Posterior reversible encephalopathy syndrome in a patient with mixed connective tissue disease: a case report

Posterior reversible encephalopathy is a syndrome highly associated with hypertension and cytotoxic therapy. The syndrome typically presents with headache, visual abnormality, seizures and characteristic vasogenic edema on magnetic resonance imaging. The entity warrants a prompt diagnosis to avoid deteriorating consequences.

In this report, we describe a 15-year-old Iranian boy who was diagnosed with mixed connective tissue disease, and cyclophosphamide pulse therapy was administered. Three days after the second pulse of cyclophosphamide, when he was receiving prednisolone and hydroxycholoroquine, our patient developed generalized tonic-clonic seizures. Magnetic resonance imaging findings showed high signal intensities in the posterior areas of his brain. After 8 days, the brain magnetic resonance imaging abnormalities were resolved following the control of his blood pressure and antiepileptic treatment. These observations have been indicative of posterior reversible encephalopathy syndrome. Nevertheless, our patient developed uncontrollable respiratory distress and eventually died.

To the best of our knowledge, this case is the first report of posterior reversible encephalopathy syndrome in a patient with mixed connective tissue disease. As the patient developed posterior reversible encephalopathy syndrome 3 days after cyclophosphamide pulse therapy to reduce the disease activity, it is hard to accurately determine whether posterior reversible encephalopathy syndrome in this case is a complication of cyclophosphamide or a condition that resulted from the mixed connective tissue disease flare-up.