|Year : 2022 | Volume
| Issue : 1 | Page : 23-25
A rare case of myelin oligodendrocyte glycoprotein antibody disease with keratoconus
Sunny Makkar1, Nisha Ahuja2, Sonal Khandelwal3
1 Department of Ophthalmology, IOL Fellow, Sankara Eye Hospital, Anand, Gujarat, India
2 Department of Ophthalmology, CMO, Sankara Eye Hospital, Anand, Gujarat, India
3 Consultant Radiologist, Zydus Hospital, Anand, Gujarat, India
|Date of Submission||25-Jun-2021|
|Date of Acceptance||16-Aug-2021|
|Date of Web Publication||07-Jan-2022|
Dr. Sunny Makkar
Department of Ophthalmology, IOL Fellow, Sankara Eye Hospital, Anand, Gujarat
Source of Support: None, Conflict of Interest: None
Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) consists of a group of inflammatory demyelinating disorders generally associated with optic neuritis and acute disseminated encephalomyelitis (ADEM). We present a case report of MOGAD, who along with optic neuritis and seizures was detected to have keratoconus. No association of keratoconus with this condition has been documented in the literature. The presence of keratoconus is just an incidental finding or has an association with MOGAD needs to be ascertained by studies and documentation of similar cases. Further data need to be collected to establish any association between the two.
Keywords: Keratoconus, MOGAD, optic neuritis, seizures
|How to cite this article:|
Makkar S, Ahuja N, Khandelwal S. A rare case of myelin oligodendrocyte glycoprotein antibody disease with keratoconus. Indian J Ophthalmol Case Rep 2022;2:23-5
|How to cite this URL:|
Makkar S, Ahuja N, Khandelwal S. A rare case of myelin oligodendrocyte glycoprotein antibody disease with keratoconus. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Jan 19];2:23-5. Available from: https://www.ijoreports.in/text.asp?2022/2/1/23/334901
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) represents a group of inflammatory demyelinating disorders characterized by the presence of Immunoglobulin G (IgG) antibodies to myelin oligodendrocyte glycoprotein (MOG). Over the recent years, it is becoming evident that MOGAD represents a distinct clinical entity separate from acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorder (NMOSD), and multiple sclerosis (MS).
| Case Report|| |
A 16-year-old male patient, a known case of MOG antibody-related disease on treatment by a neuro physician presented in March 2021 with complaints of severe diminution of vision of the right eye for the last 1 year. His magnetic resonance imaging (MRI) brain and orbit revealed right optic neuritis, and hence, was referred to our eye center. No previous detailed ophthalmic evaluation was available.
On clinical examination of the patient, retinoscopy scissoring reflex was noted in his right eye. His best-corrected visual acuity in the right eye was counting fingers at 1 m and that of the left eye was 6/6. The near vision in the right eye was less than N60 and N6 in the left eye.
The anterior segment examination of the right eye revealed central corneal thinning, Fleischer's ring, and grade 1 relative afferent pupillary defect. The anterior segment examination of the left eye was within normal limits. The fundus examination of both eyes was normal.
Keeping a suspicion of keratoconus, the patient was further advised pachymetric mapping and corneal topography whose findings are tabulated in [Figure 1] and [Table 1]. These clinical findings confirmed the diagnoses of the right eye keratoconus and was advised corneal collagen cross-linking of the right eye for the same. He was referred to his treating neuro physician for clearance for collagen cross-linking. The patient was treated with intravenous methylprednisolone for 3 days by the neurologist.
|Figure 1: Corneal topography of both eyes suggestive of right eye keratoconus|
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Post his treatment, his 3-week follow-up showed that his best-corrected visual acuity in the right eye improved from counting fingers at 1 m to 6/24. The patient then underwent the corneal collagen cross-linking procedure and has been under regular follow-up. His subsequent right eye distant vision has stabilized at 6/24.
| Discussion|| |
In this case report, we present our experience with a 16-year-old boy who was diagnosed with Mogad with two episodes of seizures with optic neuritis and keratoconus in the right eye.
The MOGAD is caused by the autoimmune production of antibodies against MOG1, located on the surface of the myelin sheath, and thought to be responsible for cell adhesion as well as other functions. While the antibodies to MOG have been detected for many years, MOGAD itself has been recognized as a distinct disease only since 2013.
The coexistence between seizures and MOG Abs is emerging as a relevant and challenging association, primarily for its prognostic and therapeutic implications. However, a causal relationship between epileptic manifestations, which substantially follow neuronal cell dysfunctions, and MOG Abs, which selectively targets oligodendrocytes, is hard to establish at present. The seizures can be absent in patients with very high MOG Ab titers, and occur both as part of the demyelinating manifestations of MOG-Encephalomyelitis (EM), or as isolated events in MOG Ab-positive patients with normal brain MRI. These latter cases are particularly interesting as they seem to contradict the hypothesis that seizures are just secondary to cortical involvement during an encephalitic process. Our patient also had two episodes of seizures, while his MRI brain was normal with no cortical involvement with features of the right eye optic neuritis. [Figure 2] and [Figure 3] In MOG antibody–associated ADEM, the following findings have been described in MRI: bilateral, multiple, poorly demarcated and widespread lesions that are hyperintense on T2-weighted and fluid-attenuated inversion-recovery (FLAIR) images with involvement of several regions including the subcortical, periventricular, and deep white matter; cortical gray matter; deep gray matter (most commonly the thalamus); brainstem; and cerebellum.
|Figure 2: MRI brain-T2W image showing normal white matter and cortical gray matter|
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|Figure 3: MRI brain-T1W post-contrast image showing thickening and enhancement of the right optic nerve suggestive of optic neuritis depicted by the arrow|
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In the study conducted by A Cobo- Calvo, A Ruiz, and F Rollot, the most common presentation for children was optic neuritis (41%), while ADEM (37%) was a close second.,,,
The other symptoms include the following:
Neuromyelitis optica (6–24%), longitudinally extensive spinal cord lesions, and seizures—Fluid attenuated inversion recovery sequence (FLAMES).
Dubey et al. concluded in their study that myelitis was an early manifestation of MOG-IgG-related disease.
No study till now,(at the time of writing, 2021) has shown an association of keratoconus with MOGAD.
| Conclusion|| |
In confirmation with the various studies and literature available, our patient, a 16-year-old boy also had optic neuritis and seizures. However, his condition was also associated with keratoconus.
No association of MOGAD with keratoconus has been documented in the literature. The presence of keratoconus in our patient is merely a coincidental finding or consequence of MOGAD remains inconclusive and needs to be studied further. Similar findings need to be documented to establish such associations.
Also, a high index of suspicion has to be kept in mind for diagnosing keratoconus in such patients as an improvement in visual acuity will not only depend on the treatment of optic neuritis but also on keratoconus.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]