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 Table of Contents  
BRIEF COMMUNICATION
Year : 2015  |  Volume : 3  |  Issue : 1  |  Page : 32-35

Neuromyelitis optica: Devic's disease


Department of Ophthalmology, Bharati Vidyapeeth Deemed University Medical College and Hospital, Sangli, Maharashtra, India

Date of Submission17-Dec-2013
Date of Acceptance08-Jul-2014
Date of Web Publication14-Jan-2015

Correspondence Address:
Ajit Joshi
Abhang, 248, 9/A, Anand Park, Road No. 2, Shinde Mala, Abhay Nagar, Sangli - 416 416, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-3897.149371

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  Abstract 

A 14-year-old female patient presented with sudden blindness in both eyes and inability to move both upper and lower limbs for 1 day. Clinical findings, magnetic resonance imaging (MRI), and cerebro-spinal fluid (CSF) examination fulfilled the diagnostic criteria of Devic's disease, one of the rare forms of demyelinating disease. After intravenous methylprednisolone treatment, her visual acuity and motor functions returned to normal.

Keywords: Neuromyelitis optica, NMO-IgG antibodies, optic neuropathy, transverse myelitis


How to cite this article:
Joshi A, Suryawanshi M, Ayyori S. Neuromyelitis optica: Devic's disease. J Clin Ophthalmol Res 2015;3:32-5

How to cite this URL:
Joshi A, Suryawanshi M, Ayyori S. Neuromyelitis optica: Devic's disease. J Clin Ophthalmol Res [serial online] 2015 [cited 2020 Nov 25];3:32-5. Available from: https://www.jcor.in/text.asp?2015/3/1/32/149371

Devic's neuromyelitis optica (NMO) is a rare type of autoimmune channelopathy in which the target antigen is Aquaporin-4 water channel found in cell membrane of the processes of astrocytes that surround and protect the blood brain barrier in central nervous system (CNS). It is an inflammatory, demyelinating disease characterized by severe necrotizing transverse myelitis and acute optic neuropathy, which can present uniocularly or binocularly. When binocular, both optic nerves can get affected simultaneously or successively. The disease may be monophasic or multiphasic. [1] The diagnosis is bolstered by detecting the NMO antibodies in the blood. We report a case that fulfilled NMO diagnostic criteria and had a favorable response to methylprednisolone treatment. There is a considerable overlap between NMO and multiple sclerosis (MS), and the purpose of this case report is to avoid misdiagnosis of MS in place of NMO.


  Case Report Top


A 14-year-old female patient presented with complaints of sudden, painless loss of vision in both eyes followed by inability to walk for 1 day. History of previous viral illness, autoimmune disorders, HIV infection was ruled out. Ocular examination showed no perception of light (No PL) in both eyes. Both pupils were dilated and fixed with absence of light and accommodation reflexes. Optic disc and retina in both eyes were normal. Neurological examination revealed flaccid paralysis and loss of all modalities of sensation from C 3 level and further down below. A bilateral positive Babinski's sign was noted. Cognitive functions were normal. All other cranial nerves were normal. Laboratory investigations such as test for anti-DNA antibodies, retroviral antibodies (by ELISA method), Thyroid functions, Syphilis, thymoglobulin, and rheumatoid factor were done in addition to standard hematologic investigations. All investigations were normal. Testing for myasthenia gravis-related antibodies was performed (AchR Ab) and was negative. Cerebro-spinal fluid (CSF) analysis showed pleocytosis (50 cells /mm 3 ) with absence of oligoclonal bands. NMO-IgG serum antibody test was positive by HEK293 cell-based assay. Magnetic resonance imaging (MRI) scan of spinal cord on sagittal T1- and T2-weighted images indicated demyelination from C3 to C6 [Figure 1] and [Figure 2], and MRI scan of brain showed swelling and hyper intense signal of optic nerves on T1 sagittal view and T2 coronal view suggestive of transverse myelitis with bilateral optic neuropathy [Figure 3] and [Figure 4]. Visual evoked potential (VEP) test showed both prolonged latency of P-100 and reduced amplitude suggestive of demyelination due to optic neuritis. [2]
Figure 1: Cervical spinal cord T1W MRI sagittal scan indicating oedematous C3-C6 segments as shown by arrow markings

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Figure 2: Cervical spinal cord T2W MRI sagittal scan indicating oedematous C3-C6 segments as shown by arrow markings

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Figure 3: T1-weighted sagittal view MRI brain showing optic neuritis as indicated by arrow marking

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Figure 4: T2-weighted coronal view MRI brain showing bilateral optic neuritis as indicated by arrow markings

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Based on clinical examination, radiological investigations, CSF analysis, NMO antibody test, and VEP clinical diagnosis of NMO were done. Intravenous methylprednisolone was given (1 gm/day) for 5 days followed by oral steroid 50 mg/day, tapered over 2 weeks. Her vision began to improve by third day, and at the end of third week it was 6/6 in both eyes. Her motor function started improving by 10th day, and by the end of third week she started walking normally. Now, for more than 6 months, she is leading a normal life and is being followed up regularly for relapses.


  Discussion Top


In NMO, one or both optic nerves may be involved and the time interval between ON and TM varies considerably. Bilateral ON with TM is uncommon and was seen in around 10% of the patients. [3] Our case had this uncommon presentation. Co-existing autoimmune diseases like SLE, Sjogren's syndrome, and myasthenia gravis are found to be associated with AQP4-Ab positive Devic's disease patients. In our case, there was no clinical and laboratory evidence suggestive of above diseases except positive serum NMO-IgG titer. NMO-TM is usually severe and is typically extending over 3 or more cord segments. In our case, MRI showed involvement from C3-C6 segments.

D. M. Wingerchuck et al. proposed a revised set of criteria for diagnosis of Devic's diseases in 2006 shown in [Table 1]. [4]

Neuromyelitis optica is an autoimmune condition with an immunopathogenesis distinct from that of MS as highlighted in [Table 2] and [Table 3].

We have not started preventive therapy for our patient considering the following points:
Table 1: Revised diagnostic criteria for neuromyelitis optica


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Table 2: Differences between NMO and MS


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Table 3: Various modalities of treatment advised for NMO


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  1. Patient's age, severity of the attack of ON, and paraplegia are suggestive of monophasic illness, which showed complete recovery after steroid therapy;
  2. Non-specific nature of immunosuppressants in NMO, their tendency to cause adverse reactions and infections, which has an association with NMO; [11]
  3. Economic constraints of the patient;
  4. In our case, patient refused to take preventive immunosuppressive therapy, so we advised her to have regular follow-ups in order to start the treatment if patient becomes symptomatic again.


Patient has been informed about possible future relapses (which may not be there if the disease turns out to be monophasic) and unpredictability of the time of second attack in case of polyphasic illness. We stress here the need of proper strategic guidelines regarding preventive immunosuppression in such cases while working in resource poor peripheral health centers. [12]

Relapsing type is seen in 85% of patients and is characterized by relapsing episodes of ON and TM, 90% occurring within 3 years after the index event. Each relapse follows incomplete recovery leading to early incremental disability. Within 5 years of disease onset 60% of patients with relapsing NMO are blind in one or both eyes or require ambulatory help. Due to the availability of AQP4 tests allowing earlier diagnosis and treatment 5-year survival rates of >90% have been reported. [1,13] Index events are less severe in relapsing type with worse outcome and variable interval of relapses (early, in clusters and at unpredictable intervals). [14]

In monophasic type, index events are more severe with relatively better outcome. Five-year survival rate is 90%, blindness in at least one eye in 22% and 30% have permanent paralysis in one or both legs. Long-term neurologic prognosis is somewhat better due to non-accumulative disability from recurrent attacks.

AQP4-Ab-positive NMO is part of an expanding spectrum of humorally mediated autoimmune diseases of the CNS that have been identified over the past few years. AQP4 autoimmunity is associated with a much broader range of CNS symptoms than just NMO, referring it as 'NMO spectrum disorder', which includes patients who have only myelitis or optic neuritis or patients presenting with other clinical symptoms such as brainstem encephalitis with intractable hiccups and vomiting or a syndrome of inappropriate antidiuretic hormone secretion. Other uncommon symptoms and syndromes include seizures, posterior reversible encephalopathy syndrome, myeloradiculitis, meningoencephalitis, findings related to brainstem involvement such as hearing loss, diplopia, olfactory dysfunction, and other cranial nerve palsies, or endocrinological abnormalities due to diencephalic lesions, pain syndromes, and cognitive dysfunction. [15]

In conclusion, NMO is quite different from MS, and this case report may bring us one step closer in resolving dilemma between NMO and MS.

 
  References Top

1.
Jarius S, Ruprecht K, Wildemann B, Kuempfel T, Ringelstein M, Geis C, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients. J Neuroinflammation 2012;9:14.  Back to cited text no. 1
    
2.
Ringelstein M, Klieter I, Ayzenberg I, Borisow N, Paul F, Ruprecht K, et al. Visual evoked potentials in neuromyelitis optica and its spectrum disorders. Mult Scler 2014;20:617-20.  Back to cited text no. 2
    
3.
Uzawa A, Mori M, Muto M, Masuda S, Kuwabara S. When is neuromyelitis optica diagnosed after disease onset? J Neurol 2012;259:1600-5.  Back to cited text no. 3
    
4.
Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology 2006;23:1485-9.  Back to cited text no. 4
    
5.
Kim SH, Kim W, Li XF, Jung IJ, Kim HJ. Does interferon beta treatment exacerbate neuromyelitis optica spectrum disorder? Mult Scler 2012;18:1480-3.  Back to cited text no. 5
    
6.
Kleiter I, Hellwig K, Berthele A, Kümpfel T, Linker RA, Hartung HP, et al. Neuromyelitis Optica Study Group. Failure of natalizumab to prevent relapses in neuromyelitis optica. Arch Neurol 2012;69:239-45.  Back to cited text no. 6
    
7.
Min JH, Kim BJ, Lee KH. Development of extensive brain lesions following fingolimod (FTY720) treatment in a patient with neuromyelitis optica spectrum disorder. Mult Scler 2012;18:113-5.  Back to cited text no. 7
    
8.
Kitley JL, Leite MI, George JS, Palace JA. The differential diagnosis of longitudinally extensive transverse myelitis. Mult Scler 2012;18:271-85.  Back to cited text no. 8
    
9.
Jarius S, Paul F, Franciotta D, Ruprecht K, Ringelstein M, Bergamaschi R, et al. Cerebrospinal fluid findings in aquaporin 4 antibody positive neuromyelitis optica: Results from 211 lumbar punctures. J Neurol Sci 2011;306:82-90.  Back to cited text no. 9
    
10.
Jarius S, Wildemann B. Aquaporin 4 antibodies (NMO-IgG) as a serological marker of neuromyelitis optica: A critical review of the literature. Brain Pathol 2013;23:661-83.  Back to cited text no. 10
    
11.
Bienia B, Balabanov R. Immunotherapy of neuromyelitis optica. Autoimmune Dis 2013;2013:741490.  Back to cited text no. 11
    
12.
Pandit L, Mustafa S, Kunder R, Shetty R, Misri Z, Pai S, et al. Optimizing the management of neuromyelitis optica and spectrum disorders in resource poor settings: Experience from the Mangalore demyelinating disease registry. Ann Indian Acad Neurol 2013;16:572-6.  Back to cited text no. 12
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13.
Collongues N, Marignier R, Zéphir H, Papeix C, Blanc F, Ritleng C, et al. Neuromyelitis optica in France: A multicenter study of 125 patients. Neurology 2010;74:736-42.  Back to cited text no. 13
    
14.
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. 14
    
15.
Jarius S, Wildemann B, Paul F. Neuromyelitis optica: Clinical features, immunopathogenesis, and treatment. Clin Exp Immunol 2014;176:149-64.  Back to cited text no. 15
    


    Figures

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

  [Table 1], [Table 2], [Table 3]



 

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