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Case Reports: Rare disease
Sixteen syndrome: a rare presentation of central demyelination
  1. Francesca Bridge1,2,
  2. Tim Bennett3 and
  3. Katherine Buzzard4,5
  1. 1Neurosciences, Monash University Faculty of Medicine Nursing and Health Sciences, Melbourne, Victoria, Australia
  2. 2Neurology, Alfred Health, Melbourne, Victoria, Australia
  3. 3Rheumatology, Box Hill Hospital, Box Hill, Victoria, Australia
  4. 4Neurology, Box Hill Hospital, Box Hill, Victoria, Australia
  5. 5Neurosciences, Eastern Health, Monash University, Clayton, Victoria, Australia
  1. Correspondence to Dr Francesca Bridge; francesca.mary.bridge{at}gmail.com

Abstract

This case illustrates two diagnostic challenges for clinicians: the rarely described sixteen syndrome and the relationship between tumour necrosis factor (TNF)-alpha inhibitors and central demyelination. Sixteen syndrome affects horizontal eye movements and the facial nerve bilaterally reflecting a lesion in the posterior pontine tegmentum, adjacent to the fourth ventricle. Given its rarity and complexity of clinical signs, this syndrome risks misdiagnosis and mismanagement. The relationship between TNF-alpha inhibitors and demyelination is a complex issue in which causality is yet to be established. This diagnostic challenge poses a management dilemma for clinicians.

  • Brain stem / cerebellum
  • Multiple sclerosis
  • Drugs: CNS (not psychiatric)
  • Anklosing spondylitis
  • Biological agents

https://doi.org/10.1136/bcr-2022-250440

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    Footnotes

    • Contributors The patient was under the clinical care of KB, TB and FB. The case report was written by FB and was edited by KB and TB. All authors critically read and modified the manuscript. FB is the guarantor. All authors approved 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.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests FB has received travel funding from Biogen.

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