Posterior reversible encephalopathy syndrome as a complication of Henoch–Schönlein purpura in a seven-year-old girl

Abstract

Introduction

Henoch–Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch–Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury.

Case presentation

A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch–Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch–Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery.

Conclusion

Posterior reversible encephalopathy syndrome is a severe complication of Henoch–Schönlein purpura. If promptly diagnosed and treated, children with Henoch–Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch–Schönlein purpura patients to developing this complication.