Akademik Veri Yönetim Sistemi
16th European Paediatric Neurology Society Congress, Munich, Almanya, 8 - 12 Temmuz 2025, ss.902, (Özet Bildiri)
Introduction: Autoimmune encephalitis (AE) is a rare inflammatory disease of the central nervous system characterized by antibodies targeting neuronal cell surface proteins, receptors, and ion channels. Although N-methyl-D-aspartate receptor, myelin oligodendrocyte glycoprotein, and glutamic acid decarboxylase antibodies are the most common in childhood, many other antibodies have been identified. In adults, the presence of anti-Zic4 antibodies has been associated with Zic4 antibodypositive AE and paraneoplastic cerebellar syndromes. Case Presentation: A 14-year-old male presented with confusion, speech disorder, and seizures. He had headache, weakness, and increased sleepiness for the past week. On neurological examination, the patient was lethargic, deep tendon reflexes were absent in the extremities, and the Babinski sign was positive bilaterally. Laboratory tests showed normal CSF glucose, protein, and IgG index. Serum and CSF anti-MOG antibody, aquaporin-4 antibody, oligoclonal band, and autoimmune encephalitis panel were negative, except for serum anti-Zic4 antibody, which was positive. All infectious, metabolic, and rheumatological investigations were negative. Electroencephalography showed diffuse low voltage. Brain MRI showed T2/FLAIR signal enhancement and diffusion restriction in the temporal and frontal cortex and basal ganglia. Based on the clinical, laboratory, and radiological findings, the patient was diagnosed with anti-Zic4 antibody-positive autoimmune encephalitis. Intravenous methylprednisolone and intravenous immunoglobulin were administered. Conclusion: The diagnosis of AE in children is difficult due to the overlap with other diseases and the complexity of behavioral changes. To the best of our knowledge, this is the first pediatric case of isolated anti-Zic4 antibody-positive autoimmune encephalitis reported here.