Hepatic Myelopathy: Case Report and Review of the Literature

Abstract

Background: Hepatic Myelopathy (HM) is a rare complication of chronic liver disease usually associated with extensive portosystemic shunt of blood, which has been created surgically or has occurred spontaneously, causing progressive spastic paraparesis. Some single cases or short clinical reports describing patients suffering from HM have been published worldwide, but are often scattered. Material and method: One additional case of HM with typical symptoms was presented, and a retrospective survey of the literature in a manner of comprehensive review was undertaken. Results: 46 case reports with 98 patients of HM including ours have been eligibly selected. General information on all cases was summarized. Detailed analysis of the clinical characteristics of HM patients was undertaken. Conclusion: Liver cirrhosis caused by hepatitis B infection and alcoholism is the most frequent causes of HM. Portosystemic shunt which resulted in chronic exposure to toxic substances bypassing the liver play an important role in the pathogenesis. The pathology study consistently disclosed a selective and symmetrical severe loss of myelin in both lateral pyramidal tracts. The predominant neurologic abnormality of HM is the progressive spasticity and weakness in the lower extremities. A typical manifestations, such as triparesis or quadriparesis, sensory deficit, urinary or bladder incontinence and non-pyramidal manifestations such as dysarthria, tremor and ataxia can also occur, which render the disorder more complicated to be diagnosed. Plasma ammonia concentrations were frequently found to be elevated. MEP provides evidence of the early diagnosis of HM and assesses different degrees of neurological involvement. The spinal cord MRI imaging shows no abnormality. Abnormalities of brain magnetic resonance combined with syndrome of brain dysfunction, hepatocerebral degeneration should be taken into consideration. Appending case studies suggest that liver transplantation is a promising therapeutic strategy.

Liver Transplantation Reverses Hepatic Myelopathy in 2 Children With Hepatitis A Infection

“…2 Spinal cord involvement is uncommon and usually associated with extensive porto-systemic shunt of blood either surgically created or occurring spontaneously in chronic liver disease. 4,5 Spinal cord involvement is labeled as Hepatic myelopathy (HM).It is most often seen in chronic liver disease. [9][10][11] Acute hepatic myelopathy gets obscured by dominant encephalopathy in acute liver failure, hence less often reported.…”

“…Hepatic myelopathy is more often a feature of chronic liver disease but can be seen in acute hepatic encephalopathy. 4 , 5 Hepatic myelopathy following acute fulminant liver failure is rarely seen and reported. Two children in our series had hepatic myelopathy following HE after acute fulminant hepatitis A infection, which reversed after liver transplantation (LT).…”

“…3 Spinal cord involvement is an uncommon complication and is usually associated with extensive porto-systemic shunt of blood either surgically created or occurring spontaneously in chronic liver disease. 2,4 Spinal cord involvement is labeled as hepatic myelopathy. Hepatic myelopathy is more often a feature of chronic liver disease but can be seen in acute hepatic encephalopathy.…”

Etiological profile of noncompressive myelopathies in a tertiary care hospital of Northeast India

“…The cause of spinal cord dysfunction could have been multifactorial (toxic effect of adulterated alcohol, nutritional deficiency, nitrogenous breakdown products by-passing the liver through shunt, and venous hypertensive myelopathy). [ 32 ]…”

Presentation of Congenital Portosystemic Shunts in Children

“…They range from mild cognitive deficits [ 25 ] to unexplained mental retardation [ 12 , 26 ], from attention–hyperactivity disorders and behavioral problems [ 1 , 26 ] to clear signs of encephalopathy [ 25 ]. There are also reports of post-prandial loss of consciousness or lethargy [ 1 , 37 , 45 ], seizures [ 1 ], Parkinson-like syndromes [ 46 ] or hepatic myelopathy [ 47 , 48 ]. Relevant findings include hyperammonemia [ 1 , 5 , 9 , 12 , 33 , 37 , 49 ], which has been linked to the ratio of portosystemic bypass [ 1 ], although plasma ammonia is an unreliable measure of encephalopathy in the absence of acute liver failure [ 50 , 51 ].…”