Department of Neurology, University of Michigan, Ann Arbor.
Division of Neurovirology, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
JAMA Neurol. 2014 Apr;71(4):487-9. doi: 10.1001/jamaneurol.2013.4668.
Progressive multifocal leukoencephalopathy results from lytic infection of the glia by the JC polyomavirus (JCV); JCV granule cell neuronopathy is caused by infection with a mutated form of JCV, leading to a shift in viral tropism from the glia to cerebellar granule cells. This shift results in a clinical syndrome dominated by progressive cerebellar dysfunction that might elude standard diagnostic workup strategies for ataxia.
We present the case report of a patient receiving long-term rituximab therapy who developed progressive cerebellar ataxia and marked isolated cerebellar degeneration. This syndrome resulted from JCV granule cell neuronopathy associated with a novel JCV mutation.
New onset or worsening of isolated cerebellar ataxia in patients being treated with rituximab or natalizumab warrants early assessment for JCV infection.
进行性多灶性白质脑炎是由 JC 多瘤病毒（JCV）对神经胶质细胞的裂解性感染引起的；JCV 颗粒细胞神经元病是由突变形式的 JCV 感染引起的，导致病毒嗜性从神经胶质细胞转移到小脑颗粒细胞。这种转移导致以进行性小脑功能障碍为主的临床综合征，可能逃避了共济失调的标准诊断策略。
我们报告了一例接受长期利妥昔单抗治疗的患者，该患者出现进行性小脑共济失调和明显孤立性小脑退行性变。这种综合征是由 JCV 颗粒细胞神经元病伴新型 JCV 突变引起的。
接受利妥昔单抗或那他珠单抗治疗的患者出现孤立性小脑共济失调新发病或恶化，需要早期评估 JCV 感染。
