Encephalitis of Unclear Origin Diagnosed by Brain Biopsy A Diagnostic Challenge
Encephalitis of Unclear Origin Diagnosed by Brain Biopsy A Diagnostic Challenge
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DOI：
10.1001/jamaneurol.2014.2376
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发表时间：
2015-01-01
期刊：
影响因子：
29
通讯作者：
Cree, Bruce A. C.
中科院分区：
文献类型：
--
作者：
Gelfand, Jeffrey M.;Genrich, Gillian;Cree, Bruce A. C.
脑活检标本表现为脑炎，没有特定的组织病理学特征，对神经病理学家和神经学家的诊断提出了挑战。这些病例在病理学上通常称为脑炎，未另作说明（ENOS）。对这类患者进行系统的诊断评价具有挑战性，目前还没有普遍接受的algorithm. ObjectiveTo检查脑活检诊断的ENOS患者的最终诊断结果。设计、设置和参与者本回顾性病例系列在加州大学旧金山弗朗西斯科医学中心，一个三级护理城市神经科学中心，研究了1983年1月1日至2011年12月31日通过脑活检诊断的脑炎患者。暴露脑活检。主要结果和测量临床和神经病理诊断。结果在58例符合研究纳入标准的患者中，49例（84%）患者的原始病理诊断为ENOS。中位年龄为40岁（四分位距，27-53岁），35例患者为男性，13例已知患有人类免疫缺陷病毒或艾滋病。从症状发作到脑活检的中位时间为66天（四分位距，18-135天）。对于29例仍有病理学分析材料的患者，额外的神经病理学审查导致10例（34%）更具体的分类。42例患者的临床详细信息和随访信息可用，12例患者在辅助检测和/或临床随访的帮助下获得了具体诊断。尽管全面的神经病理学审查，额外的研究和信息，27例患者仍然被归类为ENOS类在研究结束时，研究。结论和相关性ENOS是最常见的初始类型的脑炎脑活检诊断。在这样的患者中，可能值得由神经病理学家再次全面审查活检材料，因为额外的审查导致我们三分之一的病例进行了更具体的分类。辅助检测、临床相关性和临床随访可在某些患者中建立更特异的诊断。在许多情况下，经过所有这些努力，ENOS仍然是一个诊断挑战。目前的算法价值有限。需要更先进的方法和更好的诊断算法来表征这些患者。
IMPORTANCE Brain biopsy specimens that exhibit encephalitis without specific histopathologic features pose a diagnostic challenge to neuropathologists and neurologists. Such cases are generally referred to pathologically as encephalitis, not otherwise specified (ENOS). A systematic approach to diagnostic evaluation in such patients is challenging, and currently there is no generally accepted algorithm.OBJECTIVE To examine ultimate diagnostic outcomes in patients with ENOS diagnosed by brain biopsy.DESIGN, SETTING, AND PARTICIPANTS This retrospective case series at the University of California, San Francisco, Medical Center, a tertiary care urban neurosciences center, studied patients with encephalitis diagnosed by brain biopsy from January 1, 1983, through December 31, 2011.EXPOSURES Brain biopsy.MAIN OUTCOMES AND MEASURES Clinical and neuropathologic diagnosis.RESULTS Among 58 patients who met the inclusion criteria for the study, the original pathologic diagnosis was ENOS in 49 patients (84%). The median age was 40 years (interquartile range, 27-53 years), 35 patients were male, and 13 had known human immunodeficiency virus or AIDS. Median time from onset of symptoms to brain biopsy was 66 days (interquartile range, 18-135 days). For the 29 patients in whom material for pathologic analysis was still available, additional neuropathologic review led to a more specific categorization in 10 (34%). Clinical detail and follow-up information was available for 42 patients, and a specific diagnosis was reached with the help of ancillary testing and/or clinical follow-up in 12 patients. Despite a comprehensive neuropathologic review with additional studies and information, 27 patients still had to be classified in the ENOS category at the end of the study.CONCLUSIONS AND RELEVANCE ENOS is the most common initial type of encephalitis diagnosed by brain biopsy. In such patients, it may be worth having the biopsy materials reviewed again in a comprehensive fashion by a neuropathologist because additional review led to a more specific categorization in one-third of our cases. Ancillary testing, clinical correlation, and clinical follow-up establish more specific diagnoses in some patients. ENOS still remains a diagnostic challenge after all these efforts in many cases. Current algorithms are of limited value. More advanced methods and better diagnostic algorithms are needed to characterize these patients.