37-year-old woman with acute right visual loss and tingling along spine

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Findings:

Brain: several relatively nonspecific white matter foci but greater than usual for age
Orbits: Right optic nerve swelling and edema without enhancement
Cervical Spine: C3 focus of T2 prolongation (nonenhancing)

DDX:

Multiple Sclerosis with optic neuritis
Neuromyelitis Optica (Devic’s Disease or Syndrome)

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Diagnosis:

Devic’s disease but with possible early progression to multiple sclerosis

Discussion:

Devic’s disease is characterised by attacks of acute optic neuritis, usually bilateral/ At the same time or within a few days, weeks or occasionally months, there is severe transverse myelopathy – acute inflammation of the spinal cord. However, in about 20% of cases, TM can precede the ON. Neurologists argue as to whether Devic’s syndrome is a completely different disease to MS or whether it a variant of it. Many consider Devic’s syndrome to be a variant of post-viral Acute Disseminated EncephaloMyelitis (ADEM) perhaps related to the varicella zoster virus. Other believe that, like MS, Devic’s disease is an autoimmune condition.

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Submitted by Ahmed Hassanin Negila, MD and Jerry Jarvik, MD, UW Neuroradiology