Central neurological manifestations of primary Sjogren's syndrome are very polymorphic, including pachymeningitis, one of the rarest manifestations, which is exceptionally inaugural.
A 50-year-old patient was admitted for an intracranial hypertension syndrome with paralysis of the III and VII cranial nerves. Brain MRI revealed pachymeningitis of the right cerebral hemisphere. History taking revealed the existence of xerostomy and xerophthalmos. The accessory salivary gland biopsy demonstrated Chisholm stage III sialadenitis. Search for anti-SSA antibodies and anti-SSB antibodies was positive. The diagnosis of primary Sjogren's syndrome was retained. The patient improved with corticosteroid therapy and cyclophosphamide.
This case illustrates the unusual observation of pachymeningitis in primary Sjogren's syndrome in the rare setting as an inaugural manifestation.