Progressive multifocal leukoencephalopathy and CD4+ T-lymphocytopenia in a patient with Sjögren syndrome

J Neurol Sci. 2008 May 15;268(1-2):195-8. doi: 10.1016/j.jns.2007.12.015. Epub 2008 Jan 29.


We report progressive multifocal leukoencephalopathy (PML) and CD4+ T-lymphocytopenia in a 71-year-old man with Sjögren syndrome (SjS). The patient was admitted to our hospital because of progressive dementia and gait disturbance. T2-weighted MR images showed high-intensity lesions in his left frontal white matter thalamus, cerebellum and brainstem. A pathological diagnosis of PML was made by brain biopsy. SjS is frequently accompanied with immunological complications; however, there are few reports on PML in patients with SjS. Recently, isolated CD4+ T-lymphocytopenia is reported to be one of the based immunological conditions associated with the development of PML. In the present case, CD4+ T-lymphocytopenia was also observed on admission, which is also associated with SjS.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Humans
  • Leukoencephalopathy, Progressive Multifocal / complications*
  • Leukoencephalopathy, Progressive Multifocal / diagnostic imaging
  • Leukoencephalopathy, Progressive Multifocal / pathology
  • Magnetic Resonance Imaging
  • Male
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / diagnostic imaging
  • Sjogren's Syndrome / pathology
  • T-Lymphocytopenia, Idiopathic CD4-Positive / complications*
  • T-Lymphocytopenia, Idiopathic CD4-Positive / diagnostic imaging
  • T-Lymphocytopenia, Idiopathic CD4-Positive / pathology
  • Time Factors
  • Tomography, Emission-Computed, Single-Photon
  • Viral Regulatory and Accessory Proteins / metabolism


  • Viral Regulatory and Accessory Proteins
  • agnoprotein, polyomavirus