NMOSD attacks are often unpredictable and could be severe and recurrent1-3
Neuromyelitis Optica Spectrum Disorder (NMOSD) is an autoimmune disease of the central nervous system; when recurrent, relapses* can result in cumulative disability, potentially including blindness, paralysis, and premature death.2-4*The terms “attack” and “relapse” are used interchangeably.
Presentation
NMOSD TYPICALLY ATTACKS THE OPTIC NERVES OR SPINAL CORD FIRST5,6
Initial presentation varies depending on the type and location of neuronal damage.5,6
Patients most commonly present with optic neuritis and/or transverse myelitis.5,6
Other CNS lesions can be found in the area postrema, with symptoms of hiccups, nausea, and vomiting.6,7
CHARACTERIZATION OF INITIAL NMOSD EVENT (% of patients)5
*Based on a retrospective study of medical records from 187 patients with NMOSD over 5 years from 3 large US medical centers.5
FIRST ATTACK
THE FIRST ATTACK COULD BE AN INDICATOR OF FUTURE DISABILITY3*
WHEN THE FIRST ATTACK WAS MYELITIS
- Motor symptoms at first myelitis attack indicated more severe long-term motor disability (compared to sensory symptoms)2,3
- Compared to 1 myelitis attack, more than 1 myelitis attack in the first year predicted more severe long-term disability2
WHEN THE FIRST ATTACK WAS OPTIC NEURITIS
- Severity of first optic neuritis attack was a predictor of visual disability over time3
*Retrospective study of 106 aquaporin-4 immunoglobulin G positive (AQP4-IgG+) NMOSD patients based on a 4-year review of medical records from 3 tertiary centers in the United Kingdom and Japan; median disease duration at last follow-up was 75 months.3
Up to 92.7%† of NMOSD patients who are AQP4-IgG+ have relapsed, with relapses often resulting in permanent disability.2-4
†From a 2009 to 2011 retrospective study of the German Neuromyelitis Optica Study Group (NEMOS) database, which included 137 AQP4-IgG+ NMOSD patients with a median disease duration of 60 months.2
THE DEVASTATING IMPACT OF NMOSD COULD BE IMMEDIATE
76% of patients did not fully recover from the first myelitis or optic neuritis attack.2*
- By the time of diagnosis, some patients may have already experienced multiple relapses and have irreversible nervous system damage2*
- After disease onset, the median time to first relapse was 8.5 to 14 months2,3*†
*Retrospective study based on the German NEMOS registry that evaluated 175 Caucasian patients with NMOSD (defined by Wingerchuk et al 2006) and known AQP4 antibody status.2
†Retrospective study of 106 AQP4-IgG+ NMOSD patients based on a 4-year review of medical records from 3 tertiary centers in the United Kingdom and Japan; median disease duration at last follow-up was 75 months.3
CHRONIC RELAPSES
NMOSD ATTACKS ARE OFTEN UNPREDICTABLE AND COULD BE SEVERE AND RECURRENT
After diagnosis, patients had an average of 1.3 relapses per year.3*†
PERCENT OF PATIENTS WHO RELAPSED WITHIN 1, 3, AND 5 YEARS OF THEIR INITIAL RELAPSE1
aTwo-part study (retrospective from 1950 to 1993 and prospective from 1993 to 1997) based on a review of medical records from the Mayo Clinic that evaluated 48 patients with relapsing NMOSD; mean disease duration at last follow-up was 7.7 years. Data based on time between first relapse (used to define a relapsing course) and final relapse.1
*Retrospective study of 106 patients with APQ4-IgG+ NMOSD based on a 4-year review of medical records from 3 tertiary centers in the United Kingdom and Japan; median disease duration at last follow-up was 75 months.1
†Calculated as the mean annualized relapse rate for patients prior to treatment with immunosuppressants.1
References: 1. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53(5):1107-1114. 2. Jarius S, Ruprecht K, Wildemann B, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients. J Neuroinflammation. 2012;9:14. 3. Kitley J, Leite MI, Nakashima I, et al. Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain. 2012;135(pt 6):1834-1849. 4. Jiao Y, Fryer JP, Lennon VA, et al. Updated estimate of AQP4-IgG serostatus and disability outcome in neuromyelitis optica. Neurology. 2013;81(14):1197-1204. 5. Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012;69(9):1176-1180. 6. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6(9):805-815. 7. Hinson SR, Lennon VA, Pittock SJ. Autoimmune AQP4 channelopathies and neuromyelitis optica spectrum disorders. Handb Clin Neurol. 2016;133:377-403.
