Systemic lupus erythematosus presenting as idiopathic intracranial hypertension

Abstract

We describe a 13-year-old girl who presented with an acute febrile disease accompanied by headache, dizziness, nausea, decreased visual acuity, and diplopia. Examination showed papilledema, enlarged blind spots, and visual field defects with an otherwise normal neurological examination. The diagnosis of idiopathic intracranial hypertension was confirmed by increased intracranial pressure (cerebrospinal pressure > 200 mm water) in the absence of any abnormal radiological findings of the brain. Initially, only positive serology tests showing elevated titers of anti-DNA antibodies and positive tests for anti-Sm and anti-RNP antibodies were found; however, 6 mo later clinical and laboratory findings were compatible with systemic lupus erythematosus (SLE). Our patient illustrates that the possibility of SLE needs to be considered in the differential diagnosis of idiopathic intracranial hypertension.