RESUMO
Neurological symptoms can be one of the over-riding symptoms in patients with liver cirrhosis. Patients can present with subtle changes in mood or neurological function due to hepatic encephalopathy (HE), to more severe presentations including stupor and coma. While HE, in its severe form, can be clinically easy to diagnose, more subtle forms may be more difficult to recognize. Other neurological diseases may indeed be overlooked in the context of cirrhosis or confuse the physician regarding the diagnosis. Chronic acquired hepatocerebral degeneration (CAHD) is an uncommon problem occurring in patients with cirrhosis characterised by a Parkinsonian-like neurological presentation with damage to the brain secondary to manganese (Mn) deposition. Here we describe a case of a patient with a neurological presentation of liver disease with a review of the current CAHD literature. In conclusion, CAHD is a rare condition occurring in liver cirrhosis that should always be considered in patients with neurological manifestations of chronic liver disease.
Assuntos
Encéfalo/metabolismo , Degeneração Hepatolenticular/metabolismo , Cirrose Hepática/complicações , Manganês/metabolismo , Doença Crônica , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/etiologia , Humanos , Cirrose Hepática/diagnóstico , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios XRESUMO
Abstract Acquired hepatocerebral degeneration is a neurological syndrome with typical clinical (extrapyramidal and neuropsychiatric) symptoms and brain magnetic resonance imaging findings (high T1 signal in the globus pallidus). It occurs mainly in patients with advanced liver disease, such as in patients co-infected with hepatitis B virus (HBV) and hepatitis delta virus (HDV). However, there are no reports relating HBV/HDV coinfection and acquired hepatocerebral degeneration. This report presents the case of a 49-year-old woman with characteristics of acquired hepatocerebral degeneration and liver cirrhosis due to HBV/HDV coinfection, and presents the main theories of the physiopathology of this condition.
Assuntos
Humanos , Feminino , Hepatite D/complicações , Hepatite B/complicações , Degeneração Hepatolenticular/virologia , Cirrose Hepática/virologia , Coinfecção/virologia , Pessoa de Meia-IdadeRESUMO
Acquired hepatocerebral degeneration is an underdiagnosed neurologic syndrome characterized by parkinsonism,ataxia or other movement disorders and by neuropsychiatric and cognitive symptoms. It occurs in patients with chronic liverdisease, especially those who develop portosystemic shunting and is often unrecognized as a cause of cognitive decline.Recently, its pathogenesis has been associated with manganese accumulation in basal ganglia and some treatmentsproposed. The aim of this article was to report a case and discuss some discoveries in connection with the disease.
Degeneração hepatocerebral adquirida é uma síndrome neurológica subdiagnosticado caracterizada porparkinsonismo, ataxia ou outros distúrbios do movimento e por sintomas neuropsiquiátricos e cognitivos. Ocorre empacientes com doença hepática crônica, especialmente aqueles que desenvolvem shunts porto-sistêmicos e frequentementenão é reconhecido como uma causa de declínio cognitivo. Recentemente, sua patogênese tem sido associada ao acúmulode manganês nos gânglios da base e alguns tratamentos foram propostos. O objetivo do artigo foi relatar um caso e discutiralgumas descobertas nesta doença.
Assuntos
Humanos , Encefalopatia Hepática , Degeneração Hepatolenticular , HepatopatiasRESUMO
Acquired hepatocerebral degeneration is an underdiagnosed neurologic syndrome characterized by parkinsonism, ataxia or other movement disorders and by neuropsychiatric and cognitive symptoms. It occurs in patients with chronic liver disease, especially those who develop portosystemic shunting and is often unrecognized as a cause of cognitive decline. Recently, its pathogenesis has been associated with manganese accumulation in basal ganglia and some treatments proposed. The aim of this article was to report a case and discuss some discoveries in connection with the disease.
Degeneração hepatocerebral adquirida é uma síndrome neurológica subdiagnosticado caracterizada por parkinsonismo, ataxia ou outros distúrbios do movimento e por sintomas neuropsiquiátricos e cognitivos. Ocorre em pacientes com doença hepática crônica, especialmente aqueles que desenvolvem shunts porto-sistêmicos e frequentemente não é reconhecido como uma causa de declínio cognitivo. Recentemente, sua patogênese tem sido associada ao acúmulo de manganês nos gânglios da base e alguns tratamentos foram propostos. O objetivo do artigo foi relatar um caso e discutir algumas descobertas nesta doença.