Pediatric autoimmune encephalitis

Abstract

ObjectiveThe aims of this study were (1) to describe the incidence of autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) in children, (2) to validate the currently used clinical criteria to diagnose AIE, and (3) to describe pitfalls in the diagnosis of pediatric autoimmune (AI) and inflammatory neurologic disorders.MethodsThis study cohort consists of 3 patient categories: (1) children with antibody-mediated AIE (n = 21), (2) children with ADEM (n = 32), and (3) children with suspicion of an AI etiology of their neurologic symptoms (n = 60). Baseline and follow-up clinical data were used to validate the current guideline to diagnose AIE. In addition, patient files and final diagnoses were reviewed.ResultsOne-hundred three of the 113 included patients fulfilled the criteria of possible AIE. Twenty-one children had antibody-mediated AIE, of whom 19 had anti-N-methyl-D-aspartate receptor (NMDAR), 1 had anti–α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, and 1 had anti–leucine-rich glioma-inactivated protein 1 encephalitis. Finally, 34 children had ADEM, and 2 children had Hashimoto encephalopathy. Mean incidence rates were 1.54 children/million (95% CI 0.95–2.35) for antibody-mediated AIE and 2.49 children/million (95% CI 1.73–3.48) for ADEM. Of the other 48 children, treating physicians' diagnoses were reviewed. In 22% (n = 6) of children initially diagnosed as having an AI/inflammatory etiology (n = 27), no support for AI/inflammation was found.ConclusionBesides anti-NMDAR encephalitis and ADEM, other AIEs are rare in children. The current guideline to diagnose AIE is also useful in children. However, in children with nonspecific symptoms, it is important to review data critically, to perform complete workup, and to consult specialized neuroinflammatory centers.

Thalamic Lesions in a Toddler with Glutamic Acid Decarboxylase Autoimmune Encephalitis

“…15 A retrospective cohort study by de Bruijn et al (2020) of 113 children diagnosed with AE showed the following characteristics: an incidence rate of 1.54 children/million, a female predominance, a median age of 14 years, and a presentation with seizures in 62% of cases. 16 Recommended investigations are as with other AE, and they should include brain imaging, EEG, and antibody testing in CSF and serum, in addition to investigations to rule out potential differential diagnoses (particularly other causes of inflammatory encephalitis). MRI and EEG findings tended to be nonspecific and occur in, but not limited to, the mesial temporal structures.…”

Autoimmune Encephalitis in Children: From Suspicion to Diagnosis

“…Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and acute disseminated encephalomyelitis (ADEM) are the most frequently described forms of autoimmune encephalitis in children. In one study of 103 children with AES, 19 children had anti-NMDAR encephalitis and 34 children had ADEM [ 20 ].…”

N2 year in review

“…The approach was mainly based on experience with adult patients, although data from children were also taken in consideration for some diseases (e.g., anti-NMDAR encephalitis). To validate these algorithmic diagnostic guidelines in children, de Bruijn and colleagues 23 investigated 113 children that were initially included in 3 categories: (1) children with antibody-mediated encephalitis (n = 21), (2) children with acute disseminated encephalomyelitis (ADEM, n = 34), and (3) children with neurologic symptoms suspected to be autoimmune (n = 60). Overall, 103 children fulfilled the criteria of possible autoimmune encephalitis.…”