Pinarbasi Binnur, Kaymakoglu Sabahattin, Matur Zeliha, Akyuz Filiz, Demir Kadir, Besisik Fatih, Ozdil Sadakat, Boztas Gungor, Cakaloglu Yilmaz, Mungan Zeynel, Okten Atilla
Department of Gastroenterohepatology, Medical Faculty, Istanbul University, Istanbul, Turkey.
J Clin Gastroenterol. 2009 Feb;43(2):176-81. doi: 10.1097/MCG.0b013e318150d399.
Acquired hepatocerebral degeneration (AHD) and hepatic myelopathy (HM) are rare complications of chronic liver disease and are usually resistant to medical therapy.
The clinical and laboratory findings of 14 male and 2 female patients with AHD or HM were evaluated.
The prevalence of AHD and HM was 2% inpatient case series in the last 10 years. The median age of the patients (5 Child's B and 11 Child's C) was 48.7 years (28 to 66 y), and the mean known duration of the liver disease was 75 months (24 to 194 mo). The median time of onset of neurologic findings after diagnosis of the liver disease was 14.5 months. Eight patients who had marked spastic paraparesis or tetraparesis were included in the HM group and all others had AHD group. Sixty-nine percent of the patients had a spontaneous or surgical portosystemic shunts, and the remaining dense retroperitoneal collaterals. During the follow-up period of median 29 months (4 to 72 mo), 12 patients died while waiting for liver transplantation, and these patients suffered from the several complications of chronic liver disease more than the living patients. A marked improvement was observed in 2 of the patients (1 with AHD and the other with HM) at 6 and 8 months after the liver transplantation, respectively.
Our data suggest that liver transplantation had an important effect on the improvement in these patients.
获得性肝性脑病(AHD)和肝性脊髓病(HM)是慢性肝病的罕见并发症,通常对药物治疗耐药。
对14例男性和2例女性AHD或HM患者的临床和实验室检查结果进行评估。
在过去10年的住院病例系列中,AHD和HM的患病率为2%。患者的中位年龄(5例Child's B级和11例Child's C级)为48.7岁(28至66岁),已知肝病的平均病程为75个月(24至194个月)。肝病诊断后神经学表现的中位发病时间为14.5个月。8例有明显痉挛性截瘫或四肢瘫的患者被纳入HM组,其他所有患者被纳入AHD组。69%的患者有自发性或手术性门体分流,其余有密集的腹膜后侧支循环。在中位29个月(4至72个月)的随访期内,12例患者在等待肝移植期间死亡,这些患者比存活患者更多地患有慢性肝病的几种并发症。分别在肝移植后6个月和8个月时,2例患者(1例AHD和1例HM)有明显改善。
我们的数据表明肝移植对这些患者的改善有重要作用。