N Collongues1, R Marignier2, A Jacob3, M I Leite4, A Siva5, F Paul6, H Zephir7, G Akman-Demir5, L Elsone3, S Jarius8, C Papeix9, K Mutch3, S Saip5, B Wildemann8, J Kitley4, R Karabudak10, O Aktas11, D Kuscu12, A Altintas5, J Palace4, C Confavreux, J De Seze13. 1. Department of Neurology, Hautepierre Hospital, University of Strasbourg, France nicolas.collongues@chru-strasbourg.fr. 2. Department of Neurology, Pierre Wertheimer Hospital, University of Lyon, France. 3. The Walton Centre for Neurology and Neurosurgery, The Walton Centre Foundation Trust, Liverpool, UK. 4. Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, UK. 5. Department of Neurology, Istanbul University, Turkey. 6. Neurocure, Charité University Medicine Berlin, Germany. 7. Department of Neurology, Robert Salengro Hospital, University of Lille Nord de France, France. 8. Division of Molecular Neuroimmunology, University of Heidelberg, Germany. 9. Department of Neurology, Groupe Hospitalier Pitié Salpétrière, Paris, France. 10. Department of Neurology, Hacettepe University, Ankara, Turkey. 11. Department of Neurology, Heinrich-Heine-University of Düsseldorf, Germany. 12. Department of Neurology, Bakýrkoy Hospital for Psychiatric and Neurological Diseases, Istanbul, Turkey. 13. Department of Neurology, Hautepierre Hospital, University of Strasbourg, France.
Abstract
BACKGROUND: Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). OBJECTIVE: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. METHODS: We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. RESULTS: We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. CONCLUSION: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.
BACKGROUND: Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). OBJECTIVE: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. METHODS: We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. RESULTS: We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. CONCLUSION: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.
Authors: Sara Salama; Hazem Marouf; M Ihab Reda; Amal R Mansour; Osama ELKholy; Michael Levy Journal: J Neuroimmunol Date: 2018-08-29 Impact factor: 3.478
Authors: Sven Jarius; Klemens Ruprecht; Ingo Kleiter; Nadja Borisow; Nasrin Asgari; Kalliopi Pitarokoili; Florence Pache; Oliver Stich; Lena-Alexandra Beume; Martin W Hümmert; Marius Ringelstein; Corinna Trebst; Alexander Winkelmann; Alexander Schwarz; Mathias Buttmann; Hanna Zimmermann; Joseph Kuchling; Diego Franciotta; Marco Capobianco; Eberhard Siebert; Carsten Lukas; Mirjam Korporal-Kuhnke; Jürgen Haas; Kai Fechner; Alexander U Brandt; Kathrin Schanda; Orhan Aktas; Friedemann Paul; Markus Reindl; Brigitte Wildemann Journal: J Neuroinflammation Date: 2016-09-27 Impact factor: 8.322
Authors: L Pandit; N Asgari; M Apiwattanakul; J Palace; F Paul; M I Leite; I Kleiter; T Chitnis Journal: Mult Scler Date: 2015-04-28 Impact factor: 6.312
Authors: Franziska Di Pauli; Romana Höftberger; Markus Reindl; Ronny Beer; Paul Rhomberg; Kathrin Schanda; Douglas Sato; Kazuo Fujihara; Hans Lassmann; Erich Schmutzhard; Thomas Berger Journal: Neurol Neuroimmunol Neuroinflamm Date: 2015-11-04