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Table of Contents    
Year : 2016  |  Volume : 64  |  Issue : 4  |  Page : 798-800

Recurrent myelopathy and optic neuritis associated with systemic lupus erythematosus

Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh, India

Date of Web Publication5-Jul-2016

Correspondence Address:
Rajesh Verma
Department of Neurology, King George Medical University, Lucknow, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.185355

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How to cite this article:
Verma R, Nagar KK, Mehta M. Recurrent myelopathy and optic neuritis associated with systemic lupus erythematosus. Neurol India 2016;64:798-800

How to cite this URL:
Verma R, Nagar KK, Mehta M. Recurrent myelopathy and optic neuritis associated with systemic lupus erythematosus. Neurol India [serial online] 2016 [cited 2020 May 28];64:798-800. Available from:


A 9-year-old girl presented with chief complaints of recurrent episodes of bilateral visual loss and paraparesis since the last 4 years. At the age of 5 years, she developed acute onset sensory motor paraparesis with bowel and bladder involvement, which recovered completely over 1 month after treatment with intravenous methylprednisolone. Two months later, she developed painless progressive bilateral visual loss that recovered completely over 6 weeks duration with treatment. After 6 months, she again developed bilateral visual loss, which recovered completely with treatment. Around 7 years of age, she, for the third time, developed bilateral visual loss, which did not recover and made her blind. Within the next 2 years, she had 3 more episodes of acute paraparesis which recovered within 6–8 weeks with treatment, recurring after a few months duration. Nearly 6 months back, she again developed acute paraparesis along with dyspnea on exertion and generalized edema. On evaluation, she was found to have proteinuria, and pericardial and pleural effusion. The antinuclear antibody and anti-double stranded deoxyribonucleic acid antibody revealed positive results. She was, therefore, diagnosed to be having systemic lupus erythematosus (SLE). She was given steroids along with hydoxychloroquine. The patient presented to us during this episode with acute onset sensory motor paraparesis of 7 days duration. On enquiry, the patient denied any history of arthralgia, rash, oral ulcers, and hair loss. On examination, she was conscious and oriented having a moon facies. General examination did not reveal rash, alopecia, or oral ulcers. Bilateral pupils were semi-dilated, sluggishly reacting to light with no perception of light. Fundus examination showed bilateral optic atrophy. Power in the upper limb was normal and in the lower limb was 2/5 (Medical Research Council classification). The lower limb examination revealed spasticity with brisk knee and ankle reflexes on both sides. Plantars were bilaterally extensor. Rest of the examination was normal. On investigation, the complete blood count, renal function test, liver function test, and urine examination were normal. Erythrocyte sedimentation rate was elevated. Cerebrospinal fluid (CSF) examination showed 10 cells, 95% lymphocytes, protein 28mg%, and sugar 65 mg%. CSF oligoclonal bands were negative and serum neuromyelitis optica (NMO)-IgG antibody was positive. Magnetic resonance imaging (MRI) of the cranium [Figure 1] showed multifocal white matter lesions, mainly involving bilateral periventricular and subcortical white matter. MRI of the spine [Figure 2] showed T2 high signal intensity extending from C5 to D7 segments of the spinal cord. The patient was given intravenous pulse methyl prednisolone therapy for 5 days. There was no change in her vision but her motor weakness improved (power 3/5 MRC); she was walking with support at discharge after 2 weeks. The diagnosis of NMO with SLE was made according to the diagnostic criteria given by Wingerchuk et al.[1]
Figure 1: Magnetic resonance imaging of the brain demonstrating multifocal white matter lesions mainly involving the bilateral periventricular and subcortical white matter

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Figure 2: Magnetic resonance imaging of the spine showing T2 signal intensity alteration in the cervicodorsal areas of the spinal cord

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SLE is a multisystem disorder with a few patients developing their first symptom before 18 years of age. It is slightly more common in females and more acute and severe in children. NMO initially was considered as a monophasic disorder but is now known to be polyphasic in more than 75% of the cases, and in more than 50% of cases, it may lead to functional blindness or physical dependency within 5 years of the initial attack if the treatment is not initiated early.[2] Our patient had become blind within 2 years of the disease onset and required support for her daily activities. [Table 1] presents the reported literature of SLE patients who developed myelitis and optic neuritis later. We are presenting this rare case of SLE, coexisting with NMO, because of its very early onset within the first decade and rapid progression. Prompt recognition and diagnosis may help in better clinical outcome of such patients.
Table 1: Reported literature of SLE patients presenting with attacks of myelitis and optic neuritis

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Conflicts of interest

There are no conflicts of interest.[7]

 » References Top

Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology 2006;66:1485-9.  Back to cited text no. 1
Wingerchuk DM, Hogancamp WF, O'Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology 1999;53:1107-14.  Back to cited text no. 2
Nasir S, Kerr DA, Birnbaum J. Nineteen episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus. Arch Neurol 2009;66:1160-3.  Back to cited text no. 3
Birnbaum J, Kerr D. Optic neuritis and recurrent myelitis in a woman with systemic lupus erythematosus. Nat Clin Pract Rheumatol 2008;4:381-6.  Back to cited text no. 4
Ferreira S, Marques P, Carneiro E, D'Cruz D, Gama G. Devic's syndrome in systemic lupus erythematosus and probable antiphospholipid syndrome. Rheumatology 2005;44:693-5.  Back to cited text no. 5
Gibbs NA, Moroney J, Foley-Nolan D, O'Connell PG. Neuromyelitis optica (Devic's syndrome) in systemic lupus erythematosus: A case report. Rheumatology 2002;41:470-7.  Back to cited text no. 6
Chan K, Boey ML. Transverse myelopathy in SLE: Clinical features and functional outcomes. Lupus 1996;5:294-9.  Back to cited text no. 7


  [Figure 1], [Figure 2]

  [Table 1]

This article has been cited by
J.R. Escosteguy,N.L. Konrad,L.B. Raguzzoni,E. Lopes,M. Teixeira,C.M. Rocha
Revista Brasileira de Reumatologia. 2017; 57: S178
[Pubmed] | [DOI]


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