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

Abstract

-bilateral and symmetric globus-pallidus hyperintensities are observed on t1-weighted MrI in most of the patients with chronic liver failure, due to manganese accumulation. We report a 53-year-old man, with rapid onset parkinsonism-dementia complex associated with accumulation of manganese in the brain, secondary to liver failure. A brain MrI was performed and a high signal on t1-weighted images was seen on globus-pallidus, as well as on t2-weighted images on the hemispheric white-matter. He was referred to a liver-transplantation. the patient passed away on the seventh postoperative day. Our findings support the concept of the toxic effects of manganese on the globus-pallidus. the treatment of this form of parkinsonism is controversial and liver-transplantation should not be considered as first line treatment but as an alternative one.Key WOrds: manganese, liver transplantation, parkinsonism-dementia, globus pallidus, high-signal.Transplante hepático em um paciente com complexo parkinsonismo-demência rapidamente progressivo induzido por manganismo devido a insuficiência hepática resuMO -Hiperintesidades simétricas e bilaterais dos gânglios da base são observadas em imagens de ressonância magnética encefálica (rM) ponderadas em t1 na maioria dos pacientes com insuficiência hepáti-ca crônica devidas ao acúmulo de manganês. Nós relatamos o caso de um homem, com 53 anos de idade, com um complexo parkinsonismo-demência rapidamente progressivo associado com o acúmulo de manganês no cérebro, secundariamente a insuficiência hepática. uma rM encefálica foi realizada e foram observadas imagens hiperintensas/hipersinal nas imagens ponderadas em t1 no globo pálido e, também, na substância branca dos hemisférios cerebrais ponderadas em t2. devido à falta de resposta ao tratamento clinico optamos pelo transplante hepático. O paciente faleceu no 7º dia de PO. Nossos achados corroboram o conceito dos efeitos tóxicos do manganês nos gânglios da base/globo pálido. O tratamento desta forma de parkinsonismo é controverso e o transplante hepático não deverá ser considerada uma opção terapêutica de primeira linha, porém como um tratamento alternativo considerando-se os riscos-benefícios dessa escolha.PAlAvrAs-cHAve: manganês, transplante hepático, complexo parkinsonismo-demência, globo pálido.

Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease

“…A recent report showed a patient with Parkinsonism and dementia associated with MRI hypersignal in T1, which was interpreted as evidence of encephalic manganese deposition. The authors considered this finding as the determinant factor for indicating a liver transplant 32 . The indication in this setting is reasonable, since about 20% of patients selected for liver transplant have extrapyramidal signs.…”

Brain Imaging and Hepatic Encephalopathy

“…It is therefore unclear whether T 1 -weighted hyperintensity represents a manifestation of HE, or if it is an occurrence secondary to cirrhosis, cholestasis and/or porto-systemic shunting (25,26). The preferential deposition of manganese in the basal ganglia has been suggested to be an explanation for the Parkinsonian-like symptoms, which can arise as a result of HE (27). So while a useful adjunct this lack of correlation with grades of HE makes a single T 1 -weighted exam insufficient in MR Brain in patients with cirrhosis.…”

Inhibition of Calpain Prevents Manganese-Induced Cell Injury and Alpha-Synuclein Oligomerization in Organotypic Brain Slice Cultures

“…The wide use of Mn in a range of industries has led to global health concerns. Indeed, Mn intoxication occurs from occupational exposure [2], administration of total parenteral nutrition [3], and chronic liver failure [4]. Concern about Mn exposure has also focused on the use of a Mn-containing fuel additive, methylcyclopentadienyl Mn tricarbonyl (MMT), as an anti-knock agent in gasoline in Canada and other Western nations [5].…”