[Spontaneous intracranial hypotension: A rare cause of headache in systemic lupus erythematosus]. / Hypotension intracrânienne : une cause rare de céphalées au cours du lupus systémique.

ABSTRACT

INTRODUCTION: Spontaneous intracranial hypotension (SIH), a rare cause of headache, may be idiopathic or secondary, in particular to Systemic Lupus Erythematosus (SLE) where it remains exceptionally evoked or documented. CASE REPORT A 36-year-old woman presented with postural headache, recurrent nausea and vomiting. The discovery of a nephrotic syndrome led to the diagnosis of SLE with lupus nephropathy (class IV-G-(A)). A brain MRI showed signs of intracranial hypotension with tonsil ptosis and a left parietal hypersignal, and leading to a diagnosis of neurolupus with SIH. Treatment with prednisone, cyclophosphamide, and then mycophenolate mofetil allowed a rapid complete response of all systemic, renal and neurological manifestations, including the iconographic signs of intracranial hypotension. CONCLUSION: Headaches are frequent and often unexplained during SLE. Their orthostatic character should, if appropriate, suggests a SIH and lead to perform a brain MRI, even in the absence of other neurological signs.