Isolated new onset ‘atypical’ optic neuritis in the NMO clinic: serum antibodies, prognoses and diagnoses at follow-up

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• 作者：L. Piccolo ; M. Woodhall ; G. Tackley ; M. Juryńczyk ; Y. Kong…
• 关键词：Optic neuritis ; Neuromyelitis optica ; Visual loss ; AQP4 ; Ab ; MOG ; Ab
• 刊名：Journal of Neurology
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：263
• 期：2
• 页码：370-379
• 全文大小：535 KB
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• 作者单位：L. Piccolo (1) (2)
  M. Woodhall (3)
  G. Tackley (1)
  M. Juryńczyk (1)
  Y. Kong (4)
  J. Domingos (1) (5)
  R. Gore (1)
  A. Vincent (3)
  P. Waters (3)
  M. I. Leite (1)
  J. Palace (1)
  
  1. Nuffield Department of Clinical Neurosciences, Level 3 West Wing, Oxford University Hospitals NHS Trust, University of Oxford, Headley Way, Oxford, OX3 9DU, UK
  2. IRCCS C. Mondino, University of Pavia, Pavia, Italy
  3. Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Level 6 West Wing, John Radcliffe Hospital, Headley Way, Oxford, OX3 9DU, UK
  4. FMRIB Centre, John Radcliffe Hospital, Oxford University, Headington, Oxford, OX3 9DU, UK
  5. Centro Hospitalar do Porto, Hospital de Santo António, Serviço de Neurologia, Porto, Portugal
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Neurology
  Neurosciences
  Neuroradiology
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1432-1459

文摘

Severe, recurrent or bilateral optic neuritis (ON) often falls within the neuromyelitis optica spectrum disorders (NMOSD), but the diagnosis can be particularly challenging and has important treatment implications. We report the features, course and outcomes of patients presenting with atypical ON when isolated at onset. We retrospectively analyzed 69 sequential patients referred to a single UK NMO center with isolated ON at onset. Aquaporin-4 antibody (AQP4-Ab) assessment was performed in all patients and IgG1 myelin-oligodenrocyte glycoprotein (MOG-Ab) in AQP4-Abneg patients. 37 AQP4-Ab positive (AQP4-Abpos) and 32 AQP4-Ab negative (AQP4-Ab neg) patients (8 with MOG-Ab) were identified. The AQP4-Abneg group included heterogeneous diagnoses: multiple sclerosis (MS), NMO, relapsing isolated ON (RION), monophasic isolated ON and relapsing acute disseminated encephalomyelitis (ADEM)-like syndromes. Compared to AQP4-Abneg patients, AQP4-Abpos patients had a worse residual visual outcome from first attack (median VFSS 4 vs. 0, p = 0.010) and at last assessment (median VFSS 5 versus 2, p = 0.005). However, AQP4-Abneg patients with RION also had poor visual outcome. Up to 35 % of AQP4-Abneg patients developed a LETM and two developed low positivity for AQP4-Ab over time. Eight AQP4-Abneg patients (25 %) were MOG-Ab positive, covering a range of phenotypes excluding MS; the first ON attack was often bilateral and most had relapsing disease with a poor final visual outcome [VFSS 4, range (0–6)]. In conlcusion, AQP4-Ab positivity is confirmed as a predictor of poor visual outcome but AQP4-Abneg RION also had a poor visual outcome. Of those without AQP4-Ab, 25 % had MOG-Ab and another 25 % developed MS; thus, MOG-Ab is associated with AQP4-Abneg non-MS ON. Keywords Optic neuritis Neuromyelitis optica Visual loss AQP4-Ab MOG-Ab