Granule Cell Neuronopathy in a Patient with Common Variable Immunodeficiency

  • Andrew McLean-TookeEmail author
  • Constantine Chris Phatouros
  • Glenys Chidlow
  • David W Smith
  • Peter Silbert
Letter to Editor

To the Editor,

The JC virus or John Cunningham virus is a member of the polyomavirus family found only in humans. Initial infection occurs mainly in childhood, and is usually asymptomatic. The virus establishes lifelong latent infection in a range of tissues, including the brain. JC virus is recognised as the cause for progressive multifocal leukoencephalopathy (PML), an often-devastating central nervous system infection resulting from reactivation of latent virus, almost exclusively seen in immunosuppressed patients. Whereas PML results from JC virus infection of brain glial cells, infection of granule neurons in the cerebellum results in granule cell neuronopathy (GCN). We report a common variable immunodeficiency (CVID) patient who developed GCN, and who clinically improved with mirtazapine and mefloquine.

The patient was referred in 2004 at 58 years of age with a 5-year history of recurrent bacterial pulmonary and sinus infections and recurrent diarrhoea. Past medical history...


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Supplementary material

10875_2019_624_MOESM1_ESM.jpg (38 kb)
Figure S1 Axial CT scan of lung bases demonstrates old left lower lobe calcific granuloma [Yellow Arrow] and left basal bronchiectasis [Red Arrow] with resulting volume loss and elevation of the left hemidiaphragm [Blue Arrows] (JPG 38 kb)
10875_2019_624_MOESM2_ESM.docx (17 kb)
ESM 2 (DOCX 17 kb)


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Clinical ImmunologySir Charles Gairdner HospitalPerthAustralia
  2. 2.Department of Laboratory ImmunologyPathWest QEII Medical CentrePerthAustralia
  3. 3.Neurological Intervention & Imaging Service of Western AustraliaSir Charles Gairdner HospitalPerthAustralia
  4. 4.Department of MicrobiologyPathWest Laboratory Medicine WAPerthAustralia
  5. 5.Faculty of Health and Medical SciencesUniversity of Western AustraliaPerthAustralia
  6. 6.School of MedicineUniversity of Western AustraliaPerthAustralia

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