Pseudotumor Cerebri as the First Manifestation of Juvenile Systemic Lupus Erythematosus.

INTRODUCTION: Headache is a common neuropsychiatric manifestation of juvenile systemic lupus erythematous (JSLE). Pseudotumor cerebri (PTC) is an uncommon cause of headache, presenting especially in active JSLE. In this paper, we report a case of missed intractable headache that was eventually diagnosed as PTC and presented as the first manifestation of JSLE. CASE PRESENTATION: A 9-year-old girl with a history of progressive headache for four months, fever, fatigue, myalgia, arthralgia, small-joint arthritis of the hands, and recent diplopia was referred to our clinic. The diagnosis of PTC in the background of JSLE was made based on her history, physical examination, positive laboratory findings, and increased intracranial pressure, with normal neuroimaging. Treatment with high-dose prednisolone led to dramatic resolution of the headache. CONCLUSIONS: It seems that a complete neurologic examination in newly diagnosed SLE patients is mandatory, especially in the presence of any neuropsychiatric manifestations, such as headache.