Hepatolenticular degeneration complicated by hepatic myelopathy： a clinical analysis of 5 cases and literature review / 中风与神经疾病杂志

ABSTRACT

@#Objective To analyze the clinical features of patients with hepatolenticular degeneration （HLD） complicated by hepatic myelopathy （HM）. Methods A retrospective analysis was performed on the clinical features，auxiliary examinations，diagnosis and treatment，and outcomes of 5 patients diagnosed with HLD complicated by HM in our hospital from January 2018 to February 2023，and the relevant literature was reviewed. Results Among the 5 patients，4 were male and 1 was female； the age of onset of HM ranged from 16 to 32 years. All 5 patients had manifestations of decompensated cirrhosis，and 3 patients had a history of transjugular intrahepatic portosystemic shunt （TIPS）. The spinal MRI showed abnormal signals in the thoracic cord in 2 patients. Electromyography showed abnormalities in 3 patients，and electroencephalography showed significantly slower background brain waves in 4 patients. Two patients underwent liver transplantation，and 2 patients received endovascular treatment. One patient died of upper gastrointestinal bleeding； the other 4 patients had varying degrees of recovery. Conclusion The prevalence of HLD complicated by HM is very low. High copper status，hyperammonemia，TIPS，anemia，hypoproteinemia，and portal hypertension are directly associated with HM.