Li Sisi, Chen Jiani, He Yongqiao, Deng Yongyi, Chen Jie, Fang Wenyu, Zeren Zhamu, Liu Yadong, Abdulaziz Ammar Taha Abdullah, Yan Bo, Zhou Dong
Department of Neurology, West China Hospital, Sichuan University, Chengdu, China.
Department of Neurology, Ganzi Tibetan Autonomous Prefecture People's Hospital, Kangding, China.
Front Neurol. 2021 Jan 15;11:537565. doi: 10.3389/fneur.2020.537565. eCollection 2020.
Intracranial alveolar echinococcosis (IAE), a zoonotic disease, is a critical health problem in the Tibetan region. We aimed to describe the clinical and radiological characteristics and outcomes among patients with IAE. We screened patients diagnosed with IAE between March 2015 and May 2019 at the Ganzi Tibetan Autonomous Prefecture People's Hospital. Detailed demographics, clinical characteristics, neuroimaging features, and outcomes were recorded. A total of 21 patients with an average age of 44.1 ± 12.7 years were included. Thirteen (61.9%) patients were male. The most common chief neurological complaint was headache ( = 17, 81.0%), followed by dizziness, seizure, visual disturbances, hemiparesis, disturbed consciousness, and dysphasia. All the patients had coexisting liver localizations. The typical neuroimaging features of IAE on cerebral magnetic resonance imaging scans showed obvious low-signal shadow with multiple small vesicles inside the lesions on T2-weighted images and FLAIR images. The pathological HE staining demonstrates vesicular lesions with several internal sacs. For hepatic alveolar echinococcosis (AE), the hepatic portal was invaded in six (28.6%) patients, and the portal vein ( = 5, 23.8%) was the mostly commonly involved vessel. As for treatment, 11 patients (52.4%) had poor compliance with albendazole. The duration of patients taken albendazole ranged from 2 months to 3 years. Cerebral AE surgery was performed in 11 patients, five of them underwent partial resection of AE lesions, and six patients received total resection. One patient with primary IAE underwent radical surgery. Ten patients (47.6%) died during the follow-up for a mean of 21.7 ± 11.9 (3-46) months. In total, 28.9% of the patients died within 5 years, and 71.6% died within 10 years. The median interval between the date of diagnosis as AE and death was 84 (19-144) months. Despite substantial advances in diagnostic and therapeutic methods, the treatment of IAE remains difficult and results in unsatisfactory outcomes. The major critical issue is surgical treatment of IAE although the disease is disseminated. Besides, lifelong albendazole would be indicated, but most patients had poor medication compliance. It is important to educate patients about the necessity of medical treatment.
颅内泡型包虫病（IAE）是一种人畜共患疾病，是西藏地区一个严重的健康问题。我们旨在描述IAE患者的临床和放射学特征及预后。我们筛查了2015年3月至2019年5月期间在甘孜藏族自治州人民医院被诊断为IAE的患者。记录了详细的人口统计学信息、临床特征、神经影像学特征及预后情况。共纳入21例患者，平均年龄为44.1±12.7岁。其中13例（61.9%）为男性。最常见的主要神经症状是头痛（n = 17，81.0%），其次是头晕、癫痫发作、视觉障碍、偏瘫、意识障碍和言语困难。所有患者均合并肝脏病变。IAE在脑磁共振成像扫描中的典型神经影像学特征显示，在T2加权像和液体衰减反转恢复（FLAIR）像上，病变内有多个小囊泡的明显低信号影。病理苏木精-伊红（HE）染色显示有含多个内囊的囊泡状病变。对于肝泡型包虫病（AE），6例（28.6%）患者的肝门受到侵犯，门静脉（n = 5，23.8%）是最常受累的血管。至于治疗，11例患者（52.4%）对阿苯达唑的依从性较差。服用阿苯达唑的时间为2个月至3年。11例患者接受了脑AE手术，其中5例患者接受了AE病变部分切除术，6例患者接受了全切除术。1例原发性IAE患者接受了根治性手术。10例患者（47.6%）在平均21.7±11.9（3 - 46）个月的随访期间死亡。总的来说，28.9%的患者在5年内死亡，71.6%的患者在10年内死亡。从被诊断为AE到死亡的中位间隔时间为84（19 - 144）个月。尽管在诊断和治疗方法上取得了重大进展，但IAE的治疗仍然困难，预后不理想。主要关键问题是IAE的外科治疗，尽管该病已扩散。此外，应使用阿苯达唑进行终身治疗，但大多数患者的药物依从性较差。对患者进行关于药物治疗必要性的教育很重要。
