Posterior Reversible Encephalopathy Syndrome in a 6-Year-Old Female Patient with Steroid-Dependent Nephrotic Syndrome with Cyclosporine A Therapy

Background: The posterior reversible encephalopathy syndrome (PRES) is a rare disease in pediatrics. Cerebral seizures, sudden onset of severe headache, visual disturbance and impairment of consciousness are common. The cerebral MRI usually shows symmetric T2-weighted bright abnormalities in the posterior white matter. PRES is a disorder of cerebral autoregulation in association with hypertension. Cytotoxic and nephrotoxic drugs enhance the risk of PRES.

Methods: Our patient presented with a steroid-sensitive nephrotic syndrome at the age of 3 5/12 years. She was treated with cyclosporine A (CSA) after she developed a steroid induced Cushing syndrome. During an afebrile gastroenteritis a relapse of nephrotic syndrome occurred and was treated with steroids. On 7th day of illness a secondary generalized seizure was reported with prolonged postictal consciousness impairment. Blood pressure was significantly increased to 155/110 mm Hg. The T2-weighted images of cMRI showed occipital regions of high signal indicating vasogenic edema. The EEG showed diffuse occipital theta/delta-slowing. Under control of blood pressure with nifedipine the patient improved rapidly and no further seizures occurred. The MRI abnormalities disappeared after 14 days. In the EEG there was still a slowing after 10 days.

Conclusion: In immunocompromised patients with cerebral seizures, headache or visual disturbance a posterior reversible encephalopathy syndrome (PRES) should be considered. A cerebral MRI should be initiated and an adequate therapy should be started to reduce the risk of complications.