perimetry results. Changes in QoL measures were correlated with changes in primary and secondary outcome measures in both groups. Results DA increase in the defective visual field was significantly larger after rtACS (41.1 6 78.9%, M 6 SD) than after sham stimulation (13.6 6 26.3%), P , 0.05. While there was a significant increase of DA in the whole tested HRP visual field after rtACS (26.8 6 76.7%, P , 0.05), DA in sham-stimulation

The aim of this paper is to report clinically various cases of intracranial tumors in patients referred to glaucoma clinic for consultation. The secondary aim was to increase the awareness of intracranial tumors in atypical cases of glaucoma. We present the retrospective analysis of five patients referred to glaucoma clinic for consultation. Due to atypical course of the disease, in addition to standard glaucoma examinations, all patients had a neurologic full visual field, color vision, and MRI done. In all patients, intracranial malignancies were found, some patients underwent surgery of the lesions with consecutive clinical improvements. Interestingly, in some patients, coexisting glaucoma was diagnosed. Patients were selected deliberately to present a wide spectrum of possible clinical scenarios when glaucoma may be complicated by intracranial tumors. Sometimes, the relevance of intracranial tumors with respect to their influence on the clinical picture of the optic nerve cannot...

Acute pancreatitis is a pancreatic inflammatory disorder that often leads to multi-organ dysfunction associated with systemic inflammatory response. Optic neuropathy is an extremely rare ocular manifestation that can occur especially in alcoholic pancreatitis most likely due to ischemic complications and is a vision-threatening condition that has to be recognized early as it can cause permanent vision loss. In this case report, a 51-year-old lady, an occasional consumer of alcohol, post-cholecystectomy status, presented with severe abdominal pain of four days' duration associated with multiple episodes of vomiting. She was diagnosed with moderate to severe acute pancreatitis and needed ionotropic support initially. She had improvement in gastrointestinal symptoms. However, she had left peri-orbital pain and lacrimation with blurring of vision on Day 11 of illness. Subsequently, an ophthalmic evaluation revealed optic disc oedema and a mild decrease in visual acuity but normal visual field and colour vision. Therefore, left optic neuropathy was diagnosed and a high-dose oral steroid was started on a tapering dose. Follow-ups after four and 12 weeks showed significant improvement in optic disc oedema and other symptoms. Therefore, though optic neuropathy is rarely reported in acute pancreatitis, it has to be considered in clinical practice along with Purtscher-like retinopathy, which presents with ocular symptoms in acute pancreatitis.

Dear Editor I enjoyed reading the manuscript titled "Hooch blindness: a community study report on a few indoor patients of toxic optic neuropathy following consumption of adulterated alcohol in West Bengal" that has been published in your journal (Samanta et al, 2012). The authors reported ten cases of blindness due to methanol intoxication in West Bengal, India. After presentation of their patients' histories, ophthalmologic examinations, fundus photographs, and assessment of the functional condition of the optic nerve, they have described the treatment performed for their patients. Their treatment included intramuscular injection of hydoxycobalamine (a bolus of 1000 mg repeated after one week), administration of anti-oxidant tablet once daily with local neuro-protective agents for 3 weeks. Since they believed that this treatment resulted in partial recovery of visual disturbances (VD) in all but one of their patients, they recommended the physicians to treat all such patients using this protocol irrespective of their severity of visual disturbances and/or the time elapsed between methanol ingestion and hospital presentation. However, there are some major points worth mentioning about the general management of these methanol-intoxicated patients and the management performed for their VD, in particular. It is not clear why the physicians of the local hospital (District Hospital) have not properly managed the patients in acute stage of methanol intoxication. They have only administered intravenous (IV) infusion of sodium bicarbonate and absolute alcohol. As you know, in methanol intoxication, hemodialysis is absolutely indicated in any patient with VD of any degree accompanied by metabolic acidosis or a detectable methanol level in addition to the antidotal therapy (ethanol or fomepizole), administration of folic or folinic acid, and sodium bicarbonate if indicated (Hovda et al, 2004; Hovda et al, 2008; Sanaei-Zadeh et al, 2011; Shah et al, 2012). Also, interestingly, the authors have not mentioned anything about the type and dose of the administered anti-oxidant tablet and local neuro-protective agents that have suggested. To my knowledge, to date, no other published study has reported managing methanol-induced optic neuropathy in such a way. Instead, in addition to the successful management of a case of methanol-induced VD with prednisone and vitamin B1 (Rotenstreich et al, 1997), IV prednisolone for methanol-induced VD has been used in 6 other studies (36 cases), so far. This treatment has resulted in complete or incomplete recovery of VD in all but three of the patients (

The peak latency of the pattern-reversal visual evoked potential is a sensitive measure of conduction delay in the optic nerve caused by demyelination. Despite its clinical utility, the pattern-reversal visual evoked potential has not previously been used in multicenter clinical trials, presumably because of difficulty in standardizing conditions between centers. To establish whether the pattern-reversal visual evoked potential could be adequately standardized for use as a measure in multicenter therapeutic trials for optic neuropathy or multiple sclerosis, stimulus and recording variables were equated at four centers and pattern-reversal visual evoked potentials were recorded from 64 normal subjects and 15 patients with resolved optic neuritis. Results showed equivalent latency and amplitude data from all centers, suggesting that stimulus and recording variables can be satisfactorily standardized for multicenter clinical trials. N70 and P100 peak latencies and N70-P100 interocular amplitude difference were sensitive measures of resolved optic neuritis.