H1N1-associated Acute Necrotizing Encephalopathy of Childhood: Successful Treatment with the "Zipper Method" and Long-Term Outcome

Abstract

Acute necrotizing encephalopathy of childhood (ANEC) is a devastating childhood disease characterized by rapid neurologic deterioration after a viral febrile illness. Seizures, encephalopathy, and fatal acute necrotizing encephalopathy are well-defined neurologic complications of H1N1 virus infections. Symmetrical, multifocal lesions on cranial magnetic resonance imaging (MRI) are the best-known features of ANEC. Various treatment options include glucocorticoids, intravenous immunoglobulin (IVIG), and plasma exchange (PEX). Herein, we present a 45-month-old girl diagnosed with ANEC and treated with a novel immunomodulation technique, the "zipper method." It is a combined treatment method in which PEX and IVIG treatments are used together. In the first session of plasma exchange, one and a half volumes of patients' plasma were removed using 5% albumin as a replacement solution. At the end of the PEX session, 0.4 g/kg IVIG infusion was started. The second PEX session was applied with one volume 24 hours after the end of the IVIG infusion. This plasma exchange-intravenous immunoglobulin cycle was repeated five times. Furthermore, this case report presents her outcome 3-years after discharge: full recovery. This case is a unique example of ANEC treated successfully with the zipper method.

Keywords: Acute necrotizing encephalopathy of childhood; H1N1; Outcome; Treatment; Zipper method.