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DOI: 10.1055/a-2383-5660
Diagnostik und Therapie bei Kindern mit antikörperbedingten ZNS-Erkrankungen
Diagnostic workup and treatment in children with antibody-mediated CNS diseases
Autoimmunerkrankungen des zentralen Nervensystems gewinnen in der Pädiatrie zunehmend an Bedeutung. In den letzten Jahren wurden verschiedene Antikörper gegen neuronale Antigene, aber auch Astrozyten und die weiße Substanz entdeckt. Die autoimmune Enzephalitis präsentiert sich mit vielfältiger Symptomatik. Auch Erkrankungen mit Beteiligung der weißen Substanz führen zu einer Vielzahl von Krankheitsbildern. Eine frühe Diagnose und Therapie sind entscheidend für die Prognose.
Abstract
Autoimmune diseases of the central nervous system (CNS) have gained importance in pediatric patients. In recent years, various antibodies against neuronal antigens, and against astrocytes and white matter, have been discovered. Autoimmune encephalitis (AE) presents with a broad spectrum of symptoms such as altered consciousness, epileptic seizures, and psychiatric abnormalities. AE is most often caused by NMDA-receptor antibodies in children. Clinical presentation can hardly be distinguished from encephalitis of a different origin (e.g. infectious), alleviating the correct diagnosis. In patients with white matter involvement, frequent clinical phenotypes include optic neuritis, transverse myelitis, or ADEM. In this subgroup, MOG-antibody associated disease (MOGAD) are most often found. Diagnosis can be challengeing and infectious and metabolic causes must be excluded in the differential diagnosis. To narrow down the diagnosis, the diagnostic criteria by Graus et al. or the diagnostic criteria for pediatric AE by Cellucci et al. can be used. Early therapy is crucial for prognosis. In patients with relapsing disease course, adjusted maintenance therapy has to be administered.
Schlüsselwörter
Autoimmunenzephalitis - MOGAD - NMDAR-Enzephalitis - Autoantikörper - Demyelinisierung - SchubprophylaxePublication History
Article published online:
03 June 2025
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