Akute demyelinisierende Erkrankungen des Zentralnervensystems mit Antikörpern gegen die weiße Substanz im Kindesalter

Acute demyelinating diseases in pediatrics encompass diseases such as pediatric multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and the myelin oligodendrocyte glycoprotein antibody-associated diseases (MOGAD), a group of diseases which have been increasingly more described in recent years. In the latter diseases antibodies against aquaporin‑4 (AQP4), which in terms of numbers are only rarely detected in German-speaking countries and against myelin oligodendrocyte glycoprotein (MOG) play an important role in the pathogenesis. The MOGAD, in particular is characterized by a broad age-related spectrum of different clinical presentations, such as acute demyelinating encephalomyelitis (ADEM), AQP4 negative NMOSD, transverse myelitis and various relapsing subtypes. Acute manifestations are treated with high-dose steroids followed by a 3-month treatment with low dose oral prednisolone in order to reduce the risk of relapses in the early phase. Children with relapsing forms of MOGAD (roughly one third of affected children) appear to benefit from a monthly immunoglobulin treatment.