肝性脊髓病合并肝性脑病13例临床影像特点与预后

Clinical and neuroimaging characteristics and prognosis of patients with hepatic myelopathy and hepatic encephalopathy

目的探讨肝性脊髓病合并肝性脑病的发病机制、临床表现、影像特点、治疗和预后。方法回顾性分析13例肝性脊髓病合并肝性脑病患者的临床表现、影像特点及预后,并结合文献进行分析。结果本病多发生于肝硬化自发性或手术门体分流后,临床主要表现可逆的脑病症状,进行性以双下肢为主的痉挛性瘫痪,仅1例有括约肌功能障碍,无肌萎缩、感觉障碍。9例血氨增高,9例脑电图表现弥漫性θ波,其中2例有三相波,8例MRIT1像显示基底神经节高信号,脊髓MRI阳性率低。所有患者均限制蛋白摄入量,给予护肝、降血氨、神经营养等治疗,3例加用糖皮质激素。9例患者脑病症状缓解,但瘫痪改善不明显;死亡3例,仅1例肝移植患者预后良好。结论脊髓症状与脑病症状并不平行消长,可能存在不同的发病机制。神经影像学研究为慢性肝病神经系统并发症的诊断提供了新思路。常规治疗脑病症状好转,但脊髓病变预后不良,肝移植为慢性肝病神经系统并发症带来曙光。

Objective To analyze the clinical and neuroimaging characteristics and prognosis of patients with hepatic myelopathy and hepatic encephalopathy. Methods Clinical data of 13 cases with hepatic myelopathy and hepatic encephalopathy were retrospectively reviewed. Results All patients had chronic liver disease and 11 developed extensive porto-systemic shunting due to surgical porto-caval shunting or spontaneous shunting through collateral vessels. The major clinical characteristics were reversible encephalopathy symptoms,disabling progressive spastic paraparesis （most in the legs,only one with quadriplegia）,no sphincter involvements except the patient with quadriplegia,and no sensory disturbances. The blood ammonia level increased in 9 patients. T1 weighted cranial MR imaging in 8 cases showed bilateral basal ganglia high signal intensity. MRI of the spine did not reveal any abnormal findings except 2 cases. Electroencephalogram （EEG） of 8 patients showed diffuse θ waves,2 of whom had triphasic wave. Therapy protocol included protein restriction,branched-chain amino acid,acetylglutamide,oral neomycin,lactulose,neurotrophy and colonic exclusion,3 with glucocorticoid. One patient experienced improved neurological symptoms after liver transplantation,whereas three patients died and the other patients who only received medical treatments gained partial improvements of encephalopathy symptoms but not prevent the progression of spastic paraparesis. Conclusion Hepatic myelopathy was not parallel with hepatic encephalopathy. The pathophysiology may be different between hepatic myelopathy and hepatic encephalopathy. Neuroimaging techniques cast new lights on the diagnosis of neurological syndromes in patients with liver failure. Those medical treatments decreased the numbers of encephalopathic episodes but can not improve the poor prognosis of spastic paraparesis. Liver transplantation may be a promising method to improve the neurological syndromes of liver failure.