Ruxolitinib
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JC virus granule cell neuronopathy: case report A 73-year-old woman developed JC virus granule cell neuronopathy (JCV-GCN) during treatment with ruxolitinib for postessential thrombocythaemia (ET) myelofibrosis. The woman, who was diagnosed with ET in 2002, was treated with anagrelide and ranimustine. In November 2017, she was diagnosed with CALR-mutated post-ET myelofibrosis. Therefore, she started receiving ruxolitinib 5mg twice daily [route not stated]. However, one year later, she presented with slowly progressive gait inability and left-sided intention tremor. Five months later, she developed gradually progressive dysarthria. Additionally, she had difficulty in walking and needed a cane to walk. A brain MRI revealed multiple hyperintense lesions in the left middle cerebellar peduncle (MCP), left cerebellar hemisphere and brainstem on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images. Approximately 7 months after the onset of symptoms, she was hospitalised. Neurological examination showed ataxic dysarthria, left-sided limb ataxia, slight left hemiparesis and gait ataxia. The scale for the assessment and rating of the ataxia (SARA) score was 19 points. Laboratory examination showed anaemia and thrombocytopenia. Lymphocyte counts were normal. However, CD4+ T-cell counts were decreased. Serum levels of blood urea nitrogen, creatinine and ferritin were found to have increased. Tests for auto-antibodies were all negative. A cerebrospinal fluid analysis revealed elevated levels of protein without pleocytosis. The IgG index was 0.58. Repeat MRI showed extension of the hyperintense lesions. Her CSF PCR was positive for JC virus. The JCV genome had deletion in the region-D within the non-coding control region, which is characteristic of progressive multifocal leucoencephalopathy (PML)-type (prototype) JCV. Initially, infratentorial PML was suspected. The woman’s ruxolitinib therapy was discontinued. She was treated with mirtazapine and mefloquine. Thereafter, her condition slightly improved. At 10 months after the onset of symptoms, copy numbers of JCV DNA also decreased. A follow-up MRI showed progressive cerebellar atrophy with a new lesion in the right MCP. Hypointense cerebellar cortical lesions on FLAIR images became prominent. She developed slightly right-sided limb ataxia. However, her SARA score remained stable at 17 points. At 16 months after the initial symptoms, the copy numbers of JCV DNA decreased further. A final diagnosis of JCV-GCN secondary to ruxolitinib therapy was made. Nakayama K, et al. JC virus granule cell neuronopathy associated with Ruxolitinib: A case report and review of the literature. eNeurologicalSci 21: Dec 2020. Available from: 803504438 URL: http://doi.org/10.1016/j.ensci.2020.100269
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Reactions 3 Oct 2020 No. 1824
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