Case Study: Headache, Confusion, and Seizures in Patient With SLE

Reversible Posterior Leukoencephalopathy Syndrome

The patient is admitted to the neurology service for treatment of presumptive reversible posterior leukoencephalopathy syndrome (RPLS). RPLS is a neurologic syndrome defined by clinical and radiologic features. It has also been referred to as posterior reversible encephalopathy syndrome, reversible posterior cerebral edema syndrome, and posterior leukoencephalopathy syndrome.

The typical clinical presentation includes headache, confusion, visual symptoms, and seizures. RPLS most often occurs in those in hypertensive crisis (as in this case) or with preeclampsia, and in those undergoing cytotoxic immunosuppressive therapy.1,2 RPLS is associated with good prognosis in most patients with SLE1; thus, prompt diagnosis and initiation of early goal-directed treatment is crucial.

The differential diagnosis for this patient includes possible ischemic stroke, venous thrombosis, toxic or metabolic encephalopathy, demyelinating disorder, vasculitis, or encephalitis.3 Early differentiation of RPLS from ischemic stroke is important because blood pressure treatment goals are different for stroke and RPLS. Additionally, in those with sudden onset of neurologic symptoms, bilateral posterior cerebral artery infarction must be ruled out.2 Radiographic imaging can help distinguish RPLS from bilateral basilar infarcts, as infarcts of the posterior cerebral artery territory usually involve the calcarine and paramedian occipital lobe.4


This contrast-enhanced, axial (cross-sectional), T1-weighted MR image shows gyral swelling and patchy enhancement (white areas) in the parietal and occipital lobes. The enhancement is related to a breakdown in the blood-brain barrier. Credit: Living Art Enterprises / Science Source

Neurologic imaging is an important diagnostic tool for RPLS. The cardinal radiologic feature of RPLS is bilateral white matter vasogenic edema in the posterior regions of the cerebral hemispheres, concentrated in the parieto-occipital region.1 

RPLS in the Setting of SLE

SLE is one of the inflammatory autoimmune disorders associated with RPLS.5 Healthcare providers should have a high clinical suspicion for RPLS when a person who has SLE presents with headache, confusion, seizures, and vision abnormalities or is pregnant, receiving cytotoxic therapy, or in hypertensive crisis.5

Distinguishing RPLS from neuropsychiatric lupus is important because the treatment approaches differ.6 In RPLS, MRI demonstrates posterior cerebral vasogenic edema that is not confined to a single specific vascular territory.6 Diffusion-weighted MRI can help distinguish between the vasogenic edema of RPLS and the cytotoxic edema seen in lupus-related infarction.

Next: RPLS Pathogenesis

This article originally appeared on Rheumatology Advisor