Article Text

PDF
Delayed onset of a second paraneoplastic neurological syndrome in eight patients
  1. François Ducray1,2,
  2. Francesc Graus3,
  3. Maria Claudia Vigliani4,
  4. Jean-Christophe Antoine2,5,
  5. Veronique Rogemond1,2,
  6. Albert Saiz3,
  7. Jérôme Honnorat1,2
  1. 1Hospices Civils de Lyon, Hôpital Neurologique, Centre de Référence Maladie Rare ‘Syndromes Neurologiques Paranéoplasiques’, Lyon, France
  2. 2INSERM, U842, Lyon, Université Lyon 1, Lyon, France
  3. 3Department of Neurology, Hospital Clinic, Universitat de Barcelona and Institut d'Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Barcelona, Spain
  4. 4Dipartimento di Neuroscienze, Università di Torino, Torino, Italy
  5. 5Hôpital Bellevue, Neurologie, Saint-Etienne, France
  1. Correspondence to Professor J Honnorat, Neurologie B, Hôpital Neurologique de Lyon, 59 boulevard Pinel, 69677 Bron Cedex, France; jerome.honnorat{at}chu-lyon.fr

Abstract

Background The occurrence of a second delayed paraneoplastic neurological syndrome (PNS), different from the first, in the same patient, is not well known. However, recognition of this possibility is important in the management of the patient with PNS.

Objective To describe eight patients who presented with two different PNS with a time delay of months to years.

Design, setting and patients Retrospective analysis of eight patients, included in the European PNS database, with two different PNS that occurred months to years apart.

Results The median delay between the two different PNS was 15 months. The antibody repertoire did not change between the two episodes. Anti-Hu was detected in five patients, and anti-CV2 and anti-Ri in one each. One patient did not present onconeural antibodies. Lung cancer was diagnosed in six patients. In five patients, the second PNS revealed a cancer relapse (n=4) or a second cancer (n=1) and in three it occurred without evidence of tumour recurrence. In these three patients, all with anti-Hu antibodies, the second episode consisted of a lower motor neuron disease. Median survival was not reached after a median follow-up of 5 years.

Conclusions A second PNS can reveal a cancer relapse but can also arise in its absence. The long survival of patients with a second PNS suggests that the immune response might be more effective in controlling the cancer.

Statistics from Altmetric.com

Footnotes

  • Competing interests None.

  • Patient consent Obtained.

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

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.