A Case of Sjogren's Syndrome Associated with Neuromyelitis Optica

ABSTRACT

Neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating disease, characterized by optic neuritis and myelitis. NMO is a very uncommon and serious neurologic manifestation of Sjogren's syndrome. We report on a 32-year-old female with NMO as central nerve system involvement of Sjogren's syndrome. She had a transverse myelitis ten years ago and did not have symptoms for a long period of time. She visited the emergency center because of worsening weakness of both limbs. She had an appendectomy three days ago before hospitalization. Cervical spinal magnetic resonance imaging showed increased signal intensity in T2-weighted images from the cervical (C2) to the upper thoracic (T4) spinal cord. As serum NMO-IgG was positive, we diagnosed neuromyelitis optica and treated with high dose steroid, but failed. Therefore, we treated with plasmapheresis and the patient was discharged without any neurological deficits.