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Abstract Details

Outcomes in Acute Necrotizing Encephalopathy of Childhood
Child Neurology and Developmental Neurology
P12 - Poster Session 12 (12:00 PM-1:00 PM)
5-011
To understand the prognosis of children with Acute Necrotizing Encephalopathy of Childhood (ANEC) treated with intensive immunosuppression

ANEC is a disorder characterized by acute neurological symptoms associated with haemorrhagic lesions in the deep grey matter on brain MRI in a post infectious setting that has been associated with mutations in RANBP2. ANEC traditionally is treated with pulse steroids. Historically, it has been associated with a 30% mortality rate with only 10% achieving full recovery. However, literature regarding outcomes in patients receiving more intensive immunosuppressive therapy is currently lacking.

We performed a retrospective chart review in patients with ANEC (2012-2019) seen at a tertiary pediatric center in North America. Children were included if they had acute inflammatory lesions of the CNS with hemorrhage isolated primarily to the basal ganglia and pons. Variables of interest included presenting features, RANBP2 gene testing results, nasopharyngeal swab findings, therapies, and long-term outcomes as measured with the Estimated Disability Status Scale (EDSS).  
13 patients were included (Avg. age at presentation 2.9 yrs., F:M 3.3:1). 2/13 experienced recurrences; 1/13 died. Average length of hospital stay was 25.33 days; 11/13 required ICU admission. 100% presented with fever. Viral pathogens isolated from nasopharyngeal samples included influenza (7/13: Influenza A 6/7, B 1/7), Adenovirus 1/13; Salmonella Typhii 1/13. No organisms were isolated from the CSF. 5/13 patients were positive for the RANBP2 mutation. 12/13 patients received pulse steroids (30 mg/kg/d for 5 days), 5/13 received IVIG (2 g/kg), 5/13 received plasmapheresis. EDSS was 9.5 at clinical nadir and 2.5 at last follow up. 4/13 had an EDSS score of 0-1, indicating no disability.
Mortality and disability were lower than previously reported. The majority of children with ANEC experience good motor recovery after immunosuppressive therapies. Studies evaluating larger cohorts are needed to clarify the relationship between immunosuppressive therapy and outcomes.
Authors/Disclosures
Nurin Chatur, MD
PRESENTER 		No disclosure on file
Giulia Longoni, MD (The Hospital for Sick Children) Dr. Longoni has nothing to disclose.
E. Ann Yeh, MD, MA, FRCPC (Hospital for Sick Children) Dr. Yeh has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Pipeline therapeutics. Dr. Yeh has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Yeh has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for SCN. Dr. Yeh has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. The institution of Dr. Yeh has received research support from CIHR. The institution of Dr. Yeh has received research support from National MS Society. The institution of Dr. Yeh has received research support from SickKids Foundation. The institution of Dr. Yeh has received research support from MSSC. The institution of Dr. Yeh has received research support from Garry Hurvitz Foundation. The institution of Dr. Yeh has received research support from Leong Centre. The institution of Dr. Yeh has received research support from OMSLife. The institution of Dr. Yeh has received research support from Canada's Drug Agency. Dr. Yeh has received personal compensation in the range of $0-$499 for serving as a Author with Medscape. Dr. Yeh has a non-compensated relationship as a Editorial Board with Neurology that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. Dr. Yeh has a non-compensated relationship as a Editorial Board with MSJ that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.