Use of model for end-stage liver disease exception points for early liver transplantation and successful reversal of hepatic myelopathy with a review of the literature

Abstract

Hepatic myelopathy (HM) is a rarely reported disorder characterized by progressive spastic paraparesis due to impaired corticospinal tract function in the setting of cirrhosis or portosystemic shunting. HM has not to date been recognized as a Model for End-Stage Liver Disease (MELD) exception for transplantation. Outcomes for a small number of patients from Europe and Asia who have undergone liver transplantation (LT) for HM suggest a potential neurological benefit, especially with earlier transplantation. We report the first use of MELD exception points for the condition of HM to enable early LT resulting in the reversal of marked spastic paraparesis. Our patient, whose myelopathy had markedly progressed without further hepatic decompensation, underwent LT 14 months after the diagnosis of HM with an adjusted MELD score of 30, which was granted as a United Network for Organ Sharing exception. After LT, there was significant neurological improvement as the patient progressed from wheelchair dependency to full ambulation. We reviewed the literature of other HM patients who had undergone LT. With our patient, there were in all 15 reported cases of LT in individuals with HM. LT can lead to a marked improvement in HM, particularly in the earlier clinical stages of the disorder. Early LT can be accomplished, as in our case, by the submission of an appeal for a MELD upgrade. Liver Transpl 16:818-826, 2010. V C 2010 AASLD.Received November 22, 2009; accepted March 16, 2010. Hepatic encephalopathy or portosystemic encephalopathy (PSE) is a well-established and common complication in patients with end-stage liver disease (ESLD), whereas hepatic myelopathy (HM) is a rare and possibly underrecognized neurological complication of cirrhosis. Findings of spastic paraparesis, hyperreflexia, and extensor plantar responses characterize HM.

Reversal of hepatic myelopathy after liver transplantation: Fifteen plus one

“…This degree of reversal of hepatic myelopathy after transplantation is possibly the highest described to date. [1][2][3] In most reported cases of hepatic myelopathy and in our patient, an association has been observed between myelopathy, encephalopathy, and portosystemic shunting. [1][2][3] Interestingly, Caldwell and colleagues 1 state that their patient had no overt encephalopathy.…”

“…[1][2][3] In most reported cases of hepatic myelopathy and in our patient, an association has been observed between myelopathy, encephalopathy, and portosystemic shunting. [1][2][3] Interestingly, Caldwell and colleagues 1 state that their patient had no overt encephalopathy. However, the clinical diagnosis of low-grade overt encephalopathy can be difficult, and Caldwell and colleagues seem to have made no attempt to diagnose minimal encephalopathy by psychometric or neurophysiological testing.…”

“…The link between hepatic myelopathy and encephalopathy is supported by the following: (1) their association with portosystemic shunting, [1][2][3] (2) the analogies between cerebral and spinal MRI abnormalities in patients with cirrhosis, 4 and (3) the high prevalence of motor impairment in encephalopathic patients. 5 Indeed, in our patient, the full anti-encephalopathy regime relieved lower limb spasticity to an extent.…”

Central motor and sensory conduction in patients with hepatic myelopathy

“…22 In a recently reported patient the abnormal MRI finding of increased fluid-attenuated inversion recovery signal in the subcortical white matter and subcortical spinal tracts suggests the possible occurrence of HM-related pathology above the level of the foramen magnum. 15 We cannot find any MRI abnormalities in our patients. Moreover, the normality of the N24-P40 interval also excludes a subclinical involvement of the intracranial segment of the central sensory pathways in our patients.…”

“…2,14 Occasionally, demyelination has also been found in the ventral pyramidal tracts, in the posterior columns and spinocerebellar tracts. A recent study also documented in an HM patient a delayed onset posterior column dysfunction (proprioception and vibratory sensory loss) and a small fiber length-dependent axonal polyneuropathy, 15 both progressing concomitantly with the motor deficits.…”