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Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses
Abstract
Objective: Neuromyelitis optica (NMO) attacks often are severe, are difficult to treat, and leave residual deficits. Here, we analyzed the frequency, sequence, and efficacy of therapies used for NMO attacks. Methods: A retrospective review was made of patient records to assess demographic/diagnostic data, attack characteristics, therapies, and the short‐term remission status (complete remission [CR], partial remission [PR], no remission [NR]). Inclusion criteria were NMO according to Wingerchuk's 2006 criteria or aquaporin‐4 antibody–positive NMO spectrum disorder (NMOSD). Remission status was analyzed with generalized estimating equations (GEEs), a patient‐based statistical approach. Results: A total of 871 attacks in 185 patients (142 NMO/43 NMOSD, 82% female) were analyzed. The 1,153 treatment courses comprised high‐dose intravenous steroids (HD‐S; n = 810), plasma exchange (PE; n = 192), immunoadsorption (IA; n = 38), other (n = 80), and unknown (n = 33) therapies. The first treatment course led to CR in 19.1%, PR in 64.5%, and NR in 16.4% of attacks. Second, third, fourth, and fifth treatment courses were given in 28.2%, 7.1%, 1.4%, and 0.5% of attacks, respectively. This escalation of attack therapy significantly improved outcome ( p < 0.001, Bowker test). Remission rates were higher for isolated optic neuritis versus isolated myelitis ( p < 0.001), and for unilateral versus bilateral optic neuritis ( p = 0.020). Isolated myelitis responded better to PE/IA than to HD‐S as first treatment course ( p = 0.037). Predictors of CR in multivariate GEE analysis were age (odds ratio [OR] = 0.97, p = 0.011), presence of myelitis (OR = 0.38, p = 0.002), CR from previous attack (OR = 6.85, p < 0.001), and first‐line PE/IA versus HD‐S (OR = 4.38, p = 0.006). Interpretation: Particularly myelitis and bilateral optic neuritis have poor remission rates. Escalation of attack therapy improves outcome. PE/IA may increase recovery in isolated myelitis. Ann Neurol 2016;79:206–216
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Cites
A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis
Lennon, V. A., Wingerchuk, D. M., Kryzer, T. J., Pittock, S. J., Lucchinetti, C. F., Fujihara, K., Nakashima, I., & Weinshenker, B. G. (2004) · The Lancet · doi:10.1016/s0140-6736(04)17551-x · Cited by 3,239
Revised diagnostic criteria for neuromyelitis optica
Wingerchuk, D. M., Lennon, V. A., Pittock, S. J., Lucchinetti, C. F., & Weinshenker, B. G. (2006) · Neurology · doi:10.1212/01.wnl.0000216139.44259.74 · Cited by 2,574
The spectrum of neuromyelitis optica
Wingerchuk, D. M., Lennon, V. A., Lucchinetti, C. F., Pittock, S. J., & Weinshenker, B. G. (2007) · The Lancet Neurology · doi:10.1016/s1474-4422(07)70216-8 · Cited by 2,179
Weinshenker, B. G., O'Brien, P. C., Petterson, T. M., Noseworthy, J. H., Lucchinetti, C. F., Dodick, D. W., Pineda, A. A., Stevens, L. N., & Rodriguez, M. (1999) · Annals of Neurology · doi:10.1002/1531-8249(199912)46:6<878::aid-ana10>3.0.co;2-q · Cited by 894
Jarius, S., Ruprecht, K., Wildemann, B., Kuempfel, T., Ringelstein, M., Geis, C., Kleiter, I., Kleinschnitz, C., Berthele, A., Brettschneider, J., et al. (2012) · Journal of Neuroinflammation · doi:10.1186/1742-2094-9-14 · Cited by 764
Self-citationEfficient semiparametric estimation of multi-valued treatment effects under ignorability
Cattaneo, M. D. (2010) · Journal of Econometrics · doi:10.1016/j.jeconom.2009.09.023 · Cited by 620
Plasma exchange for severe attacks of CNS demyelination: Predictors of response
Keegan, M., Pineda, A. A., McClelland, R. L., Darby, C. H., Rodriguez, M., & Weinshenker, B. G. (2002) · Neurology · doi:10.1212/wnl.58.1.143 · Cited by 600
Neuromyelitis Optica Study Group (NEMOS), Trebst, C., Jarius, S., Berthele, A., Paul, F., Schippling, S., Wildemann, B., Borisow, N., Kleiter, I., Aktas, O., et al. (2014) · Journal of Neurology · doi:10.1007/s00415-013-7169-7 · Cited by 595
Self-citationEFNS guidelines on diagnosis and management of neuromyelitis optica
Sellner, J., Boggild, M., Clanet, M., Hintzen, R. Q., Illes, Z., Montalban, X., Du Pasquier, R. A., Polman, C. H., Sorensen, P. S., & Hemmer, B. (2010) · European Journal of Neurology · doi:10.1111/j.1468-1331.2010.03066.x · Cited by 461
Self-citationKeegan, M., König, F., McClelland, R., Brück, W., Morales, Y., Bitsch, A., Panitch, H., Lassmann, H., Weinshenker, B., Rodriguez, M., et al. (2005) · The Lancet · doi:10.1016/s0140-6736(05)67102-4 · Cited by 433
Cited by
Optic neuritis and autoimmune optic neuropathies: advances in diagnosis and treatment
Bennett, J. L., Costello, F., Chen, J. J., Petzold, A., Biousse, V., Newman, N. J., & Galetta, S. L. (2023) · The Lancet Neurology · doi:10.1016/s1474-4422(22)00187-9 · Cited by 226
Atypical inflammatory demyelinating syndromes of the CNS
Hardy, T. A., Reddel, S. W., Barnett, M. H., Palace, J., Lucchinetti, C. F., & Weinshenker, B. G. (2016) · The Lancet Neurology · doi:10.1016/s1474-4422(16)30043-6 · Cited by 161
Neuromyelitis optica and neuromyelitis optica spectrum disorders
Marignier, R., Cobo Calvo, A., & Vukusic, S. (2017) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000455 · Cited by 45
Metadata
- Authors
- Kleiter, I., Gahlen, A., Borisow, N., Fischer, K., Wernecke, K., Wegner, B., Hellwig, K., Pache, F., Ruprecht, K., Havla, J., Krumbholz, M., Kümpfel, T., Aktas, O., Hartung, H., Ringelstein, M., Geis, C., Kleinschnitz, C., Berthele, A., Hemmer, B., Angstwurm, K., Stellmann, J., Schuster, S., Stangel, M., Lauda, F., Tumani, H., Mayer, C., Zeltner, L., Ziemann, U., Linker, R., Schwab, M., Marziniak, M., Then Bergh, F., Hofstadt‐van Oy, U., Neuhaus, O., Winkelmann, A., Marouf, W., Faiss, J., Wildemann, B., Paul, F., Jarius, S., Trebst, C., & on behalf of the Neuromyelitis Optica Study Group
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 2015-11-26
- Volume
- 79
- Issue
- 2
- Pages
- 206-216
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W2155049452
- Cited by
- 432
- References
- 26
- FWCI
- 19.27
- Type
- journal article
- Language
- en
- Open access
- closed
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- Kleiter, I., Gahlen, A., Borisow, N., Fischer, K., Wernecke, K., Wegner, B., Hellwig, K., Pache, F., Ruprecht, K., Havla, J., Krumbholz, M., Kümpfel, T., Aktas, O., Hartung, H., Ringelstein, M., Geis, C., Kleinschnitz, C., Berthele, A., Hemmer, B., . . . on behalf of the Neuromyelitis Optica Study Group. (2016). Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses. Annals of Neurology. https://doi.org/10.1002/ana.24554
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