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Abstract：
BACKGROUND: Hepatic myelopathy is a very rare neurological complication of chronic liver disease. Patients habitually present with progressive pure motor spastic paraparesis. This neurological dysfunction is almost always due to cirrhosis and portocaval shunt, either surgical or spontaneous.
METHODS: We report two cases of a 57-year-old man and a 37-year-old woman with progressive spastic paraparesis linked to cirrhosis and portal hypertension. The two patients are of Tunisian origin (north Africa). Magnetic resonance imaging of the spinal cord of two patients was normal, while brain magnetic resonance imaging showed a T2 hypersignals of the pallidums. These signs, in favor of hepatic encephalopathy in the two patients with cirrhosis with isolated progressive spastic paraparesis without bladder or sensory disorders, help to retain the diagnosis of hepatic myelopathy.
CONCLUSIONS: Hepatic myelopathy is a severe and debilitating neurological complication of chronic liver disease. The pathogenesis is misunderstood and seems to be multifactorial, including the selective neurotoxic role both of ammonia and other pathogenic neurotoxins. Usually a pathological brain magnetic resonance imaging showing a hepatic encephalopathy was documented, contrasting with a normal spinal cord magnetic resonance imaging that contributed to diagnosis of hepatic myelopathy. Conservative therapies such as ammonia-lowering measures, diet supplementation, antispastic drugs, and endovascular shunt occlusion show little benefit in improving disease symptoms. Liver transplantation performed at early stage can prevent disease progression and could probably allow for recovery.
METHODS: We report two cases of a 57-year-old man and a 37-year-old woman with progressive spastic paraparesis linked to cirrhosis and portal hypertension. The two patients are of Tunisian origin (north Africa). Magnetic resonance imaging of the spinal cord of two patients was normal, while brain magnetic resonance imaging showed a T2 hypersignals of the pallidums. These signs, in favor of hepatic encephalopathy in the two patients with cirrhosis with isolated progressive spastic paraparesis without bladder or sensory disorders, help to retain the diagnosis of hepatic myelopathy.
CONCLUSIONS: Hepatic myelopathy is a severe and debilitating neurological complication of chronic liver disease. The pathogenesis is misunderstood and seems to be multifactorial, including the selective neurotoxic role both of ammonia and other pathogenic neurotoxins. Usually a pathological brain magnetic resonance imaging showing a hepatic encephalopathy was documented, contrasting with a normal spinal cord magnetic resonance imaging that contributed to diagnosis of hepatic myelopathy. Conservative therapies such as ammonia-lowering measures, diet supplementation, antispastic drugs, and endovascular shunt occlusion show little benefit in improving disease symptoms. Liver transplantation performed at early stage can prevent disease progression and could probably allow for recovery.
摘要：
背景：肝性脊髓病是一种非常罕见的慢性肝病的神经系统并发症。患者习惯性出现进行性纯运动性痉挛性轻瘫。这种神经功能障碍几乎总是由于肝硬化和门腔分流，手术或自发。
方法：我们报告了两例57岁的男性和37岁的女性患者，他们患有与肝硬化和门脉高压相关的进行性痉挛性轻瘫。这两名患者来自突尼斯（北非）。两名患者的脊髓磁共振成像正常，而脑磁共振成像显示苍白球的T2超信号。这些迹象,支持肝性脑病的两名肝硬化患者伴有孤立性进行性痉挛性轻瘫，无膀胱或感觉障碍，有助于保留肝性脊髓病的诊断。
结论：肝性脊髓病是慢性肝病的一种严重且使人衰弱的神经系统并发症。发病机制被误解，似乎是多因素的，包括氨和其他致病性神经毒素的选择性神经毒性作用。通常有病理性脑磁共振成像显示肝性脑病，与有助于诊断肝性脊髓病的正常脊髓磁共振成像相反。保守治疗如降氨措施，饮食补充，抗痉挛药物,和血管内分流闭塞在改善疾病症状方面几乎没有益处。早期进行的肝移植可以防止疾病进展，并可能允许恢复。
方法：我们报告了两例57岁的男性和37岁的女性患者，他们患有与肝硬化和门脉高压相关的进行性痉挛性轻瘫。这两名患者来自突尼斯（北非）。两名患者的脊髓磁共振成像正常，而脑磁共振成像显示苍白球的T2超信号。这些迹象,支持肝性脑病的两名肝硬化患者伴有孤立性进行性痉挛性轻瘫，无膀胱或感觉障碍，有助于保留肝性脊髓病的诊断。
结论：肝性脊髓病是慢性肝病的一种严重且使人衰弱的神经系统并发症。发病机制被误解，似乎是多因素的，包括氨和其他致病性神经毒素的选择性神经毒性作用。通常有病理性脑磁共振成像显示肝性脑病，与有助于诊断肝性脊髓病的正常脊髓磁共振成像相反。保守治疗如降氨措施，饮食补充，抗痉挛药物,和血管内分流闭塞在改善疾病症状方面几乎没有益处。早期进行的肝移植可以防止疾病进展，并可能允许恢复。
