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[A case of mixed connective tissue disease with subacute transverse myelopathy].

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1991

Year

Abstract

A rare case of mixed connective tissue disease (MCTD) with subacute transverse myelopathy and various neurological signs was reported. The patient, a 53 year-old woman, was admitted to our hospital with subacute progressive muscle weakness of left lower limb and sensory disturbance of bilateral lower extremities. At the age of 40, she suffered from sensory disturbance of her face, which improved in about three years. She had a high fever, Raynaud phenomenon, dyshydrosis on right side of her face at the age of 43. On the admission, physical examination revealed swollen fingers and telangiectasia of her face. Neurologically, she had transverse myelopathy at the level of Th6, bilateral trigeminal neuropathy, tonic, pupils, polyneuropathy and dyshydrosis. Laboratory examination showed positive antinuclear antibody, a high titer of antibody to RNase-sensitive components of extractable nuclear antigen, positive antinuclear RNP antibody and negative anti-Sm antibody. Her myelopathy improved with corticosteroid therapy, and ESR and the level of immunoglobulin were normalized. But, other neurological signs showed no improvement.