Online Articles: Clinical and Laboratory ObservationsSurvival and Neurologic Recovery After Prompt Diagnosis and Aggressive Management of Severe Idiopathic Hyperammonemic Encephalopathy in a Patient with Acute Myeloid LeukemiaMangum, Ross MD*; Soler-Alfonso, Claudia MD†; Birchansky, Sherri MD‡,§; Stevens, Alexandra MD, PhD*Author Information *Department of Pediatrics, Texas Children’s Cancer and Hematology Center †Department of Molecular and Human Genetics §Department of Radiology, Baylor College of Medicine ‡Department of Pediatric Radiology, Texas Children’s Hospital, Houston, TX The authors declare no conflict of interest. Reprints: Ross Mangum, MD, Texas Children’s Cancer and Hematology Center, 6701 Fannin Street, 14th Floor, Houston, TX 77030 (e-mail: email@example.com). Journal of Pediatric Hematology/Oncology: August 2020 - Volume 42 - Issue 6 - p e452-e455 doi: 10.1097/MPH.0000000000001579 Buy Metrics Abstract A case of a 19-year-old female with low-risk acute myeloid leukemia is presented who was diagnosed with idiopathic hyperammonemic encephalopathy following the development of abrupt neurologic decline, respiratory alkalosis, and elevated plasma ammonia levels of unknown etiology. Delayed symptom recognition of this exceedingly rare condition contributes to the often fatal outcomes of idiopathic hyperammonemic encephalopathy. As illustrated by this case, prompt diagnosis and utilization of a variety of ammonia-modulating treatment modalities can result in remarkable clinical recovery. This case provides guidance to clinicians in counseling families about the possibility of neurologic recovery in similar clinical scenarios. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.