{
  "abstract": "Background/Objectives Aquaporin-4 positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD) is a rare neuroinflammatory disease, with pediatric-onset cases comprising 3–5% of all diagnoses. This study aims to study the impact of age of onset on relapse rates, treatment responses and disability accrual.Method We conducted an international cohort study using the MSBase registry. Patients were categorised by age of onset: paediatric (<18 years), adult (18–55 years), and late-onset (>55 years). Primary outcomes were annualized relapse rates (ARR) and time to first relapse in patients receiving high-efficacy therapy (HET) versus low-efficacy therapy (LET).Results Amongst 442 patients (86.2% female), median age at onset was 35.0 (Q1 24.4, Q3 48.3), with a median disease duration of 8.4 years (Q1 4.3, Q3 13.4). There were no significant differences (p= 0.72) between the ARR on HET for the late-onset group (n=52; median 0.22, Q1 0.0, Q3 0.45) compared to paediatric (n=30; median 0.16, Q1 0.0, Q3 0.36) and adult-onset groups (n=251; median 0.15, Q1 0.0, Q3 0.39). Similarly, no significant differences (p=0.85) were observed on LET between the ARR of late-onset patients (median 0.12, Q1 0.0, Q3 0.41), compared to paediatric-onset (median 0.15, Q1 0.06, Q3 0.36) and adult-onset (median 0.15, Q1 0.0, Q3 0.38) cohorts. Cox modelling showed no significant age-related differences in second relapse risk.Conclusion This multi-centre study shows no significant effect of age on treatment effectiveness for relapse prevention. Further research is needed to clarify age-related therapeutic responses.",
  "authors": [
    {
      "affiliations": [
        "Neurosciences, The Central Clinical School, Monash University, Melbourne, VIC, Australia",
        "Neurology, Alfred Health, Melbourne, VIC, Australia",
        "Department of Neurology, Walton Centre NHS Trust, Liverpool, UK"
      ],
      "name": "Pakeeran Siriratnam"
    },
    {
      "affiliations": [
        "Department of Neurology, Walton Centre NHS Trust, Liverpool, UK"
      ],
      "name": "Saif Huda"
    },
    {
      "affiliations": [
        "Neurosciences, The Central Clinical School, Monash University, Melbourne, VIC, Australia",
        "Neurology, Alfred Health, Melbourne, VIC, Australia"
      ],
      "name": "Paul Sanfilippo"
    },
    {
      "affiliations": [
        "Neurosciences, The Central Clinical School, Monash University, Melbourne, VIC, Australia",
        "Neurology, Alfred Health, Melbourne, VIC, Australia"
      ],
      "name": "Vilija Jokubaitis"
    },
    {
      "affiliations": [
        "Neurosciences, The Central Clinical School, Monash University, Melbourne, VIC, Australia",
        "Neurology, Alfred Health, Melbourne, VIC, Australia"
      ],
      "name": "Anneke Van der Walt"
    },
    {
      "affiliations": [
        "Neurosciences, The Central Clinical School, Monash University, Melbourne, VIC, Australia",
        "Neurology, Alfred Health, Melbourne, VIC, Australia"
      ],
      "name": "Helmut Butzkueven"
    },
    {
      "affiliations": [
        "Neurosciences, The Central Clinical School, Monash University, Melbourne, VIC, Australia",
        "Neurology, Alfred Health, Melbourne, VIC, Australia"
      ],
      "name": "Mastura Monif"
    }
  ],
  "title": "3621 Paediatric versus adult-onset neuromyelitis optica spectrum disorder: a multi-centre study",
  "uid": "aa0c93a2-11a9-5d0f-bfe0-d6264d475982"
}