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Neuromyelitis optica (NMO; Devic’s disease) is an aggressive inflammatory disorder characterized by recurrent attacks of optic neuritis (ON) and myelitis; the more inclusive term NMO Spectrum Disorders (NMOSD) incorporates pts with partial forms, and with involvement of additional CNS structures (Table 193-1). NMO is more frequent in women than men (>3:1), and typically begins in adulthood but can arise at any age. An important consideration is distinguishing between NMO and multiple sclerosis (MS; Chap. 192). In NMO, attacks of ON can be bilateral and produce severe visual loss (uncommon in MS); myelitis can be severe and transverse (rare in MS) and is typically longitudinally extensive (Fig. 193-1) involving three or more contiguous vertebral segments. Also in contrast to MS, progressive symptoms typically do not occur in NMO.

FIGURE 193-1

Imaging findings in neuromyelitis optica: longitudinally extensive transverse myelitis, optic neuritis and brainstem involvement. (A) Sagittal fluid attenuation inversion recovery (FLAIR) cervical-spine magnetic resonance image (MRI) showing an area of increased signal change on T2-weighted imaging spanning more than 3 vertebral segments in length. (B) Sagittal T1-weighted cervical-spine MRI following gadolinium-DPTA infusion showing enhancement. (C) Coronal brain MRI shows hyperintense signal on FLAIR imaging within the left optic nerve. (D) Coronal T1-weighted brain MRI following gadolinium-DPTA infusion shows enhancement of the left optic nerve. (E) Axial brain MRI shows an area of hyperintense signal on T2-weighted imaging within the area postrema (arrows). (F) Axial T1-weighted brain MRI following gadolinium-diethylene triamine pentaacetic acid (DPTA) infusion shows punctate enhancement of the area postrema (arrows).

TABLE 193-1Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorder

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