Neuromyelitis optica

Zusammenfassung

Die Neuromyelitis optica (NMO) ist eine autoimmun vermittelte entzündliche Erkrankung des zentralen Nervensystems (ZNS), die insbesondere das Rückenmark und die Sehnerven betrifft. Neuropathologisch ist die NMO durch Antikörper- und Komplementablagerungen, Astrozytenverlust, nekrotische Veränderungen mit Infiltration neutrophiler und eosinophiler Granulozyten und sekundären Untergang von Oligodendrozyten und Neuronen gekennzeichnet. Bei der Abgrenzung gegenüber der klinisch ähnlichen, aber deutlich häufigeren Multiplen Sklerose (MS) hilft insbesondere der Nachweis von Autoantikörpern gegen Aquaporin-4 (AQP4-Ak, NMO-IgG), die im Serum von über 80 % der NMO-Patienten nachgewiesen werden können. Das Zielantigen des Autoantikörpers, das Wasserkanalprotein Aquaporin-4, wird in hoher Konzentration in den Astrozyten-Endfüßchen der Glia limitans interna und externa exprimiert und ist als wichtiger Bestandteil der Blut-Hirn-Schranke an der Aufrechterhaltung der Wasserhomöostase des ZNS beteiligt. Neben den beiden Indexmanifestationen Optikusneuritis und Myelitis kommen häufig auch Hirnstamm- und Dienzephalitiden vor. Als besonders charakteristisch gilt das Area-postrema-Syndrom, das durch entzündliche Läsionen in der dorsalen Medulla verursacht wird und durch unstillbaren Schluckauf und/oder unstillbares Erbrechen gekennzeichnet ist. Die klinischen Symptome der NMO treten zumeist schubförmig auf, selten verläuft die Erkrankung monophasisch. Die Behandlung akuter Schübe erfolgt mit hochdosierten intravenösen Steroiden und/oder Plasmapherese, zur Rezidivprophylaxe ist eine Therapie mit Immunsuppressiva oder B-Zell-depletierenden Therapien essentiell.

Abstract

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system (CNS) that predominantly affects the spinal cord and optic nerves. The neuropathologic hallmarks comprise deposits of antibodies and complement as well as loss of astrocytes, secondary degeneration of oligodendrocytes and neurons, and necrotic lesions with infiltration of neutrophilic and eosinophilic granulocytes. Pathognomonic serum autoantibodies against aquaporin-4 (AQP4-IgG, also termed NMO-IgG) are detectable in around 80 % of NMO patients and help to distinguish this rare entity from multiple sclerosis. The target antigen of NMO-IgG, the water channel protein AQP4, is ubiquitously expressed within the CNS and, as a component of the blood-brain barrier, highly concentrated in the endfeet of astrocytes. New international consensus criteria for NMO spectrum disorders, published in 2015, allow earlier diagnosis. Besides the two index manifestations, optic neuritis and transverse myelitis, involvement of the brainstem and diencephalon is relatively common in NMO. Inflammatory lesions of the area postrema typically cause intractable nausea and vomiting and/or hiccups. NMO mostly follows a relapsing course, especially in AQP4-IgG-positive cases. The treatment of acute exacerbations comprises intravenous methylprednisolone pulses and/or plasma exchange, and prevention of attacks requires long-term therapy with immunosuppressants and/or B-cell-depleting monoclonal antibodies.

Schlüsselwörter

Neuromyelitis optica - Autoimmunerkrankung - Neuromyelitis - Sehnerv - Aquaporin-4

Keywords

neuromyelitis optica - autoimmune disorder - myelitis - optic nerve - aquaporin-4

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