Discussion Author(s): Faesel, Paul
Neuromyelitis Optica (Devic's syndrome) is an uncommon illness marked by both optic neuropathy and myelopathy in a close temporal relationship. Although the diagnosis overlaps with Multiple Sclerosis, recent articles have demonstrated distinct differences in imaging. The two entities are clincally different in that Neuromyelitis Optica is associated with frequent and severe exacerbations and an overall poorer prognosis.
Pathologically, there is a destruction involving gray and white matter with dense macrophage invasion. This may progress to cord atrophy with cystic degeneration and gliosis, especially in chronic lesions. The disease typically affects young women.
MRI findings which suggest the diagnosis include high signal within the optic nerves with an otherwise normal brain. Lesions in the spinal cord demonstrate longitudinal high signal on T2 images with moderate enhancement on T1 images. The lesions are typically confluent and may extend across three or more vertebral segments. Cord swelling and/or atrophy may be present.
NOTE: Diagnostic criteria for Devic Disease may need to be modified to include possible brain lesions, based on a 2006 article by Pittock et al "Brain Abnormalities in Neuromyelitis Optica" Arch Neurol. 2006;63:390-396. PMID: 16533966
"Asymptomatic brain lesions are common in NMO, and symptomatic brain lesions do not exclude the diagnosis of NMO. These observations justify revision of diagnostic criteria for NMO to allow for brain involvement."
The diagnosis of Neuromyelitis Optica involves both clinical and imaging findings. Recently proposed criteria include the following:
Absolute--1. Optic Neuritis 2. Acute Myelitis 3. No evidence of disease outside the optic nerves or spinal canal.
Major--1. Negative brain MRI 2. Spinal cord MRI with signal abnormality extending for 3 or more vertebral segments 3. CSF pleocytosis
Minor--1. Bilateral optic neuritis 2. Severe optic neuritis with decreased visual acuity 3. Severe, fixed, attack related weakness in one or more limbs.
Literature search for NMO:
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NMO Revised (2011) Diagnostic Criteria
• Optic neuritis and myelitis
• Myelopathy (MR) 4 or more segments
• CSF+ for NMO Ig-G
PMID: 17401734 PMID:16717206
Revised McDonald Criteria for MS - PMID:2138737
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