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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 102-105

Optic Nerve Demyelination Along with Cardiorespiratory Involvement: A Rare Presentation of Multisystem Inflammatory Syndrome in Children

Department of Pediatrics, IQ City Medical College and Hospital, Durgapur, West Bengal, India

Date of Submission29-Dec-2022
Date of Decision06-Apr-2023
Date of Acceptance06-Apr-2023
Date of Web Publication24-May-2023

Correspondence Address:
Dr. Meenakshi Mitra
Department of Pediatrics, IQ City Medical College and Hospital, Bijra, Sovapur, Durgapur - 713 206, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_294_22

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Background: Multisystem inflammatory syndrome in children (MIS-C) has been described as an illness consisting of inflammation of more than one system of the body with raised inflammatory biomarkers following an infection of COVID-19. There is growing literature regarding the clinical spectrum of the condition. We report an unusual presentation of optic nerve demyelination in a child diagnosed with MIS-C. Clinical Description: A 3 years, 10-month-old boy presented with fever, generalized maculopapular rash, conjunctivitis, and bilateral palmar erythema. On examination, the child was febrile with tachypnea, tachycardia, and engorged jugular veins. Chest auscultation revealed basal crepitation. The cardiovascular examination was unremarkable. He had tender hepatomegaly. He developed photophobia and poor vision on the fourth day of admission with weakness of limbs. Ophthalmological examination showed a loss of visual acuity (perception of light only), while the fundoscopy examination was normal. Management: Inflammatory biomarkers and COVID-19 antibody titers were elevated. Echocardiography (ECHO) showed dilatation of the coronary arteries and poor ejection fraction. Magnetic resonance imaging of the brain showed bilateral optic nerve demyelination. Thus, the diagnosis was consistent with MIS-C with optic neuritis. Treatment was initiated with intravenous immunoglobulin (IVIG) and intravenous methylprednisolone with other supportive measures. At the 3-week follow-up, the child's vision, power in all four limbs, and echo parameters improved. Conclusion: This case creates awareness regarding optic neuritis as a rare presentation of MIS-C with cardiorespiratory and neurological involvement, successfully managed with IVIG and steroids.

Keywords: Central nervous system, COVID-19, hypotonia, optic nerve demyelination

How to cite this article:
Muduli JK, Mitra M, Mahipal S. Optic Nerve Demyelination Along with Cardiorespiratory Involvement: A Rare Presentation of Multisystem Inflammatory Syndrome in Children. Indian Pediatr Case Rep 2023;3:102-5

How to cite this URL:
Muduli JK, Mitra M, Mahipal S. Optic Nerve Demyelination Along with Cardiorespiratory Involvement: A Rare Presentation of Multisystem Inflammatory Syndrome in Children. Indian Pediatr Case Rep [serial online] 2023 [cited 2023 Jun 6];3:102-5. Available from: http://www.ipcares.org/text.asp?2023/3/2/102/377518

Reports of a condition termed multisystem inflammatory syndrome in children (MIS-C), or pediatric multisystem inflammatory syndrome, gradually emerged around the world since April 2020, which was found to be temporally associated with SARS-CoV-2 infection.[1] Knowledge about its epidemiology, pathophysiology, and clinical presentation is advancing over the months, and guidelines have been made defining the diagnostic criteria of MIS-C.[2],[3]

While cardiac and respiratory systems are commonly described, we report an unusual presentation of optic nerve demyelination in a child diagnosed with MIS-C.

  Case Description Top

A 3 years, 10-month-old boy presented to the emergency with a history of fever for 5 days, generalized maculopapular rash for 2 days, palmar erythema, bilateral nonexudative conjunctivitis for 4 days, increasing irritability, and refusal to feed. The child was diagnosed with autism at the age of 2 years and was receiving speech therapy and cognitive behavioral therapy. There was a history of COVID-19 infection in his elder brother 2 weeks back.

On examination, the child was found to be conscious and febrile (temperature was 101.4 F), with a heart rate of 160/min, blood pressure of 90/60 mm Hg, respiratory rate of 52/min, and SpO2 of 92% in room air. He was nutritionally healthy with a weight of 14 kg and a height of 95 cm. There was no pallor, icterus, cyanosis nor clubbing, or pedal edema. He had unilateral right tender cervical lymphadenopathy (3 cm × 2 cm), engorged neck veins, and tender hepatomegaly of 4 cm below the right costal margin, firm in consistency and smooth surface. Chest auscultation revealed basal crepitation with normal heart sounds and tachycardia. The rest of the cardiovascular examination was normal.

The child was hospitalized and started on supportive treatment with intravenous fluids and oxygen by nasal prongs at 3 l/min. Injection ceftriaxone was started for fever. On the fourth day of fever, the child developed photophobia and poor vision associated with weakness of all four limbs. The tone was increased, power was 2/5, and deep tendon reflexes were brisk in all four limbs. The child was irritable but conscious. However, the meningeal signs were absent. The plantar reflex was equivocal. There were no other focal neurological deficits or vomiting. Ophthalmological examination showed a loss of visual acuity (perception of light only), while fundus examination was normal.

  Management and Outcome Top

Initial investigations showed leukocytosis with neutropenia and an increase in inflammatory biomarkers such as C-reactive protein (CRP) and increased D-dimer [Table 1], with an increased SARS-CoV2 immunoglobulin G antibody titer (160 AU/ml). COVID-19 antigen test was negative by immunochromatographic method; reverse transcription-polymerase chain reaction (RT-PCR) was not done. The ECG showed sinus tachycardia with a short PR interval. The chest X-ray showed infiltrates in both the basal lung fields. 2D-echocardiography (ECHO) revealed dilatation of the coronary arteries with high Z-scores (left main coronary artery [LMCA] – 3.1 mm [z-score +2.81]), lack of tapering of the left anterior descending artery, and pericardial and pleural effusion with a poor ejection fraction of 35% [Figure 1]. The pro-B-type natriuretic peptide (NT-proBNP) count was very high confirming myocardial involvement.
Figure 1: Echocardiography showing dilated LMCA. LMCA: Left main coronary artery. Arrow indicates dilated LMCA

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Table 1: Investigation findings in the children with multisystem inflammatory syndrome during hospitalization

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Blood culture, malarial antigen, Dengue NS1, and Typhidot tests failed to reveal an infectious etiology. The cerebrospinal fluid study, including anti-neuromyelitis optica (NMO) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies, was normal ruling out any central nervous system (CNS) infection and antibody-mediated demyelinating disorders. Nerve conduction velocity (NCV) and electromyography (EMG) studies were normal. Magnetic resonance imaging (MRI) of the brain revealed bilateral optic nerve demyelination only [Figure 2]. MRI of the spine was not done.
Figure 2: T2-weighted plain MRI brain axial section showing bilateral optic nerve demyelination. Arrow indicates optic nerve demyelination. MRI: Magnetic resonance imaging

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Thus, the child was diagnosed as a case of MIS-C with cardiopulmonary involvement, associated with bilateral optic nerve demyelination. The weakness of limbs could neither be explained as an upper or lower motor neuron type of neuropathy nor as a myopathy. The child was started on intravenous immunoglobulin (IVIG) (1 g/kg IV over 24 h) and methylprednisolone (30 mg/kg IV for 5 days) and aspirin (5 mg/kg/day, per orally for 4 weeks) with other supportive care. The child was discharged after one week of hospital stay. At the time of discharge, there was an improvement of vision, and had a power of 4/5 in all limbs. At the 3-week follow-up, the child's vision, power in all four limbs, and echo parameters (z-score of LMCA = 0.31 mm, ejection fraction: 60%, and no evidence of pericardial effusion) had improved. At the 1-year follow-up, the neurological evaluation of the child was normal.

  Discussion Top

The case described above had features consistent with MIS-C with bilateral optic nerve demyelination as an unusual association.

After the initial reports of MIS-C from the United Kingdom in April 2020,[1] more such cases have been reported from other parts of the world, including India.[4] The understanding of the disease pathogenesis and its varied manifestations is evolving over the days. Most of the MIS-C cases have been reported in children of age 5 years and above, in contrast to Kawasaki disease, which with many similar features, is classically seen in infants and young children. Furthermore, while acute symptomatic COVID-19 disease tends to be seen more in children with underlying comorbidities, MIS-C is seen in healthy children.[5] Our case had a relatively younger age of presentation.

The case definition for MIS-C, provided by the CDC and WHO, is more or less similar, with essential parameters being fever, multisystem involvement, raised inflammatory markers, and evidence of SARS-CoV-2 RT-PCR in the form of any of the following: positive SARS-CoV-2 RT-PCR, positive serology, or positive antigen test, or contact with an individual with COVID-19 infection.[2],[3]

Our case fulfilled the criteria for MIS-C and had household contact with COVID-19 two weeks ago. While cardiorespiratory involvement has been widely reported in MIS-C,[6] the association with optic nerve demyelination is interesting vand rarely reported in children.[7]

The mechanisms proposed for SARS-CoV-2-induced optic injury include possible entry of the virus into the brain through the olfactory bulb and crossing the blood–brain barrier following viremia, or through infected leukocytes,[8] or capillary ischemic event associated with endotheliitis or microthrombosis known to be caused by the virus, resulting in optic atrophy or a parainfectious or postinfectious demyelinating condition linked to a prodromal viral illness. The third cause described here is likely to be the one for our case, as CSF cultures were negative for other pathogens, and there were no brain lesions indicative of multiple sclerosis. Another proposed mechanism is molecular mimicry, in which viral antigens induce an immune response against self-proteins.[9]

Recently, authors have published a pediatric case series of acute CNS demyelinating disorders in the context of acute or recent COVID-19 infection.[10] Weakness in limbs with exaggerated deep tendon reflexes was seen in our case, although CSF was negative for anti-NMO and anti-MOG antibodies, and NCV and EMG were also normal. Even though there was a weakness of all four limbs associated with brisk reflexes clinically, an MRI of the brain revealed bilateral optic nerve demyelination only. It has been mentioned in the literature that an MRI of the brain in the initial stages of demyelinating disorders may not reveal any pathology. This could explain normal findings in the MRI of the brain of our case.[11] Intravenous or oral steroids and IVIG are the treatments commonly used in such conditions.[10] In our case too, we used a course of pulse methylprednisolone and IVIG, which resulted in a favorable outcome.

  Conclusion Top

As more and more reports of MIS-C are being published, our understanding of the varied clinical presentations of the condition is evolving. Our case creates awareness of the possible association of demyelinating optic neuritis with cardiorespiratory involvement in a child with MIS-C. However, the debilitating pathology has an excellent prognosis with appropriate treatment.

Patient consent

Patient consent was obtained.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published, and due efforts will be made to conceal the patient's identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Riphagen S, Gomez X, Gonzalez-Martinez C, et al. Hyperinflammatory shock in children during COVID-19 pandemic. Lancet 2020;395:1607-8.  Back to cited text no. 1
Melgar M. Council of State and Territorial Epidemiologists/CDC Surveillance Case Definition for Multisystem Inflammatory Syndrome in Children Associated with SARS-COV-2 Infection – United States. Centers for Disease Control and Prevention; 2022. Available from: https://www.cdc.gov/mmwr/volumes/71/rr/rr7104a1.htm. [Last accessed on 2023 Mar 27].  Back to cited text no. 2
Freedman S. Multisystem Inflammatory Syndrome in Children and Adolescents with COVID-19. World Health Organization; 2020. Available from: https://www.who.int/publications/i/item/multisystem-inflammatory-syndrome-in-children-and-adolescents-with-covid-19. [Last accessed on 2023 Mar 25].  Back to cited text no. 3
Dhanalakshmi K, Venkataraman A, Balasubramanian S, et al. Epidemiological and clinical profile of pediatric inflammatory multisystem syndrome – Temporally associated with SARS-CoV-2 (PIMS-TS) in Indian Children. Indian Pediatr 2020;57:1010-4.  Back to cited text no. 4
Wessels PA, Bingler MA. A comparison of Kawasaki disease and multisystem inflammatory syndrome in children. Prog Pediatr Cardiol 2022;65:101516.  Back to cited text no. 5
Alsaied T, Tremoulet AH, Burns JC, et al. Review of cardiac involvement in multisystem inflammatory syndrome in children. Circulation 2021;143:78-88.  Back to cited text no. 6
Rodríguez-Rodríguez MS, Romero-Castro RM, Alvarado-de la Barrera C, et al. Optic neuritis following SARS-CoV-2 infection. J Neurovirol 2021;27:359-63.  Back to cited text no. 7
Ellul MA, Benjamin L, Singh B, et al. Neurological associations of COVID-19. Lancet Neurol 2020;19:767-83.  Back to cited text no. 8
Insausti-García A, Reche-Sainz JA, Ruiz-Arranz C, et al. Papillophlebitis in a COVID-19 patient: Inflammation and hypercoagulable state. Eur J Ophthalmol 2022;32:P168-72.  Back to cited text no. 9
Khair AM, Nikam R, Husain S, et al. Para and Post-COVID-19 CNS acute demyelinating disorders in children: A case series on expanding the spectrum of clinical and radiological characteristics. Cureus 2022;14:e23405.  Back to cited text no. 10
Anilkumar AC, Foris LA, Tadi P. Acute Disseminated Encephalomyelitis. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2023. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430934/. [Last updated on 2023 Jan 21].  Back to cited text no. 11


  [Figure 1], [Figure 2]

  [Table 1]


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