Neuromyelitis Optica

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]

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