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JC Virus Granule Cell Neuronopathy as AIDS-Presenting Illness

  • Simon Grandjean Lapierre (a1) (a2), Xin Dang (a3), Danielle Gilbert (a4), Sylvie Lauzier (a5), Igor J. Koralnik (a3) and Louiselle LeBlanc (a2) (a6)...

Abstract

JC virus is the etiological agent of progressive multifocal leukoencephalopathy, a white matter demyelinating disease that mostly affects immunocompromised patients. JC virus can also infect neurons and meningeal cells and cause encephalitis, meningitis and granule cell neuronopathy. We report a patient with JC virus granule cell neuronopathy, without concomitant progressive multifocal leukoencephalopathy, presenting as inaugural acquired immune deficiency syndrome-related illness. This patient’s human immunodeficiency virus infection remained undiagnosed for several months after neurological symptoms onset. We review JC virus pathophysiology, clinical manifestations, treatment and prognosis, and emphasize the importance of considering human immunodeficiency virus infection and related opportunistic infections in the differential diagnosis of new-onset isolated cerebellar disease.

Un cas de neuropathie des cellules granulaires causé par le virus JC et relié au syndrome d’immunodéficience acquise. Le virus JC est l’agent étiologique de la leucoencéphalopathie multifocale progressive, une maladie démyélinisante de la substance blanche qui affecte surtout des patients immunodéprimés. Ce virus peut aussi infecter les cellules des neurones et des méninges et entraîner des cas d’encéphalite, de méningite et de neuropathie des cellules granulaires. Nous voulons justement faire état ici du cas d’un patient atteint de neuropathie des cellules granulaires causée par le virus JC et reliée au syndrome d’immunodéficience acquise (SIDA). À noter que ce patient n’a pas développé, de façon concomitante, la leucoencéphalopathie multifocale progressive. Dans son cas, l’infection au VIH est demeurée non diagnostiquée plusieurs mois après l’apparition des premiers symptômes neurologiques. Nous nous sommes donc penchés sur la physiopathologie du virus JC, sur ses manifestations cliniques, sur ses traitements et sur le pronostic pouvant être établi. Nous avons aussi voulu souligner l’importance de considérer l’infection au VIH et d’autres infections opportunistes y étant reliées à la lumière du diagnostic différentiel d’une maladie cérébelleuse isolée nouvellement déclarée.

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Copyright

Corresponding author

Correspondence to: S. Grandjean Lapierre, Infectious Diseases and Medical Microbiology, Universitair Medisch Centrum Groningen, Montreal, QC, Canada 9700 RB. Email: simon.grandjean.lapierre@umontreal.ca

References

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1. Miskin, DP, Koralnik, IJ. Novel syndromes associated with JC virus infection of neurons and meningeal cells: no longer a gray area. Curr Opin Neurol. 2015;28(3):288-294.
2. Agnihotri, SP, Wuthrich, C, Dang, X, et al. A fatal case of JC virus meningitis presenting with hydrocephalus in a human immunodeficiency virus-seronegative patient. Ann Neurol. 2014;76(1):140-147.
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7. Koralnik, IJ, Wuthrich, C, Dang, X, et al. JC virus granule cell neuronopathy: a novel clinical syndrome distinct from progressive multifocal leukoencephalopathy. Ann Neurol. 2005;57(4):576-580.
8. Dang, X, Vidal, JE, Oliveira, AC, et al. JC virus granule cell neuronopathy is associated with VP1 C terminus mutants. J Gen Virol. 2012;93(Pt 1):175-183.
9. Agnihotri, SP, Dang, X, Carter, JL, et al. JCV GCN in a natalizumab-treated MS patient is associated with mutations of the VP1 capsid gene. Neurology. 2014;83(8):727-732.
10. Henry, C, Jouan, F, De Broucker, T. JC virus granule cell neuronopathy: a cause of infectious cerebellar degeneration. J Neurol Sci. 2015;354(1-2):86-90.

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