A case of primary Sjogren`s syndrome manifested as CNS symptoms mimicking multiple sclerosis

Abstract

Central nervous system (CNS) involvement in primary Sjogren`s syndrome is less common, and its manifestations may be localized (optic neuropathy, hemiparesis, transverse myelitis, and dystonia) or diffuse (encephalopathy, and dementia). A combination of lesions and the presence of relapse and remissions often suggests multiple sclerosis. Case : A 47-year-old woman was initially diagnosed as a primary Sjogren`s syndrome on the basis of typical sicca symptomatology, objective documentation of keratoconjunctivitis sicca, xerostomia, and a positive minor labial salivary gland biopsy, 4 years ago. And then, for 2 years, she was admitted with relapsing & remitting patterns of right hemiparesis. Brain MRI demonstrated multifocal high signal intensity on T2-weight image just like CNS vasculitis. One years later, paraparesis, sensory deficit below T6 dermatome and urinary disturbance were developed. And, T-spine MRI revealed the high signal intensity at T6 level in T2-weight image. Clinically, multiple sclerosis was suspected. But, CSF finding was not compatible with multiple sclerosis. Finally, she was diagnosed as primary Sjogren`s syndrome manifestating as vasculitis and myelitis, not multiple sclerosis. she was treated with steroid and cyclophosphamide.