Original Research| Volume 74, 104656, June 2023

Clinical and radiological profile of neuromyelitis optica spectrum disorders in a Pakistani cohort


      • Patients presented with a combination of all known presentations of NMOSD, including ON and myelitis/ symptomatic narcolepsy.
      • 28 patients (77.8%) had longitudinal extensive transverse myelitis on MRI spine, whereas 27 patients (75%) had normal MRI brains.
      • In terms of treatment, 26 patients (72.2%) received pulse steroid while 10 patients (27.8%) received both pulse therapy and plasmapheresis.
      • 29 patients were treated with azathioprine (80.6%), 4 patients were treated with rituximab (11.1%), and 2 (8.6%) were treated with mycophenolate mofetil (5.6%) as immunomodulatory treatment of NMOSD.
      • More than half of the patients were diagnosed within 12 months of the index event (range 0–144 months), and multiple sclerosis was the most common initial misdiagnosis.



      The clinical and radiological characteristics of neuromyelitis optica spectrum disorder (NMOSD) from Pakistan is unknown. Our study aimed to describe the clinical and radiological features of NMOSD patients presenting to a Pakistani tertiary care center.

      Materials and methods

      This retrospective, observational study was conducted at the Neurology Department, Pakistan Institute of Medical Sciences between January 2017 and September 2021 (56 months). The study included patients diagnosed with neuromyelitis optica spectrum disorder (NMOSD) according to the 2015 International Panel for NMO Diagnosis (IPND) criteria, with the exclusion of patients under 12 years of age and those who tested positive for Myelin oligodendrocyte glycoprotein (MOG) IgG antibody. The patients were divided into two groups based on clinical presentation and the presence of NMO-IgG antibodies: NMO-IgG positive NMO (Seropositive NMO) and NMO-IgG negative (Seronegative NMO). The clinical features of NMOSD were recorded, and data was analyzed using SPSS version 26.0.


      Among 204 patients with suspected demyelination, multiple sclerosis was diagnosed in 100 individuals (49.02%), while acute disseminated encephalomyelitis (ADEM), clinically isolated syndrome (CIS), and neuromyelitis optica (NMO) were found in 5 patients each (2.45%, 2.45%, and 17.65%, respectively). Out of 36 patients with NMO, 32 (88.89%) tested positive for NMO-Ab, while the remaining 4 (11.11%) were seronegative for both NMO and anti-MOG Abs. The mean age of NMO-positive patients who tested positive for NMO antibodies was 31.03±10.12 years, compared to 27.95±2.5 years for NMO-negative patients, though this difference was not statistically significant (p>0.05). Females were more commonly affected by NMO, accounting for 72.2% of the NMO-positive group, and there was a significant difference in clinical phenotypes between the two groups (p<0.05). The NMO-positive group predominantly had relapsing NMO presentation (75%), and 72.8% of these patients showed longitudinally extensive transverse myelitis on the MRI spine. Azathioprine was the most frequently administered treatment for positive NMO patients (69.44%), followed by rituximab and MMF. The follow-up period for the study participants lasted 24 months.


      This is the first study on NMOSD cases in Pakistan. According to the present study, NMOSD is most prevalent among women in their forties. Relapsing NMO was the most common form of presentation. 89% of patients had antibodies against AQP4. 72.8% of patients suffered from LETM during the course of their disease. There are some features of our NMOSD cases that appear comparable with those around the world, despite some limitations in testing and access to care. It is clear that the clinical and radiological spectrums of patients with NMO and NMOSD in this cohort are similar. It is reasonable to suspect NMO if demyelinating episodes are not characteristic of MS.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Multiple Sclerosis and Related Disorders
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Jarius S.
        • Wildemann B.
        The history of neuromyelitis optica.
        J. Neuroinflammat. 2013; 10: 8
        • Wingerchuk D.M.
        • Hogancamp W.F.
        • O'Brien P.C.
        • Weinshenker B.G.
        The clinical course of neuromyelitis optica (Devic's syndrome).
        Neurology. 1999; 53: 1107-1114
        • Lennon V.A.
        • Wingerchuk D.M.
        • Kryzer T.J.
        • Pittock S.J.
        • Lucchinetti C.F.
        • Fujihara K.
        • et al.
        A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis.
        Lancet. 2004; 364: 2106-2112
        • Wingerchuk D.M.
        • Lennon V.A.
        • Pittock S.J.
        • Lucchinetti C.F.
        • Weinshenker B.G.
        Revised diagnostic criteria for neuromyelitis optica.
        Neurology. 2006; 66: 1485-1489
        • Wingerchuk D.M.
        • Banwell B.
        • Bennett J.L.
        • Cabre P.
        • Carroll W.
        • Chitnis T.
        • et al.
        International Panel for NMO Diagnosis. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.
        Neurology. 2015; 85: 177-189
        • Sepúlveda M.
        • Aldea M.
        • Escudero D.
        • Llufriu S.
        • Arrambide G.
        • Otero-Romero S.
        • et al.
        Epidemiology of NMOSD in Catalonia: influence of the new 2015 criteria in incidence and prevalence estimates.
        Mult. Scler. 2018; 24: 1843-1851
        • Papp V.
        • Magyari M.
        • Aktas O.
        • Berger T.
        • Broadley S.A.
        • Cabre P.
        • et al.
        Worldwide Incidence and Prevalence of Neuromyelitis Optica: a Systematic Review.
        Neurology. 2021; 96: 59-77
        • Jonsson D.I.
        • Sveinsson O.
        • Hakim R.
        • Brundin L.
        Epidemiology of NMOSD in Sweden from 1987 to 2013: a nationwide population-based study.
        Neurology. 2019; 93: e181-e189
        • Eskandarieh S.
        • Nedjat S.
        • Azimi A.R.
        • Moghadasi A.N.
        • Sahraian M.A.
        Neuromyelitis optica spectrum disorders in Iran.
        Mult. Scler. Relat. Disord. 2017; 18: 209-212
        • Waters P.J.
        • McKeon A.
        • Leite M.I.
        • Rajasekharan S.
        • Lennon V.A.
        • Villalobos A.
        • et al.
        Serologic diagnosis of NMO: a multicenter comparison of aquaporin-4-IgG assays.
        Neurology. 2012; 78: 665-671
        • Rajput H.M.
        • Wasay M.
        • Badshah M.
        • Hassan M.
        Multiple sclerosis in Pakistan and need for multiple sclerosis registry.
        Pak. J. Neurological. Sci-PJNS. 2019; 14: 52-58
        • Waters P.J.
        • McKeon A.
        • Leite M.I.
        • Rajasekharan S.
        • Lennon V.A.
        • Villalobos A.
        • et al.
        Serologic diagnosis of NMO. A multicenter comparison of aquaporin-4-IgG assays.
        Neurology. 2012; 78: 665-671
        • Bano S.
        • Javed M.A.
        • Numan A.
        Longitudinally extensive transverse myelitis and neuromyelitis optica spectrum disorder: a tertiary care centre experience.
        Pak. J. Neurological. Sci. -PJNS. 2020; 15: 36-40
        • Athar Javed M.
        • Zaman D.Q.
        • Alam D.N.
        Enam A. spectrum of acquired demyelinating disorders of the central nervous system in adults in a tertiary care hospital.
        Pak. J. Neurological. Sci. -PJNS. 2014; 9: 1-5
        • Ijaz U.
        • Habib A.
        • Rathore H.S.
        • Khan M.I.
        Spectrum of optic neuropathies presenting in neuroophthalmology clinic at AFIO.
        Pak. Armed Forces Med. J. 2021; 71: 112-116
        • Rafique S.
        • Wasim A.
        • Sultan T.
        • Ahmad A.
        Post-COVID neuromyelitis optica spectrum disorder.
        J. Coll. Physicians Surg. Pak. 2021; 31: 138-140
        • Jan D.
        • Shabir A.
        • Ibrahim S.
        Neuromyelitis optica (devic's disease) in a 10 years old boy.
        Pak. J. Neurological. Sci.-PJNS. 2016; 11: 27-30
        • Siddiqi S.A.
        • Hashmi M.
        • Azmat Z.
        • Mustafa S.
        • Siddiqui K.A.
        Pulmonary tuberculosis with neuromyelitis optica: an uncommon association of a common disease.
        J. Coll. Physicians Surg. Pak. 2012; 22: 527-528
        • Athar Javed M.
        • Zaman D.Q.
        • Alam D.N.
        Enam A. spectrum of acquired demyelinating disorders of the central nervous system in adults in a tertiary care hospital.
        Pak. J. Neurological. Sci.-PJNS. 2014; 9: 1-5
        • Iftikhar S.
        • Mahmood A.
        • Hashmat M.
        Neuromyelitis Optica as an Initial Presentation of Systemic Lupus Erythematosus in a Young Male.
        J. Coll. Physicians Surg. Pak. 2020; 30: 321-323
        • Waheed S.
        • Hussian J.
        • Saood Y.
        • Shehzadi A.
        Neuromyelitis Optica (nmo); A case report.
        Pak. J. Neurological. Sci. -PJNS. 2018; 13: 21-26
        • Shahzad W.
        • Rajput H.M.
        • Hassan M.
        • Inayat T.
        • Badshah M.
        A Case of Neuromyelitis Optica with Systemic Lupus Erythematosus.
        J. Coll. Physicians Surg. Pak. 2022; 32: 1498-1500
        • Shahmohammadi S.
        • Doosti R.
        • Shahmohammadi A.
        • Mohammadianinejad S.E.
        • Sahraian M.A.
        • Azimi A.R.
        • et al.
        Autoimmune diseases associated with Neuromyelitis Optica Spectrum Disorders: a literature review.
        Mult Scler Relat Disord. 2019; 27: 350-363
        • Man B.L.
        • Mok C.C.
        • Fu Y.P.
        Neuro-ophthalmologic manifestations of systemic lupus erythematosus: a systematic review.
        Int J Rheum Dis. 2014; 17: 494-501
        • Kovacs B.
        • Lafferty T.L.
        • Brent L.H.
        • DeHoratius R.J.
        Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature.
        Ann. Rheum. Dis. 2000; 59: 120-124