Concomitant granule cell neuronopathy in patients with natalizumab-associated PML

Journal of Neurology
Martijn T WijburgMike P Wattjes

Abstract

Granule cell neuronopathy (GCN) is a rare JC virus infection of the cerebellar granule cell neurons in immunocompromised patients. On brain imaging, GCN is characterized by cerebellar atrophy which can be accompanied by infratentorial white matter lesions. The objective of this study is to investigate the prevalence of MRI findings suggestive of GCN in a large natalizumab-associated progressive multifocal leukoencephalopathy (PML) cohort. MRI scans from before, at the time of, and during follow-up after diagnosis of PML in 44 natalizumab-treated MS patients, and a control group of 25 natalizumab-treated non-PML MS patients were retrospectively reviewed for imaging findings suggestive of GCN. To assess and quantify the degree of cerebellar atrophy, we used a 4 grade rating scale. Three patients in the PML group showed imaging findings suggestive of GCN and none in the control group. In two of these PML patients, cerebellar atrophy progressed from grade 0 at the time of diagnosis of isolated supratentorial PML to grade 1 and 2 after 2.5 and 3 months, respectively, in the absence of infratentorial white mater lesions. The third patient had grade 1 cerebellar atrophy before diagnosis of infra- and supratentorial PML, and showed pro...Continue Reading

References

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Citations

Aug 18, 2016·Journal of Neurology, Neurosurgery, and Psychiatry·Martijn T WijburgMike P Wattjes
Mar 13, 2018·Expert Opinion on Pharmacotherapy·Paulus S Rommer, Uwe K Zettl
Aug 2, 2017·Neurology. Neuroimmunology and Neuroinflammation·Salwa KamouriehRichard Nicholas
Nov 22, 2020·Nature Reviews. Neurology·Irene CorteseAvindra Nath
Nov 25, 2020·Journal of Neurology, Neurosurgery, and Psychiatry·Martijn T WijburgMike P Wattjes

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