Park Jae Hyun, Ryoo Sookyung, Noh Hyun Jin, Seo Jung Min, Kang Hyun Hee, Shin Ji Soo, Seo Sang Won, Na Duk L
Departments of Neurology, Sungkyunkwan University School of Medicine, Seoul, Korea.
Case Rep Neurol. 2011 Sep;3(3):258-62. doi: 10.1159/000333780. Epub 2011 Oct 14.
Progressive multifocal leukoencephalopathy (PML) is a demyelinating central nervous system disease caused by JC virus (JCV) reactivation in immunocompromised patients. The disease course of PML is often progressive, fatal and at present, there are few reports on successful treatment outcomes.
A 45-year-old man with systemic sarcoidosis presented with rapidly progressive dementia and right hemiparesis. The patient was diagnosed with PML as confirmed via brain biopsy and JCV PCR. With a combination treatment of cidofovir and mirtazapine, there was significant improvement of neurological symptoms without measurable functional deficit.
This case suggests that dual therapy with cidofovir and mirtazapine might be an effective treatment option in PML patients with sarcoidosis.
进行性多灶性白质脑病(PML)是一种由免疫功能低下患者体内JC病毒(JCV)重新激活引起的中枢神经系统脱髓鞘疾病。PML的病程通常呈进行性且致命,目前关于成功治疗结果的报道很少。
一名45岁的系统性结节病男性患者出现快速进展性痴呆和右侧偏瘫。经脑活检和JCV聚合酶链反应(PCR)确诊为PML。采用西多福韦和米氮平联合治疗后,神经症状有显著改善,且无明显功能缺陷。
该病例表明,西多福韦和米氮平联合治疗可能是结节病相关PML患者的一种有效治疗选择。