Possible Autoimmune Association Between Herpes Simplex Virus Infection and Subsequent Anti–N-Methyl- d -Aspartate Receptor Encephalitis: A Pediatric Patient With Abnormal Movements

• Published in: Pediatric neurology 2015-04, Vol.52 (4), p.454-456
• Main Authors: Yushvayev-Cavalier, Yefim, DO, Nichter, Charles, MD, Ramirez-Zamora, Adolfo, MD
• Format: Article
• Language: English
• Subjects: Anti-N-Methyl-D-Aspartate Receptor Encephalitis - drug therapy; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - pathology; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - physiopathology; Brain - pathology; dyskinesias; Dyskinesias - drug therapy; Dyskinesias - pathology; Dyskinesias - physiopathology; encephalitis; Encephalitis, Herpes Simplex - pathology; Encephalitis, Herpes Simplex - physiopathology; Female; Follow-Up Studies; herpes simplex; Herpes simplex virus 1; Herpesvirus 1, Human; Humans; Infant; Magnetic Resonance Imaging; Neurology; NMDA; Pediatrics; rituximab; tetrabenazine
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/j.pediatrneurol.2014.10.011

Abstract Aim We describe a child with severe generalized choreoathetosis and anti–N-methyl- d -aspartate receptor encephalitis after herpes simplex virus type 1 encephalitis. Recent evidence supports an autoimmune trigger for anti–N-methyl- d -aspartate receptor encephalitis following a viral infection. This is emerging as a common and potentially treatable autoimmune condition in the pediatric population. Patient description A 6-month-old girl presented with fever, diarrhea, and partial seizures and was subsequently treated for proven herpes simplex virus type 1 encephalitis. Shortly thereafter, she developed irritability, insomnia, dysautonomia, orolingual and facial choreodystonic movements, spontaneous vocalizations, and choreoathetoid movements of her trunk and limbs. Cerebrospinal fluid analysis confirmed anti–N-methyl- d -aspartate receptor antibodies. Management of her movements required titrated doses of clobazam, valproate, tetrabenazine, and immunotherapy. At 3 months' follow-up, her abnormal movements had completely resolved. Conclusions Our patient adds to recent evidence linking a viral trigger for brain autoimmunity. Movement disorders appear early, leading to severe patient and family distress, and pose a serious management dilemma because of a paucity of clinical trials assessing treatments in the pediatric population. Abnormal hyperkinetic movements present early and prominently, requiring a combination of symptomatic and immune-modulating therapies for successful treatment.