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患有结外中枢神经系统Rosai-Dorfman病且新发癫痫的孕妇:区分疾病进展与子痫的难题。

Pregnant patient with extranodal CNS Rosai-Dorfman disease with new-onset seizures: the conundrum of differentiating disease progression from eclampsia.

作者信息

Swor Katie, Zutshi Deepti, Dubey Elizabeth, Gonik Bernard

机构信息

Obstetrics-Gynecology, Wayne State University, Detroit, Michigan, USA

Neurology, Wayne State University, Detroit, Michigan, USA.

出版信息

BMJ Case Rep. 2021 Feb 18;14(2):e239427. doi: 10.1136/bcr-2020-239427.

DOI:10.1136/bcr-2020-239427
PMID:33602767
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7896577/
Abstract

A 31-year-old woman at 30 weeks gestation presented to the emergency department following multiple seizures. Her history was significant for extranodal Rosai-Dorfman Disease (RDD) with central nervous system (CNS) lesions. RDD, a rare form of non-Langerhans cell histiocytosis, commonly presents with non-tender cervical lymphadenopathy. CNS involvement accounts for a small number of cases in those with extranodal disease. Patients with CNS RDD can have a variety of neurological symptoms, including seizures. Eclampsia, a relatively rare obstetric hypertensive disorder, is always within the differential diagnosis for patients presenting with late gestation seizures. We present the challenging evaluation and treatment of a patient whose clinical picture did not clearly differentiate eclampsia from new onset seizures related to progression of her RDD. This conundrum perhaps resulted in unnecessary preterm operative delivery of a critically ill patient. Only follow-up of the patient helped clarify the likely antepartum diagnosis.

摘要

一名31岁、孕30周的女性在多次癫痫发作后被送往急诊科。她有显著的病史,患有结外Rosai-Dorfman病(RDD)并伴有中枢神经系统(CNS)病变。RDD是一种罕见的非朗格汉斯细胞组织细胞增多症,通常表现为无痛性颈部淋巴结病。中枢神经系统受累在结外疾病患者中占少数病例。患有中枢神经系统RDD的患者可出现多种神经症状,包括癫痫发作。子痫是一种相对罕见的产科高血压疾病,始终在妊娠晚期癫痫发作患者的鉴别诊断范围内。我们介绍了一名患者具有挑战性的评估和治疗过程,该患者的临床表现未能明确区分子痫与因RDD进展导致的新发癫痫发作。这个难题可能导致了对一名危重症患者进行了不必要的早产手术分娩。只有对该患者的随访才有助于明确可能的产前诊断。

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Pregnant patient with extranodal CNS Rosai-Dorfman disease with new-onset seizures: the conundrum of differentiating disease progression from eclampsia.患有结外中枢神经系统Rosai-Dorfman病且新发癫痫的孕妇:区分疾病进展与子痫的难题。
BMJ Case Rep. 2021 Feb 18;14(2):e239427. doi: 10.1136/bcr-2020-239427.
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本文引用的文献

1
Pregnancy in a Rare Case of Intracranial Rosai Dorfman Disease (RDD).罕见的颅内Rosai-Dorfman病(RDD)合并妊娠
J Obstet Gynaecol India. 2020 Feb;70(1):78-80. doi: 10.1007/s13224-019-01254-y. Epub 2019 Aug 2.
2
Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease.罗萨-多夫曼-德斯东贝病的诊断和临床管理共识建议。
Blood. 2018 Jun 28;131(26):2877-2890. doi: 10.1182/blood-2018-03-839753. Epub 2018 May 2.
3
Cranio-spinal Rosai Dorfman disease: case series and literature review.颅-脊髓型罗萨伊-多夫曼病:病例系列及文献综述
Br J Neurosurg. 2019 Apr;33(2):176-183. doi: 10.1080/02688697.2017.1329517. Epub 2017 May 22.
4
Rosai-Dorfman Disease Masquerading as Cholangiocarcinoma.伪装成胆管癌的罗萨伊-多夫曼病
Clin Gastroenterol Hepatol. 2017 Feb;15(2):e55-e56. doi: 10.1016/j.cgh.2016.08.039. Epub 2016 Sep 5.
5
Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature.中枢神经系统Rosai-Dorfman病:6例报告并文献复习
Medicine (Baltimore). 2014 May;93(3):165-175. doi: 10.1097/MD.0000000000000030.
6
Imaging characteristics of Rosai-Dorfman disease in the central nervous system.中枢神经系统 Rosai-Dorfman 病的影像学特征。
Eur J Radiol. 2012 Jun;81(6):1265-72. doi: 10.1016/j.ejrad.2011.03.006. Epub 2011 Mar 25.
7
Rosai-Dorfman disease of the mandible.下颌骨的罗萨伊-多夫曼病
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006 Oct;102(4):506-12. doi: 10.1016/j.tripleo.2005.10.071. Epub 2006 Mar 20.
8
Pregnancy and sinus histiocytosis with massive lymphadenopathy.妊娠与伴有巨大淋巴结病的窦性组织细胞增生症。
Ann Intern Med. 1985 Feb;102(2):278. doi: 10.7326/0003-4819-102-2-278.