Cerebral large vessels vasculitis following Guillain-Barré syndrome as first clinical manifestations of primary Sjogren’s syndrome : a case report and review of the literature

Abstract

Abstract Background : Primary Sjogren’s syndrome (pSS) is an autoimmune exocrinopathy in which sicca syndrome of the exocrine glands represent the main clinical manifestation. Severe extraglandular signs of pSS are determinant for the prognosis of this disease. Involvement of both peripheral and central nervous system (CNS) are known to be among the sites of high systemic activity in pSS. Case presentation : We, herein, report a case of a 57-year-old female patient with pSS presenting with typical Guillan-Barré syndrome (GBS), shortly followed by acute headaches accompanied by cortical blindness. Cerebral magnetic resonance imaging (MRI) demonstrated T2 signal abnormalities on the occipital region with narrowing and irregularities of the cerebral arteries, suggestive of CNS vasculitis. Subtle sicca symptoms occurring prior to neurological symptoms by 8 months together with immunological disturbances (anti-SSA, anti-SSB antibodies positivity, type II cryoglobulins positivity, and C4 hypocomplementemia) allowed us to retain the diagnosis of pSS. Recovery of motor symptoms was possible under the combined use of immunoglobulins and corticotherapy during the initial phase. A three-years follow-up confirmed progressive motor recovery and disease stabilization under 6-months cyclophosphamide cycles relayed by azathioprine immunosuppressive therapy. However, severe residual visual loss persisted Conclusions : Neurological complications can be inaugural in pSS and they lead to urgent investigations and treatment. Peripheral and central neurological manifestations can coexist. The approach should integrate careful clinical assessment, as well as radiological and immunological findings.