Neuromyelitis optica (NMO; Devic syndrome) is a clinically defined, severe CNS demyelinating syndrome characterized by optic neuritis (ON) and acute myelitis; the presence of CNS symptoms outside the optic nerves and spinal cord has until recently excluded the diagnosis.

Diagnosis requires all absolute criterion and one major supportive criteria or two minor supportive criteria

Absolute criteria:

• Optic neuritis
• Acute myelitis
• No evidence of clinical disease outside of the optic nerve or spinal cord

Major supportive criteria:

• Negative brain MRI at onset (does not meet criteria for multiple sclerosis)
• Spinal cord MRI with signal abnormality extending over >/=3 vertebral segments
• CSF pleocytosis of >50 WBC/mm3 or >5 PMNs/mm3

Minor supportive criteria:

• Bilateral optic neuritis
• Severe optic neuritis with fixed visual acuity worse than 20/200 in at least one eye
• Severe, fixed, attack-related weakness (MRC </=2) in one or more limbs

References:

1. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006 May 23;66(10):1485-9. [Medline]
2. Saiz A, Zuliani L, Blanco Y, Tavolato B, Giometto B, Graus F; Spanish-Italian NMO Study Group. Revised diagnostic criteria for neuromyelitis optica (NMO). Application in a series of suspected patients. J Neurol. 2007 Sep;254(9):1233-7. [Medline]

Created May 24, 2011