Cerebral large vessels vasculitis following Guillain-Barré syndrome as first clinical manifestations of primary Sjogren's syndrome: A case based - Review

Abstract

Background: Primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy in which extraglandular signs of pSS are determinant for the prognosis. 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 stabilization under 6-months cyclophosphamide cycles relayed by azathioprine therapy.

Conclusions: Neurological complications can be inaugural in 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.

Keywords: Central nervous system; Guillan-Barré syndrome; Primary Sjogren syndrome; Vasculitis.

Fig. 1

(a) Initial magnetic resonance (MRI) of the brain. The initial MRI scan showed hyperintenses white matter lesions involving right occipital regions and left subcortical occipital, periventricular regions in the axial T2 Flair ponderation (Arrows). (b) The initial MRI scan of the brain, axial T2 Flair ponderation showed hyperintense lesion involving the left parietal lobe (Arrow). (c) The initial Angio-MRI 3D-TOF (time-of-flight) showed the narrowing of the M 1 and M 3 segments of the right middle cerebral artery and M 1 segment of the left middle cerebral artery (Arrows).