視神経炎および末梢神経障害を合併したシェーグレン症候群の1例

Abstract

A 68-year-old woman was admitted because of progressive dryness of the mouth. She had suffered from optic neuritis of the right eye 2 years before admission. Physical examination revealed marked dry mouth, keratoconjunctivitis, optic nerve atrophy in the right eye and glove and stocking type paresthesia. Laboratory examination showed positive antinuclear antibody, positive anti SS-A antiboby and mild pleocytosis of cerebrospinal fluid. Sialography and salivary gland biopsy confirmed her Sjögren's syndrome. Once she temporarily recovered her visual acuity partially by corticosteroid therapy, but lost right eyesight progressively over the past 3 years. Her symptoms did not meet the criteria of systemic lupus erythematosus, of other collagen diseases or of multiple sclerosis. Though central nerve system involvement in Sjögren's syndrome had been considered rare, some patients with Sjögren's syndrome may present severe central nervous system manifestations.