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; Yang-Yang Wang; Ling-Yu Pang; Gui-Xia Zhang; Li-Ping Zou

doi:10.1055/s-0038-1623478

Journal of Pediatric Neurology, Table of Contents

Journal of Pediatric Neurology 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

¹Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China

,

Yang-Yang Wang

¹Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China

,

Ling-Yu Pang

¹Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China

,

Gui-Xia Zhang

¹Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China

,

Li-Ping Zou

¹Department of Pediatrics, Chinese PLA General Hospital, Center for Brain Disorders Research, Capital Medical University, Beijing Institute for Brain Disorders, Beijing, China

› Author Affiliations

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.

Keywords

child - anti-N-methyl-D-aspartate receptor - encephalitis - autonomic nervous system disease - immunotherapy

Full Text

References

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