Hepatolenticular degeneration complicated by hepatic myelopathy: a clinical analysis of 5 cases and literature review / 中风与神经疾病杂志
Journal of Apoplexy and Nervous Diseases
; (12): 1030-1034, 2023.
Article
in Zh
| WPRIM
| ID: wpr-1031826
Responsible library:
WPRO
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.
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Database:
WPRIM
Language:
Zh
Journal:
Journal of Apoplexy and Nervous Diseases
Year:
2023
Document type:
Article