Nardone Raffaele, Höller Yvonne, Storti Monica, Lochner Piergiorgio, Tezzon Frediano, Golaszewski Stefan, Brigo Francesco, Trinka Eugen
Raffaele Nardone, Yvonne Höller, Stefan Golaszewski, Eugen Trinka, Department of Neurology, Christian Doppler Klinik, Paracelsus Medical University, 5020 Salzburg, Austria.
World J Gastroenterol. 2014 Mar 14;20(10):2578-85. doi: 10.3748/wjg.v20.i10.2578.
A severe spinal cord involvement may rarely occur in patients with cirrhosis and other chronic liver diseases; this complication is usually associated with overt liver failure and surgical or spontaneous porto-systemic shunt. Hepatic myelopathy (HM) is characterized by progressive weakness and spasticity of the lower extremities, while sensory and sphincter disturbances have rarely been described and are usually less important. The diagnosis is assigned in the appropriate clinical setting on clinical grounds after the exclusion of other clinical entities leading to spastic paraparesis. Magnetic resonance imaging is often unremarkable; however, also intracerebral corticospinal tract abnormalities have been reported recently. The study of motor evoked potentials may disclose central conduction abnormalities even before HM is clinically manifest. HM responds poorly to blood ammonia-lowering and other conservative medical therapy. Liver transplantation represents a potentially definitive treatment for HM in patients with decompensated cirrhosis of Child-Pugh B and C grades. Other surgical treatment options in HM include surgical ligation, shunt reduction, or occlusion by interventional procedures.
肝硬化和其他慢性肝病患者很少会出现严重的脊髓受累情况;这种并发症通常与明显的肝衰竭以及手术或自发性门体分流有关。肝性脊髓病(HM)的特征是下肢进行性无力和痉挛,而感觉和括约肌功能障碍很少被描述且通常不太严重。在排除其他导致痉挛性截瘫的临床病症后,根据临床情况在适当的临床背景下做出诊断。磁共振成像通常无明显异常;然而,最近也有关于脑内皮质脊髓束异常的报道。运动诱发电位研究甚至在HM出现临床症状之前就可能发现中枢传导异常。HM对降低血氨及其他保守药物治疗反应不佳。对于Child-Pugh B级和C级失代偿性肝硬化患者,肝移植是治疗HM的一种可能的确定性方法。HM的其他手术治疗选择包括手术结扎、分流减少或通过介入程序进行封堵。