Article Text

Download PDFPDF

069 Progressive multifocal leukoencephalopathy in good’s syndrome
  1. M Edwards1,
  2. R Cousins2,
  3. R Smith1,
  4. NP Hinds1
  1. 1Morriston Hospital, Swansea
  2. 2University Hospital of Wales, Cardiff

Abstract

A 65-year-old retired paramedic presented with progressive visual disturbance, cognitive impairment and behavioural change over a course of 3 months. At the time of admission he was cortically blind and disoriented to time, place and person. His past medical history included a recently resected thymoma. An MRI Head with contrast revealed bilateral white matter signal change mainly in occipital lobes extending to both temporal and parietal lobes. This was non-enhancing with no obvious oedema. His basic CSF parameters were normal and JCV antibodies werenegative. However his CSF JCV PCR was significantly high and consistent with a diagnosis of progressive multifocal leukoencephalopathy (PML). HIV testing was negative and he was not taking any immunosuppressive therapy. The patient was found to be hypogammaglobulinaemic as well having a low B-cell count. In the context of a thymoma this was a case of Good’s syndrome. Unfortunately the patient deteriorated further due to a hospital acquired pneumonia and he was palliated prior to his death. PML is a rare diagnosis and most often related to HIV or immunosuppression. It has been reported in cases of hypogammaglobulinaemia but this is thought to be one of only a handful of cases of PML in Good’s syndrome.

Statistics from Altmetric.com

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.