Neuropsychiatric lupus with posterior reversible encephalopathy syndrome: a rare presentation

Systemic lupus erythematosus presenting with neuropsychiatric symptoms (NPSLE) along with posterior reversible encephalopathy syndrome (PRES) is rare. A young woman of 29 years presented with various neuropsychiatric symptoms along with low-grade fever, occasional headache, skin rash, arthralgias and gradually became non-ambulatory over last 6 months. After admission, she had an episode of generalised tonic-clonic seizure, followed by drowsiness. She was normotensive. Investigations revealed no evidence of any underlying infection, normal renal functions and electrolytes; but other parameters were supportive to a diagnosis of NPSLE. MRI brain showed vasogenic oedema characterised by symmetrical hyperintensities over posterior brain regions in T2 and fluid attenuated inversion recovery images with no restricted diffusion in diffusion weighted image suggestive of PRES. A diagnosis of NPSLE presenting with PRES, particularly in the absence of hypertension and abnormal renal functions was made, which is a rare presentation. She responded well to immunomodulatory therapy with methylprednisolone and monthly cyclophosphamide.