ABSTRACT
INTRODUCTION AND OBJECTIVE: Acquired chronic hepatocerebral degeneration, acquired hepatolenticular degeneration or pseudo-Wilson is an infrequent disorder with a hepatic origin. Cases in the literature are scarce and it is frequently confused with hepatic encephalopathy and Wilson s disease. The aim of this essay is to report a patient suffering from this disorder due to cirrhosis from non-alcoholic steatohepatitis. CASE REPORT: We present a 54-year-old man diagnosed from cirrhosis grade B9 of the Child Pugh classification. He progressively developed a picture with bradylalia, mild postural and action tremor and spatial and temporal disorientation. Further studies demonstrated an increase of the values of hepatic transaminases and a hyperintensity in the basal nuclei in the cerebral magnetic resonance imaging. Clinical and radiological data established the diagnosis of hepatocerebral degeneration. CONCLUSIONS: Acquired chronic hepatocerebral degeneration is a disorder rarely reported in the literature that it is usually confused with other diseases. We alert about the need of having this diagnosis into account with patients developing neurological symptoms after hepatic disease.
Subject(s)
Fatty Liver/complications , Hepatolenticular Degeneration/etiology , Liver Cirrhosis/complications , Chronic Disease , Humans , Liver Cirrhosis/etiology , Male , Middle AgedABSTRACT
Fundamento y objetivo: el síndrome hepatocerebral crónico,también denominado degeneración hepatolenticular crónicaadquirida (DHCA) o pseudo-Wilson, es un trastorno poco frecuentede origen hepático. Los casos recogidos en la literatura son escasosy frecuentemente es confundido con la encefalopatía hepáticay con la enfermedad de Wilson. El objetivo de este artículo espresentar un paciente que sufre este trastorno de forma secundariaa una cirrosis por esteatohepatitis no alcohólica.Caso clínico: se trata de un varón de 54 años diagnosticadode cirrosis en grado funcional de Child-Pugh B9 que presentó uncuadro progresivo de bradilalia, temblor postural y de acción levey, en ocasiones, desorientación temporoespacial. Los estudioscomplementarios evidenciaron una elevación de las transaminasashepáticas y una hiperintensidad de los núcleos basales en la resonanciamagnética cerebral estableciéndose el diagnóstico deDHCA.Conclusiones: la DHCA es un trastorno poco descrito en laliteratura que en ocasiones es confundido con otras entidades. Esnecesario descartar la presencia de esta patología en los pacientescirróticos con alteraciones neurológicas tras una enfermedad hepática(AU)
Introduction and objective: acquired chronic hepatocerebraldegeneration, acquired hepatolenticular degeneration orpseudo-Wilson is an infrequent disorder with a hepatic origin.Cases in the literature are scarce and it is frequently confused withhepatic encephalopathy and Wilsons disease. The aim of this essayis to report a patient suffering from this disorder due to cirrhosisfrom non-alcoholic steatohepatitis.Case report: we present a 54-year-old man diagnosed fromcirrhosis grade B9 of the Child Pugh classification. He progressivelydeveloped a picture with bradylalia, mild postural and actiontremor and spatial and temporal disorientation. Further studiesdemonstrated an increase of the values of hepatic transaminasesand a hiperintensity in the basal nuclei in the cerebral magneticresonance imaging. Clinical and radiological data established thediagnosis of hepatocerebral degeneration.Conclusions: acquired chronic hepatocerebral degenerationis a disorder rarely reported in the literature that it is usually confusedwith other diseases. We alert about the need of having thisdiagnosis into account with patients developing neurologicalsymptoms after hepatic disease(AU)
