273 ANTIPHOSPHOLIPID ANTIBODY SYNDROME-RELATED DEMYELINATING DISORDER WITH MULTIPLE CRANIAL NEUROPATHIES TREATED WITH RITUXIMAB. Issue 1 (1st January 2007)

• Record Type: Journal Article
• Authors: Vasudevan, A. R.; Kang, J.; Moldovan, I.; Krick, E. H.; Katsaros, E. P.; Colburn, K. K.
• Is Part Of: Journal of investigative medicine. Volume 55:Issue 1(2007)
• Page Start: S121
• Page End: S121
• Publication Date: 2007-01-01
• Subjects: Clinical medicine -- Periodicals; Medicine -- Research -- Periodicals; Medicine; Research -- United States; Clinical medicine; Medicine -- Research; Periodicals; 616.075
• Languages: English
• ISSNs: 1081-5589

Abstract

Abstract : We report the case of a 39-year-old African-American lady diagnosed with a cutaneous vasculitis manifesting as hyperpigmented areas over the lower extremities. She was ANA and ds-DNA positive and was started on immunosuppressive agents, which were discontinued due to side effects. Over the next 2 months, she developed III, IV, VI, VII, and VIII cranial nerve (CN) palsy on the left side. The patient's MRI showed abnormal T2 lengthening in the pons, midbrain, and medulla without abnormal enhancement, with a differential diagnosis of thiamine deficiency, vitamin B12 deficiency, multiple sclerosis, and postviral encephalopathy. Her cerebrospinal fluid studies were negative for infections. Her antiphospholipid antibodies and anti-β2-glycoprotein 1 antibodies were strongly positive. She was treated with intravenous thiamine, high-dose corticosteroids with no effect. She was then given rituximab, a CD20 monoclonal antibody, infusions 375 mg/m 2 once a week for 4 weeks. The clinical changes involving the IV and VI CN completely resolved in 2 weeks, and there was significant improvement in her III, VII, and VIII CN palsy. The MRI changes resolved completely 2 months after rituximab infusions. Cranial neuropathies occur in 3 to 16% of systemic lupus erythematosus patients and can be due to vascular occlusion, demyelination, vasculitis, or meningitis. There are only two such case reports of multiple cranial nerve involvement secondary to antiphospholipid antibody syndrome-associated demyelination in the foreign language literature and none in English-language journals.