Unit of Endocrinology and Internal Medicine, University of Pavia, ICS Maugeri SPA Società Benefit, Pavia 27100, Italy.
Unit of Interventional Radiology, Radiology Department, IRCCS Fondazione Policlinico San Matteo, Pavia 27100, Italy.
World J Gastroenterol. 2017 Dec 21;23(47):8426-8431. doi: 10.3748/wjg.v23.i47.8426.
Hepatic encephalopathy is suspected in non-cirrhotic cases of encephalopathy because the symptoms are accompanied by hyperammonaemia. Some cases have been misdiagnosed as psychiatric diseases and consequently patients hospitalized in psychiatric institutions or geriatric facilities. Therefore, the importance of accurate diagnosis of this disease should be strongly emphasized. A 68-year-old female patient presented to the Emergency Room with confusion, lethargy, nausea and vomiting. Examination disclosed normal vital signs. Neurological examination revealed a minimally responsive woman without apparent focal deficits and normal reflexes. She had no history of hematologic disorders or alcohol abuse. Her brain TC did not demonstrate any intracranial abnormalities and electroencephalography did not reveal any subclinical epileptiform discharges. Her ammonia level was > 400 mg/dL (reference range < 75 mg/dL) while hepatitis viral markers were negative. The patient was started on lactulose, rifaximin and low-protein diet. On the basis of the doppler ultrasound and abdomen computed tomography angiography findings, the decision was made to attempt portal venography which confirmed the presence of a giant portal-systemic venous shunt. Therefore, mechanic obliteration of shunt by interventional radiology was performed. As a consequence, mesenteric venous blood returned to hepatopetally flow into the liver, metabolic detoxification of ammonia increased and hepatic encephalopathy subsided. It is crucial that physicians immediately recognize the presence of non-cirrhotic encephalopathy, in view of the potential therapeutic resolution after accurate diagnosis and appropriate treatments.
疑诊非肝硬化性脑病患者存在肝性脑病,因为其症状伴有血氨升高。部分病例误诊为精神疾病,进而导致患者住院于精神科或老年科。因此,应强烈强调准确诊断该病的重要性。一名 68 岁女性患者因意识模糊、嗜睡、恶心和呕吐到急诊就诊。检查显示生命体征正常。神经系统检查显示患者为反应迟钝的女性,无明显局灶性缺损和正常反射。她无血液系统疾病或酒精滥用史。脑部 CT 未显示任何颅内异常,脑电图未显示任何亚临床癫痫样放电。她的血氨水平>400mg/dL(参考范围<75mg/dL),而肝炎病毒标志物为阴性。给予乳果糖、利福昔明和低蛋白饮食。根据多普勒超声和腹部 CT 血管造影结果,决定尝试门静脉造影,该检查证实存在巨大门-体静脉分流。因此,通过介入放射学进行了分流机械闭塞。肠系膜静脉血因此改为经肝向心性回流进入肝脏,氨的代谢解毒增加,肝性脑病缓解。鉴于准确诊断和适当治疗后可能具有治疗缓解作用,医生应立即识别非肝硬化性脑病的存在。