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Case Reports
. 2018 Jul;45(4):466-469.
doi: 10.1017/cjn.2018.28.

JC Virus Granule Cell Neuronopathy as AIDS-Presenting Illness

Simon Grandjean Lapierre  1 , Xin Dang  2 , Danielle Gilbert  3 , Sylvie Lauzier  4 , Igor J Koralnik  2 , Louiselle LeBlanc  5
Affiliations
Case Reports

JC Virus Granule Cell Neuronopathy as AIDS-Presenting Illness

Simon Grandjean Lapierre et al. Can J Neurol Sci. 2018 Jul.

Abstract
in English, French

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.

Keywords: Demyelination; Granule cell neuronopathy; HIV; JC virus; Neuroimaging.

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Figures

Figure 1:
Figure 1:
JC virus (JCV) DNA sequence variations among variants responsible for granule cell neuronopathy (GCN). JCV DNA sequencing revealing co-existence of JCVGCN1 and JCVGCN8.
Figure 2:
Figure 2:
Evolution of MRI abnormalities at (A) 1 month, (B) 10 months and (C) 22 months after symptoms onset. Axial FLAIR sequences showed marked posterior fossa atrophy without signal abnormality (left) and Sagittal T1 sequences showed progressive pontocerebellar and pons atrophy (right).
See this image and copyright information in PMC

Comment in

References

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