Neurological manifestations such as
polyneuropathy are reported in 8-49% of cases with
Sjögren'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 ofsulci and restriction ofperitruncal
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) ofprobable autoimmune
etiology in the context of SjS was proposed, and management with high-
dose corticosteroids was initiated with favorable clinical and imaging response.