Neurol Sci. 2026 Jul 3;47(8):605. doi: 10.1007/s10072-026-09209-8.
ABSTRACT
INTRODUCTION: Primary Sjögren's syndrome (pSS) is a chronic autoimmune disorder primarily affecting exocrine glands but frequently associated with extra glandular manifestations, including neurological involvement. Central nervous system (CNS) involvement is a rare and potentially severe manifestation of pSS, often leading to diagnostic uncertainty. We describe a case of pSS presenting with systemic manifestations and suspected CNS inflammatory involvement.
METHODS: We report the clinical, laboratory, and imaging findings of a 43-year-old woman presenting with progressive focal neurological deficits and systemic symptoms. Diagnostic workup included brain MRI with magnetic resonance angiography (MRA) and vessel wall imaging (VWI), cerebrospinal fluid (CSF) analysis, autoimmune and infectious screening, and renal biopsy.
RESULTS: Brain MRI revealed cerebral severe small vessel disease (cSVD), while MRA showed diffuse intracranial arterial irregularities, predominantly involving the vertebrobasilar circulation. VWI demonstrated vascular enhancement. Laboratory findings included high-titer antinuclear antibodies and anti-SSA/SSB positivity. Renal biopsy revealed tubulointerstitial nephritis with microangiopathic vascular changes. The overall clinical, laboratory, histopathological and imaging findings confirmed the diagnosis of pSS and suggested suggested a possible inflammatory vascular mechanism underlying the CNS involvement. The patient was treated with high-dose corticosteroids followed by rituximab.
CONCLUSIONS: CNS inflammatory involvement may represent a rare manifestation of pSS and should be considered in patients with unexplained neurological symptoms and systemic autoimmune features. Advanced imaging techniques such as VWI may provide complementary informations. Early recognition and a multidisciplinary approach are essential to guide treatment and improve outcomes.
PMID:42397588 | DOI:10.1007/s10072-026-09209-8