Synonyms:

a.k.a. Devic’s disease

Diagnosis:

A combination of clinical features, NMO-IgG antibodies and imaging

Clinical features:

simultaneous or sequential occurrence of optic neuritis and myelitis (usually acute complete transverse myelitis).

Findings on Investigations:

MRI:

• Spinal cord lesion =or> 3 segments long
• Non-diagnostic for Multiple sclerosis
• White matter lesions that are long & extend from the ventricle to the cerebrum
• Periaqueductal lesions
• Floor of the fourth ventricle lesions

NMO- IgG (targets Aquaporin-4): positive in blood.
Other tests:

• Other antibodies: occasionally positive
• CSF: pleocytosis >50 WBC, >5 PMN

Pathology:

Demyelinating plaques
Loss of Aquaporin-4.

Treatment:

Acutely:

• Plasmaphoresis [case series]
• IVIg [case reports]

Chronically:

• Rituximab, Monitor B-cell flow cytometry panel [case series]
• Azathioprine [case series]

Related articles:

• Approach to weakness, approach to visual deficits,
• Multiple sclerosis, idiopathic transverse myelitis, optic neuritis,