IMAGES IN NEUROLOGY
|Year : 2022 | Volume
| Issue : 6 | Page : 1174-1176
NMOSD and MOGAD dual positivity: An extremely rare phenomenon
Ayush Agarwal1, Aminu Aliyar1, Shilpa Rao2, Anita Mahadevan2, Ajay Garg3, Achal Srivastava1
1 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
3 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
|Date of Submission||07-Jul-2022|
|Date of Decision||06-Aug-2022|
|Date of Acceptance||06-Aug-2022|
|Date of Web Publication||3-Dec-2022|
Department of Neurology, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Agarwal A, Aliyar A, Rao S, Mahadevan A, Garg A, Srivastava A. NMOSD and MOGAD dual positivity: An extremely rare phenomenon. Ann Indian Acad Neurol 2022;25:1174-6
|How to cite this URL:|
Agarwal A, Aliyar A, Rao S, Mahadevan A, Garg A, Srivastava A. NMOSD and MOGAD dual positivity: An extremely rare phenomenon. Ann Indian Acad Neurol [serial online] 2022 [cited 2023 Feb 6];25:1174-6. Available from: https://www.annalsofian.org/text.asp?2022/25/6/1174/361566
Myelin oligodendrocyte glycoprotein-associated disorder (MOGAD) is an autoimmune oligodendrocytopathy, whereas neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy. Both cause central nervous system demyelination, and serum IgG-MOG and anti-aquaporin-4 antibodies serve as their respective biomarkers. Both these disorders, although distinct, share common clinical features, such as optic neuritis and myelitis. There are rare reports (0.05%) of concomitant occurrence of these otherwise distinct disorders, and we present one such rare occurrence in a woman who presented with left hemiparesis and persistent hiccups. Magnetic resonance imaging (MRI) brain revealed a tumefactive demyelinating lesion involving the right temporal lobe, lentiform nucleus, and cerebral peduncle, and another small lesion in the area postrema. Both these lesions showed patchy contrast enhancement [Figure 1]. MRI spine was normal. A possibility of NMOSD was kept and serum was tested for antibodies against aquaporin-4 and myelin oligodendrocyte glycoprotein (cell-based assay), both of which were strongly positive (tested twice). A diagnosis of concomitant NMOSD and MOGAD was made, and she was treated with intravenous methylprednisolone pulse (1 g daily for 5 days), followed by plasma exchange (5 cycles done on alternate days) with some symptomatic improvement (left upper and lower limb power 3/5 on Medical Research Council (MRC)). Thereafter, she was treated with rituximab infusion (1 g) with next dose planned after 2 weeks and discharged on tapering steroids. Dual-positive patients tend to have a multiphasic disease course, higher relapse rates, and more disability and should be aggressively managed.
|Figure 1: (a) Sagittal T2- weighted imaging (T2-WI) shows focal hyperintensity (arrow) in area postrema (b) Axial T2-WI shows ill-defined hyperintensity in the right medial temporal lobe, hippocampal head (thick arrow), right optic tract (thin arrow), and ventral midbrain with foci of diffusion restriction in the region of the optic tract (thin arrow in c and d) and (c) ventral midbrain (thick arrow in c and d) in diffusion trace image and (d) Apparent Diffusion Co-efficient (ADC) map (e) The punctate focus of enhancement (arrow) is seen in the medial temporal lobe following contrast administration (f) Coronal FLuid Attenuated Inversion Recovery (FLAIR) image shows hyperintensity in the medial temporal lobe, including the hippocampal head (arrow), posterior limb of the internal capsule, and ventral midbrain (g) Axial T2-WI at the level of basal ganglia shows hyperintensity involving the right putamen, posterior limb of the internal capsule, right thalamus, and peri-atrial white matter (thin arrow) (h) with foci of diffusion restriction in diffusion trace image and (i) ADC map (j) No contrast enhancement is seen in post-gad T1-WI. Cell-based assay shows strong immunofluorescence to aquaporin (neuromyelitis optica, A) and myelin oligodendrocyte protein (B, myelin oligodendrocyte glycoprotein) in the serum tested on transfected cell lines|
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