Article Text

Download PDFPDF
CASE REPORT
Sensory neuronopathy associated with cholangiocarcinoma diagnosed 6 years after symptom onset
  1. Alexander M Rossor1,
  2. Julian Blake2,
  3. Theodora Pissanou3,
  4. Mary M Reilly1
  1. 1 MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, London, UK
  2. 2 Department of Neurophysiology, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK
  3. 3 Sheila Sherlock Liver Unit, Royal Free London NHS Foundation Trust, London, UK
  1. Correspondence to Dr Alexander M Rossor, a.rossor{at}ucl.ac.uk

Summary

A pure sensory neuronopathy (also referred to as a sensory ganglionopathy) is one of a handful of classical neurological paraneoplastic syndromes. Current guidelines recommend that in cases of sensory neuronopathy, a search for an underlying malignancy be pursued for up to 4 years. We report the case of a 52-year-old woman with a sensory neuronopathy who was eventually diagnosed with a cholangiocarcinoma 6 years after the onset of her disease. A CT fluorodeoxyglucose positron emission tomography (FDG-PET) scan performed 18 and 24 months after disease onset failed to identify an underlying neoplasm. Immunomodulatory treatment with corticosteroids, intravenous immunoglobulins and plasma exchange were ineffective. Investigations for Sjogren’s disease were negative. A third FDG-PET performed 6 years after symptom onset identified a cholangiocarcinoma, which was confirmed histologically following open resection. Since the tumour was removed, our patient’s condition has not progressed, but there has been no improvement and she remains severely disabled.

  • neuromuscular disease
  • peripheral nerve disease
  • hepatic cancer
View Full Text

Statistics from Altmetric.com

Footnotes

  • Contributors AMR wrote the first draft of the manuscript. JB, TP and MMR revised the manuscript. All authors were involved in the clinical care of the patient.

  • Funding AM Rossor is funded by a Wellcome Trust post doctoral fellowship for clinicians (110043/Z/15/Z).

  • Competing interests None declared.

  • 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.