Limb Weakness and Vision Loss in a 30-Year-Old Man

Sumaira Nabi, MBBS; Shahzad Ahmed, MBBS; Muhammad Tariq, MBBS, FRCP

Disclosures

November 10, 2015

Discussion

The patient was given pulse therapy with methylprednisolone for 5 days; he was later switched to oral corticosteroids with azathioprine. Improvement in his motor power was negligible. His vision loss persisted. He was then referred to the physiotherapy and rehabilitation unit because he was confined to bed due to severe disability. Unfortunately, because this case occurred in Pakistan, neuromyelitis optica (NMO) antibody testing was not possible. Most serologic assays are not available in the resource-limited settings of the third-world countries.

NMO, also known as Devic syndrome or disease, is an idiopathic, inflammatory, demyelinating disease specific to the spinal cord and optic nerves resulting in acute attacks of severe myelitis and acute or subacute unilateral or bilateral optic neuritis.[1] The recovery may be complete or partial. These attacks commonly spare the brain in the early stages.[2] NMO was traditionally thought to be a monophasic illness but is now recognized as a discrete multiphasic or relapsing demyelinating disease.[3]

The first account of an association between myelitis and an optic nerve disorder was reported by T.C. Allbutt in 1870.[4] Eugene Devic, a French physician, subsequently described a monophasic illness of bilateral optic neuritis and myelitis occurring in quick succession.[5] In 1894, his student Fernand Gault proposed it to be a distinct clinical entity: neuromyélite optique aiguë.[6] Neuromyelitis optica has a very strong female preponderance, with prevalence reportedly nine times higher in women than in men. Although the first episode of NMO can occur at any time from childhood to old age, the reported mean age of onset is almost 35 years.[7] Worldwide, NMO is more common in nonwhite populations, especially East Asians.

The etiology of NMO is the subject of much debate. In some cases, a specific cause has been identified[8,9]; in most cases of uncertain cause, whether it is a variant of multiple sclerosis or a distinct clinical syndrome is unclear. However, optic neuritis, myelitis, and inflammatory demyelination are features of both disorders.[3,5]

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