Clinical features of hepatic myelopathy: An analysis of 562 cases / 临床肝胆病杂志

ObjectiveTo investigate the clinical features of patients with hepatic myelopathy (HM) in China. MethodsThe articles on HM, published in China from January 2009 to December 2018, were collected to analyze the clinical features, laboratory examination results, diagnosis, treatment, and prognosis of HM patients. ResultsA total of 94 articles were included, with 562 patients in total, among whom there were 489 male patients and 73 female patients. Their age ranged from 17-81 years, with a mean age of 46.3±17.5 years. Hepatitis B cirrhosis was the most common etiology (64.4%), followed by alcoholic cirrhosis (10.3%) and hepatitis C cirrhosis (9.2%). The clinical manifestations of HM mainly included decline of muscle strength (89.50%), tendon hyperreflexia (76.87%), ataxia, and movement disorder (76.51%). Laboratory examination showed an increase in blood ammonia by 92.31% and a reduction in albumin by 88.96%. Imaging diagnosis mainly depended on electromyography (64.92%) and spinal cord MRI (22.82%), and abdominal color Doppler ultrasound, CT, or MRI alone has limited clinical value. After comprehensive medical treatment, no patient (0%) was cured, 106 patients (18.86%) were improved, 211 patients (37.54%) had no response, 31 patients (5.52%) worsened, 129 patients (22.95%) died, and 53 patients (9.43%) were not explained. A total of 39 patients underwent liver transplantation, with an improvement rate of 5641%. ConclusionHM is a rare complication of end-stage liver disease and is mainly observed in middle-aged men. It has the main manifestation of chronic and progressive spastic paraplegia of both lower limbs. Currently, there is no effective treatment method, and liver transplantation is feasible for some patients, with poor treatment response and poor prognosis.

Hepatic myelopathy after transjugular intrahepatic portosystemic shunt: natural course, survival analysis, and treatment / 临床肝胆病杂志

ObjectiveTo investigate the features of hepatic myelopathy (HM) after transjugular intrahepatic portosystemic shunt (TIPS). MethodsA retrospective analysis was performed for a consecutive cohort of patients with cirrhotic portal hypertension who were successfully treated with TIPS in Department of Liver Disease and Digestive Interventional Radiology in Xijing Hospital of Digestive Diseases, Fourth Military Medical University, from 2005 to 2014 and then developed HM. Routine follow-up was performed for all patients at 1, 3, and 6 months after TIPS and every half a year subsequently. The time to disease progression was used to summarize the natural course of HM, the comparison of continuous data was made by independent samples t-test, the comparison of categorical date was made by chi-square test. The Kaplan-Meier method was used for survival analysis, and the Cox proportional hazards regression model was used to determine prognostic factors. Relief of symptoms in the lower limbs was analyzed to investigate the therapeutic effect. ResultsThe time to HM progression from grade 1 to grades 2, 3, and 4 was 40 months (range, 1-36 months), 8.5 months (range, 1-44 months), and 18.8 months (range, 54-48.6 months), respectively. In HM patients, the 1-, 3-, and 5-year cumulative survival rates were 84.19%, 5186%, and 4521%, respectively. Age of HM onset (HR=1.034, 95% CI: 1.003-1.065) and recurrent ascites (HR=3.869, 95% Cl: 1623-9225) were independent prognostic factors in patients with HM. The patients who underwent liver transplantation had a significantly higher proportion of patients with relief of symptoms than those who did not undergo liver transplantation (χ2=13.5, P=0.003), while the effects of stent flow limitation and occlusion showed no significant differences (P＞0.05). ConclusionThe course of HM consists of rapid progression stage and plateau stage, and HM patients after TIPS show good survival. At present, liver transplantation is an effective method to treat HM.

Discussion of liver transplantation for the treatment of hepatic myelopathy / 中华消化外科杂志

Hepatic myelopathy is one of special category changes of nervous system,which was secondary to the end-stage hepatic diseases and is a syndrome of myeleterosis.It usually occurred after portosystemic shunt surgery or collateral circulation of portosystemic vein.The prognosis of hepatic myelopathy is poor,and the progression of this disease is slow.Surgical approaches such as dissociation of colon and anastomosis of ileum and rectum aimed at reducing the absorption of toxic substance and thus to breakdown the blood ammonia and improve the symptoms of nervous system,but the effects are not satisfactory.The clinical data of 1 patient with hepatic myelopathy who received liver transplantation at the Second Affiliated Hospital of Dalian Medical University in April 2012 were retrospectively analyzed.The clinical symptoms and physical signs were improved,and muscle strength was effectively recovered in the patient.Liver transplantation might be an effective method for the treatment of hepatic myelopathy.

Hepatic Myelopathy Involving the Posterior Column

Hepatic myelopathy is a rare neurological complication of chronic liver disease, and is characterized by a progressing spastic paraparesis without any sensory loss. However, a few recent reports suggest that involvement of the sensory system is also possible in hepatic myelopathy. We present a patient with hepatic myelopathy, who had impaired proprioception and a delayed cortical response of somatosensory evoked potentials. This supports the hypothesis that hepatic myelopathy may involve the sensory system as well as the motor system.

Hepatic myelopathy as a presenting neurological complication in patients with cirrhosis and spontaneous splenorenal shunt / 대한간학회지

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.