|Year : 2023 | Volume
| Issue : 2 | Page : 455-457
Widespread retinal hemorrhages in a patient with atypical optic neuritis: A case report
Madhurima Kaushik1, Karthik Kumar Mani1, Shriram Varadharajan2, Virna M Shah1
1 Neuro-Ophthalmology Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Coimbatore, Tamil Nadu, India
2 Department of Imaging Sciences and Interventional Radiology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India
|Date of Submission||30-Oct-2022|
|Date of Acceptance||02-Jan-2023|
|Date of Web Publication||28-Apr-2023|
Virna M Shah
Consultant and Head of the Department, Neuro-Ophthalmology Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Avinashi Road, Coimbatore - 641 014, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Retinal hemorrhages are rare in optic neuritis. We report a case of atypical optic neuritis with widespread retinal hemorrhages. A 30-year-old Indian woman presented with sudden painful and severe visual loss in the left eye for 3 days. Fundus examination showed minimal optic disk edema associated with intraretinal hemorrhages extending to the periphery in the left eye. On neuroimaging, long segment thickening of the intraorbital segment of the left optic nerve was appreciated with T2 hyperintensity. Serum myelin oligodendrocyte (MOG) antibodies and neuromyelitis optica (NMO) antibodies were negative. Visual acuity improved rapidly after the administration of intravenous high-dose steroids, and fundus abnormalities resolved rapidly. Retinal hemorrhages can also be present in atypical optic neuritis in the absence of severe optic disk edema. Clinicians should be mindful of the variable presentations of optic neuritis to initiate proper and timely treatment.
Keywords: Atypical optic neuritis, neuroimaging, optic neuritis, retinal hemorrhage
|How to cite this article:|
Kaushik M, Mani KK, Varadharajan S, Shah VM. Widespread retinal hemorrhages in a patient with atypical optic neuritis: A case report. Indian J Ophthalmol Case Rep 2023;3:455-7
|How to cite this URL:|
Kaushik M, Mani KK, Varadharajan S, Shah VM. Widespread retinal hemorrhages in a patient with atypical optic neuritis: A case report. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 10];3:455-7. Available from: https://www.ijoreports.in/text.asp?2023/3/2/455/374994
Atypical optic neuritis displays characteristics that deviate from the classic features. Presenting features include painless presentation, poor response to or relapse upon tapering corticosteroids, or optic nerve head or peripapillary hemorrhages., The most common causes of atypical optic neuritis include neuromyelitis optica spectrum disorders (NMOSDs), myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), and infections. Atypical optic neuritis is generally encountered in Asia.
We report a case of atypical optic neuritis where widespread intraretinal hemorrhages were more notable than the appearance of the disk.
| Case Report|| |
A 30-year-old nondiabetic, nonhypertensive young Indian woman presented to the neuro-ophthalmology clinic with the complaints of sudden-onset pain and diminution of vision in the left eye for 3 days. The pain was more pronounced on moving the eye. There was no previous history of neurologic symptoms. She was not on any medications toxic to the optic nerve and had no other significant history, except for a history of infertility. She was a nonsmoker and was not habituated to alcohol. There was no relevant family history.
On general examination, the patient weighed 80 kg and had a body mass index (BMI) of 34.3 kg/m2. Blood pressure was 134/84 mmHg. She was afebrile. Her neurologic examination was normal.
Uncorrected visual acuity was counting fingers (CF) in the affected eye and 5/60 in the right eye. Best corrected visual acuity was CF in the affected eye and 6/6 in the right eye with a −4 D lens. Intraocular pressure was normal in both eyes. Color vision was defective in the left eye. A grade 4 relative afferent pupillary defect (RAPD) was elicited while examining the left eye. The media were very clear, with no cells in the anterior and posterior vitreous on slit-lamp examination. The disk was mildly edematous and hyperemic on fundus examination. Superficial and deep dot blot hemorrhages were the more notable finding, which started in the peripapillary region, were most prominently associated with the vascular arcades and extended till the near periphery. Hemorrhages were present in all layers of the retina. The veins appeared slightly tortuous and dilated in the affected eye. There was no obvious vasculitis or ischemic whitening of the retina, which negated the possibility of branch retinal artery occlusion. Ocular coherence tomography (OCT) confirmed disk edema and the absence of cells in the posterior vitreous, with normal macular anatomy. B-scan ultrasound also showed mild disk edema. T sign was absent. The right eye was normal on examination [Figure 1]. We suspected a diagnosis of atypical optic neuritis due to the presence of painful visual loss in the setting of an RAPD with confirmed disk edema.
|Figure 1: (a) Normal fundus picture of the right eye. (b) Hyperemic disk with tortuous veins and scattered deep hemorrhages over the posterior pole of the affected eye|
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Magnetic resonance imaging (MRI) of the orbits showed long segment thickening of the intraorbital segment of the left optic nerve. T2 hyperintensity, altered diffusivity, and post-contrast enhancement with gadolinium were also noted [Figure 2]. MRI of the brain revealed few focal patchy fluid-attenuated inversion recovery (FLAIR) hyperintensities in the left parietal white matter with no diffusion restriction or enhancement. Reduced amplitude and prolonged P100 latency were noted in the left optic nerve on visually evoked potentials (VEP).
|Figure 2: Long segment thickening of intraorbital segment of the left optic nerve with T2 signal changes (a and b) and enhancement (c) suggestive of optic neuritis|
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Both neuromyelitis optica (NMO) and MOG antibodies were negative. Clotting tests were within normal limits, with normal platelet counts. The Mantoux test was negative. Other investigations like erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were also unremarkable, except for mild microcytic hypochromic anaemia noted on a peripheral blood smear. At this point, we felt that fundus fluorescein angiography (FFA) could be avoided as it was an invasive procedure and would not impact the diagnosis. OCT angiography (OCTA) of the macula of the left eye was also normal [Figure 3]. Spinal tap was not considered necessary in this case.
|Figure 3: A normal 6 mm× 6 mm OCTA en face image of the superficial retinal vascular layer of the left eye. OCTA = optical coherence tomography angiography|
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She received three doses of intravenous methylprednisolone (IVMP) 1000 mg/day followed by oral steroids (prednisolone) in the dose of 1 mg/kg body weight, tapered over 6 weeks. There was an excellent response to steroids, with quick restoration of visual acuity to 6/6. Spectacular response to IVMP indicated that the pathology involved the optic nerve. On her follow-up visit at 6 weeks, the optic disk appeared normal with less numerous hemorrhages in the fundus. The patient had no other similar episodes during a follow-up period of 3 months after the cessation of oral steroids.
| Discussion|| |
Typical optic neuritis associated with multiple sclerosis frequently presents with retrobulbar neuritis. Sudden-onset defective vision, RAPD, and a normal optic disk are the diagnostic pointers in such cases. Diagnosis of retrobulbar neuritis is confirmed on MRI. Peripapillary hemorrhages have been rarely described in cases of atypical optic neuritis.,
Recently, widespread retinal hemorrhages have been described in literature in association with MOGAD, with reports showing associated severe optic disk edema and vascular tortuosity. We agree with the hypothesis that blocked outflow at the optic nerve head can cause venous outflow obstruction, stasis, and resultant hemorrhages in such cases.,, However, in our case, only mild disk edema was present with diffuse intraretinal hemorrhages near the vascular arcades and extending till the near periphery. Systemic diagnoses like hypertensive retinopathy and diabetic retinopathy were excluded because of a normal fundus in the unaffected eye. The resolution of the hemorrhages after the administration of steroids supports the idea that they formed due to edema, compression, and venous congestion.
Significant inflammation of the optic nerve and optic nerve sheath was demonstrated on MRI of the orbits. Long segment thickening of the optic nerve on T2 images was appreciated with post-contrast enhancement. Given the MRI results, we had a strong suspicion for MOGAD, but this diagnosis could not be confirmed because of a negative antibody test. The case was labeled as atypical optic neuritis and the patient kept on follow-up to monitor for relapses.
| Conclusion|| |
Clinicians should keep in mind that optic neuritis can have a variable presentation. RAPD is an important clue in the diagnosis of these cases. Retinal hemorrhages can be present even with a near-normal disk. It is important that such cases of atypical optic neuritis are recognized early to initiate proper treatment with corticosteroids.
MK and VMS conceived the study; MK, VMS, and KKM performed the clinical assessment; SV performed the radiological assessment; all authors were involved in analysis and interpretation of the data; MK prepared the initial manuscript; and VMS, KKM, and SV revised the manuscript for its intellectual content. The final manuscript was approved by all authors. MK and VMS are the guarantors of the paper.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We are grateful to the patient for giving her consent to the publication of this case. We thank Dr. V. R. Saravanan for his valuable suggestions during preparation of the manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Gaier ED, Boudreault K, Rizzo JF 3rd
, Falardeau J, Cestari DM. Atypical optic neuritis. Curr Neurol Neurosci Rep 2015;15:76.
Abel A, McClelland C, Lee MS. Critical review: Typical and atypical optic neuritis. Surv Ophthalmol 2019;64:770-9.
Sarkar P, Mehtani A, Gandhi HC, Dubey V, Tembhurde PM, Gupta MK. Atypical optic neuritis: An overview. Indian J Ophthalmol 2021;69:27-35.
] [Full text]
Wilhelm H, Schabet M. The diagnosis and treatment of optic neuritis. Dtsch Arztebl Int 2015;112:616-25.
Balcer LJ. Clinical practice. Optic neuritis. N Engl J Med 2006;354:1273-80.
Lukewich MK, Micieli JA. Venous stasis retinopathy secondary to myelin-oligodendrocyte glycoprotein antibody-positive optic neuritis. Retin Cases Brief Rep 2022;16:305-7.
Aboab J, Errera MH, Espinoza S, Girmens JF, Heron E. Atypical case of MOG antibody-associated optic neuritis with Roth spots. Ocul Immunol Inflamm 2022:1-5. doi: 10.1080/09273948.2022.2062386.
Mittal A, Baig IF, Merchant AG, Chen JJ, Choi JJ, Goldberg A, et al
. Sjogren disease and myelin oligodendrocyte glycoprotein antibody-associated optic neuritis. J Neuro-Ophtalmol 2021;41:e48-50.
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