Zahedan J Res Med Sci. In Press(In Press):e10336. Published online 2017 June 30. doi: 10.5812/zjrms.10336. Case Report Presentation of Neuromyelitis Optica with Recurrent Severe Myelitis and Acute Respiratory Failure in an Old Woman Saeed Razmeh, 1,* Zahra Ghurchian, 1 Amir Hasan Habibi, 1 Farzad Sina, 1 and Mostafa Almasi 1 1 Neurology Department, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran * Corresponding author: Saeed Razmeh, First unit, Number 57, West 17th St, Ghalam St, Shahinvilla, Tehran, Iran. Tel: +98-9397631901, E-mail: srazme82@gmail.com Received 2017 June 03; Revised 2017 March 07; Accepted 2017 June 09. Abstract Neuromyelitis Optica (NMO) is a rare disease of the central nervous system that causes optic nerve and spinal cord involvement. The our patient first developed acute paraplegia that was treated with intravenous methylprednisolone with diagnosis of acute thoracic myelitis according to magnetic resonance imaging (MRI) finding , concurrently with tapering of oral prednisolone, again affected by quadriplegia and respiratory failure. She was seropositivity for NMO-IgG that was negative in first admission and MRI of spine shows hyperintense lesion in whole cervical and upper thoracic MRI. With considering the findings, NMO was diagnosed and the plasmapheresis starts for her. We report a case of this syndrome because it can increase the physician’s awareness of the unusual manifestations of this syndrome. Keywords: Neuromyelitis Optica, Myelitis, Respiratory Failure 1. Introduction NMO, also called Devic disease, is an uncommon form of inflammatory demyelinating diseases of the central ner- vous system (CNS) that cause optic neuritis and transverse myelitis [1]. In other words, it is an autoimmune inflam- matory disease of the CNS in which NMO-IgG binds to the aquaporin-4 (AQP4) water channel. it is may be con- fused with multiple sclerosis but it has distinctive clinical and pathological features, mainly it causes necrosis of the spinal cord and affecting white matter and gray matter of the brain and spinal cord and usually with an absence of OCB in cerebrospinal fluid (CSF) [2-4]. Anti-aquaporin 4 an- tibody (NMO IgG AB) is the useful test that is highly spe- cific and sensitive. The treatment for this disease involves acute management of acute attacks with intravenous (IV) methylprednisolone and plasma exchange, and preven- tion of future attacks with immunosuppressants includ- ing mycophenolate mofetil, azathioprine and rituximab [5, 6]. We report a severe seropositive NMO in old Iranian woman with recurrent severe myelitis and respiratory fail- ure. 2. Case Presentation A 73-year-old lady who was referred to our hospital with 7 days history of lower limbs weakness, gait disturbance and urinary retention that progress to paraplegia during 2 days. She had no history of medical problems, doesn’t take any medication and her family history, social history, and review of systems were negative. On physical examina- tion she was conscious and had stable vital signs, he had no neck vein engorgement, no carotid bruit. Chest examina- tion was normal. She had, normal rate and regular rhythm, no murmur and without respiratory distress. Abdomen was soft, non-tender, without hepatosplenomegaly. In the extremities the pulses were good and symmetric. The neu- rological examination showed paraplegia with hypotonia, hyporeflexia and bilateral extensor plantar response with T6 level. Basic blood test shows hyperglycemia. B12 level was normal. HIV, HTLV1 and both vasculitis and malig- nancy workup were negative. NMO antibody was posi- tive. Brain and whole spine MRI show hyperintensity in periventricular, juxtacortical area with a hyperintense lon- gitudinal lesion in T2 weighted sequences of the thoracic spinal cord. Cerebrospinal fluid (CSF) was normal in cell count, protein and glucose. Viral polymerase chain reac- tion (PCR) and Oligoclonal bands (OCB) were negative. The patient was treated with a 5 -day course of intravenous methylprednisolone (1gr daily) with diagnosis of NMO that achieved mild improvement. She was discharged on oral prednisolone and folic acid and mineral. Concurrently with the tapering of prednisolone, again approximately 2 monthly later, the patient presented to emergency room with quadriplegia, dyspnea and bladder and bowel dys- function. MRI of spine shows hyperintense lesion in whole cervical and upper thoracic MRI (Figure 1). Plasma ex- change was started for patient with total dose 250 mL/kg but in 4 days of admission, she developed respiratory ar- rest and intubated with mechanical ventilation in ICU and Copyright © 2017, Zahedan Journal of Research in Medical Sciences. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.