Heterogeneous imaging characteristics of JC virus granule cell neuronopathy (GCN): a case series and review of the literature

Autor: Mike P. Wattjes, Joep Killestein, Martijn T. Wijburg, Ouafae Karimi, Bob W. van Oosten, Jean-Luc Murk
Přispěvatelé: Neurology, Medical Microbiology and Infection Prevention, Internal medicine, Radiology and nuclear medicine, NCA - Neuroinflamation
Rok vydání: 2014
Předmět:
Zdroj: Journal of Neurology, 262(1), 65-73. D. Steinkopff-Verlag
Wijburg, M T, van Oosten, B W, Murk, J L A N, Karimi, O, Killestein, J & Wattjes, M P 2015, ' Heterogeneous imaging characteristics of JC virus granule cell neuronopathy (GCN): a case series and review of the literature ', Journal of Neurology, vol. 262, no. 1, pp. 65-73 . https://doi.org/10.1007/s00415-014-7530-5
ISSN: 1432-1459
0340-5354
Popis: Granule cell neuronopathy (GCN) is a rare JC virus (JCV)-related disease in immunocompromised patients, characterized by lytic infection of the cerebellar granule cell layer. To enable early diagnosis and intervention, we identify features of GCN and describe possible aspects of disease heterogeneity. We report on two new cases of GCN in HIV-infected patients of whom we retrospectively assessed clinical and radiologic data. In addition, we carried out a literature search and review of clinical, radiologic and histopathologic findings of all published GCN cases. Including the two new cases reported here, a total of 18 GCN cases were included in this study. HIV infection, present in 12 of the cases, was the most common underlying condition, followed by monoclonal antibody treatment which was present in three cases. Cerebellar atrophy was detected in all except two cases. In 12 patients a heterogeneous distribution pattern of white matter changes in the cerebellum and brainstem was observed. Imaging findings in GCN are remarkably heterogeneous; exhibiting cerebellar atrophy, as well as white matter pathology, particularly in the adjacent infratentorial white matter. This suggests an overlap of GCN with other JCV-related diseases, such as progressive multifocal leukoencephalopathy.
Databáze: OpenAIRE