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海绵状白质脑病:一例经活检确诊的继发于有毒、非炎性暴露的白质脑病的独特病例。

Spongiform leukoencephalopathy: A unique case of biopsy confirmed leukoencephalopathy secondary to toxic, non-inflammatory exposure.

作者信息

Zemina Kristen, Piña Yolanda, Malafronte Patrick, Suresh Niraja, Hurst Rebeca

机构信息

Department of Neurology. Morsani College of Medicine, University of South Florida, Tampa, FL, USA.

Department of Neuro-Oncology. H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.

出版信息

SAGE Open Med Case Rep. 2021 Sep 1;9:2050313X211042984. doi: 10.1177/2050313X211042984. eCollection 2021.

DOI:10.1177/2050313X211042984
PMID:34484793
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8414607/
Abstract

Toxin-induced leukoencephalopathy is a rare neurological condition that has been previously associated with intracranial radiation, chemotherapy, drugs of abuse, and environmental exposures. Herein, we present a patient with brain-biopsy proven toxin-induced leukoencephalopathy, likely secondary to multiple environmental offenders including insecticides and non-Food and Drug Administration approved anabolic steroids, opioids, and benzodiazepines. A 60-year-old man presented to our service as a direct transfer from an outside facility for evaluation of a rapidly progressive neuropsychiatric decline. Extensive workup with blood work, cerebrospinal fluid analysis, paraneoplastic panel, serial magnetic resonance imaging brain with and without contrast, and electroencephalograms were unrevealing. Magnetic resonance imaging brain showed diffuse confluent white matter disease, which was non-specific. The patient was treated with high-dose methylprednisolone and trials of intravenous immunoglobulin without any significant improvement. Finally, a brain biopsy was performed, and pathology confirmed a spongiform leukoencephalopathy, favoring a toxin-related etiology. The diagnosis of toxin-induced leukoencephalopathy should be considered in patients with steep neuropsychiatric decline and associated diffuse white matter disease. Diagnosis relies heavily on history of exposure, clinical presentation, imaging findings, and ultimately, histopathology from brain biopsy. The recognition of the clinical presentation is important to pursue the appropriate diagnostic workup and treatment.

摘要

毒素诱导性白质脑病是一种罕见的神经系统疾病,此前曾与颅内放疗、化疗、滥用药物及环境暴露有关。在此,我们报告一例经脑活检证实为毒素诱导性白质脑病的患者,可能继发于多种环境因素,包括杀虫剂以及未获美国食品药品监督管理局批准的合成代谢类固醇、阿片类药物和苯二氮䓬类药物。一名60岁男性患者从外部机构直接转入我院,以评估其快速进展的神经精神衰退情况。进行了全面检查,包括血液检查、脑脊液分析、副肿瘤相关检查、对比剂增强及未增强的头颅磁共振成像序列检查以及脑电图检查,但均未发现异常。头颅磁共振成像显示弥漫性融合性白质病变,表现不具特异性。该患者接受了大剂量甲泼尼龙治疗及静脉注射免疫球蛋白试验,但均无明显改善。最后进行了脑活检,病理证实为海绵状白质脑病,倾向于毒素相关病因。对于神经精神状态急剧衰退且伴有弥漫性白质病变的患者,应考虑毒素诱导性白质脑病的诊断。诊断很大程度上依赖于暴露史、临床表现、影像学表现,最终还需依靠脑活检的组织病理学结果。认识临床表现对于进行适当的诊断检查和治疗很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdba/8414607/dbe89d2461db/10.1177_2050313X211042984-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdba/8414607/f49894201e87/10.1177_2050313X211042984-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdba/8414607/8edcd5012495/10.1177_2050313X211042984-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdba/8414607/dbe89d2461db/10.1177_2050313X211042984-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdba/8414607/f49894201e87/10.1177_2050313X211042984-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdba/8414607/8edcd5012495/10.1177_2050313X211042984-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bdba/8414607/dbe89d2461db/10.1177_2050313X211042984-fig3.jpg

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本文引用的文献

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Chasing the wrong dragon: A new presentation of heroin-induced toxic leukoencephalopathy mimicking anoxic brain injury.追逐错误的“龙”:一种酷似缺氧性脑损伤的海洛因所致中毒性白质脑病的新表现。
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大脑中的毒素!中毒性白质脑病的磁共振成像——图文并茂的文章
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J Neuropsychiatry Clin Neurosci. 2017 Fall;29(4):308-318. doi: 10.1176/appi.neuropsych.17010006. Epub 2017 May 16.
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Pathology of toxic leucoencephalopathy in drug abuse supports hypoxic-ischemic pathophysiology/etiology.药物滥用中毒性白质脑病的病理学支持缺氧缺血性病理生理学/病因学。
Neuropathology. 2017 Aug;37(4):321-328. doi: 10.1111/neup.12377. Epub 2017 Mar 9.
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Chlorfenapyr-Induced Toxic Leukoencephalopathy with Radiologic Reversibility: A Case Report and Literature Review.氯虫苯甲酰胺诱发的具有放射学可逆性的中毒性白质脑病:一例报告及文献综述
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Intravenous Heroin-Associated Delayed Spongiform Leukoencephalopathy: Case Report and Reviews of the Literature.静脉注射海洛因相关的迟发性海绵状白质脑病:病例报告及文献综述
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