Cause Of Optic Neuropathy Research Articles

Glaucoma as a cause of optic nerve abnormalities on magnetic resonance imaging.

To report a series of patients with glaucoma and optic nerve abnormalities on magnetic resonance imaging (MRI) in at least one-eye, and to determine whether these findings correlate with the severity of glaucoma. Retrospective study of all patients who underwent a brain/orbits MRI without and with contrast at our institution between 07/1/2019-6/30/2022. Patients with optic nerve T2-hyperintensity and/or MRI optic nerve atrophy in at least one-eye and a diagnosis of isolated glaucoma in at least one-eye were included. Demographic information, glaucoma clinical characteristics, glaucoma severity parameters, and MRI indication were collected. Fifty-six patients (112 eyes) (age 65 years-old [range 26-88]; 70% male) had isolated bilateral glaucoma with at least one-eye MRI optic nerve abnormality. The indication for MRI was atypical/asymmetric glaucoma in 91% of patients. Of the 112 eyes, 23 had optic nerve T2-hyperintensity alone; 33 had both optic nerve T2-hyperintensity and MRI optic nerve atrophy; 34 had MRI optic nerve atrophy alone; and 22 did not have abnormal optic nerve MRI-findings. None had optic nerve enhancement. A statistically significant association between optic nerve T2-hyperintensity or MRI optic nerve atrophy and glaucoma severity parameters was found. Glaucoma is a clinical diagnosis and MRI brain is usually not required, except in atypical or asymmetric cases. Optic nerve T2-hyperintensity and MRI optic nerve atrophy are nonspecific MRI-findings that can be found in severe glaucomatous optic nerves and should not systematically prompt investigations for another cause of optic neuropathy.

Optic Nerve MRI T2-Hyperintensity: A Nonspecific Marker of Optic Nerve Damage.

MRI abnormalities are common in optic neuropathies, especially on dedicated orbital imaging. In acute optic neuritis, optic nerve T2-hyperintensity associated with optic nerve contrast enhancement is the typical imaging finding. In chronic optic neuropathies, optic nerve T2-hyperintensity and atrophy are regularly seen. Isolated optic nerve T2-hyperintensity is often erroneously presumed to reflect optic neuritis, frequently prompting unnecessary investigations and neuro-ophthalmology consultations. Our goal was to determine the significance of optic nerve/chiasm T2-hyperintensity and/or atrophy on MRI. Retrospective study of consecutive patients who underwent brain/orbital MRI with/without contrast at our institution between July 1, 2019, and June 6, 2022. Patients with optic nerve/chiasm T2-hyperintensity and/or atrophy were included. Medical records were reviewed to determine the etiology of the T2-hyperintensity and/or atrophy. Four hundred seventy-seven patients (698 eyes) were included [mean age 52 years (SD ±18 years); 57% women]. Of the 364 of 698 eyes with optic nerve/chiasm T2-hyperintensity without atrophy, the causes were compressive (104), inflammatory (103), multifactorial (49), glaucoma (21), normal (19), and other (68); of the 219 of 698 eyes with optic nerve/chiasm T2-hyperintensity and atrophy, the causes were compressive (57), multifactorial (40), inflammatory (38), glaucoma (33), normal (7), and other (44); of the 115 of 698 eyes with optic nerve/chiasm atrophy without T2-hyperintensity, the causes were glaucoma (34), multifactorial (21), inflammatory (13), compressive (11), normal (10), and other (26). Thirty-six eyes with optic nerve/chiasm T2-hyperintensity or atrophy did not have evidence of optic neuropathy or retinopathy on ophthalmologic examination, and 17 eyes had clinical evidence of severe retinopathy without primary optic neuropathy. Optic nerve T2-hyperintensity or atrophy can be found with any cause of optic neuropathy and with severe chronic retinopathy. These MRI findings should not automatically prompt optic neuritis diagnosis, workup, and treatment, and caution is advised regarding their use in the diagnostic criteria for multiple sclerosis. Cases of incidentally found MRI optic nerve T2-hyperintensity and/or atrophy without a known underlying optic neuropathy or severe retinopathy are rare. Such patients should receive an ophthalmologic examination before further investigations.

Clinical Etiology of Optic Neuropathy patients visiting BPKLCOS, Institute of Medicine, Nepal

Background & Objective: The clinical etiology of Optic neuropathy is vast and may be associated with life threatening conditions which might demand initiation of treatment. Therefore, the aim of the study was to evaluate clinical etiology among patients visiting ophthalmology OPD of Institute of medicine, Nepal.
 Material and Methods: This is a descriptive study conducted at B.P. Koirala Lions Centre for Ophthalmic Studies, Institute of Medicine, Nepal. All cases of optic neuropathy who presented to outpatient department (OPD), indoor patients from 7th July 2017-6th July 2018 were included in the study. A detailed clinical history was obtained which was followed by ophthalmic examination and relevant investigation. In addition, assessment of visual acuity, color vision, contrast sensitivity, visual field were done. Data was analyzed with SPSS version 24.
 Results: A total of 86 eyes of 66 patients with optic neuropathy meeting all the inclusion criteria during the study period were enrolled with 20 patients having bilateral eye involvement and 46 having unilateral eye involvement accounting for a total of 86 eyes with optic neuropathy. The mean age of the affected population was 39.12±13.57 years with male to female ratio of 1:1.1. The most common etiology for optic neuropathy was optic neuritis (n=40 patients, 60.6%). Diminution of vision was the most common presenting complaints. Best corrected visual acuity was 6/24 –6/60 in 39.5% cases (n=34). Majority of the eyes had presence of RAPD (n=53 eyes, 61.7%) The most common color vision defect was nonspecific defect (n=41eyes, 47.7%).There was reduced contrast in 55.8% of the affected eyes in cases of optic neuropathy. Majority of affected eyes had blurred disc margin (n=39 eyes, 45.3%) followed by hyperemic disc and pale disc. Majority of the patients could not perform visual field due to low vision but those who could perform had enlarged blind spot.
 Conclusion: The most frequent etiological factor for optic neuropathy was optic neuritis. In cases of optic neuropathy, the main complaint of the majority of patients was a dimunition in vision. The majority of cases of optic neuropathy involved abnormalities in both colour vision and contrast sensitivity. The most typical visual field pattern in eyes with optic neuropathy was an enlarged blind spot. Optic neuritis is the most common cause of optic neuropathy, followed by traumatic optic neuropathy, and toxic metabolic optic neuropathy.

Diagnostic Yield of Investigations in Symmetric Optic Neuropathy.

Symmetric optic neuropathy (SON) is commonly seen in neuro-ophthalmic practice and is often discovered incidentally. Although multiple investigations might be performed to discover the underlying cause, they are not always indicated. The aim of this study was to report a clinically reasonable and cost-effective approach to investigating patients with SON. SON was defined as bilateral optic neuropathy with normal and/or symmetrically decreased central visual acuity, absence of relative afferent pupillary defect, presence of symmetric optic disc pallor, symmetric thinning of peripapillary retinal nerve fiber layer on optical coherence tomography, and absence of other identifiable causes of optic neuropathy. Records of all patients diagnosed with SON seen at a tertiary university-affiliated neuro-ophthalmology practice from 2016 to 2022 were reviewed to identify the yield of various investigations. Clinical data from the initial and last follow-up visit were obtained. Subgroup analysis was performed to ascertain whether diagnostic yield is higher in patients with severe visual loss (central acuity worse than 20/40) compared with those with mild visual loss (acuity 20/40 or better). One hundred thirty-six patients met inclusion criteria. Testing for OPA1 and OPA2 mutations had the highest diagnostic yield (16.0%), followed by mitochondrial genome sequencing (13.6%), serum vitamin B12 (6.1%), and serum folate (1.6%). MRI brain was performed in 54.4% of patients and had a diagnostic yield of only 5%. Both patients who had abnormal MRI had symptoms of demyelination at presentation. Patients were followed for a mean of 15.0 (SD 21.3) months. The most frequently identified etiologies of SON were Leber hereditary optic neuropathy (8.1%), alcohol/tobacco amblyopia (7.4%), vitamin B12 deficiency (5.9%), and dominant optic atrophy (2.9%). Patients with severe visual impairment were more likely to have a final diagnosis compared with those with milder visual impairment (63.9% vs 12.0%, P < 0.001). The diagnostic yield of investigating SON in patients with preserved visual function, normal diet, and absence of other neurological symptoms is very low. It is reasonable to observe patients with SON with mild visual impairment, reserving costly investigations for those with the visual acuity worse than 20/40 or progressive course.

Neuropatía óptica en imagen

The aim of this work is to provide a diagnostic approach to the potential causes of optic neuropathy, focusing on the radiological findings associated with this pathology. Various etiologies have been identified, including inflammatory and demyelinating optic neuritis, developmental and hereditary diseases, neurodegenerative disorders, infectious conditions, post-traumatic causes, ischemic optic neuropathy (with anterior ischemic optic neuropathy being the most common form), and neoplastic etiologies. Optical coherence tomography and magnetic resonance imaging play a fundamental role in the diagnosis of optic neuropathy, allowing to distinguish patterns of optic nerve involvement. These studies are essential to locate and characterize the different pathologies, increasing the precision of the diagnosis in diseases presenting optic neuropathy as the main symptom. In conclusion, the findings obtained from magnetic resonance imaging are essential in the differential diagnosis of optic nerve diseases, aiding in the localization and characterization of various pathologies affecting either the optic pathway alone or multiple levels of the central nervous system and thereby increasing diagnostic accuracy.

MRI Signal Intensity Varies Along the Course of the Normal Optic Nerve.

MRI can help distinguish various causes of optic neuropathy including optic neuritis. Importantly, neuromyelitis optica spectrum disorder (NMOSD) has a propensity to cause enhancement of the prechiasmatic optic nerves. To determine whether the prechiasmatic optic nerve (PC-ON) demonstrates a different intensity from the midorbital optic nerve (MO-ON) on MRI among patients without optic neuropathy. Data were retrospectively obtained from 75 patients who underwent brain MRI for an ocular motor nerve palsy between January 2005 and April 2021. Inclusion criteria were patients aged 18 years or older with visual acuities of at least 20/25 and no evidence of optic neuropathy on neuro-ophthalmic examination. A total of 67 right eyes and 68 left eyes were assessed. A neuroradiologist performed quantitative intensity measurements of the MO-ON and PC-ON on precontrast and postcontrast T1 axial images. Normal-appearing temporalis muscle intensity was also measured and used as a reference to calculate an intensity ratio to calibrate across images. The mean PC-ON intensity ratio was significantly higher than the MO-ON intensity ratio on both precontrast (19.6%, P < 0.01) and postcontrast images (14.2%, P < 0.01). Age, gender, and laterality did not independently affect measurements. The prechiasmatic optic nerve shows brighter intensity ratios on both precontrast and postcontrast T1 images than the midorbital optic nerve among normal optic nerves. Clinicians should recognize this subtle signal discrepancy when assessing patients with presumed optic neuropathy.

Leukemic Optic Neuropathy

Background: Leukemic infiltration of the optic nerve is a neuro-oncologic emergency and also a sign of extramedullary central nervous system relapse. It presents a clinical dilemma in the early stages due to multiple differentials. Patients with leukemia receive radiation and chemotherapy are thus, susceptible to inflammatory, toxic, and infectious causes of optic neuropathy, besides infiltration with tumor cells. Clinical Description: A 15-year-old boy treated for acute lymphoblastic leukemia (ALL) and in remission for 7 months, presented with the unilateral decreased vision for 7 days. A structured evaluation was done, comprising visual acuity, color vision, field of vision, fundus, ophthalmoscopy, ultrasound b-scan, and magnetic resonance imaging of the orbit. The final diagnosis was leukemic infiltration of the optic nerve. Cerebrospinal fluid (CSF) analysis confirmed the presence of atypical lymphocytes. Management: The patient was diagnosed with extramedullary relapse of ALL. Since there are no standard guidelines, a literature review was performed, and the treating team decided to start the patient on stand-alone chemotherapy. Symptomatic resolution became apparent within 10 days. On follow-up, the optic nerve lesion resolved with residual gliosis in the surrounding retina. The CSF has become clear, and the patient is now considered to be in remission. Conclusion: It is important to use a structured clinical approach coupled with investigations to recognize the ocular involvement of ALL (especially in younger patients). There is a need for a regular routine ophthalmological examination in patients in remission for early detection of a relapse. There is a strong felt need for pediatric hemato-oncologists to plan research in this area to generate data so that recommendations for the management of extramedullary relapses are formulated.

Approach to the diagnosis and management of nutritional optic neuropathies.

Nutritional deficiency is an under-recognized cause of optic neuropathy. The purpose of this review is to discuss how to identify, diagnose, and appropriately manage patients with nutritional optic neuropathy. Nutritional deficiencies have long been thought to be more prevalent in the developing countries. However, with the advent of bariatric surgery, restrictive/selective diets, and the increase in alcohol dependence, it is not uncommon to see nutritional optic neuropathies in the developed world. Although nutritional optic neuropathy can cause severe and debilitating vision loss, it is often reversible when it is diagnosed and treated in a timely manner.

Factors Associated With Visual Acuity in Non-arteritic Ischemic Optic Neuropathy Patients: A Five-Year Cross-Sectional Study.

Background and objectiveNon-arteritic ischemic optic neuropathy (NAION) is a common cause of optic neuropathy in elderly patients. Currently, there is no definitive treatment for this condition, and the factors influencing visual outcomes have not yet been conclusively identified. In this study, we aimed to evaluate factors that affect visual outcomes and those that are predictors of the development of NAION in a Thai population.MethodsAll patients diagnosed with NAION at the Rajavithi Hospital between January 1, 2016, and December 31, 2020, were retrospectively reviewed to evaluate the improvement in their best-corrected visual acuity (BCVA) and determine the factors that are predictive of visual outcomes.ResultsThe 80 patients reviewed were predominantly male (55%) with a mean age of 55.8 ±9.89 years. Their most common comorbidities were dyslipidemia (DLP) (67.5%), diabetes mellitus (DM) (61.3%), and hypertension (HT) (48.8%). At the 12-week follow-up visit, there was a significant improvement of at least 0.2 logarithm of the minimum angle of resolution (logMAR) in BCVA (p=0.001). A significantly greater percentage of patients with higher age, DM, and HT was observed in the unfavorable visual recovery (UVR) group (p=0.002, p=0.001, and p=0.005 respectively). In contrast, neither baseline visual acuity nor cup-to-disc ratio (CDR) affected the result of visual recovery (p=0.275 and p=0.076, respectively). In multivariate logistic analysis, older age increased the odds of worse visual recovery [odds ratio (OR): 4.014; 95% CI: 1.038-15.515; p=0.044], as did having DM (OR: 3.809; 95% CI: 1.168-12.421; p=0.027), and HT (OR: 4.577; 95% CI: 1.491-14.049; p=0.008).ConclusionsNone of the baseline visual status parameters (visual acuity, CDR, or visual field defect) was able to determine the outcome of visual recovery at 12 weeks in our NAION patients. Regarding systemic vascular diseases, diabetes and HT are significant risk factors and also predictors of poor visual improvement in Thai populations. NAION patients who are elderly or have vascular diseases such as DM or HT should be closely followed up and advised about the likelihood of having inferior visual recovery.

Vitamin B12 in Leber hereditary optic neuropathy mutation carriers: a prospective cohort study

BackgroundLeber hereditary optic neuropathy (LHON) is the most common mitochondrial disorder, frequently resulting in acute or subacute severe bilateral central vision loss. Vitamin B12 deficiency is also a known cause of optic neuropathy through mitochondrial dysfunction. Here we evaluated the prevalence and clinical significance of vitamin B12 deficiency in a large cohort of LHON patients and asymptomatic mutation carriers from a tertiary referral center.MethodsFrom the Munich LHON prospective cohort study, participants included all LHON patients and asymptomatic LHON mutation carriers, who were recruited between February 2014 and March 2020 and consented to participate. Neurological, general, and ophthalmological examinations were regularly performed, as were laboratory tests. Vitamin B12 deficiency was diagnosed if serum vitamin B12 was below 201 pg/mL, or if 201–339 pg/mL plus low serum holotranscobalamin or elevated serum methylmalonic acid or elevated total plasma homocysteine.ResultsWe analyzed 244 subjects, including 147 symptomatic LHON patients (74% males) and 97 asymptomatic mutation carriers (31% males). Median age at study baseline was 34 years (range 5–82 years). The prevalence of vitamin B12 deficiency was higher for LHON mutation carriers than for the general population in all age categories. This was statistically significant for the LHON mutation carriers under 65 years (21% vs. 5–7%, p = 0.002). While vitamin B12 deficiency prevalence was not statistically different between LHON patients and asymptomatic mutation carriers, its clinical correlates, e.g., macrocytosis and polyneuropathy, were more frequent in the subgroup of LHON patients. Excessive alcohol consumption was a significant predictor of vitamin B12 deficiency (p < 0.05).ConclusionsThe high prevalence of vitamin B12 deficiency in LHON mutation carriers, both asymptomatic mutation carriers and LHON patients, highlights the need for regular vitamin B12 screening in this population, in order to ensure early treatment, aiming for better outcomes. Our study is not conclusive regarding vitamin B12 deficiency as determinant for disease conversion in LHON, and further research is warranted to disentangle the role of vitamin B12 in the pathophysiology and prognosis of LHON.

Optic nerve neuropathies – causative factors, methods of diagnostics, current and future possibilities of treatment

Introduction: Optic neuropathies are a broad group of diseases in which the dominant disturbance is damage to the optic nerve, often irreversible. Underlying causes of neuropathy are both genetic and environmental. Despite limited treatment options, much research is currently being done on substances that could improve optic nerve function and alleviate the clinical consequences of optic nerve damage.&#x0D; Purpose: This article describes current findings in both the optic nerve neuropathy pathophysiology and diagnosis of the disorder, as well as treatment options and future perspectives.&#x0D; State of knowledge: The pathogenetic cause of neuropathy is mainly demyelination within the neural sheath, often caused by inflammation. It is characterized by progressive loss of vision. The most common genetic cause of optic neuropathy is mitochondrially inherited Leber hereditary optic neuropathy (LHON) and is characterized by mostly sudden and painless loss of visual acuity. Toxic neuropathies are a group of diseases caused by heavy metals, pharmaceuticals, methanol and carbon monoxide. Nutritional neuropathy is mainly related to vitamin B1, B9 and B12 deficiency, and is a rare example of neuropathy that can be curable at the early stage of the disease. Another group of neuropathies is caused by ischemia and can be divided according to the place of the optic nerve affected – AION (anterior ischemic optic neuropathy) and PION (posterior ischemic optic neuropathy).&#x0D; Conclusions: The therapeutic options in the treatment of optic neuropathy strictly depend on the causative factor. Nutritional deficiencies are treated with appropriate supplementation, so it is vital to truly determine the missing vitamins and elements.

Toxic and Nutritional Optic Neuropathies-An Updated Mini-Review.

Optic neuropathies constitute a group of conditions with various etiologies and might be caused by different factors; we can distinguish the genetic and acquired causes of optic neuropathies. Even though the symptoms are not highly specific, this condition is primarily characterized by unilateral or bilateral vision loss with worsening color detection. The loss may be acute or gradual depending on the causation. In this article, we included a specification of toxic optic neuropathy (TON) mainly triggered by alcohol abuse and also the usage of other substances, including drugs or methanol, as well as intoxication by metals, organic solvents, or carbon dioxide. Nutritional deficiencies, vitamin absorption disorder, and anemia, which usually appear during excessive alcohol intake, and their effect on the etiology of the optic neuropathy have been likewise discussed.

Anomalous Course of the Internal Carotid Artery Resulting in Optic Nerve Compression

Compression of the optic nerve by the internal carotid artery can occur due to their proximity as they exit the cavernous sinus. This report details a case of unilateral compressive optic neuropathy resulting from an anomalous course of the internal carotid artery. It presents optical coherence tomography scans, Humphrey visual field results, and magnetic resonance images, as well as reviews other causes of optic neuropathy that can have a similar clinical presentation.

The Usefulness of the Erythrocyte Sedimentation Rate and C-reactive Protein for the Differential Diagnosis of Non-Arteritic Anterior Ischemic Optic Neuropathy in the Era of Microinflammation

ABSTRACT Purpose The presence of a microinflammatory response is one of the possible pathological mechanisms related to the development of nonarteritic anterior ischemic optic neuropathy (NAAION), a common cause of optic neuropathy in old age. We tested whether individuals with NAAION harbor a heightened microinflammatory response compared to controls. Methods We measured the erythrocyte sedimentation rate (ESR) and high sensitivity C-reactive protein (hs-CRP) in NAAION patients during hospital admission and in four matched controls for each patient, retrieved from a large cohort of 20,000 apparently healthy individuals. Results We included 128 NAAION patients and 512 controls. No significant differences were found between patients and controls regarding the inflammatory biomarkers. Conclusions This is the first report showing a lack of difference in ESR and hs-CRP levels between NAAION patients and matched controls, suggesting NAAION is not associated with a heightened inflammatory response, such as the one associated with multiple atherothrombotic risk factors.

Tacrolimus-Related Optic Neuropathy

Optic neuropathy has a broad differential diagnosis, including demyelinating disease, infection, and inflammatory etiologies such as sarcoidosis, radiation, and drug toxicity. Tacrolimus is a rare cause of optic neuropathy, but radiologists can benefit patients by suggesting the diagnosis. We report a case of tacrolimus optic neuropathy initially suggested on the basis of MR imaging findings. The patient improved clinically and had near resolution of MR imaging findings after treatment, which has not been previously reported.

ATPase Domain AFG3L2 Mutations Alter OPA1 Processing and Cause Optic Neuropathy.

ObjectiveDominant optic atrophy (DOA) is the most common inherited optic neuropathy, with a prevalence of 1:12,000 to 1:25,000. OPA1 mutations are found in 70% of DOA patients, with a significant number remaining undiagnosed.MethodsWe screened 286 index cases presenting optic atrophy, negative for OPA1 mutations, by targeted next generation sequencing or whole exome sequencing. Pathogenicity and molecular mechanisms of the identified variants were studied in yeast and patient‐derived fibroblasts.ResultsTwelve cases (4%) were found to carry novel variants in AFG3L2, a gene that has been associated with autosomal dominant spinocerebellar ataxia 28 (SCA28). Half of cases were familial with a dominant inheritance, whereas the others were sporadic, including de novo mutations. Biallelic mutations were found in 3 probands with severe syndromic optic neuropathy, acting as recessive or phenotype‐modifier variants. All the DOA‐associated AFG3L2 mutations were clustered in the ATPase domain, whereas SCA28‐associated mutations mostly affect the proteolytic domain. The pathogenic role of DOA‐associated AFG3L2 mutations was confirmed in yeast, unraveling a mechanism distinct from that of SCA28‐associated AFG3L2 mutations. Patients' fibroblasts showed abnormal OPA1 processing, with accumulation of the fission‐inducing short forms leading to mitochondrial network fragmentation, not observed in SCA28 patients' cells.InterpretationThis study demonstrates that mutations in AFG3L2 are a relevant cause of optic neuropathy, broadening the spectrum of clinical manifestations and genetic mechanisms associated with AFG3L2 mutations, and underscores the pivotal role of OPA1 and its processing in the pathogenesis of DOA. ANN NEUROL 2020 ANN NEUROL 2020;88:18–32

Randomized control trial of evaluation of Clemastine effects on visual evoked potential, nerve fiber layer and ganglion cell layer complex in patients with optic neuritis

ObjectivesOptic neuritis (ON) is the most common cause of optic neuropathy; typically presenting with a unilateral visual loss in young adults, with incidence of 1–5 in 100,000 per year. We evaluated the effect of Clemastine, a first-generation and CNS (central nervous system)-penetrant H1 receptor antagonist on visual evoked potential (VEP), retinal nerve fibre layer (RNFL) and ganglion cell layer (GCL) complex in patients with optic neuritis. Patients and methodsThis is a prospective comparative interventional case series in 25 patients with acute optic neuritis. Patients were randomly assigned to group 1 (treated with Clemastine 1 mg orally twice a day for 90 days; 16 patients) or group 2 (received placebo for 90 days; 9 patients) and both groups received standard treatment of optic neuritis. We recorded VEP and peripapillary OCT (optical coherence tomography) of patients before and after three months of treatment. ResultsIn contrast to patients treated with Clemastine, RNFL thickness loss between base line phase and after three months follow up in control group were statistically significant in temporal, supra temporal, Infrotemporal and almost global sections of RNFL map. The reduction in GCL thickness between base line phase and after three months follow up in control group were significant, while it did not reach significance in treatment group except in inferior region. ConclusionIn contrast to treatment group, RNFL and GCL thickness of most quadrants are decreased significantly after three months in patients with ON in control group. In contrast to control group, p100 wave’s amplitude recovered in a significant manner in treatment group.

Magnetic resonance imaging indicator of the causes of optic neuropathy in IgG4-related ophthalmic disease

BackgroundThe following study investigates the involvement of optic neuropathy in IgG4-related ophthalmic diseases (IgG4-ROD) based on the magnetic resonance imaging (MRI) data, and different imaging features of IgG4-ROD related optic neuropathy related to other orbital diseases.MethodsThis retrospective study included 225 patients with IgG4-RD admitted at two ophthalmology centers between January 2014 and December 2017. Twenty-six patients had both pre-therapeutic orbital MRI and optic never injury. The causes of optic neuropathy were analyzed, and the special sign in MRI to diagnose IgG4-ROD was also evaluated.ResultsTwelve cases had inflammation of the optic nerve sheath, while 14 cases had compression due to extraocular muscles and pseudo tumor masses. Two cases had hypertrophic cranial pachymeningitis, while one case had hypophysis involving optic chiasma.ConclusionThe most common causes of optic nerve injury in IgG-4 ROD are inflammation of optic nerve sheath, compression of extraocular muscles, pseudo tumor mass and hypertrophic cranial pachymeningitis, and hypophysis involving optic chiasma.

English

AIM OF THE STUDY: To analyse Disc Damage likelihood ratio in patients with established open angle glaucoma and to correlate it with field defects and to thereby obtain an anatomical versus functional correlation. This study also evaluates the diagnostic ability of disc damage likelihood scale in glaucoma. MATERIALS AND METHODS: 50 cases of established open angle glaucoma were included in this study. Patients more than 45 yrs, BCVA more than 6/36, with open angles were included. Other causes of optic neuropathy, angle closures, BCVA less than 6/36, patients operated less than a year were excluded. All of the cases underwent detailed anterior segment examination, fundus examination. Visual fields were recorded by Octopus 301 using G1 program and TOP strategy. After field testing, DDLS scores were calculated after dilataion with tropicamide. The DDLS score and field parameters of the patients were analysed. RESULTS: Of the 50 patients included in this study, 30% were in the age group of 45-50 yrs and 24% were aged more than 66 years. 62% were male patients and 38% were female patients. 70% were on topical antiglaucoma medications and 30% had undergone trabeculectomy. The average vertical disc diameter was 1.86 mm. Among the 50 patients, 4 patients had asymmetry of disc diameter between the right and left eye. The cup disc ratio in all four patients showed significant asymmetry, of more than 0.2. But the DDLS score in both the eyes in all four patients were the same. DDLS score and average loss variance showed a strong positive correlation as the r value was 0.95. CONCLUSION: Disc diameter evaluation is an important part of optic nerve head evaluation. In cases with asymmetry of the cup disc ratio between two eyes, asymmetry of the disc size should also be considered if the neuroretinal rim is healthy. Disc Damage Likelihood Scale (DDLS) is a better indicator of optic nerve head status and has strong positive correlation with visual field indices.

Yield of screening blood work and MRI of the brain and orbits in the work-up of unilateral chronic optic neuropathy.

No guidelines exist for the investigation of treatable causes of chronic optic neuropathy, including sarcoidosis, lupus, and syphilis. The purpose of this study was to determine the diagnostic yield of screening blood work (ACE (Angiotensin Converting Enzyme)for sarcoidosis, Antinuclear Antibodies (ANA) for lupus, CMIA(chemiluminescence microparticle enzyme immunoassay) for syphilis) and contrast-enhanced MRI brain and orbits in atypical unilateral chronic optic neuropathy. Retrospective review from February 2012 to June 2018 at a neuro-ophthalmology practice. Six hundred and eighty-three consecutive charts with optic neuropathy were reviewed. Inclusion criteria were unilateral chronic optic neuropathy and a work-up including contrast-enhanced MRI brain and orbits, CBC, ESR, CRP, ANA, CMIA, and ACE. Exclusion criteria were optic nerve swelling in either eye on initial assessment or an established cause of optic neuropathy. The main outcome measure was diagnostic yield. Fifty-seven patients were included. One patient had elevated ACE, seven had positive ANA titers, and three had positive CMIA. Zero patients were diagnosed with sarcoidosis, one patient was diagnosed with lupus-related optic neuropathy, and one patient was diagnosed with syphilitic optic neuropathy. The diagnostic yield of ACE was 0%, ANA was 1.75%, and CMIA was 1.75%. MRI revealed planum sphenoidale meningioma causing compressive optic neuropathy in one patient, giving it a diagnostic yield of 1.82%. Routine screening blood work (ACE, ANA, CMIA) and MRI brain and orbits for chronic idiopathic unilateral optic neuropathy has low diagnostic yield, especially if clinical suspicion for syphilis, lupus, and sarcoidosis is low. MRI should still be performed in all cases in order to rule out compressive lesions.