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[1例子痫前期患者急诊剖宫产术后被诊断为伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)]

[A Case of Preeclampsia Diagnosed with CADASIL after Emergency Cesarean Section].

作者信息

Kaneko Haruka, Kato Rie, Okutomi Toshiyuki, Okamoto Hirotsugu

出版信息

Masui. 2016 Oct;65(10):1043-1047.

PMID:30358285
Abstract

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a rare inherited disease which begins with migraine and later develops repeated cerebral subcortical infarction and dementia. We present an anesthetic experience of an undiagnosed CADASIL woman complicated with preeclampsia. She developed headache, slurred speech, cognitive dysfunction and restlessness at 35 weeks' gestation and was diagnosed as hypertensive encepha- lopathy. Urgent cesarean section was decided. After ruling out meningitis by physical examination, and intracranial hemorrhage, cerebral swelling and hydro- cephalus by brain CT, spinal anesthesia was chosen. Mild sedation was necessary because the patient became restless and uncooperative during surgery. The anesthetic course was uneventful otherwise. She was either restless or lethargy and had hallucinatory episodes on 1st post-operative day. The neurologist suspected CADASIL because of multiple lacunar infarct lesions on MRI and her family history. The diagnosis was confirmed by skin biopsy and a genetic test.

摘要

伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)是一种罕见的遗传性疾病,始于偏头痛,随后发展为反复的脑皮质下梗死和痴呆。我们介绍了一位未确诊的CADASIL女性并发先兆子痫的麻醉经验。她在妊娠35周时出现头痛、言语不清、认知功能障碍和烦躁不安,被诊断为高血压脑病。决定紧急剖宫产。通过体格检查排除脑膜炎,通过脑部CT排除颅内出血、脑肿胀和脑积水后,选择了脊髓麻醉。由于患者在手术过程中变得烦躁不安且不配合,需要轻度镇静。否则麻醉过程顺利。术后第1天,她要么烦躁不安,要么嗜睡,并出现幻觉发作。由于MRI上有多个腔隙性梗死灶以及她的家族病史,神经科医生怀疑为CADASIL。皮肤活检和基因检测确诊了该诊断。

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Masui. 2016 Oct;65(10):1043-1047.
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Lacunar infarcts are the main correlate with cognitive dysfunction in CADASIL.腔隙性脑梗死是伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)中认知功能障碍的主要相关因素。
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CADASIL: MRI may be normal in the fourth decade of life - a case report.伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL):在生命的第四个十年,磁共振成像(MRI)可能正常——病例报告
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[Case of CADASIL showing spontaneous subcortical hemorrhage with a novel mutation of Notch3 gene].[伴有Notch3基因新突变的大脑常染色体显性动脉病伴皮质下梗死和白质脑病(CADASIL)自发性皮质下出血病例]
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