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一名被诊断患有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)患者的麻醉管理。

Anesthetic management of a patient diagnosed with CADASIL (cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy).

作者信息

Errando C L, Navarro L, Vila M, Pallardó M A

机构信息

Servicio de Anestesiología, Reanimación y Tratamiento del Dolor, Consorcio Hospital General Universitario de Valencia, Valencia, España.

出版信息

Rev Esp Anestesiol Reanim. 2012 Feb;59(2):102-6. doi: 10.1016/j.redar.2012.02.021.

DOI:10.1016/j.redar.2012.02.021
PMID:22480557
Abstract

CADASIL (cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leu-koencephalopathy) is an infrequent inherited disease that could have anesthetic implica-tions. However these have rarely been reported. We present a male patient previously diagnosed with CADASIL, who had suffered an ischemic vascular cerebral accident with a MRI compatible with leukoencephalopathy, and who was dependent for daily activities, and sustained dementia, mood alterations, apathy, and urine incontinence. He had famil-ial antecedents of psychiatric symptoms and ischemic stroke events in several relatives including his father, two brothers and one sister. He was scheduled for arthrodesis of the left knee because of multiple infectious complications of prosthetic knee surgery. He was under clopidogrel treatment which was withdrawn seven days before surgery. The pro-cedure was performed under combined spinal-epidural anesthesia, intraoperative seda-tion with midazolam, and postoperative multimodal analgesia including epidural patient controlled analgesia. The perioperative management was uneventful and we outline the adequacy of managing these patients under regional anesthesia and analgesia, as these permit to maintain hemodynamic stability leading to adequate cerebral perfusion, key to avoid an increase in the effects of the chronic arteriopathy patients with CADASIL sustain.

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,可能会对麻醉产生影响。然而,相关报道很少。我们报告一例先前被诊断为CADASIL的男性患者,他曾发生过缺血性脑血管意外,其磁共振成像(MRI)结果符合白质脑病表现,日常生活需要依赖他人,且患有痴呆、情绪改变、淡漠和尿失禁。他有家族精神症状及缺血性卒中病史,其父亲、两个兄弟和一个姐妹等多名亲属均有此类情况。由于人工膝关节手术出现多种感染并发症,他计划接受左膝关节融合术。他正在接受氯吡格雷治疗,术前7天停药。手术在腰麻-硬膜外联合麻醉下进行,术中使用咪达唑仑镇静,术后采用多模式镇痛,包括硬膜外患者自控镇痛。围手术期管理顺利,我们概述了在区域麻醉和镇痛下管理这些患者的适宜性,因为这有助于维持血流动力学稳定,从而实现充足的脑灌注,这是避免加重CADASIL慢性动脉病患者病情的关键。

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