Persistence of mild parkinsonism 4 months after liver transplantation in patients with preoperative minimal hepatic encephalopathy: a study on neuroradiological and blood manganese changes

Abstract

Pallidal hyperintensity at magnetic resonance imaging (MRI) correlates to blood manganese (Mn) levels and parkinsonian signs in patients with cirrhosis. Similarly, metabolite changes in the basal ganglia (BG) at proton spectroscopy are related to these neurological signs. The evolution of these abnormalities after liver transplantation (OLT) is incompletely described. We evaluated 14 unselected consecutive patients with cirrhosis (minimal hepatic encephalopathy [HE] n = 8, no HE n = 6) before and 4 months after successful OLT for the evolution of parkinsonism using a validated scale (the United Parkinson's Disease Rating Scale, or UPDRS). Pallidal intensity at MRI, spectroscopic changes in the BG at magnetic resonance spectroscopy (MRS), and whole blood manganese concentrations were measured. After OLT in patients with preoperative minimal HE, the UPDRS scores improved, but mild parkinsonism persisted (16.1 2r3.6 to 6.2f4.8, P < 0.05). Pallidal hyperintensity remained abnormal in 5/8 of cases, but spectroscopic changes normalized in all patients. Blood Mn remained elevated in 4/6 patients. In patients without HE, UPDRS values remained negligible (2.42 f 1.5 to 2.5 + 1.4). Pallidal hyperintensity normalized in 7/8 patients and spectroscopic changes normalized in all patients. Blood Mn remained elevated in 5/6 patients. Four months after successful OLT, patients with preoperative minimal HE and severe pallidal hyperintensity showed persistent mild parkinsonism. The role of blood manganese determination appears limited in the monitoring of MRI and parkinsonian signs changes after OLT.

Acquired hepatocerebral degeneration

“…Although long assumed to be irreversible, mounting evidence suggests that even long-standing AHD may improve remarkably following liver transplantation [118,143], including hyperkinetic movement disorders [107,117,131,142], parkinsonism [77,118,121,134,139,142], ataxia [110,131], myelopathy [98,114,150,157] and cognitive dysfunction [67,86,142]. Blood manganese levels and MRI changes also improve following restoration of hepatic function, although normalization of these parameters is gradual and sometimes incomplete.…”

“…Blood manganese levels and MRI changes also improve following restoration of hepatic function, although normalization of these parameters is gradual and sometimes incomplete. Magnetic resonance spectroscopy (MRS) of the brain is an alternate means of tracking recovery following liver transplantation, since MRS abnormalities resolve in unison with clinical improvement and in advance of changes on standard MRI [77]. In this population, the changes on MRS that correlate with the presence of parkinsonism or encephalopathy include reduced choline and myoinositol resonance [139] and increased glutamate/glutamine resonance [96].…”

“…Finally, AHD-related parkinsonism and Parkinson disease differ in their response to therapy; although levodopa benefits some patients with AHD [8, 55,67,83,122], the response is far less consistent than in Parkinson disease [2,68,134,164]. AHD-related parkinsonism, however, may improve following liver transplantation [77,134,139,142]. Ataxia is a ubiquitous feature of AHD in most series, but alcoholic cerebellar degeneration is a potential confounder for patients with a history of alcohol-related cirrhosis.…”

Surgical attenuation of spontaneous congenital portosystemic shunts in dogs resolves hepatic encephalopathy but not hypermanganesemia

“…Aside from a case report demonstrating a reduction in globus pallidus hyperintensity, a finding which is pathognomic for Mn deposition, after occlusion of a portosystemic shunt and treatment for hepatic encephalopathy, there is only one study in humans which measured whole blood manganese before and after an improvement in hepatic function (Taguchi et al 1999;Lazeyras et al 2002;Barron et al 1994). This study examined Mn concentrations before and after orthotopic liver transplantation (OLT) which failed to demonstrate a significant reduction in Mn concentrations 4 months after transplant.…”

Liver transplantion in a patient with rapid onset parkinsonism-dementia complex induced by manganism secondary to liver failure

“…rocha et al reported an interesting case of reversible parkinsonism and port systemic encephalopathy following embolization of a congenital intrahepatic venous shunt. Nonetheless these evidences, liver transplantation might be the only choice for some patients; even though the parkinsonian and the neuropsychiatric changes might persist even after liver-transplantation in some patients [9][10][11] .…”