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Red flags: Findings atypical for neuromyelitis optica spectrum disorders

Red flags: Findings atypical for neuromyelitis optica spectrum disorders
Red flags (clinical/laboratory)
1. Clinical features and laboratory findings
Progressive overall clinical course (neurologic deterioration unrelated to attacks; consider MS)
Atypical time to attack nadir: Less than four hours (consider cord ischemia/infarction); continual worsening for more than four weeks from attack onset (consider sarcoidosis or neoplasm)
Partial transverse myelitis, especially when not associated with LETM MRI lesion (consider MS)
Presence of CSF oligoclonal bands (oligoclonal bands occur in <20% of cases of NMO versus >80% of MS)
2. Comorbidities associated with neurologic syndromes that mimic NMOSD
Sarcoidosis, established or suggestive clinical, radiologic, or laboratory findings thereof (eg, mediastinal adenopathy, fever and night sweats, elevated serum angiotensin converting enzyme or interleukin-2 receptor levels)
Cancer, established or with suggestive clinical, radiologic, or laboratory findings thereof; consider lymphoma or paraneoplastic disease (eg, collapsin response mediator protein-5 associated optic neuropathy and myelopathy or anti-Ma-associated diencephalic syndrome)
Chronic infection, established or with suggestive clinical, radiologic, or laboratory findings thereof (eg, HIV, syphilis)
Red flags (conventional neuroimaging)
1. Brain
a. Imaging features (T2-weighted MRI) suggestive of MS (MS-typical)
Lesions with orientation perpendicular to a lateral ventricular surface (Dawson fingers)
Lesions adjacent to lateral ventricle in the inferior temporal lobe
Juxtacortical lesions involving subcortical U-fibers
Cortical lesions
b. Imaging characteristics suggestive of diseases other than MS and NMOSD
Lesions with persistent (>3 months) gadolinium enhancement
2. Spinal cord
Characteristics more suggestive of MS than NMOSD
Lesions <3 complete vertebral segments on sagittal T2-weighted sequences
Lesions located predominantly (>70%) in the peripheral cord on axial T2-weighted sequences
Diffuse, indistinct signal change on T2-weighted sequences (as sometimes seen with longstanding or progressive MS)
These are some common or key findings that should prompt thorough investigation for competing differential diagnoses before making a diagnosis of NMOSD.
CSF: cerebrospinal fluid; LETM: longitudinally extensive transverse myelitis lesions; MS: multiple sclerosis; NMO: neuromyelitis optica; NMOSD: neuromyelitis optica spectrum disorders.
From: Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 2015; 85:177. DOI: 10.1212/WNL.0000000000001729. Copyright © 2015 American Academy of Neurology. Reproduced with permission from Wolters Kluwer Health. Unauthorized reproduction of this material is prohibited.
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