a.k.a. Devic’s disease
A combination of clinical features, NMO-IgG antibodies and imaging
simultaneous or sequential occurrence of optic neuritis and myelitis (usually acute complete transverse myelitis).
Findings on Investigations:
- 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 antibodies: occasionally positive
- CSF: pleocytosis >50 WBC, >5 PMN
Loss of Aquaporin-4.
- Plasmaphoresis [case series]
- IVIg [case reports]
- Rituximab, Monitor B-cell flow cytometry panel [case series]
- Azathioprine [case series]
- Approach to weakness, approach to visual deficits,
- Multiple sclerosis, idiopathic transverse myelitis, optic neuritis,