University of Calgary
Department of Pediatrics

Author Of 1 Presentation

Neuromyelitis Optica and Anti-MOG Disease Poster Presentation

P0726 - Longitudinally extensive transverse myelitis and NMOSD associated with dengue infection: a case report and systematic review of cases (ID 1155)

Speakers
Presentation Number
P0726
Presentation Topic
Neuromyelitis Optica and Anti-MOG Disease

Abstract

Background

Neuromyelitis optica spectrum disorder (NMOSD) has been linked to para-and post-infectious triggers. Dengue virus (DENV) is one of the most common arbovirus infections in the world, with a wide spectrum of neurological clinical manifestations, including longitudinally extensive transverse myelitis (LETM), albeit rarely. It is unclear if the association is coincidental, permissive, or causal.

Objectives

We present a case of DENV-associated aquaporin-4 positive (AQP4) NMOSD with LETM with a systematic review of cases reported in the literature.

Methods

In addition to our case report, for the systematic review, we searched Medline/PubMed through April 2020, without language or design restrictions, for case reports, series and observational studies that described patients with DENV-associated LETM and/or NMOSD. We also hand-searched references and relevant conference abstracts.

Results

Case report: An adolescent girl who recently had immigrated from the Philippines presented with a 2-day history of paraparesis and urinary incontinence. Magnetic resonance imaging revealed spinal hyperintensity with patchy enhancement from T4-T7. AQP4 antibodies were positive on cell-based assay testing (Mayo Clinic Laboratories) with a titer of 1:10,000. She was diagnosed with AQP+ NMOSD. Infectious workup revealed serum +IgM and IgG antibodies against DENV, consistent with an acute dengue infection. She responded to high-dose steroids and subsequently started on rituximab maintenance. Literature review: Of 59 unique articles, 15 publications describing 18 patients met inclusion criteria. Age ranged from 8 months to 71-years (mean: 37.3) with no sex predominance. Imaging and CSF findings were heterogeneous. Four cases met 2015 criteria for NMOSD: two had LETM and optic neuritis, two had recurrent myelitis and area postrema syndrome, and one had LETM with AQP4 antibodies. The mainstay of treatment was IV methylprednisolone, although some also had IVIG and/or plasmapheresis. Prognosis varied, but few had long-term follow-up to assess for ongoing NMOSD activity.

Conclusions

DENV-associated LETM is rare, typically monophasic, and presents with severe disability, typically flaccid paraparesis. Immunotherapy should be instituted rapidly, particularly because the presentation could represent NMOSD. Decisions regarding longterm immunotherapy may depend on index of suspicion of true NMOSD, and this is where AQP4 status might be helpful. It is unknown whether there is an epidemiological or pathophysiological association between DENV infection and AQP4+ NMOSD.

Collapse