Neuromyelitis Optica Spectrum Disorder: A Case Report

Figures & data

Table 1 The 2015 International Panel for NMOSD Diagnostic Criteria for Adult Patients

Diagnostic criteria for NMOSD with AQP4-IgG

1. At least one core clinical characteristic

2. Positive test for AQP4-IgG using best available detection method (cell-based assay strongly recommended)

3. Exclusion of alternative diagnoses

Diagnostic criteria for NMOSD without AQP4-IgG or NMOSD with unknown AQP4-IgG status

1. At least two core clinical characteristics occurring as a result of one or more clinical attacks and meeting all of the following requirements:

(a) At least one core clinical characteristic must be optic neuritis, acute myelitis with LETM, or area postrema syndrome

(b) Dissemination in space (two or more different core clinical characteristics)

(c) Fulfillment of additional MRI requirements, as applicable

2. Negative tests for AQP4-IgG using best available detection method, or testing unavailable

3. Exclusion of alternative diagnoses

Core clinical characteristics

1. Optic neuritis

2. Acute myelitis

3. Area postrema syndrome: episode of otherwise unexplained hiccups or nausea and vomiting

4. Acute brainstem syndrome

5. Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions

6. Symptomatic cerebral syndrome with NMOSD-typical brain lesions

Additional MRI requirements for NMOSD without AQP4-IgG and NMOSD with unknown

AQP4-IgG status

1. Acute optic neuritis: requires brain MRI showing (a) normal findings or only nonspecific white matter lesions OR (b) optic nerve MRI with T2-hyperintense lesion or T1-weighted gadolinium enhancing lesion extending over >1/2 optic nerve length or involving optic chiasm

2. Acute myelitis: requires associated intramedullary MRI lesion extending over >3 contiguous segments (LETM) OR >3 contiguous segments of focal spinal cord atrophy in patients with history compatible with acute myelitis

3. Area postrema syndrome: requires associated dorsal medulla/area postrema lesions

4. Acute brainstem syndrome: requires associated periependymal brainstem lesions

Abbreviations: AQP4, aquaporin-4; IgG, immunoglobulin G; LETM, longitudinally extensive transverse myelitis lesions; NMOSD, neuromyelitis optica spectrum disorders.

Figure 1 T2-weighted mid-sagittal image centered at T3. Long contiguous segment (T3 to T6) central T2 hyperintense lesion mildly expanding the cord.

Figure 2 T2-weighted mid-sagittal image proximal to the level on previous image shows long segment expansion of the central canal (syrinx).

Figure 3 Axial T2-weighted image at the level of T3 revealed central cord hyperintensity.