{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T23:58:41Z","timestamp":1780963121906,"version":"3.54.1"},"reference-count":41,"publisher":"Georg Thieme Verlag KG","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Fortschr Neurol Psychiatr"],"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n                  <jats:p>Die Neuromyelitis optica (NMO) und ihre Varianten, zusammengefasst als Neuromyelitis-optica-Spektrum-Erkrankungen (engl. \u201eNMO spectrum disorders\u201c, NMOSD), sind seltene, potenziell schwere Autoimmunerkrankungen des zentralen Nervensystems (ZNS). Typischerweise erkranken Frauen zwischen dem 30. und 40. Lebensjahr. In rund 80\u202f% der F\u00e4lle k\u00f6nnen Aquaporin-4-Antik\u00f6rper (AQP4-Ak) nachgewiesen werden.Kardinale Manifestationen sind R\u00fcckenmarksentz\u00fcndung (Myelitis), Sehnervenentz\u00fcndung (Optikusneuritis, ON), aber auch Hirnstammenzephalitis.NMOSD verlaufen in den meisten F\u00e4llen schubf\u00f6rmig und ohne Fortschreiten der Symptome zwischen den Krankheitssch\u00fcben. NMOSD-Sch\u00fcbe sind h\u00e4ufig schwer und resultieren oft in einer inkompletten Remission sowie bleibender Behinderung. Monophasische Verl\u00e4ufe sind selten.<\/jats:p>\n                  <jats:p>Wir stellen den Fall einer 86-j\u00e4hriger Patientin vor, bei der subakut Kopfschmerzen occipital auftraten, gefolgt von einer beinbetonten Hemiparese rechts mit rascher Progredienz zur Tetraparese<\/jats:p>\n                  <jats:p>Im MRT zeigte sich eine langstreckige Myelitis (HWK I\u2013V). Im Liquor cerebrospinalis fanden sich eine milde Pleozytose, Eiwei\u00dferh\u00f6hung und oligoklonale Banden. Der AQP4-Ak-Titer war stark positiv, sodass wir die Diagnose einer NMOSD stellten. Wir klassifizierten die Entit\u00e4t als Very Late Onset (VLO)-NMOSD. Nach einer f\u00fcnft\u00e4gigen intraven\u00f6sen Hochdosistherapie mit Methylprednisolon kam es zu einer Besserung der Symptome. Etwa elf Wochen nach der Entlassung wurde mit einer Therapie mit monoklonalem Anti-CD20-Antik\u00f6rper Rituximab begonnen. Der Zustand der Patientin blieb w\u00e4hrend der Therapie stabil und frei von neuen Sch\u00fcben.<\/jats:p>\n                  <jats:p>Eine NMOSD kann auch sehr sp\u00e4t auftreten, \u00fcber das 75. Lebensjahr hinaus. Angesichts einer, wie in unserem Fall, prim\u00e4r aufgetretenen beinbetonten Hemiparese rechts, des Alters der Patientin und mehreren Gef\u00e4\u00dfrisikofaktoren kann eine NMOSD zun\u00e4chst nicht als Erstliniendiagnose in Betracht gezogen werden. Die begleitenden okzipitalen Kopfschmerzen weisen h\u00e4ufig migr\u00e4neartige Charakteristika auf.Eine intraven\u00f6se Therapie mit Methylprednisolon f\u00fchrte zu einer Besserung der Symptome. Rituximab wurde gut vertragen.<\/jats:p>","DOI":"10.1055\/a-2878-1828","type":"journal-article","created":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T23:05:12Z","timestamp":1780959912000},"source":"Crossref","is-referenced-by-count":0,"title":["Seltener, sehr sp\u00e4ter Beginn einer NMO (Neuromyelitis optica)"],"prefix":"10.1055","author":[{"given":"Maxim","family":"Maximov","sequence":"additional","affiliation":[{"name":"Medius Klinik Kirchheim, BW, Germany","place":["Kirchheim unter Teck"],"department":["Neurologie"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Rodriguez Lago","sequence":"additional","affiliation":[{"name":"Medius Klinik Kirchheim, BW, Germany","place":["Kirchheim unter Teck"],"department":["Neurologie"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2026,6,8]]},"reference":[{"key":"ref2","doi-asserted-by":"publisher","first-page":"3341","DOI":"10.1007\/s00415-023-11634-0","article-title":"Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders\n                    (NMOSD) \u2013 revised recommendations of the Neuromyelitis Optica Study Group\n                    (NEMOS). 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