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NICOLE M. ORZECHOWSKI, DO, AND CLEMENT J. MICHET JR, MD Division of Rheumatology, Mayo Clinic, Rochester, MN
31-year-old woman with systemic lupus erythematosus (SLE) presented with headache, fever, and urinary retention. Within 36 hours, progressive ascending paresthesias and weakness developed. Neurologic examination revealed near-complete lower extremity paralysis with upper extremity hyperreflexia. Laboratory studies showed lymphopenia, thrombocytopenia, elevated doublestranded DNA levels, and low complement levels. Pleocytosis and an elevated protein level were found on analysis of cerebrospinal fluid. A sagittal fast spin echo magnetic resonance image of the thoracic spine demonstrated diffusely abnormal, increased intramedullary T2 signal (arrows) and edema. The heterogeneous signal extended from the cervicomedullary junction to the conus medullaris. These findings were consistent with acute transverse myelitis involving the entire spinal cord, also referred to as longitudinal myelitis. The patient was initially treated with methylprednisolone, 1000 mg/d, for 5 days and 7 cycles of plasmapheresis. She then received cyclophosphamide, 1000 mg per month, for 6 months and a tapering dose of prednisone, which was started at 60 mg/d. Within 1 month of hospital discharge, the patient’s weakness had completely resolved. A magnetic resonance image obtained 6 months after cyclophosphamide therapy had been initiated revealed near-complete resolution of the imaging abnormalities. Transverse myelitis is a rare and serious complication of SLE, occurring in 1% to 2% of patients.1 Longitudinal
involvement of the spinal cord is even less common, with only 13 reported cases, including 2 in which longitudinal myelitis was the initial manifestation of SLE. 2-8 Therapeutic options include intravenous methylprednisolone, plasmapheresis, and monthly intravenous cyclophosphamide. Prognosis is poor; most patients experience only a partial response to treatment or relapse within the first year of diagnosis.
1. West SG. Neuropsychiatric lupus. Rheum Dis Clin North Am. 1994; 20(1):129-158. 2. Deodhar AA, Hochenedel T, Bennett RM. Longitudinal involvement of the spinal cord in a patient with lupus related transverse myelitis. J Rheumatol. 1999;26(2):446-449. 3. Kimura Kato Y, Seino Y, Hirayama Y, et al. Systemic lupus erythematosus related transverse myelitis presenting longitudinal involvement of the spinal cord. Intern Med. 2002;41(2):156-160. 4. Téllez-Zenteno JF, Remes-Troche JM, Negrete-Pulido RO, DávilaMaldonado L. Longitudinal myelitis associated with systemic lupus erythematosus: clinical features and magnetic resonance imaging of six cases. Lupus. 2001;10(12):851-856. 5. Chen HS, Lai JH, Juan CJ, Kuo SY, Chen CH, Chang DM. Longitudinal myelitis as an initial manifestation of systemic lupus erythematosus. Am J Med Sci. 2004;327(2):105-108. 6. Moranne O, Hachulla E, Valat AS, Sotoares G, Pagniez D, Boulanger E. Longitudinal myelitis in a pregnant patient with SLE [letter]. Am J Med. 2004; 116(5):355-357. 7. Lehnhardt FG, Impekoven P, Rubbert A, et al. Recurrent longitudinal myelitis as primary manifestation of SLE. Neurology. 2004;63(10):1976. 8. Rheu CW, Lee SI, Yoo WH. A catastrophic-onset longitudinal myelitis accompanied by bilateral internuclear ophthalmoplegia in a patient with systemic lupus erythematosus. J Korean Med Sci. 2005;20(6):1085-1088.
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