Intracranial hypertension syndrome as an unusual manifestation of Sjogren's syndrome. Report of one case

Neurological manifestations such as polyneuropathy are reported in 8-49% of cases with Sjogren's Syndrome (SjS), but central nervous system involvement is seldom described. We report a 46-year-old woman with a history of SjS with distal renal tubular acidosis and autoimmune thyroiditis. She consulted in the emergency room for a five-days history of holocranial headache and explosive vomiting. Fundoscopy showed bilateral papilledema. Brain computed tomography (CT) without contrast showed diffuse encephalic edema, with effacement of sulci and restriction of peritruncal cisterns. Brain AngioCT ruled out thrombosis, and brain magnetic resonance (MRI) was without structural alterations or hydrocephalus. Lumbar puncture had increased cerebrospinal fluid output pressure but without cytochemical alterations, and negative gram, cultures and filmarray. The diagnosis of Intracranial Hypertension Syndrome (ICHTS) of probable autoimmune etiology in the context of SjS was proposed, and management with high-dose corticosteroids was initiated with favorable clinical and imaging response.