From the Henry Ford Health System, Detroit, MI.
Am J Forensic Med Pathol. 2021 Jun 1;42(2):186-190. doi: 10.1097/PAF.0000000000000656.
Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease caused by reactivation of John Cunningham virus affecting typically subcortical and periventricular white matter of immunocompromised hosts (human immunodeficiency virus infection, hematologic malignancies). Cerebral hemispheric white matter is most commonly affected by lytic infections, leading to progressive damage to oligodendrocytes in the central nervous system. Neuroimaging usually highlights scattered foci of white matter hypodensity not attributable to contrast enhancement or mass effect. In contrast, we present an unusual case of PML predominantly affecting cervical spinal cord and brainstem in an immunocompetent host. This is a rare subset of PML case that can occur in association with connective tissue disorders (Sjögren Syndrome in this case), systemic lupus erythematosus being the most common. Progressive multifocal leukoencephalopathy should be considered in the differential diagnosis of spinal cord or brainstem lesions, particularly in the patients with connective tissue disorders.
进行性多灶性白质脑病（PML）是一种罕见的脱髓鞘疾病，由约翰·坎宁安病毒（JC 病毒）激活引起，影响通常为免疫功能低下宿主（人类免疫缺陷病毒感染、血液系统恶性肿瘤）的皮质下和脑室周围白质。大脑半球白质最常受到溶质性感染的影响，导致中枢神经系统的少突胶质细胞进行性损伤。神经影像学通常突出显示散在的脑白质密度降低病灶，不能归因于对比增强或肿块效应。相比之下，我们报告了一例免疫功能正常宿主中以颈髓和脑干为主的不常见 PML 病例。这是 PML 的一个罕见亚组病例，可与结缔组织疾病（本例为干燥综合征）相关发生，系统性红斑狼疮是最常见的。在鉴别诊断脊髓或脑干病变时，应考虑进行性多灶性白质脑病，特别是在结缔组织疾病患者中。
