1. Fatal JC-virus Granular Cerebellar Neuronopathy in a Patient Diagnosed with ALPS and Hypogammaglobulinemia.
- Author
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Helweg-Larsen, Jannik, Rasmussen, Morten, Marquart, Hanne Vibeke, Kondziella, Daniel, Marvig, Rasmus L., Rosenstierne, Maiken Worsøe, Bay, Jakob Thaning, Ryder, Lars Peter, Gjerdrum, Lise Mette Rahbek, Bangsgaard, Regitze, Gideon, Peter, Sellebjerg, Finn Thorup, and Fomsgaard, Anders
- Subjects
IMMUNOGLOBULIN light chains ,DIAGNOSIS ,SOMATIC mutation ,IMMUNE reconstitution inflammatory syndrome ,PROGRESSIVE multifocal leukoencephalopathy ,AGAMMAGLOBULINEMIA - Abstract
Progressive multifocal leukoencephalopathy (PML) is caused by lytic JC-virus (JCV) infection of oligodendroglial cells with progressive white matter lesions. Very rarely, JCV may cause granular cerebellar neuronopathy (JCV-GCN), caused by selective infection of cerebellar granule cell neurons by JCV variants, which commonly have a small deletion in the VP1 capsid (3). Previously, no cases of PML or JCV-GCN have been reported in patients with ALPS. B Timeline of JCV-CSF findings and treatment from diagnosis of JCV-GN to PML and death. [Extracted from the article]
- Published
- 2022
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