Koo Ja Eun, Lim Young-Suk, Myung Sun Jeong, Suh Kyung Suk, Kim Kang Mo, Lee Han Chu, Chung Young-Hwa, Lee Yung Sang, Suh Dong Jin
Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Hepatol. 2008 Mar;14(1):89-96. doi: 10.3350/kjhep.2008.14.1.89.
Hepatic myelopathy is a rare complication of chronic liver disease that is associated with extensive portosystemic shunts. The main clinical feature of hepatic myelopathy is progressive spastic paraparesis in the absence of sensory or sphincter impairment. Early and accurate diagnosis of hepatic myelopathy is important because patients with early stages of the disease can fully recover following liver transplantation. Motor-evoked potential studies may be suitable for the early diagnosis of hepatic myelopathy, even in patients with preclinical stages of the disease. Here we describe two patients who presented with spastic paraparesis associated with a spontaneous splenorenal shunt and without any previous episode of hepatic encephalopathy. One patient experienced improved neurologic symptoms after liver transplantation, whereas the other patient only received medical treatment, which did not prevent the progression of spastic paraparesis.
肝性脊髓病是慢性肝病的一种罕见并发症,与广泛的门体分流有关。肝性脊髓病的主要临床特征是在无感觉或括约肌功能障碍的情况下出现进行性痉挛性截瘫。肝性脊髓病的早期准确诊断很重要,因为疾病早期的患者在肝移植后可完全康复。运动诱发电位研究可能适用于肝性脊髓病的早期诊断,即使是处于疾病临床前期的患者。在此,我们描述两名表现为与自发性脾肾分流相关的痉挛性截瘫且既往无肝性脑病发作的患者。一名患者在肝移植后神经症状改善,而另一名患者仅接受了药物治疗,这未能阻止痉挛性截瘫的进展。