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Posterior semicircular canal cupulolithiasis during acute pontomedullary demyelination
  1. Sara Matos1,
  2. Ana Inês Martins1,2,
  3. André Jorge1,
  4. Maria do Carmo Macário1,2,
  5. Daniela Pereira3,
  6. Michael Strupp4,5,
  7. João Lemos1,2
  1. 1 Neurology Department, Coimbra University Hospital Centre, Coimbra, Portugal
  2. 2 Faculty of Medicine, Coimbra University, Coimbra, Portugal
  3. 3 Neuroradiology Unit/Imaging Department, Coimbra University Hospital Centre, Coimbra, Portugal
  4. 4 Ludwig-Maximilians-Universitat, Munchen, Germany
  5. 5 Department of Neurology and German Center for Vertigo and Balance Disorders, Ludwig Maximilians Universitat Hospital, Munchen, Germany
  1. Correspondence to Dr João Lemos, Neurology, Hospital and University Centre of Coimbra, Coimbra 3000-075, Coimbra, Portugal; merrin72{at}


Positional vertigo poses a diagnostic challenge in people with multiple sclerosis (MS). The characteristics of positional nystagmus and its response to repositioning manoeuvres are usually sufficient to diagnose benign paroxysmal positional vertigo (BPPV). However, certain BPPV variants respond poorly to repositioning manoeuvres and their nystagmus pattern can resemble that of central positional vertigo caused by infratentorial demyelination. This diagnostic difficulty is particularly challenging if positional vertigo occurs during an MS relapse. We describe a woman with MS who developed a sixth nerve palsy and gaze-evoked nystagmus, caused by demyelination near or within areas classically involved in central positional vertigo. However, she also had positional vertigo from coincident BPPV (and not central positional vertigo). This was initially a treatment resistant-posterior semicircular canal cupulolithiasis but it later progressed to a posterior semicircular canal canalolithiasis, with symptoms promptly resolving after a repositioning manoeuvre.


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Data are available on reasonable request. Not applicable.

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Data availability statement

Data are available on reasonable request. Not applicable.

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  • Contributors JL and MS contributed to the study conception and design. Material preparation, data collection and analysis were performed by SM, AIM, AJ, DP, JL and MS. The first draft of the manuscript was written by SM and AIM and all authors commented on previous versions of the manuscript. All authors read and approved the final 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 MS is Joint Chief Editor of the Journal of Neurology, Editor in Chief of Frontiers of Neuro-otology and Section Editor of F1000. He has received speaker’s honoraria from Abbott, Auris Medical, Biogen, Eisai, Grünenthal, GSK, Henning Pharma, Interacoustics, J&J, MSD, Otometrics, Pierre-Fabre, TEVA, UCB, and Viatris. He is a shareholder and investor in IntraBio. He distributes 'M-glasses' and the 'Positional vertigo App'. He acts as a consultant for Abbott, AurisMedical, Heel, IntraBio and Sensorion.

  • Provenance and peer review Not commissioned: externally reviewed by Diego Kaski, London, UK.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.