Abstract
PurposeEarly diagnosis and treatment are of paramount importance for pediatric patients with autoimmune encephalitis (AE). The aim is to evaluate the usefulness of FDG PET/CT in pediatric patients with suspected AE from a prospective study.MethodsThe prospective study was conducted over a period of 23.5 months from May 14, 2019, to April 30, 2021. All patients (< 18-year-old) were hospitalized at the department of pediatric neurology and met the criteria of clinical suspected AE. The children underwent the tests of blood samplings, CSF, EEG, MRI, and 18F-FDG PET/CT. The criteria for FDG PET/CT diagnosis of AE were large lobar hypometabolism with or without focal hypermetabolism found on PET/CT. The clinical final diagnosis of AE includes seropositive and seronegative AE based on the diagnostic criteria.ResultsOne hundred four pediatric inpatients (57 boys, 47 girls) were included, of which 58 children were diagnosed with AE (seropositive, 16; seronegative, 42), 45 children were diagnosed with non-AE, and one boy remained indeterminate diagnosis. Large lobar hypometabolism was found in 61 children, of which 54 (88.5%) children were finally diagnosed with AE. The sensitivity, specificity, and accuracy of FDG PET/CT for diagnosis of AE were 93.1%, 84.4%, and 89.3%, respectively, with a positive predictive value of 88.5% and a negative predictive value of 90.5%. The most common involved with hypometabolism was the parietal lobe, followed by occipital and frontal lobes, finally the temporal lobe on PET/CT in children with AE.ConclusionBrain FDG PET/CT imaging has high specificity, sensitivity, and accuracy for diagnosis of AE in clinical suspected AE children.Trial registration.Clinical Trials.gov. NCT02969213. Registered 17 October 2016.
Highlights
Autoimmune encephalitis (AE) generally refers to a group of severe inflammatory brain disease due to abnormal autoimmunity mechanism
Forty-five children were diagnosed with non-AE, and one child remained indeterminate diagnosis, who was an 11-year-old boy with neuropsychiatric changes, infection, negative results of cerebrospinal fluid (CSF), EEG, and MRI, and no response to immunotherapy
Sixteen seropositive AE children presented with positive neuronal autoantibody test for anti-NMDAR (7 children), anti-Ri (2 children), anti-MOG (2 children), anti-Hu and anti-Amphiphysin (1 girl), anti-DPPX (1 girl), anti-GQ1b (1 boy), anti-AMPAR1 (1 boy), and anti-Recoverin (1 girl)
Summary
Autoimmune encephalitis (AE) generally refers to a group of severe inflammatory brain disease due to abnormal autoimmunity mechanism. AE is one of the most challenging clinical conditions to diagnose because of the heterogeneity in disease manifestations and diagnostic test results, overlap in clinical presentations between AE, other inflammatory brain diseases, metabolic diseases, and psychiatric disorders. It is especially difficult for children because of the complexity of normal behavioral changes during childhood and the limited capacity of younger children to describe their symptoms. Abnormal brain metabolism detected on FDG-PET/CT is most often in early stage of AE, compared to initial EEG, MRI, and CSF studies in inpatients with AE [17]
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