TABLE 2.
Neuromyelitis Optica Spectrum Disorders—Magnetic Resonance Imaging features and systemic autoimmunity
| Associated MRI and Autoimmune Features of NMOSD |
|---|
| Optic nerve MRI |
| Increased T2 signal (standard T2 or STIR sequences) or T1 gadolinium enhancement of one or both optic nerves (Fig. 1C) |
| Additional characteristic features |
| Lesions are typically long (encompassing more than half of the optic nerve) or involve multiple regions of the nerve |
| Lesions typically involve the posterior, intracranial portion of the optic nerve and optic chiasm (Fig. 1D) |
| Spinal cord MRI |
| Longitudinally extensive lesion demonstrating increased T2 signal (standard T2, proton density, or STIR sequences) involving 3 or more contiguous vertebral segments (Fig. 1A) |
| Central cord predominance (>70% of the lesion residing within the central gray matter) (Fig. 1B) |
| Postcontrast enhancement of the lesion on T1 sequences (Fig. 1B′) |
| Additional characteristic features |
| Rostral extension into brainstem (Fig. 1A) |
| Cord edema |
| Cord atrophy may be observed in cases with long-standing injury |
| Cerebral MRI lesions |
| Large, confluent subcortical or deep white matter lesions (Fig. 1I, J) |
| Long diffuse or edematous corpus callosum lesions (typically involving half the length of the corpus callosum) |
| Diencephalic MRI Lesions |
| Thalamic (Fig. 1J) |
| Hypothalamic lesions (Fig. 1H) |
| Periependymal lesions around the third ventricle |
| Brainstem MRI lesions |
| Lesions of the dorsal medulla (area postrema) (Fig. 1E) |
| Additional characteristic features |
| Unilateral or bilateral lesions (Fig. 1F) |
| Often contiguous with an upper cervical spinal cord lesion (Fig. 1A) |
| Periependymal lesions around the fourth ventricle (Fig. 1G) |
| Extensive postcontrast enhancing periependymal brain lesions |
| Long corticospinal tract lesions involving internal capsule and cerebral peduncle |
| Associated systemic autoimmunity (1,17) |
| Antinuclear autoantibodies |
| Neural autoantibodies (anti-GAD, anti-CRMP5, anti-Ro, anti-VGC, anti-AchR) |
| Autoimmune disorders: thyroiditis, Sjogren disease, systemic lupus erythematosus, myasthenia gravis |
AchR, acetylcholine receptor; CRMP5, collapsin response-mediator protein 5; GAD, glutamic acid decarboxylase; MRI, magnetic resonance imaging; NMOSD, neuromyelitis optica spectrum disorder; STIR, short tau inversion recovery; VGC, voltage-gated channel.