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临床推理:一位 24 岁孕妇,头痛并逐渐出现行为改变和昏迷。

Clinical Reasoning: A 24-Year-Old Pregnant Woman With Headache and Behavioral Change Progressing to Coma.

机构信息

From the Neurology Department (C.B., J.L., C.L., S.B.); Functional Unit of Neurorradiology (R.P.), Department of Medical Imaging; and Ophthalmology Department (C.F.), Coimbra University Hospital Centre, Portugal.

出版信息

Neurology. 2023 Nov 27;101(22):e2331-e2337. doi: 10.1212/WNL.0000000000207859.

DOI:10.1212/WNL.0000000000207859
PMID:37648530
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10727214/
Abstract

The broad differential diagnosis associated with progressive subacute encephalopathy can be intimidating, especially in a young, pregnant woman. In this case, a 24-year-old woman at 21 weeks of gestation presented with persistent, drug-resistant fronto-parietal headache, with subsequent progressive development of psychomotor lentification and inappropriate behavior. Physical examination was normal, as were routine laboratory parameters and CT findings, and these symptoms were initially interpreted in the context of chronic depression. Later, the patient developed generalized dystonia and fever, with rapid clinical deterioration, depression of consciousness and, eventually, progression to coma. This case emphasizes the complexity and challenges involved in the diagnostic approach to a patient with progressive subacute encephalopathy framed by worsening CNS symptoms. It highlights the clinical considerations and complementary investigation of various etiologies, in a step-by-step approach, ultimately leading to the final diagnosis. Early recognition and appropriate treatment of these conditions can lead to more favorable outcomes, particularly in gestating patients, where prompt intervention is crucial, and where critical decisions may have to be made regarding pregnancy and the safety of treatment options.

摘要

进行性亚急性脑病相关的广泛鉴别诊断可能令人望而生畏,尤其是在年轻的孕妇中。在本例中,一位 24 岁、妊娠 21 周的女性出现持续性、耐药性额顶头痛,随后逐渐出现精神运动迟缓及不当行为。体格检查正常,常规实验室参数和 CT 结果也正常,这些症状最初被解释为慢性抑郁症。后来,患者出现全身肌张力障碍和发热,病情迅速恶化,意识障碍,最终进展为昏迷。本病例强调了以逐渐加重的中枢神经系统症状为特征的进行性亚急性脑病患者的诊断方法的复杂性和挑战。它突出了在逐步的方法中对各种病因的临床考虑和补充性检查,最终导致最终诊断。早期识别和适当治疗这些疾病可以带来更好的结果,尤其是在妊娠患者中,及时干预至关重要,并且可能必须就妊娠和治疗选择的安全性做出关键决策。

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本文引用的文献

1
Susac syndrome: challenges in the diagnosis and treatment.Susac综合征:诊断与治疗中的挑战
Brain. 2022 Apr 29;145(3):858-871. doi: 10.1093/brain/awab476.
2
Susac syndrome and pregnancy: a review of published cases and considerations for patient management.Susac综合征与妊娠:已发表病例综述及患者管理考量
Ther Adv Neurol Disord. 2021 Jan 30;14:1756286420981352. doi: 10.1177/1756286420981352. eCollection 2021.
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Guidelines for treatment of Susac syndrome - An update.Susac 综合征治疗指南——更新版。
Int J Stroke. 2020 Jul;15(5):484-494. doi: 10.1177/1747493017751737. Epub 2018 Jan 10.
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Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study.Susac 综合征的临床、辅助检查和血清学表现:一项国际多中心研究。
J Neuroinflammation. 2014 Mar 8;11:46. doi: 10.1186/1742-2094-11-46.
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Characteristics of Susac syndrome: a review of all reported cases.Susac 综合征的特征:所有报道病例的回顾。
Nat Rev Neurol. 2013 Jun;9(6):307-16. doi: 10.1038/nrneurol.2013.82. Epub 2013 Apr 30.
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MRI findings in Susac's syndrome.苏萨克综合征的磁共振成像表现。
Neurology. 2003 Dec 23;61(12):1783-7. doi: 10.1212/01.wnl.0000103880.29693.48.