Di Pauli Franziska, Berger Thomas, Walder Alois, Maier Hans, Rhomberg Paul, Uprimny Christian, Steurer Michael, Stockhammer Guenther
Clinical Department of Neurology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
Clinical Department of Neurology, Innsbruck Medical University, Anichstr. 35, 6020 Innsbruck, Austria.
J Clin Virol. 2014 Aug;60(4):424-7. doi: 10.1016/j.jcv.2014.05.007. Epub 2014 May 22.
A 58-year old female with a four-year history of previously untreated CLL at Binet stage A complained about word finding problems, impaired vision, and gait unsteadiness. Concerning her CLL she was asymptomatic and had never required any specific treatment. Her neurological examination disclosed cognitive alterations, homonyme hemianopia to the right, aphasia, and mild right-sided hemiparesis. Cerebral MRI showed a hyperintense lesion on T2 weighted images without contrast enhancement. CSF examination revealed normal findings, including CSF protein, cell count, cytology and PCR-analysis was negative for the presence of JC virus DNA. On follow-up MRI, performed 2 weeks later, the T2 lesion was further enlarging. Subsequent stereotactic brain biopsy was diagnostic for PML revealing abnormal oligodendrocytes staining positive against antibodies specific for simian vacuolating virus 40. In addition, repeated CSF analyses for JC-Virus DNA in the course of the disease became positive. After confirmation of diagnosis treatment with mirtazapine (30 mg/d) and mefloquine (250 mg/d) was initiated. Rapid clinical progression correlated to further worsening on MRI. Therefore this treatment was terminated after 16 days and the regime was changed to a five-day courses of cytarabine (2 mg/kg/d) combined with intrathecal administration of liposomal cytarabine (50 mg). Due to further clinical progression with global aphasia, blindness and severe right-sided hemiparesia, medication was stopped. The Patient died three and a half months after onset of symptoms.
一名58岁女性，有四年未经治疗的Binet A期慢性淋巴细胞白血病病史，主诉存在找词困难、视力受损和步态不稳。关于她的慢性淋巴细胞白血病，她无症状，从未需要任何特殊治疗。她的神经系统检查发现认知改变、右侧同向性偏盲、失语和轻度右侧偏瘫。脑部MRI在T2加权图像上显示一个高强度病变且无强化。脑脊液检查结果正常，包括脑脊液蛋白、细胞计数、细胞学检查，PCR分析未检测到JC病毒DNA。两周后进行的随访MRI显示，T2病变进一步扩大。随后的立体定向脑活检诊断为进行性多灶性白质脑病，显示异常少突胶质细胞对猴空泡病毒40特异性抗体染色呈阳性。此外，在病程中重复进行的脑脊液JC病毒DNA分析呈阳性。确诊后开始用米氮平（30毫克/天）和甲氟喹（250毫克/天）治疗。快速的临床进展与MRI上的进一步恶化相关。因此，该治疗在16天后终止，治疗方案改为为期五天的阿糖胞苷疗程（2毫克/千克/天）联合鞘内注射脂质体阿糖胞苷（50毫克）。由于出现全面失语、失明和严重右侧偏瘫等进一步的临床进展，停止用药。患者在症状出现三个半月后死亡。
