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Case report
‘Twenty syndrome’ in neuromyelitis optica spectrum disorder
  1. Ritwik Ghosh1,
  2. Souvik Dubey2,
  3. Subhankar Chatterjee3 and
  4. Durjoy Lahiri4
  1. 1General Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
  2. 2Neuromedicine, Institute of Postgraduate Medical Education and Research, Bangur Institute of Neurology, Kolkata, West Bengal, India
  3. 3General Medicine, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India
  4. 4Neuromedicine, RG Kar Medical College and Hospital, Kolkata, West Bengal, India
  1. Correspondence to Dr Souvik Dubey; drsouvik79{at}gmail.com

Abstract

A 30-year-old woman presented with recurrent hiccups, vomiting and painful diminution of vision and gait instability for 1 day. She had one-and-a-half syndrome, bilateral seventh cranial nerve paresis with bilateral symptomatic optic neuritis and left-sided ataxic haemiparesis. We described her disorder as the ‘twenty syndrome’ (11/2+7+7+2+2+½=20). MRI of her brain revealed demyelination predominantly in right posterolateral aspect of pons, medulla and bilateral optic nerves. Serum antiaquaporin-4 antibody came out positive. Thus, she was diagnosed as neuromyelitis optica spectrum disorder (NMOSD). She responded brilliantly to immunosuppressive therapy. This is the first ever reported case of the ‘twenty syndrome’ secondary to cerebral NMOSD.

  • visual pathway
  • neuroopthalmology
  • neuroimaging
  • multiple sclerosis
  • brain stem / cerebellum
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Footnotes

  • Contributors RG and SD diagnosed the case. RG wrote the draft after critical discussion with DL, SD and SC. SC and DL critically analysed the draft, edited the draft. All authors agreed with the final version of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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