Abstract
Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a rare autoimmune illness characterized by a constellation of often severe, but treatable, psychiatric and neurological symptoms. Whereas symptoms such as psychosis and bizarre and abnormal motor behavior are common in adults, pediatric patients typically present with behavioral changes, irritability and sleep dysfunction. The recovery phase is usually slow and may be associated with longstanding adaptive, behavioral and neuropsychological problems. Very few studies explored the cognitive and adaptive sequelae in children with anti-NMDAR encephalitis. The present review article suggests that, although most children and adolescents return to their daily life and previous activities, they may have a low quality of life and show neuropsychological sequelae involving language, memory, especially verbal memory, and attentional resources, even after several months from the hospital discharge. In particular, the available results reveal difficulties in cognitive skills involving executive functions. This impairment is considered the “core” of the cognitive profile of young patients with anti-NMDAR encephalitis. On the other hand, some cognitive skills, such as general intelligence, show good overall recovery over time. Additional neuropsychological research evaluating larger samples, more homogenous methods and longitudinal studies is required.
Highlights
Similar results have been found in both patients with anti-NMDAR encephalitis and healthy subjects [30]
With regard to general intelligence, few studies and case reports available on children/adolescents have described a positive outcome over time
In 2010, few years after the original characterization of anti-NMDAR encephalitis, Poloni et al described the case of an 8-year-old girl diagnosed with Sebire syndrome and whose serum was found positive for anti-NMDA
Summary
First described in 2007, anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rare autoimmune illness [1]. Initially identified as a paraneoplastic disease in young women with ovarian teratoma [1,5,6], anti-NMDAR encephalitis has very often an unknown etiology, especially in pediatric age [7,8,9]. This disease has been recognized in patients of all ages and spans a broad age range (4 months–85 years) [2,3,8].
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