J Pediatr Neurol 2019; 17(01): 041-044
DOI: 10.1055/s-0038-1623478
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Anti-N-Methyl-D-Aspartate Receptor Encephalitis in a 3-Year-Old Toddler with the Involvement of Severe Autonomic Dysfunctions in Gastrointestinal System: A Case Report

Li-Ying Liu
1  Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China
,
Yang-Yang Wang
1  Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China
,
Ling-Yu Pang
1  Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China
,
Gui-Xia Zhang
1  Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China
,
Li-Ping Zou
1  Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China
› Author Affiliations
Further Information

Publication History

04 August 2017

04 December 2017

Publication Date:
23 January 2018 (eFirst)

Abstract

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is under-recognized in children and adults. It is characterized by prominent neurologic and psychiatric features at first onset. Autonomic manifestations are rarer in children than in adults. We report a 3-year-old male patient with rare manifestation of severe autonomic dysfunctions of the gastrointestinal system and atypical prodromal symptoms. The serum and CSF of the patient were positive for anti-NMDA receptor antibodies, and his electroencephalogram results showed an “extreme delta brush” pattern. The patient had completely recovered by the sixth-month assessment after receiving first-line immunomodulatory treatment. Thus, anti-NMDAR encephalitis should be considered in the differential diagnosis of children with mental status changes and widespread autonomic dysfunction, particularly if symptoms are accompanied by gastrointestinal dysmotility and anhidrosis.

Funding

This study was funded by the National Key Research Program of China (No. 2016YC1000700) and the National Nature Fund (No. 81471329).