Facial Nerve Involvement Accompanying Optic Neuritis Secondary to Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder

We present the first reported case of facial nerve involvement accompanying an optic neuritis in myelin oligodendrocyte glycoprotein antibody-associated disorder.

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Facial Nerve Involvement Accompanying Optic Neuritis Secondary to Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disorder Zhiyong Chen, MRCP, Monica Saini, MRCP, Jing Liang Loo, FRCS (Ed) FIG. 1. Lack of lower facial movement (A), eyebrow elevation and forehead furrowing (B) on the right side, in keeping with a lower motor neuron weakness. Resolution of facial weakness after treatment (C, D). Abstract: We present the first reported case of facial nerve involvement accompanying an optic neuritis in myelin oligodendrocyte glycoprotein antibody–associated disorder. Journal of Neuro-Ophthalmology 2021;41:e378–380 doi: 10.1097/WNO.0000000000001145 © 2020 by North American Neuro-Ophthalmology Society Department of Neurology (ZC, MS), National Neuroscience Institute, Singapore; and Department of Neuro-Ophthalmology (JLL), Singapore National Eye Centre, Singapore. The authors report no conflicts of interest. Address correspondence to Zhiyong Chen, MRCP, Department of Neurology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore 308433; E-mail: [e-mail] e378 A 21-year-old Malay man presented with acute painful, blurry vision of the right eye, followed by right facial weakness 3 days thereafter. These symptoms were preceded by a 2-week history of fever, with no other localizing symptoms. On examination, the right eye visual acuity was hand motion, and pupillary examination revealed a right relative afferent pupillary defect. The left eye visual acuity was intact. Bilateral retinal and optic disc examination was normal. There was a concomitant right lower motor neuron facial weakness (Fig. 1). Syphilis, Lyme, and HIV serologies were negative. Cerebrospinal fluid analysis revealed 5 nucleated cells (reference range, 0–5 cells/mm3), 28 red blood cells, protein of 0.27 g/L (reference range, 0.1–0.4 g/L), and Chen et al: J Neuro-Ophthalmol 2021; 41: e378-e380 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay FIG. 2. MRI orbits coronal T2 image (A) shows hyperintensity of the right optic nerve, whereas coronal T1 postgadolinium image (B) shows mild contrast enhancement. glucose of 4.6 mmol/L (reference range, 2.4–5.5 mmol/L); however, serum antimyelin oligodendrocyte glycoprotein antibody (MOG-Ab) was positive. Serum examination of antinuclear antibodies, antineutrophil cytoplasmic antibodies, anti-Ro, anti-La, aquaporin 4 antibodies, and extractable nuclear antibodies was negative. Serum testing for Bartonella henselae, dengue virus, Epstein–Barr virus, measles virus, and tuberculosis was negative. Cerebrospinal fluid testing for cryptococcosis, Cytomegalovirus, Enterovirus, herpes simplex virus, and varicella-zoster virus was negative. MRI of the brain and orbits showed T2 hyperintensity associated with postcontrast T1 enhancement of a short segment of the right retrobulbar optic nerve (Fig. 2) and equivocal T2 hyperintensity without postcontrast T1 enhancement of the right facial nerve exit zone. The patient was treated on Day 5 after onset of visual symptoms with pulsed methylprednisolone (1000 mg/d, over 5 days), 5 cycles of plasma exchange, and initiated on high dose (1 mg/kg) oral prednisolone. He responded well to treatment. On examination, 4 months after symptom onset, the patient exhibited a residual right red desaturation with intact visual acuity. The right facial palsy had also completely resolved. MOG-Ab–associated disorder was originally reported in acute disseminated encephalomyelitis (children) and neuromyelitis optica spectrum disorders (adults). Recently, there have been a few reports of oculomotor, trigeminal, and vestibulocochlear neuropathies in MOG-Ab–associated disorder (1,2). Imaging may show lesions either in the root, root exit, or cisternal level. The underlying pathophysiology is uncertain (1). Facial nerve involvement has not previously been reported. In clinical practice, the presentation of a cranial neuropathy accompanying an inflammatory optic neuropathy is rarely TABLE 1. Causes and clinical features of inflammatory optic neuropathy associated with cranial neuropathies.(4–7) Diagnosis Anti-MOG-Ab disorder(4) Syphilis Age (yrs); Demographics 2–79 High-risk sexual behavior Onset Gender Predilection Painful Optic Neuropathy Acute F = 57% 86% M=F Variable F = 66% .90% Acute, subacute, or chronic Acute Fundus Findings Normal optic disc or optic disc swelling Severe disc edema and neuroretinitis Multiple sclerosis(5) 20–50 Normal optic disc or mild edema Lyme disease Lyme endemic areas Acute to subacute M=F Variable Papillitis, neuroretinitis, and papilledema Sarcoidosis(6) 20–71; more prevalent in African, Caribbean, and Scandinavians Acute to subacute F = 64% 27% Normal optic disc, edematous, or atrophic optic discs Possible Associated Features Opticospinal, ADEM Uveitis, neuroretinitis, meningitis, and other CNS deficits Other manifestations of multiple sclerosis Rash, uveitis, signs of raised intracranial pressure, and other cranial neuropathies Pulmonary disease and lacrimal gland enlargement Diagnostic Test (s) Anti-MOG antibodies Syphilis serology McDonald criteria Western blot assay and intrathecal antibodies Chest imaging and tissue diagnosis ADEM, acute demyelinating encephalomyelitis; CNS, central nervous system; F, female; M, male. Chen et al: J Neuro-Ophthalmol 2021; 41: e378-e380 e379 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay encountered. A combination of optic and facial neuropathy carries several differential diagnoses of both autoimmune and infective etiologies that need to be considered (Table 1). Early recognition and treatment of an onset attack of MOG-Ab– associated disorder is imperative because there is suggestion that time-to-treatment of disease is a major factor influencing the degree of disability in this disorder (3). Prompt diagnosis and treatment possibly contributed to the good clinical outcome seen in our patient despite his initial severe neurological symptoms. In summary, we present the first reported case of facial nerve involvement accompanying an optic neuritis in MOG-Ab–associated disorder. AUTHOR CONTRIBUTIONS: Data collection and analysis, drafting, and revision of manuscript: Z. Chen and M. Saini. Patient recruitment, data collection, and analysis: J. L. Loo. ACKNOWLEDGMENTS The authors are indebted to Dr. Sindhu John for her aid in the selection of radiology images. e380 REFERENCES 1. Cobo-Calvo A, Ayrignac X, Kerschen P, Horellou P, Cotton F, Labauge P, Vukusic S, Deiva K, Serguera C, Marignier R. Cranial nerve involvement in patients with MOG antibody– associated disease. Neurol Neuroimmunol Neuroinflamm. 2019;6:e543. 2. Kawakami S, Akamine S, Chong PF, Yamashita F, Maeda K, Takahashi T, Kira R. Isolated cranial neuritis of the oculomotor nerve: expanding the MOG phenotype? Mult Scler Relat Disord. 2020;41:102040. 3. Reindl M, Waters P. Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Nat Rev Neurol. 2019;15:89–102. 4. 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