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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 59  |  Issue : 1  |  Page : 80-82

Devic's disease: A rare case presentation


Consultant Ophthalmologist, Arunodaya Deseret Eye Hospital, Gurugram, Haryana, India

Date of Submission02-Sep-2020
Date of Acceptance13-Jan-2020
Date of Web Publication27-Mar-2021

Correspondence Address:
Dr. Sahebaan S Sethi
Arunodaya Deseret Eye Hospital, NH-4, Sector 55, Gurugram - 122 011, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_129_20

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  Abstract 


We report a case of a young patient with sudden drop in vision and lower limb weakness. On examination was found to have left eye disc edema and abnormal CNS function. On further investigation, the patient was diagnosed to have optic neuritis with associated transverse myelitis-a syndrome referred to neuromyelitis optica or Devic's Disease. This case emphasizes the need for timely intervention to prevent permanent vision loss.

Keywords: Devic's disease, neuromyelitis optica, optic neuritis, transverse myelitis


How to cite this article:
Sethi SS, Sethi A. Devic's disease: A rare case presentation. TNOA J Ophthalmic Sci Res 2021;59:80-2

How to cite this URL:
Sethi SS, Sethi A. Devic's disease: A rare case presentation. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Apr 22];59:80-2. Available from: https://www.tnoajosr.com/text.asp?2021/59/1/80/312278




  Introduction Top


Neuromyelitis optica (NMO), characterized by concurrent inflammation and demyelination of the optic nerve (optic neuritis [ON] and the spinal cord (myelitis), is an immune-mediated inflammatory disorder of the central nervous system.[1] It occurs as an autoimmune disorder of the central nervous system, mainly involving the optic nerves and spinal cord, and sometimes in the brain.

NMO may occur after an infection but the cause remains largely unknown, although it may be associated with another autoimmune condition. Thus, it is often misdiagnosed as multiple sclerosis (MS) or as a type of MS, even though it is a distinct condition.[2]

NMO though extremely rare, affecting between 0.05 and 4.4 in every 100,000 people worldwide, affects the age group above 40 years more often.[3] NMO episodes may be reversible, but can be severe enough to cause unilateral or bilateral blindness, weakness, or paralysis in the legs or arms.


  Presentation Top


A 26-year-old male presented to us with complaints of sudden diminution of vision and pain on movement in the left eye since a day. He gave a history of suffering from fever with chills with rigors and loss of bladder control 2 days back which was associated with pain and distention of abdomen. He also gave a history of weakness in right lower limb, followed by left lower limb since the past 3 days.

The patient was conscious, alert, and oriented. On systemic examination, he was found to be essentially normal. Examination of the central nervous system revealed GCS of 15/15,

  • Power


    • Right lower limb-3+/5
    • Left lower limb-4+/5
    • Both upper limbs-4+/5


  • Sensation: Bilaterally reduced below the sternum. Altered sensation D10-D11
  • Plantar reflex: Right–exaggerated; left-reduced
  • Deep tendon reflexes: brisk more in lower limb
  • Tone: Both upper and lower limb-normal.


Ocular examination findings showed right eye normal findings, left eye showed a drop in vision to counting finger at 50 m, drop in color vision to 10/21 (Ishihara Chart). Pupil examination of the left eye showed a relative afferent pupillary defect. Fundus examination revealed left eye disc edema and right eye normal fundus, which was captured on fundus photography [Figure 1]. Magnetic resonance imaging (MRI) brain was done [Figure 2] and was suggestive of demyelination at T7-T9 level with left ON. Cerebrospinal fluid oligoclonal bands were positive. NMO antibodies were positive.
Figure 1: Right eye normal optic disc; left eye disc edema

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Figure 2: Magnetic resonance imaging brain suggestive of left optic neuritis

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On the basis of clinical examination and investigations, a diagnosis of acute transverse myelitis with left on also known as Devic's disease was made.

The patient was immediately treated with high-dose intravenous steroids iv methylprednisolone 1 gm/day for 3 days followed by tapering dose of oral steroids 1 mg/kg body weight over 3 weeks. The patient's visual function recovered fully at 1 month follow-up.


  Discussion Top


Devic's disease is an autoimmune inflammatory disorder wherein patient's own immune system-antibodies called NMO-immunoglobulin G (IgG) attack protein of nervous system cells called aquaporins in the cell membrane of astrocytes. This produces ON and myelitis. Symptoms include loss of vision or decreased visual acuity, visual field defect, loss of color vision, loss of spinal cord function leading to muscle weakness, spastic paraparesis or tetraparesis, reduced sensation, and loss of bladder/bowel control. Optic nerve involvement can be unilateral or bilateral[4] and is a painful condition worsened by movement with decreased visual acuity in the affected eye and is the most common initial symptom.[5] Half of the patients lose functional vision within 5 years of onset.[6] Myelitis involvement classically involves more than three spinal cord segments.[4]

Currently, three supposed causes of the disease are:

  1. A first subset of patients is anti-AQP4+. In them, the cause of the NMO (understood as the syndrome) is an autoimmune aquaporin-4 channelopathy, due to these specific autoantibodies.[7] In these cases, astrocytes are the victims of the autoimmune attack
  2. A second subset of patients is anti-MOG+. In them, the cause is an anti-MOG associated encephalomyelitis[8]
  3. In a third subset of patients, NMO is due to the presence of anti-neurofascin auto-antibodies which damage the Ranvier nodes of the neurons.[9] These antibodies are more related to the peripheral nervous demyelination, but they were also found in NMO.[10]


The etiology is actually unknown. It is said to have been associated with other autoimmune diseases such as collagen vascular disease, autoantibody syndrome, infections such as varicella zoster, Epstein–Barr virus, human immunodeficiency virus, and exposure to clioquinol and antituberculosis drugs.

The Mayo Clinic proposed a revised set of criteria for the diagnosis of Devic's disease in 2006. Those new guidelines require two absolute criteria plus at least two of three supportive criteria.[11]

Absolute criteria: (1) optic neuritis, (2) acute myelitis.

Supportive criteria: (1) Brain MRI not meeting criteria for MS at disease onset. (2) Spinal cord MRI with continuous T2-weighted signal abnormality extending over three or more vertebral segments, indicating a relatively large lesion in the spinal cord. (3) NMO-IgG seropositive status (The NMO-IgG test checks the existence of antibodies against the aquaporin 4 antigen.)

Currently, there is no cure for Devic's disease, but symptoms can be treated. Some patients recover, but many are left with impairment of vision and limbs, which can be severe.


  Conclusion Top


Devic's disease is a rare encounter which when diagnosed correctly and intervened timely can have a good prognosis. This case also emphasizes the need for good history taking and thorough examination including general and systemic.

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.
Zarei S, Eggert J, Franqui-Dominguez L, Carl Y, Boria F, Stukova M, et al. Comprehensive review of neuromyelitis optica and clinical characteristics of neuromyelitis optica patients in Puerto Rico. Surg Neurol Int 2018;9:242.  Back to cited text no. 1
  [Full text]  
2.
Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805-15.  Back to cited text no. 2
    
3.
Etemadifar M, Nasr Z, Khalili B, Taherioun M, Vosoughi R. Epidemiology of neuromyelitis optica in the world: A systematic review and meta-analysis. Mult Scler Int 2015;2015:174720.  Back to cited text no. 3
    
4.
Masters-Israilov A, Robbins MS. Headache in neuromyelitis optica. Curr Pain Headache Rep 2017;21:20.  Back to cited text no. 4
    
5.
Morrow MJ, Wingerchuk D. Neuromyelitis optica. J Neuroophthalmol 2012;32:154-66.  Back to cited text no. 5
    
6.
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. 6
    
7.
Pittock SJ, Lucchinetti CF. Neuromyelitis optica and the evolving spectrum of autoimmune aquaporin-4 channelopathies: A decade later. Ann N Y Acad Sci 2016;1366:20-39.  Back to cited text no. 7
    
8.
Spadaro M, Gerdes LA, Mayer MC, Ertl-Wagner B, Laurent S, Krumbholz M, et al. Histopathology and clinical course of MOG-antibody-associated encephalomyelitis. Ann Clin Transl Neurol 2015;2:295-301.  Back to cited text no. 8
    
9.
Kira JI, Yamasaki R, Ogata H. Anti-neurofascin autoantibody and demyelination. Neurochem Int 2019 Nov;130:104360.  Back to cited text no. 9
    
10.
Jia K, Zhang X, Zhang LJ, Li LM, Qi Y, Yi M2, et al., Anti-neurofascin-155 antibody-positive neuromyelitis optica spectrum disorders. J Neurol Sci 2019;398:16-8.  Back to cited text no. 10
    
11.
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. 11
    


    Figures

  [Figure 1], [Figure 2]



 

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