Recognizing psychiatric presentations of anti‐NMDA receptor encephalitis in children and adolescents: A synthesis of published reports

Abstract

Aim The aim of this study was to improve early recognition of anti‐N‐methyl‐d‐aspartate receptor encephalitis (anti‐NMDArE) in children and adolescents by identifying characteristic temporal patterns of clinical features in patients likely to be referred for psychiatric evaluation. In this form of autoimmune encephalitis, NMDAr hypofunction is caused by autoantibodies to receptor surface components. Clinical outcomes following prompt immunotherapy are usually good, but delayed treatment often results in a protracted course with significant residual disability or death. Anti‐NMDArE frequently mimics psychiatric disorders, so most patients are referred initially to a psychiatrist and treated for days or weeks before being correctly diagnosed. Methods A systematic search of PubMed and EMBASE electronic databases identified all published reports of antibody‐confirmed anti‐NMDArE associated with psychiatric symptoms in patients <19 years old. Redundant reports were eliminated manually. For each patient, the order in which each feature was first observed was ranked relative to others. Median temporal ranks were used to compare the sequencing of individual features and major symptom domains. Results One hundred and sixty seven cases (121 females) met the inclusion criteria. The most common features were dyskinesias (77.8%), seizures (72.5%), mutism or staring (40.7%), insomnia (39.5%), language dysfunction (36.5%), fever (31.1%), disorientation/confusion (28.7%), reduced arousal (28.1%), and memory disturbance (26.9%). The configuration and temporal sequencing of features were highly variable between individuals. Conclusion Clinicians need to suspect this disorder: if new behavioral symptoms arise in the context of a recent viral prodrome; if they are accompanied by dyskinesias, seizures or insomnia; or if psychiatric symptoms are unusual (e.g., non‐verbal auditory hallucinations).

Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

“…And symptoms presentation varied between children and adult. In our pediatric study, the proportion of seizures (39.2%, 42/107) among initial symptoms was similar to others' studies, whereas the movement disorder (29.9%, 32/107) was more common than other studies and psychiatric symptoms (18.7%, 20/107) was less than other studies [17,3,10,20,16,14,15] (See Additional le 1: Table S1). The distribution of symptoms during whole course was similar to other studies [17,3,10,20,16,14,15].…”

“…According to previous studies, pediatric anti-NMDAR encephalitis in China and other countries was more common in children aged around 8-12 years [3,[12][13][14][15][16]. While in our study, the mean age at onset was 6.3 ± 3.1 years old which was slightly younger (See Additional le 1: Table S1).…”

“…Anti-NMDAR encephalitis is the most common type of autoimmune encephalitis [3].Some clinical features are different between children and adult with anti-NMDAR encephalitis. And they may vary from regions and racial.…”

“…Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder associated with autoantibodies binding with NR1 subunit of NMDAR receptor [1]. Since it was rst described in 2007, plenty of cases have been reported during these years [2,3]. Anti-NMDAR encephalitis is the most common form of autoimmune encephalitis.…”

Psychiatric Presentation of Anti-NMDA Receptor Encephalitis

“…In addition, movement disorders, such as dyskinesia or rigidity, autonomous instability, or central hypoventilation, typically occur as the disease progresses, as do disturbances of consciousness. Epileptic seizures may also occur at any time (3, 4). Thus, the typical clinical syndrome is neuropsychiatric in nature and thereby points to an organic cause, but there are occasional cases that present like primary idiopathic psychiatric disorders, typically with catatonia (5, 6).…”