Departement of Neurology, CHU Fattouma Bourguiba, Fattouma Bourguiba Hospital, Avenue 1Er Juin. 5000, Monastir, Tunisia.
Departement of Gastro-Enterology, CHU Fattouma Bourguiba, Monastir, Tunisia.
J Med Case Rep. 2024 Jun 17;18(1):281. doi: 10.1186/s13256-024-04495-2.
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.
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.
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.
肝性脊髓病是慢性肝病的一种罕见神经系统并发症。患者通常表现为进行性纯运动痉挛性截瘫。这种神经功能障碍几乎总是由于肝硬化和门腔分流引起的，无论是手术还是自发性的。
我们报告了两例 57 岁男性和 37 岁女性，他们均因肝硬化和门静脉高压导致进行性痉挛性截瘫。这两名患者均来自突尼斯（北非）。两名患者的脊髓磁共振成像正常，而大脑磁共振成像显示苍白球的 T2 高信号。这些迹象表明，两名肝硬化患者伴有孤立性进行性痉挛性截瘫而无膀胱或感觉障碍的肝性脑病有助于保留肝性脊髓病的诊断。
肝性脊髓病是慢性肝病的一种严重且使人虚弱的神经系统并发症。其发病机制尚未完全阐明，似乎是多因素的，包括氨和其他致病神经毒素的选择性神经毒性作用。通常，有记录显示患者的大脑磁共振成像显示肝性脑病，而脊髓磁共振成像正常，这有助于诊断肝性脊髓病。降低氨的措施、饮食补充、抗痉挛药物和血管内分流闭塞等保守治疗在改善疾病症状方面收效甚微。早期进行肝移植可以预防疾病进展，可能使病情得到恢复。
