Neuromyelitis Optica Spectrum Disorder Complicated by Posterior Reversible Encephalopathy Syndrome as an Initial Manifestation

Abstract

A 25-year-old woman was admitted to our hospital due to tonic convulsion with severe headache after having experienced symptoms of nausea and vomiting for a month. Brain MRI showed extensive symmetrical lesions in the cortical and subcortical areas of parieto-occipital lobes and basal ganglia, consistent with typical characteristics of posterior reversible encephalopathy syndrome (PRES). Furthermore, some residual lesions in the left side of dorsal medulla oblongata and central area of the cervical spinal cord along with the presence of serum anti-aquaporin-4 antibody yielded the diagnosis of neuromyelitis optica spectrum disorder (NMOSD). We herein discuss the mechanism by which PRES may occur together with NMOSD.

The pathogenesis and treatment of posterior reversible encephalopathy syndrome after neuromyelitis optica spectrum disorder: a case report and literature review

“…In conducting an analysis of the previous case reports, we found some patients’ NMOSD and PRES to have had simultaneous onsets of attack and lacked inducing factors in the prodromal stage [ 3 , 7 , 12 ]. Additionally, the asymmetric distribution, atypical location, and non-regression of some PRES lesions [ 3 , 5 , 7 , 10 , 12 ] all suggest that PRES may be a special manifestation of NMOSD. On the contrary, definite inducing factors and characteristic lesions support PRES as a complication of immunotherapy in the treatment of NMOSD [ 3 , 4 , 6 , 9 ].…”