PRIMARY SJÖGREN SYNDROME PATIENT PRESENTING CENTRAL NERVOUS SYSTEM MANIFESTATIONS SUCCESSFULLY TREATED WITH CORTICOSTEROIDS

Abstract

We treated a case of primary Sjören's syndrome with progressive central nervous systems manifestations. Central nervous system involvement in such patients is difficult to diagnose and rarely described. A 71-year-old man presenting with vertigo and vomiting was admitted to our hospital. Intension tremor developed in the left extremities and gradually spread to the right extremities, while an articulation disorder also appeared. Results of T2-weighted cranial magnetic resonance imaging on admission showed abnormally high intensity areas in the pons, medulla oblongata, and cerebellum, nearly all of which were on the left side. Laboratory findings showed a high level of serum γ-globulin, and were positive antinuclear antibodies, anti-Ro antibodies, and anti-La antibodies, while histological findings of a minor salivary gland biopsy revealed focal sialadenitis with marked lymphocytic infiltration. These serological and histopathological findings confirmed that the patient had primary Sjören's syndrome. The patient underwent pulsed intravenous methylprednisolone therapy, and blepharoptosis, miosis, kinetic tremors improved with the therapy. In addition, the intensities in T2-weighted cranial MR images in the pons and medulla oblongata disappeared, and cerebellar intensity improved. In view of the underlying autoimmune disorder, it seemed likely that the etiology of the CNS symptoms was related to inflammatory brain changes, such as from autoimmune-related vasculitis.