Devic's disease treated with plasmapharesis
Purpose: Devic's disease (neuromyelitis optica) is a rare demyelinating disease with simultaneous or sequential involvement of spinal cord and optic nerves. The brain is spared. Although considered a variant of mutiple sclerosis, there is now sufficient evidence it is a separate entity with distinct properties. Treatment options have generally been those traditional to MS. The prognosis is poor. We demonstrate a case in whom plasmapharesis was used with good effect.
Methods: The patient, a 39 year-old female, fulfilled the criteria of Devic's disease.
1). Acute onset transverse myelitis; subsequent episode of optic neuritis with no other clinical involvement.
2). Magnetic resonance imaging demonstrated spinal cord enlargement and cavitation and a lesion of the affected optic nerve. There was no other radiological involvement of the brain or brainstem.
3). Cerebrospinal spinal fluid was negative for oligoclonal bands.
Aggressive relapses occurred in the first six months of presentation.
Results: There was only partial symptom response to intravenous and oral steroids and immunotherapy. Plasmapharesis instituted on a monthly to fortnightly basis resulted in dramatic clinical and magnetic resonance imaging improvement with prevention of further relapses. Azathioprine and oral steroids were used to maintain remission. She remains attack-free for the last two years.
Conclusions: We recommend that plasmapharesis be used first-line in treatment of Devic's disease to improve neurological recovery and prevent relapses. This is supported by cases in the literature.