The role of immunotherapy in a refractory case of acute hemorrhagic leukoencephalitis in a pediatric patient.

Abstract

Acute hemorrhagic leukoencephalitis (AHLE) is a rare neurologic inflammatory disorder characterized by severe and rapidly progressive encephalopathy; typically considered a severe variant of acute demyelinating encephalomyelitis (ADEM). There is limited pediatric data on treatment modalities for refractory illness. We report here the case of an 8-year-old girl who presented with acute concern for encephalopathy, ataxia, and seizures in the context of preceding infectious symptoms and neuroimaging findings highly suspicious for ADEM. Treatment was promptly initiated with high-dose glucocorticoids followed by addition of intravenous immunoglobulin therapy. Despite ongoing treatment and permissive hypernatremia and hyperosmolar therapy to treat signs of increased intracranial pressure, she showed no clinical signs of improvement, so plasmapheresis was initiated. Follow-up magnetic resonance imaging (MRI) brain showed progression of lesions with more hemorrhagic conversion concerning for AHLE, so anakinra was started. With the combination of pheresis and anakinra, the patient began to show signs of neurologic recovery. Her course was complicated by delirium and physical deconditioning, requiring inpatient rehab, although her neurologic function showed daily improvement. Final MRI brain to date reflected these improvements. Our case highlights the importance of rapid escalation of care to improve mortality rates and reduce neurologic sequelae of AHLE. Future studies are needed to address the safety profile and efficacy of anakinra in refractory disease.