JC Virus Granule Cell Neuronopathy.pptx .

JC Virus Granule Cell Neuronopathy

1. Ade Wijaya , MD – July 2024 JC Virus Granule Cell Neuronopathy

2. Introduction JC virus  E tiological agent of progressive multifocal leukoencephalopathy , a white matter demyelinating disease that mostly affects immunocompromised patients JC virus can also cause: Meningitis Encephalitis Granule cell neuronopathy (JCV GCN) Lapierre SG, Dang X, Gilbert D, Lauzier S, Koralnik IJ, LeBlanc L. JC virus granule cell neuronopathy as AIDS-presenting illness. Canadian Journal of Neurological Sciences. 2018 Jul;45(4):466-9.

3. Pathophysiology Multiple JCV variants with mutations in the VP1 coding region were described and linked to lytic infection of cerebellar granule cell neurons and cerebellar atrophy. Infection with one of these mutants results in JCV GCN, a rare neurologic condition that can present with or without classical PML. 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):576-80

4. Clinical Features A subacute progressive cerebellar symptoms Marked RW. Teaching NeuroImage : Cerebellar Atrophy Due to JC Virus Granule Cell Neuronopathy . Neurology. 2023;101:e343-4. 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):576-80

5. Diagnosis Cerebellar atrophy PSR :CSF JC virus A histological diagnosis requires hypochromatic , enlarged granule cell neurons expressing JCV proteins on cerebellum biopsy (may occur with or without classical PML) 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):175-83. Agnihotri SP, Dang X, Carter JL, et al. JCV GCN in a natalizumabtreated MS patient is associated with mutations of the VP1 capsid gene. Neurology. 2014;83(8):727-32. 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.

6. Radiology Lapierre SG, Dang X, Gilbert D, Lauzier S, Koralnik IJ, LeBlanc L. JC virus granule cell neuronopathy as AIDS-presenting illness. Canadian Journal of Neurological Sciences. 2018 Jul;45(4):466-9.

7. Management No specific treatment R apid reversal of immunosuppression with ART only can prevent progression

8. Summary E tiology: John Cunningham Virus Subacute progressive cerebellar symptoms Cerebellar atrophy No specific treatment; ART can prevent progression

9. THANK YOU