Link A, Kaplan B T, Böhm M
Klinik für Innere Medizin III, Universität des Saarlandes, Homburg/Saar, Germany.
Dtsch Med Wochenschr. 2012 Sep;137(38):1853-6. doi: 10.1055/s-0032-1305311. Epub 2012 Sep 12.
A 21-year old woman was referred to the hospital because of progressively deteriorating consciousness. Initially agitation and irritability, later confusion and delirium predominated. Previously influenza with high temperature, headache and vomiting had occurred and been treated with acetylsalicylic acid for some days. Non preliminary diseases were reported. CLINICAL INVESTIGATION: Besides of the deterioration in consciousness no clinical or neurological abnormalities were found. Electroencephalography demonstrated general abnormalities of medium range. Cranial magnetic resonance imaging and liquor investigations showed no pathological findings. In laboratory tests a marked increase of transaminases and ammonia were found. The toxicological screening was normal.
A hepatic encephalopathy due to acute hepatic failure was diagnosed and a detoxication of ammonemia with lactulose was started immediately. Transjugular liver biopsy showed a marked fattening of liver tissue without serological or histological findings for a reason. Because of progressive deterioration in consciousness mechanical ventilation became necessary. Despite of a rapid decline of ammonium serum levels further neurological deterioration occurred. Cranial computed tomography showed oedema of the cerebrum with beginning cerebral occlusion. Despite immediate therapy of cerebral oedema the patient died because of secondary cerebral oedema in hepatic encephalopathy.
Reye's syndrome is an acute illness characterized by hepatic encephalopathy and fatty degeneration of the liver. It often occurs after viral infections. After a viral infection that was eventually treated with acetylsalicylic acid Reye's syndrome has to be discussed after exclusion of common causes of hepatic failure.
一名21岁女性因意识逐渐恶化被转诊至医院。起初表现为烦躁和易怒，随后以意识模糊和谵妄为主。此前曾患流感，伴有高热、头痛和呕吐，曾用乙酰水杨酸治疗数日。未报告有基础疾病。
除意识恶化外，未发现临床或神经学异常。脑电图显示中度广泛性异常。头颅磁共振成像和脑脊液检查未发现病理结果。实验室检查发现转氨酶和氨显著升高。毒理学筛查正常。
诊断为急性肝衰竭所致肝性脑病，立即开始用乳果糖进行氨血症解毒治疗。经颈静脉肝活检显示肝组织明显脂肪变性，但未发现血清学或组织学病因。由于意识进行性恶化，需要进行机械通气。尽管血清氨水平迅速下降，但仍出现了进一步的神经功能恶化。头颅计算机断层扫描显示大脑水肿并伴有早期脑梗死。尽管立即对脑水肿进行了治疗，但患者仍因肝性脑病继发脑水肿而死亡。
瑞氏综合征是一种以肝性脑病和肝脏脂肪变性为特征的急性疾病。它常发生在病毒感染后。在病毒感染最终用乙酰水杨酸治疗后，排除肝衰竭常见病因后必须考虑瑞氏综合征。
