Tazawa Ko-ichi, Shimojima Yasuhiro, Okano Tomomi, Yazaki Masahide, Takei Yo-ichi, Shimojo Hisashi, Kobayashi Keiko, Saheki Takeyori, Ikeda Shu-ichi
Department of Internal Medicine (Neurology), Shinshu University School of Medicine 3-1-1 Asahi, Matsumoto 390-8621, Japan.
J Neurol Sci. 2007 Feb 15;253(1-2):77-80. doi: 10.1016/j.jns.2006.11.014. Epub 2007 Jan 2.
Hepatic myelopathy is a rare neurological complication in patients with chronic liver failure and most patients who suffered from this disorder were demonstrated to have portal-systemic shunt. A 31-year-old man who was diagnosed as having adult-onset type II citrullinemia (CTLN2) and had a six-year history of recurrent hepatic encephalopathy showed progressive spastic paraparesis with no involvement of sensation and sphincter function. Examinations of cerebrospinal fluid and spinal MRI were normal. He suddenly died of acute exacerbation of hepatic encephalopathy with severe brain edema. The pathology of the spinal cord disclosed a localized degeneration of both lateral columns, the lesion being more remarkable in the lower segments of the cord. These clinical and pathological findings of hepatic myelopathy have not been noted in the many patients with CTLN2 previously reported, and our patient is unique in developing hepatic myelopathy without porto-caval shunting. Thus, repeated attacks of encephalopathy with hyperammonemia might secondarily have induced the myelopathy in this patient.
肝性脊髓病是慢性肝衰竭患者中一种罕见的神经并发症，大多数患有这种疾病的患者被证实存在门体分流。一名31岁男性被诊断为成人发作型II型瓜氨酸血症（CTLN2），有六年复发性肝性脑病病史，表现为进行性痉挛性截瘫，感觉和括约肌功能未受累。脑脊液检查和脊髓磁共振成像均正常。他突然死于肝性脑病急性加重伴严重脑水肿。脊髓病理学检查显示双侧侧柱局限性变性，病变在脊髓下段更明显。先前报道的许多CTLN2患者中尚未发现肝性脊髓病的这些临床和病理表现，我们的患者在未发生门腔分流的情况下发生肝性脊髓病是独特的。因此，高氨血症导致的反复脑病发作可能继发引起了该患者的脊髓病。
