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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 33  |  Issue : 3  |  Page : 349-352

The eyes show what the brain has: A case report on neuromyelitis optica


Department of Ophthalmology, MGM Institute of Health Sciences, Navi Mumbai, Maharashtra, India

Date of Submission05-Sep-2020
Date of Acceptance14-Sep-2020
Date of Web Publication08-Dec-2021

Correspondence Address:
Dr. Ayushi Choudhary
24, Mishra Vihar Colony, Geetabhawan, Indore - 452 001, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_135_20

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  Abstract 


Neuromyelitis optica (NMO) is a rare autoimmune disease affecting nerves of the eyes and central nervous system. Etiology of NMO is yet to be completely defined. The purpose of this report is to bring to light one such unique case of NMO.

Keywords: Disc edema, neuromyelitis optica, neuroopthalmology


How to cite this article:
Choudhary A, Ramakrishnan R, Agrawal M. The eyes show what the brain has: A case report on neuromyelitis optica. Kerala J Ophthalmol 2021;33:349-52

How to cite this URL:
Choudhary A, Ramakrishnan R, Agrawal M. The eyes show what the brain has: A case report on neuromyelitis optica. Kerala J Ophthalmol [serial online] 2021 [cited 2022 Jan 19];33:349-52. Available from: http://www.kjophthal.com/text.asp?2021/33/3/349/331919




  Introduction Top


Neuromyelitis optica (NMO) also referred to as Devic's disease and NMO spectrum disorders are a spectrum of inflammatory, autoimmune demyelinating disorders.[1] While the exact etiology of NMO remains unexplained, the disease is found to be associated with antibodies against aquaporin water channels. NMO can occur in any age, sex, or ethnicity; a certain predilection toward females and people of Asian or African descent has been observed.[2] The disease can adversely affect the spinal cord and optic nerve and may even prove life-threatening. Hence, it is important to obtain a complete understanding of the disorder to aid in better management of the same.


  Case Report Top


An 8-year-old girl presented to the ophthalmology outpatient department with the chief complaint of diminution of vision in both the eyes for the past 4 days. The diminution of vision was acute in onset, painless, progressive, and involved both peripheral and central fields of vision. There were no other ocular complaints. The patient also had complaints of fever, projectile vomiting, and headache for 4 days, for which she had been given symptomatic treatment. The parents gave no history of using spectacles, any ocular trauma or procedure. There was no history of any systemic illness. Her unaided distant visual acuity was hand movements close to the face with accurate projection of rays in both the eyes, which did not improve with the use of pinhole. Color vision and visual fields, however, could not be assessed. Pupils were bilaterally 6 mm, sluggishly reacting to light. Rest anterior segment examination was unremarkable. Intraocular pressures were 10 and 12 mmHg, respectively, on Perkin's applanation tonometer.

Dilated fundus examination showed bilateral disc edema with obscuration of all margins in both the eyes [Figure 1] and [Figure 2]. Blood vessels were dilated and telangiectatic. However, no collaterals were seen. There were no peripapillary hemorrhages seen. Blood tests including blood sugar levels, serum homocysteine, erythrocyte sedimentation rate, and c-reactive protein were normal. A lumbar puncture was carried out and cerebrospinal fluid (CSF) was studied. CSF IgG was 3.48, while the rest was within the normal limits. Magnetic resonance imaging of the brain plain and contrast studies were carried. Multiple T2 and fluid-attenuated inversion recovery hypersignal intensity was noted in the bilateral parietal and right frontal region at the corticosubcortical level. It was associated with gyral enhancement in the bilateral frontoparietal region [Figure 3]. Degenerative disorders were suspected and further blood testing was carried out. Antinuclear antibodies, p-antineutrophil cytoplasmic antibodies (ANCA), and c-ANCA were negative. Aquaporin-4 antibodies were negative, but myelin oligodendrocyte glycoprotein (MOG) antibodies were positive, thus supporting the diagnosis of NMO.
Figure 1: Fundus picture of the right eye showing disc edema with a blurring of all disc margins

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Figure 2: Fundus picture of the left eye showing disc edema with a blurring of all disc margins

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Figure 3: Magnetic resonance imaging of the brain showing multiple hyperintensities noted in the bilateral parietal and right frontal region

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Corticosteroids are the mainstay treatment for the acute phase of the disease. The patient was started on intravenous methylprednisolone at a dose of 1 mg/kg/day for 3 days and then subsequently shifted to oral prednisolone 40 mg/day for 4 weeks. The visual acuity was reassessed every 2 days. The patient was also given long-term care including medical rehabilitation, management of anxiety and depression, and symptomatic treatment for gastrointestinal problems, pain, etc.

On the 10th day follow-up, best-corrected visual acuity was same in the right eye (RE) but improved to finger counting at 2 m in the left eye (LE). After 1 month, the LE visual acuity further improved to 6/60. Fundus examination showed a pale, atrophic disc in the RE [Figure 4], whereas the LE showed resolution of disc edema [Figure 5].
Figure 4: Fundus picture of the right eye on day 10 showing pale, atrophic optic disc

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Figure 5: Fundus picture of the left eye on day 10 showing resolving disc edema

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This patient was also treated with 50 mg twice daily dose of azathioprine as maintenance therapy to prevent relapse and no relapse episodes have been noticed till date.


  Discussion Top


NMO, also known as Devic's disease, is an autoimmune, immunological disease with formation of antibodies against the central nervous system.[3] These antibodies cause demyelination of the optic nerve and spinal cord giving rise to the following distinctive features:

Optic neuritis that presents with acute optic disc swelling and can further progress to optic atrophy.

There is decreased visual acuity, abnormalities in color vision, and reduced field of vision.

A relative afferent pupillary defect might be present.

The spinal cord involvement leads to hemiparesis or even tetraparesis with bladder involvement.

NMO usually has a chronic and relapsing course with recurrent attacks of transverse myelitis or optic neuritis which are usually followed by only partial recovery.

Dyspnea may also be present.

Other clinical symptoms include brain stem symptom, posterior reversible encephalopathy syndrome, coma, hypothalamic dysfunction, depression, cognitive disorders, psychiatric symptoms, and abnormal endocrinopathy.[4]

NMO is a rare syndrome accounting for <1% of demyelinating disease. A variable incidence has been reported ranging from 0.05 to 4.4 per 100,000.[4] The typical clinical manifestations of NMO, mainly optic neuritis and transverse myelitis, can occur simultaneously or separated by a variable amount of time. While in 90% of cases, the presentations are polyphasic, 10% monophasic presentation has also been reported.

The presence of antibodies proves the definitive diagnosis of NMO. The aquaporin-4 (AQP4) immunoglobulins are detected in about 60%–90% of patients who meet the clinical and radiological criteria of NMO. However, in patients who are AQP4 antibodies negative, it is imperative to check MOG antibodies.[5] This patient was negative for AQP4 antibodies, but positive for MOG antibodies. Patients with MOG antibodies are more likely to have a spinal involvement and thus likely to have a severe disease course.

The differential diagnoses for NMO include multiple sclerosis (MS), acute disseminated encephalomyelitis, idiopathic acute transverse myelitis, and systemic lupus erythematosus. Although NMO was previously thought to be a variant of MS, it has now been recognized as a separate disease entity.[6] Distinguishing NMO from MS is very important as presentation, pathogenesis, progression, treatment, and prognosis of both diseases are different.


  Conclusion Top


Although NMO is a rare disorder, it is important to study the disease pathogenesis in details. Rapid diagnosis and early initiation of aggressive immunosuppressive treatment are essential in most NMO cases. The chances of recurrence of the disease are >90% and these subsequent attacks can prove to be life-threatening if lesions involve the spinal cord or the brain stem by affecting the respiratory centers. While NMO is a rare disorder, cases with bilateral optic neuritis should be evaluated in detail, and the possibility of NMO should be taken into account in the differential diagnosis to ensure timely diagnosis and effective management. This not only improves the life expectancy, but may also improve the quality of life of the patient by reducing the number of relapses.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sherman E, Han MH. Acute and chronic management of neuromyelitis optica spectrum disorder. Curr Treat Options Neurol 2015;17:48.  Back to cited text no. 1
    
2.
Simon KC, Schmidt H, Loud S, Ascherio A. Risk factors for multiple sclerosis, neuromyelitis optica and transverse myelitis. Mult Scler 2015;21:703-9.  Back to cited text no. 2
    
3.
Wingerchuk DM. Evidence for humoral autoimmunity in neuromyelitis optica. Neurol Res 2006;28:348-53.  Back to cited text no. 3
    
4.
Estiasari R. Neuromielitis Optik. In: Aninditha T, Wiratman W, editors. Buku Ajar Departemen Neurologi. Jakarta: Penerbit Kedokteran Indonesia; 2017. p. 258-64  Back to cited text no. 4
    
5.
Jasiak-Zatonska M, Kalinowska-Lyszczarz A, Michalak S, Kozubski W. The immunology of neuromyelitis optica-current knowledge, clinical implications, controversies and future perspectives. Int J Mol Sci 2016;17:273.  Back to cited text no. 5
    
6.
De Seze J. Neuromyelitis optica. Arch Neurol 2003;60:1336-8.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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