PC2045: An Unusual Presentation of Child Abuse and Subsequent Osmotic Demyelination Syndrome

Introduction: Osmotic demyelination syndrome (ODS), formerly central pontine myelinolysis, is often discussed but infrequently observed, especially in the pediatric population. ODS is thought to be a result of rapid osmotic shifts in the central nervous system, most commonly associated with correction of hyponatremia too rapidly, but can occur in the setting of many electrolyte derangements and malnutrition.

Case Description: Our patient is a 14 year-old female who presented to an outside hospital with obtundation, hypothermia to 28.5°C, profound septic shock, and acute kidney injury. There, she was intubated, fluid resuscitated, and vasopressors were initiated. Non-contrasted head computed tomography was negative. Blood and urine cultures were obtained, and broad spectrum antibiotics were started. During transport to our pediatric intensive care unit (PICU), she developed seizures, which were treated with benzodiazepines and levetiracetam. In the PICU, stress-dosed systemic hydrocortisone was initiated, and an echocardiogram demonstrated a structurally normal heart and function. Toxicology evaluation was negative and serum ammonia level was normal. Continuous video electroencephalogram was consistent with diffuse encephalopathy. Blood cultures grew Escherichia coli & Klebsiella oxytoca. On examination, she had extremity and facial edema but was emaciated. Her body mass index (BMI) was 13, Z-score -3.99. The initial history provided by her caregiver, including bulimia and self-harm behavior, was suspicious due to inconsistencies and later found to be largely inaccurate. Her caregiver also reported that the patient developed significant swelling of her extremities and face the previous day, which had occurred intermittently over preceding weeks. Due to persistent encephalopathy, brain magnetic resonance imaging was obtained on day 3 of admission, which was consistent with ODS (see Figure 1).

Discussion: Her ODS was presumed to be due to severe protein-calorie malnutrition secondary to maltreatment and metabolic derangements. For ODS, no specific treatment exists, rather care should be focused on prevention and awareness. The patient’s mental status improved, she was transferred out of the PICU and ultimately to inpatient rehabilitation. She was closely monitored for refeeding syndrome during her hospitalization, as her diet was advanced. Prior to discharge, she returned to her neurologic baseline, and underwent a forensic interview, which confirmed physical abuse, medical, nutritional, and educational neglect by her caregiver. After approximately 1 year of free access to nutrition, her BMI is now 27.9.

Conclusion: While the exact etiology of her ODS is not completely clear, her case emphasizes the importance of having a high index of suspicion for potential ODS and creating a broad differential when patients present with abnormal mentation, as well as inconsistent history. Our patient had a favorable outcome, however 39% of patients with ODS die within a few months of symptom onset, and only 60% recover neurologically from the insult, according to reported data.