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Finding NeMO: Respiratory Failure Secondary to Neuromyelitis Optica FREE TO VIEW

Massa Zantah, MD; Debapriya Datta, MD; Timothy Coyle, MD
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UConn Health Center, Farmington, CT

Chest. 2015;148(4_MeetingAbstracts):276A. doi:10.1378/chest.2263560
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SESSION TITLE: Critical Care Student/Resident Case Report Posters II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Neuromyelitis optica (NMO) is a distinctive severe demyelinating autoimmnune disease that involves the central nervous system . Respiratory failure due to NMO is rare.We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement.

CASE PRESENTATION: A 53-year-old female with history of NMO presented with 3 days history of left sided weakness, numbness and tingling. She was hypoxemic with oxygen saturation of 85% with mild respiratory distress. Her examination was significant for mild weakness, decreased sensation to light touch and hyper-reflexia in the left upper and lower extremities.Her chest x-ray (Figure 1) showed hypoinflation with slightly elevated left diaphragm. Lung ultrasound showed a hemiparetic left diaphragm. ABG revealed a pO2 of 59 on 40% FIO2. The patient was placed on noninvasive mechanical ventilation for respiratory failure. Cervical spine MRI (Figure 2) showed Increased T2 signal within the spinal cord parenchyma extending from C2 to T1 vertebrae, with heterogeneous enhancement in the left lateral aspect of the cord between C2 to C4-C5 disc space. The patient was started on a pulse dose steroids for NMO exacerbation and received 5 days of plasmapheresis after which she showed clinical improvement. Her respiratory failure resolved and diaphragmatic excursion improved on fluoroscopy. The patient was started on rituximab for maintainance treatment and has been doing well since, on follow-up.

DISCUSSION: NMO is a rare severe inflammatory, demyelinating, and necrotizing autoimmune disease of central nervous system characterized by recurrent attacks of optic neuritis, longitudinally extensive myelitis and presence of NMO immunoglobulin G /aquaporin-4 antibodies. Spinal cord involvement may result in different presentations. Due to expansion to the medulla, neurogenic respiratory failure and subsequent death can occur. In a case series of 71 patients with NMO by Wingerchuk et al, only two patients with monophasic NMO had respiratory failure and both recovered. Eighty per cent of patients with acute NMO respond to high dose steroids. Plasma exchange and, less commonly, intravenous immunoglobulins may be used for severe symptoms refractory to steroids. In contrast to multiple sclerosis, maintenance therapy is better achieved with immunosuppressive medications rather than immunomodulator therapy . Azathioprine is the most commonly used and studied drug for maintenance. Either low dose oral Prednisolone or Rituximab may be added for maintainence.

CONCLUSIONS: NMO involving the cervical cord can lead to respiratory failure. It requires immediate treatment and shows good response with high dose steroids and plasmapheresis.

Reference #1: Krampla W et al. Spinal cord lesions in patients with neuromyelitis optica: a retrospective long-term MRI follow-up study. European radiology. 2009;19(10):2535-43.

Reference #2: Wingerchuk DM et al. The clinical course of neuromyelitis optica. Neurology. 1999;53(5):1107-14.

DISCLOSURE: The following authors have nothing to disclose: Massa Zantah, Debapriya Datta, Timothy Coyle

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