A Case of Progressive Multifocal Leukoencephalopathy with Lung Cancer-related Brain Metastasis after Operation and Radiation Therapy
0
Citation
20
Reference
10
Related Paper
Abstract:
52歳男性, 肺癌による左後頭葉転移性脳腫瘍の術後・放射線治療後に進行性多巣性白質脳症を併発した症例を経験した. 今回, 徐々に増悪する歩行障害とADLの低下を主訴に受診され, 入院時MRIでは左前頭葉皮質下に拡散強調像で高信号領域を認めた. 当初は1カ月前に肺癌に対して施行された化学療法に伴う白質脳症を疑ったが, 徐々に臨床症状が悪化し入院2カ月後に死亡した. 正確な診断・鑑別のため, 進行性多巣性白質脳症の画像所見の特徴をおさえておく必要がある.Progressive multifocal leukoencephalopathy developed in a 33-year-old woman after renal transplantation and treatment with immunosuppressive agents. A characteristic clinical picture developed, pointing to multiple areas of progressive destruction in the cerebral hemispheres and brainstem, without evidence of increased intracranial pressure and with normal cerebrospinal fluid. The brain showed multiple areas of necrosis and demyelination, large abnormal glial cells, and papova-like virions within oligodendrocytic nuclei. Our observations show that progressive multifocal leukoencephalopathy may develop as a result of viral infection or activation of a dormant virus in the central nervous system secondary to altered immunity in the host.
JC Virus
Slow virus
Cite
Citations (68)
Natalizumab
JC Virus
Slow virus
Opportunistic infection
Cite
Citations (0)
In this issue of the Journal, there are reports describing in detail three patients in whom progressive multifocal leukoencephalopathy (PML) developed during treatment with natalizumab, a humanized monoclonal antibody against α4 integrins.13 These patients were among 3000 who had participated in clinical trials of natalizumab for the treatment of multiple sclerosis or Crohn's disease. PML is a deadly opportunistic infection of the central nervous system (CNS) for which there is no specific treatment. It is caused by reactivation of a clinically latent JC polyomavirus infection. This virus infects and destroys oligodendrocytes, leading to multifocal areas of demyelination and . . .
Natalizumab
JC Virus
Slow virus
Demyelinating disease
Cite
Citations (271)
Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common hereditary cerebral small vessel disease in adults. Many CADASIL cases were reported after NOTCH3 was identified as the causative gene of CADASIL. However, there is still no specific and effective therapies for CADASIL. In this review, we summarize recent research progress on disease models, symptomatic treatments and potential therapies for CADASIL, thereby providing a reference for follow-up CADASIL treatment research.伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy，CADASIL)是成人最常见的遗传性脑小血管病。自CADASIL的致病基因NOTCH3被鉴定以来，大量CADASIL病例被报道，但迄今仍缺乏有效的治疗药物。本文针对CADASIL的疾病模型和致病机制、对症药物治疗及潜在治疗方案研究进展进行综述，以期为后续CADASIL发病机制和治疗研究提供参考。.
CADASIL
Pathogenesis
Cite
Citations (0)
The prior diagnosis of fatal astrocytoma in a 60-year-old man with Crohn's disease treated with natalizumab, a monoclonal antibody against alpha4 integrins, was reclassified as JC virus-related progressive multifocal leukoencephalopathy (PML). Analysis of frozen serum samples showed that JC virus DNA had appeared in the serum three months after the initiation of open-label natalizumab monotherapy and two months before the appearance of symptomatic PML. There was staining of the brain lesion for polyomavirus. This case report, along with two others, suggests that anti-alpha4-integrin therapy can result in JC virus-induced PML.
Natalizumab
JC Virus
Slow virus
Cite
Citations (1,065)
Natalizumab
JC Virus
Cite
Citations (13)
Metastasectomy
Primary tumor
Cite
Citations (7)
Association (psychology)
Slow virus
Cite
Citations (0)
Progressive multifocal leukoencephalopathy is a subacute demyelinating disease of the central nervous system due to an opportunistic infection by a polyomavirus, most often JC virus, which predominantly infects oligodendrocytes. Progressive multifocal leukoencephalopathy used to be a rare condition, usually complicating lymphoproliferative diseases. Since the onset of the AIDS epidemic, its incidence has considerably increased and HIV infection has become, by far, the main risk factor for the disease. In AIDS patients, progressive leukoencephalopathy frequently shows atypical clinical and pathological features. The development of malignant glial tumors, within demyelinating regions, in patients with progressive multifocal leukoencephalopathy, has been reported in exceptional cases. The course of progressive multifocal leukoencephalopathy is invariably fatal. The diagnosis can only be made with certainty by histopathological examination of the brain, on cerebral biopsy or at postmortem. However, neuroradiological features may be extremely suggestive in many cases and PCR seems to be a reliable technique for demonstrating viral genome in the CSF. A few antiviral treatments have been proposed, however their efficacy is difficult to assess due to the low prevalence of the disease and the occurrence of rare cases with spontaneously prolonged survival.
JC Virus
Demyelinating disease
Brain biopsy
Cite
Citations (3)
Consider an underlying genetic cause in patients with progressive deterioration and leukoencephalopathy after prophylactic radiotherapy.
CADASIL
Cite
Citations (0)