Neuromyelitis-optica-Spektrum-Erkrankungen (NMOSD) und Anti-Myelin-Oligodendrozyten-Glykoprotein-Antikörper-assoziierte Erkrankungen (MOGAD)

Recurrent episodes of inflammation of the optic nerve and spinal cord are known from historical reports. In 2004, the disease-specific antibody against aquaporin‑4 on the surface of astrocytes was identified. The respective clinical manifestations are summarized as neuromyelitis optica spectrum disorders (NMOSD). Diagnostic criteria are based on clinical presentation, imaging, and antibody status. NMOSD are characterized histologically by primary astrocyte damage and secondary demyelination. Acute treatment comprising methylprednisolone and, when necessary, plasma exchange is followed by long-term immunosuppression to prevent relapses. This is based on B‑cell depletion, interleukin‑6 antagonism, and complement inhibition. Clinical presentation with recurrent optic neuritis and/or transverse myelitis may also be associated with antibodies against myelin–oligondedrocyte–glycoprotein (anti-MOG-antibody-associated diseases, MOGAD). In these diseases, primary inflammatory demyelination occurs in the central nervous system. In contrast to NMOSD associated with antibodies against aquaporin‑4, there are still no controlled studies for the treatment of MOGAD.