Tabla de la normalidad de las pruebas psicométricas para el diagnóstico de encefalopatía hepática subclínica en la población Dominicana / Score of normality psychometric tests for the diagnosis of subclinical hepatic encephalopathy in the Dominican Population

Introducción: La encefalopatía hepática mínima (EHM), es una enfermedad definida por la existencia de varias alteraciones neurofisiológicas, indetectables a la exploración neurológica y el examen clínico. Dentro de las estrategias diagnosticas para la EHM se contemplan las pruebas psicométricas (PHE), pero para su aplicación es indispensable la estandarización previamente en la población de estudio. Objetivo: El estudio se propuso determinar la tabla de la normalidad de las PHE para diagnosticar la encefalopatía hepática subclínica en una muestra de la población dominicana. Método: Se realizó un estudio descriptivo, prospectivo y transversal en un hospital de referencia nacional. Se analizaron 134 personas clasificados por grupos de edades (18-70 años de edad) y años de escolaridad. Se diseñó una tabla de 5x5. Se estudió la influencia de la edad, sexo, uso de espejuelo y de los años de escolarización en el rendimiento de cada uno de las PHE, para lo cual se utilizaron las siguientes pruebas estadísticas: análisis de varianza (ANOVA), prueba t de Student y regresión lineal. Resultado: La escolaridad y la edad fueron variables determinantes en el desempeño de las 5 pruebas psicométricas. Pero, la correlación univariable de la edad con el desempeño de la prueba TMS no hubo diferencias intra e inter grupos estadísticamente significativas (p>0.171). Conclusión: se confecciono la fórmula de predicción de resultados de los test psicométricos. Ninguno sobrepasó el punto de corte de la puntuación que oscila entre los -4 y los +2 puntos.

Introduction: Minimal hepatic encephalopathy (MHE) is a disease defined by the existence of several neurophysiological alterations, undetectable by neurological examination and clinical examination. Among the diagnostic strategies for EHM, psychometric tests (PHE) are contemplated, but for their application, prior standardization in the study population is essential. Objective: The study will need to determine the normality table of PHE to detect subclinical hepatic encephalopathy in a sample of the Dominican population. Method: A descriptive, prospective and cross-sectional study was carried out in a national reference hospital. 134 people classified by age groups (18-70 years of age) and years of schooling were analyzed. A 5x5 board is recommended. The influence of age, sex, use of glasses and years of schooling on the performance of each one of the PHEs was studied, for which the following statistical tests were used: analysis of variance (ANOVA), Student's t test and linear regression. Result: Schooling and age were determining variables in the performance of the 5 psychometric tests. But, the univariate coincidence of age with the performance of the TMS test, there were no statistically significant intra and inter group differences (p>0.171). Conclusion: the formula for predicting the results of the psychometric tests was made. None exceeded the cut-off point of the score that oscillates between -4 and +2 points.

Progress in the diagnosis of minimal hepatic encephalopathy / 中华肝脏病杂志

Hepatic encephalopathy (HE) is a common serious complication of liver cirrhosis, with sudden onset, indicating a poor prognosis in patients with chronic liver disease. Minimal hepatic encephalopathy (MHE) is an early stage of HE with no apparent symptoms, but it shows abnormal results in neuropsychological and/or neurophysiological tests. MHE affects patients' quality of life, employability, driving ability, and has a high risk of developing overt hepatic encephalopathy (OHE). This article aims to explore various diagnostic methods, strengthen the routine work of clinicians in diagnosis and treatment, and develop an effective MHE screening protocol.

Uso do Stroop EncephalApp para o screening de encefalopatia hepática mínima em pacientes cirróticos no sul do Brasil / Use of the stroop encephalapp for covert hepatic encephalopathy screening in cirrhotic patients in southern brazil

ABSTRACT BACKGROUND: Covert hepatic encephalopathy (CHE) is difficult to detect due to the lack of easily applicable screening tools. The Stroop EncephalApp is a smartphone application already validated for CHE screening. However, its applicability to the Brazilian population is not known. OBJECTIVE: To estimate the prevalence of CHE and evaluate the use of Stroop EncephalApp in a cirrhotic population in Brazil. METHODS: In this cross-sectional study, we evaluated 99 patients previously diagnosed with liver cirrhosis in a Private Hospital in Curitiba/PR. Patients were initially submitted to the mini mental state examination (MMSE) to exclude individuals with dementia. After, the Psychometric Hepatic Encephalopathy Score (PHES) test was performed and lastly, the Stroop EncephalApp test. Results were adjusted for age, sex and education levels to evaluate the accuracy of the app on detecting the disease, comparing its results with the gold standard method (PHES). Patients with one or more of the following were excluded: dementia, inadequate MMSE score, illiteracy, color blindness, history of drugs/alcohol abuse within the past 3 months and previous or actual episodes of encephalopathy. The statistical analysis was performed by SPSS 2.0 and the significance adopted by 5%. RESULTS: We included 82 individuals in the final analysis. Among these patients, 29 were diagnosed with CHE by the PHES test (35.36% prevalence) and 28 of those obtained equal diagnosis by the Stroop EncephalApp (96.6% sensitivity). A total of 53 patients obtained negative results for CHE by PHES, while the Stroop test classified 27 of them as having the disease. In the multivariate analysis, high levels of education were associated with better performance during the tests. No significant relationship was observed between age and sex with the probability of diagnosing CHE through the PHES test.

RESUMO CONTEXTO: A encefalopatia hepática mínima (EHM) é uma complicação neuro-psiquiátrica da cirrose cuja detecção é dificultada pela falta de ferramentas práticas. O Stroop EncephalApp é um aplicativo de smartphones capaz de detectar a doença, entretanto sua aplicabilidade na população brasileira ainda não é conhecida. OBJETIVO: Avaliar o uso do Stroop EncephalApp para diagnóstico e avaliação de EHM em uma população de pacientes cirróticos no Brasil. MÉTODOS: Através de um estudo observacional transversal, 99 indivíduos sabidamente cirróticos foram recrutados do ambulatório de hepatologia de um hospital privado em Curitiba/PR. Primeiramente, foram aplicados o mini exame do estado mental (MEEM) para excluir indivíduos com demência; após, foram aplicados o Escore Psicométrico da Encefalopatia Hepática (PHES), atual padrão-ouro para diagnóstico de EHM, e posteriormente o Stroop EncephalApp, ajustando para idade, sexo e anos de formação acadêmica, buscando avaliar a eficiência do aplicativo em detectar a doença e comparar seus resultados com o atual padrão-ouro. Foram excluídos do estudo indivíduos com demência, pontuação insuficiente no MEEM, analfabetos, daltônicos, e com histórico de abuso de álcool/drogas ilícitas nos últimos 3 meses e paciente com episódios prévios ou atuais de encefalopatia hepática. A análise estatística foi realizada pelo SPSS 2.0 e a significância adotada em 5%. RESULTADOS: Um total de 82 indivíduos foram incluídos na análise final. Destes, 29 foram diagnosticados com EHM (35,36% de prevalência) através do PHES e 28 obtiveram o mesmo resultado após a aplicação do Stroop (96,6% de sensibilidade). Cinquenta e três pacientes obtiveram um resultado negativo para EHM através do PHES, sendo que 27 desses obtiveram um resultado positivo para EHM através do Stroop. Na análise multivariada, níveis elevados de escolaridade estiveram associados com melhor desempenho durante a execução dos testes. Não houve associação significativa entre idade e sexo com a probabilidade de apresentar encefalopatia através do PHES. CONCLUSÃO: O Stroop EncephalApp é uma ferramenta viável e com boa sensibilidade para o screening de EHM, mas possui baixa especificidade na população estudada.

Observational Cohort Study of Hepatic Encephalopathy After Transjugular Intrahepatic Portosystemic Shunt (TIPS)

Abstract: Introduction and aim. Hepatic encephalopathy (HE) is a common complication of transjugular intrahepatic portosystemic shunting (TIPS). It is associated with a reduced quality of life and poor prognosis. The aim of this study was to compare two groups of patients who did and did not develop overt HE after TIPS. We looked for differences between these groups before TIPS. Material and methods. A study of 895 patients was conducted based on a retrospective analysis of clinical data. Data was analyzed using Fisher’s exact test, χ2, Mann Whitney test, unpaired t-test and logistic regression. After the initial analyses, we have looked at a regression models for the factors associated with development of HE after TIPS. Results. 257 (37.9%) patients developed HE after TIPS. Patients’ age, pre-TIPS portal venous pressure, serum creatinine, aspartate transaminase, albumin, presence of diabetes mellitus and etiology of portal hypertension were statistically significantly associated with the occurrence of HE after TIPS (p < 0.01). However, only the age, pre-TIPS portal venous pressure, serum creatinine, presence of diabetes mellitus and etiology of portal hypertension contributed to the regression model. Patients age, serum creatinine, presence of diabetes mellitus and portal vein pressure formed the model describing development of HE after TIPS for a subgroup of patients with refractory ascites. Conclusion. We have identified, using a substantial sample, several factors associated with the development of HE after TIPS. This could be helpful in further research.

Minimal Hepatic Encephalopathy in Cirrhosis- How Long to Treat?

Abstract: Introduction. Minimal hepatic encephalopathy (MHE) can reverse after short-term treatment. However, relapse rate of MHE after stopping treatment has not been studied so far. We aimed to evaluate long-term (9 months) efficacy of a short-term (3 months) treatment of MHE with lactulose/rifaximin, for maintenance of remission from MHE. Material and methods. In this prospective study, consecutive patients with cirrhosis and MHE were treated with lactulose/rifaximin for 3 months. After treatment, they were followed up for 6 months. Psychometric testing for diagnosis of MHE was performed at baseline, 3 months and 9 months. Results. Of the 527 patients screened, 351 were found eligible and tested for MHE. Out of these, 112 (31.9%) patients had MHE (mean age 55.3 years; 75% males). They were randomized to receive Rifaximin (n = 57; 1,200 mg/day) or Lactulose (n = 55; 30-120 mL/day) for three months. At 3 months, 73.7% (42/57) patients in Rifaximin group experienced MHE reversal compared to 69.1% (38/55) in Lactulose group (p = 0.677). Six months after stopping treatment, 47.6% (20/42) in rifaximin group and 42.1% (16/38) patients in lactulose group experienced MHE relapse (p = 0.274). The overt hepatic encephalopathy development rate (7.1% vs. 7.9%) and mortality rate (0.23% vs. 0%) were similar in both groups. The Child-Turcotte-Pugh score and model for end stage liver disease (MELD) scores of patients who had MHE relapse were higher compared to those who didn’t. On multivariate regression analysis, MELD score was an independent predictor of MHE relapse. Conclusion. Of the patients who became MHE negative after short-term (3 months) treatment with rifaximin/lactulose, almost 50% had a relapse of MHE at 6 months follow-up.

Tablas de normalidad de la población en Cuba para los test psicométricos utilizados en el diagnóstico de la encefalopatía hepática mínima / Normality tables of the population in Cuba for the psychometric tests used in the diagnosis of minimal hepatic encephalopathy

La encefalopatía hepática mínima constituye el estadio subclínico previo al desarrollo de encefalopatía hepática clínica en el paciente cirrótico, además de asociarse a un deterioro de la calidad de vida de estos pacientes y a un riesgo incrementado de accidentes laborales e incapacidad de conducción de vehículos. El Psychometric Hepatic Encephalopathy Score es considerado actualmente el método diagnóstico de elección en el diagnóstico de la EHM. El objetivo de este trabajo fue diseñar las tablas de normalidad del PHES para la población cubana. Material y métodos: Se estudió una muestra conformada por 520 personas sanas de las provincias de Villa Clara, Sancti Spíritus y Cienfuegos. Las mismas realizaron los 5 tests incluidos en el PHES. Se analizaron las variables edad, sexo, años de escolarización, procedencia, y consumo diario de alcohol. Mediante la prueba de la t de Student, ANOVA y el coeficiente de correlación de Pearson se realizó el análisis univariante. Se efectuó un análisis de regresión lineal múltiple para cada test y se construyeron las tablas de normalidad. Resultados: En el análisis multivariante (regresión lineal múltiple) la edad y los años de escolarización fueron las 2 variables independientes relacionadas con el rendimiento en cada uno de los 5 tests. Conclusiones: La disponibilidad de las tablas de normalidad del PHES permitirá contar con un método diagnóstico de referencia aplicable a los pacientes con cirrosis hepática cubanos, sin la necesidad de configurar grupos controlados por edad y nivel de escolaridad regionalmente...

Minimal hepatic encephalopathy (MHE) is the subclinical stage pre-clinical development of hepatic encephalopathy in cirrhotic patients, in addition to associated with impaired quality of life of these patients and an increased risk of accidents and disability of driving. The Psychometric Hepatic Encephalopathy Score (PHES) is currently considered the diagnostic method of choice in the diagnosis of MHE. The objective of this work was to design tables PHES normality for the Cuban population. Material and method: A sample composed of 520 healthy people in the provinces of Villa Clara, Sancti Spiritus and Cienfuegos were studied. They made the 5 tests included in the PHES. The variables age, sex, years of education, origin, and daily alcohol consumption were analyzed. By t test of Student, ANOVA and Pearson correlation coefficient univariate analysis. an analysis of multiple linear regression for each test was performed and normal tables were constructed. Results: In the multivariate analysis (multiple linear regression) age and years of schooling were the two independent variables related to performance in each of the 5 tests. Conclusions: The availability of tables PHES normality will provide a diagnostic method applicable reference to patients with liver cirrhosis Cubans, without the need to configure controlled for age and education level regional groups...

Alteraciones neuropsicológicas en pacientes con hipertensión portal prehepática / Neuropsychological alterations found in patients with prehepatic portal hypertension

INTRODUCCIÓN: en la hipertensión portal prehepática secundaria a obstrucción trombótica, pueden aparecer trastornos neurocognoscitivos, similares a la encefalopatía sistémica vista en afecciones hepáticas crónicas, la que puede estar bien definida clínicamente o en una forma subaguda, actualmente llamada encefalopatía hepática mínima. Esta consiste en la detección de déficits neuropsicológicos en pacientes sin alteraciones en la exploración neurológica rutinaria. En los niños, es difícil su estudio debido a la falta de pruebas neuropsicológicas estandarizadas para todas las edades. OBJETIVO: identificar la presencia de alteraciones neuropsicológicas en los pacientes pediátricos con hipertensión portal prehepática. MÉTODOS: se estudiaron 12 pacientes con hipertensión portal prehepática secundaria a cateterismo umbilical, mediante diferentes técnicas psicológicas. Las variables fueron: edad, tiempo de evolución de la enfermedad, forma clínica de inicio, alteraciones neuropsicológicas y procedimiento quirúrgico empleado. RESULTADOS: predominaron los pacientes entre 10 y 14 años de edad, y con un tiempo de evolución de la enfermedad entre 6 y 10 años. El sangrado digestivo alto fue la forma clínica de inicio más frecuente. En 11 casos se detectaron déficits en la atención involuntaria, concentración de la atención, memoria inmediata y dinámica de la actividad de la memoria. En 8 pacientes se afectó el pensamiento en su aspecto operacional, y en 6 la dinámica de la actividad del pensamiento. La mayoría de los niños con alteraciones neuropsicológicas no estaban intervenidos quirúrgicamente. CONCLUSIONES: existen trastornos predictivos de encefalopatía hepática mínima en pacientes con hipertensión portal prehepática. No utilizar el tratamiento quirúrgico puede estar relacionado con la aparición de las alteraciones neuropsicológicas. Teóricamente la solución sería derivaciones portoportales realizadas precozmente, o procedimientos que eliminen la obstrucción portal. Se requiere profundizar y generalizar esta investigación al resto de los pacientes con el diagnóstico de esta enfermedad

INTRODUCTION: in the prehepatic portal hypertension secondary to thrombotic obstruction, there may appear neurocognitive disorders similar to systemic encephalopathy seen in chronic hepatic illnesses. This portal hypertension may be clinically well-defined or occurs in a subacute form currently known as minimal hepatic encephalopathy. This consists of neurophyshcological deficits in patients without alterations in the routine neurological exploration. It is difficult to study it in children due to lack of standardized neuropsychological tests for all ages. OBJECTIVES: to identify the presence of neupsychological alterations in pediatric patients with prehepatic portal hypertension. METHODS: twelve patients with prehepatic portal hypertension secondary to umbilical catheterism were studied through different psychological techniques. The study variables were age, time of progression of disease, initial clinical form, neuropsychological alterations and surgical procedure used. RESULTS: predominance of patients aged 10 to 14 years and time of progression ranging 6 to 10 years. The upper digestive bleeding was the most common initial clinical form. Eleven patients showed deficit in involuntary attention, focused attention, immediate memory and dynamics of the memorizing activity. Eight patients suffered problems in the operational aspect of their thinking and 6 had the dynamics of their thinking affected. Most of children with neuropsychological alterations were not operated on. CONCLUSIONS: there are predictive disorders of minimal hepatic encephalopathy in patients suffering prehepatic portal hypertension. The failure to use the surgical treatment may be related to occurrence of neuropsychological alterations. Theoretically speaking, the solution would lie in performing early portoportal shunts or procedures eliminating the portal obstruction. It is necessary to delve into this research and generalize the results to the rest of patients diagnosed with this disease.

Osler-Weber-Rendu Disease Presenting as Recurrent Portosystemic Encephalopathy in a 75-year-old Female Patient / 대한소화기학회지

Osler-Weber-Rendu disease is a rare autosomal dominant disorder of fibrovascular tissues, characterized by a classic triad of mucocutaneous telangiectasias, recurrent hemorrhages, and a familial occurrence. Portosystemic encephalopathy in a patient with Osler-Weber-Rendu disease is rare, but we experienced a case presenting with recurrent portosystemic encephalopathy in Osler-Weber-Rendu disease. We report on a case of a 75-year-old female presenting with an altered mentality. Initial studies including brain imaging study did not reveal any specific cause for her mental status. She was diagnosed with the rare disease after a series of tests and received conservative treatment. Her neurological status recovered fully without complication after conservative treatment and she was discharged after 18 hospital days. This case demonstrated an extremely rare case of Osler-Weber-Rendu disease presenting as portosystemic encephalopathy treated successfully with conservative treatment. For patients who have shown hepatic encephalopathy without a definite cause, we recommend evaluation for the possibility of Osler-Weber-Rendu disease. Conservative treatment based on treatment of advanced liver cirrhosis could be an alternative solution.

Ammonia levels and the severity of hepatic encephalopathy

To evaluate the correlation between ammonia levels with the severity of HE in patients coming to the tertiary care hospital with liver cirrhosis and hepatic encephalopathy [HE]. Descriptive, analytical study. Shifa International Hospital, Islamabad, from January 2011 to February 2012. A total of 135 patients with liver cirrhosis and HE had serum ammonia levels measured on admission. The diagnosis of HE was based on clinical criteria, and its severity was graded according to the West Haven Criteria for grading of mental status. Ammonia levels were correlated with the severity of HE using Spearman rank correlation. Out of 20 patients with normal ammonia levels, 13 [65%] were in HE I-II, 6 [30%] were in grade-III, while 1 [5%] patient was in grade-IV HE. Out of 45 patients with mild hyperammonemia, 27 [60%] were in grade I-II, 12 [26%] were in grade-III and 6 [13%] were in grade-IV HE. Out of 34 patients with moderate hyperammonemia, 9 [26%] were in grade I-II, 18 [53%] were in grade-III, and 7 [20%] were in grade-IV HE. Out of 36 patients with severe hyperammonemia, 31 [86%] patients were in grade-IV HE [p < 0.001]. Ammonia levels correlated with the severity of hepatic encephalopathy. Greater the ammonia level, severe is the grade of hepatic encephalopathy

Encefalopatias: etiologia, fisiopatologia e manuseio clínico de algumas das principais formas de apresentação da doença / Encephalopathies: etiology, pathophysiology, and clinical management of some major forms of disease presentation

JUSTIFICATIVA E OBJETIVOS: As encefalopatias compõem um grupo heterogêneo de etiologias, onde a pronta e correta atuação médica direcionada à causa da doença, pode modificar o prognóstico do paciente. O objetivo deste estudo foi rever os aspectos fisiopatológicos das diferentes encefalopatias bem como seus principais fatores desencadeantes e manuseio clínico.CONTEÚDO: Foram selecionadas as mais frequentes encefalopatias observadas na prática clínica e discutir sua fisiopatologia, bem como sua abordagem terapêutica, destacando: encefalopatia hipertensiva, hipóxico-isquêmica, metabólica, Wernicke-Korsakoff, traumática e tóxica.CONCLUSÃO: Trata-se de uma complexa condição clínica que exige rápida identificação e preciso manuseio clínico com o intuito de reduzir sua elevada taxa de morbimortalidade. O atraso no reconhecimento dessa condição clínica poderá ser extremamente prejudicial ao paciente que estará sofrendo lesão cerebral muitas vezes irreversível.

BACKGROUND AND OBJECTIVES: Encephalopathies comprise a heterogeneous group of clinical conditions, in which the prompt and adequate medical intervention can modify patient prognosis. This paper aims to discuss the pathophysiological aspects of different encephalopathies, their etiology, and clinical management.CONTENTS: We selected the main encephalopathies observed in clinical practice, such as hypertensive, hypoxic-ischemic, metabolic, Wernicke-Korsakoff, traumatic, and toxic encephalopathies, and to discuss their therapeutic approaches.CONCLUSION: This is a complex clinical condition that requires rapid identification and accurate clinical management with the aim of reducing its high morbidity and mortality rates. Delay in recognizing this condition can be extremely harmful to the patient who is suffering from often irreversible brain injury.

Encefalopatía hepática mínima: conceptos actuales sobre una entidad emergente / Minimal hepatic encephalopathy: current concepts of an emerging clinical entity

Hepatic encephalopathy (HE) is a reversible neuropsychiatric disorder, consequence of hepatocellular failure and/or portal shunt to the systemic circulation. The neurological abnormalities are heterogeneous, affecting different cognitive and motor functions and its magnitude has a wide spectrum, ranging from a subclinical level (minimal hepatic encephalopathy, MHE) to coma. The relevance of MHE has been recognized only recently, with studies that have shown high prevalence among cirrhotic patients and significant complications such as poor quality of life, risk of vehicular accidents, predisposition to development overt HE and increased mortality. Despite this, one of the limitations for routine screening is the lack of a simple and objective diagnostic method, since MHE is not detectable by conventional medical assessment and diagnosis is only made through specialized tests that assess different cognitive domains. There isn´t a diagnostic gold standard; and some of the most recognizable methods that have been implemented in clinical practice in some countries are: Psychometric Hepatic Encephalopathy Score, Critical Flicker Frequency, and Inhibitory Control Test. The main therapies that have been evaluated for EHM are lactulose and rifaximin, with favorable impact on different outcomes. More multicentric studies with longer follow-up and clinically relevant outcomes that demonstrate the cost-effectiveness of screening and treatment of this important complication are needed.

La encefalopatía hepática (EH) corresponde a un trastorno neuropsiquiátrico reversible producido como consecuencia de una falla hepatocelular y/o de la desviación del flujo portal a la circulación sistémica.El compromiso producido es heterogéneo, afectando diversas funciones cognitivas y motoras y su magnitud presenta un amplio espectro que abarca desde un nivel subclínico correspondiente a la encefalopatíahepática mínima (EHM), hasta el coma. La relevancia de la EHM se ha reconocido sólo recientemente, conestudios que han mostrado su alta prevalencia entre los pacientes cirróticos y una trascendencia clínicasignificativa, asociándose a una pobre calidad de vida, riesgos de accidentalidad vial, predisposición a desarrollo de EH evidente y mayor mortalidad. A pesar de lo anterior, una de las limitantes para su pesquisarutinaria es la falta de un método diagnóstico simple y objetivo, ya que la EHM no es detectable medianteuna evaluación médica convencional y sólo se diagnostica a través de test especializados que evalúan distintosdominios cognitivos. No existe un estándar de oro diagnóstico; entre los métodos más reconocidos y que se han implementado en la práctica clínica enalgunos países se encuentran el Score Psicométrico de Encefalopatía Hepática, el Test de Frecuencia Críticade Parpadeo, y el Test de Control Inhibitorio. Entre las terapias para EHM más evaluadas se encuentran la lactulosa y rifaximina, con impacto favorable en distintos outcomes, sin embargo, faltan estudios multicéntricos, con seguimientos prolongados y con objetivos clínicamente relevantes que demuestren la costo-efectividad de la pesquisa y tratamiento de estarelevante complicación.

Encefalopatía hepática fulminante vinculada a hiperintensidad de la corteza cerebral en la resonancia magnética / Fulminant hepatic encephalopathy vinculated with hyperintensity of the cerebral cortex in magnetic resonance

Background: The presence of manganese deposits in basal ganglia is an expression of chronic liver damage in neuroimaging, whereas diffuse edema of the white matter and hyperintensity of the internal capsule are seen in MRI in acute decompensation. In fulminant encephalopathy, changes in intensity in the cerebral cortex have been described, suggesting different pathogenic aspects. Purpose: To describe and try to interpret hyperintensities images of the cerebral cortex in fulminant hepatic encephalopathy. Case report: A 42 years old woman, with history of hyperthyroidism treated with propylthiouracil developed abdominal pain, choluria and general malaise, without fever. At admission, she had jaundice, elevated liver enzymes, and hyperammronemia of 481 ug. She developed progressive impairment of consciousness falling into a non-reactive coma with intermediate size and poorly reactive pupils, absence of oculocephalic reflexes and diminished osteotendinous reflexes, with indifferent plantar reflexes. Brain CT showed brain edema without focal lesions. The MRI showed hyperintense signal abnormalities in the fronto-parietal cortex in T2 and FLAIR with restriction in difussion sequency. She became brain dead. Comment: These exceptional images on MRI are considered an expression of cytotoxic damage, consistent with high levels of ammonium in fulminant hepatic encephalopathy. The swelling of astrocytes and cortical neurons is caused by the accumulation of intracellular glutamine, highly osmophilic, and explains the restriction on the difussion and lower values in the ADC. Interstitial edema would be part of the chronic forms by acquisition of compensatory mechanisms capable of preventing the accumulation of glutamine.

Antecedentes: La presencia de depósitos de manganeso en los núcleos basales es una expresión neuroimagenológica de daño hepático crónico, así como el edema difuso de la substancia blanca y la hiperintensidad de la cápsula interna con RM en descompensaciones agudas. En encefalopatías fulminantes se ha visualizado cambio de intensidad en la corteza cerebral, sugiriendo aspectos patogénicos distintos. Propósito: Describir e intentar interpretar imágenes hiperintensas de la corteza cerebral en una encefalopatía hepática fulminante. Caso clínico: Mujer de 42 años, hipertiroidea en tratamiento con propiltiouracilo. Consultó por dolor abdominal, coluria y compromiso del estado general, sin fiebre. Ingresó con ictericia, enzimas hepática elevadas, e hiperamonemia de 481 ug. Desarrolló progresivo compromiso de conciencia cayendo en un coma no reactivo, con pupilas de tamaño intermedio pobremente reactivas, ausencia de reflejos oculocefálicos y reflejos osteotendíneos apagados, con reflejos plantares indiferentes. TAC de cerebro mostró edema cerebral, sin lesiones focales. La RM definió áreas hiperintensas en la corteza fronto-parietal en T2 y FLAIR, que presentaban incremento de señal en la difusión. Evolucionó hasta la muerte cerebral. Comentario: Estas imágenes excepcionales en la RMson consideradas expresión de daño citotóxico, en concordancia con los altos niveles de amonio de una encefalopatía hepática fulminante. El edema de astrocitos y neuronas corticales se origina por la acumulación de glutamina intracelular, altamente osmofílica, y explica la restricción en la difusión y valores menores en el ADC. El edema intersticial, sería atributo de las formas crónicas, debido a la adquisición de mecanismos compensatorios capaces de impedir la acumulación de glutaminas.

Revision and update on clinical practice guideline for liver cirrhosis / 대한간학회지

No abstract available.

Encefalopatia hepática: relatório da 1ª reunião monotemática da Sociedade Brasileira de Hepatologia / Hepatic encephalopathy: report 1st monothematic meeting of the Brazilian Society

A encefalopatia hepática (EH) é um distúrbio funcional do sistema nervoso central (SNC) associado à insufi ciência hepática, de fisiopatologia multifatorial e complexa. Devido aos avanços no conhecimento sobre o manejo da EH na cirrose e na insuficiência hepática aguda (IHA), a diretoria da Sociedade Brasileira de Hepatologia (SBH) promoveu uma reunião monotemática acerca da fi siopatologia, diagnóstico e tratamento da EH, abordando aspectos controversos relacionados ao tema. Com a utilização de sistemática da medicina baseada em evidências, foram abordados o manejo da EH e da hipertensão intracraniana na IHA, o manejo da EH episódica na cirrose, as controvérsias no manejo da EH e a abordagem da EH mínima. O objetivo desta revisão é resumir os principais tópicos discutidos na reunião monotemática e apresentar recomendações sobre o manejo da síndrome votadas pelo painel de expertos da SBH.

Hepatic encephalopathy (HE) is a functional disorder of the central nervous system (CNS) associated with liver failure, either end-stage chronic liver disease or fulminant hepatic failure. Its pathogenesis remains complex and poorly understood. In view of recent advances in the management of HE, the Brazilian Society of Hepatology endorsed a monothematic meetingregarding HE in order to gather experts in the to discuss related data and to draw evidence-based recommendations concerning: management of HE and intracranial hypertension in FHF, treatment of episodic HE in cirrhosis, controversies in the management of EH including difficult to treat cases and diagnostic and treatment challenges for minimal HE. The purpose of this review is to summarize the lectures and recommendations made by the panel of experts of the Brazilian Society of Hepatology.

Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease / Degeneração hepatocerebral adquirida e encefalopatia hepática: correlações e variedade de apresentações clínicas na doença hepática evidente e subclínica

Acquired hepatocerebral degeneration (AHD) and hepatolenticular degeneration can have similar clinical presentations, but when a chronic liver disease and atypical motor findings coexist, the distinction between AHD and hepatic encephalopathy (HE) can be even more complicated. We describe three cases of AHD (two having HE) with different neuroimaging findings, distinct hepatic diseases and similar motor presentations, all presenting chronic arterial hypertension and weight loss before the disease manifestations. The diagnosis and physiopathology are commented upon and compared with previous reports. In conclusion, there are many correlations among HE, hepatolenticular degeneration and AHD, but the overlapping of AHD and HE could be more common depending on the clinical knowledge and diagnostic criteria adopted for each condition. Since AHD is not considered a priority that affects the liver transplant list, the prognosis in AHD patients remains poor, and flow interruption in portosystemic shunts must always be taken into account.

A degeneração hepatocerebral adquirida (AHD) e a degeneração hepatolenticular podem ter apresentações clínicas semelhantes, mas quando uma doença hepática crônica e achados motores atípicos coexistem, a distinção entre AHD e encefalopatia hepática (HE) pode ser ainda mais complicada. Descrevemos três casos de AHD (dois tendo HE) com diferentes achados em neuroimagem, doenças hepáticas distintas e apresentações motoras semelhantes, todos com hipertensão arterial e perda de peso antes das manifestações motoras. O diagnóstico e a fisiopatologia são comentados e comparados com relatos prévios. Concluímos que existem muitas correlações entre HE, degeneração hepatolenticular e AHD, mas a sobreposição de HE e AHD pode ser mais comum dependendo do conhecimento clínico e da acurácia dos critérios diagnósticos adotados para cada enfermidade. Como a AHD não é considerada prioridade na lista de transplante hepático, o prognóstico dos pacientes com AHD permanece ruim, e a interrupção do fluxo nos shunts portossistêmicos deve ser sempre considerada.

Cognitive performance and mood in patients on the waiting list for liver transplantation and their relation to the model for end-stage liver disease / Desempenho cognitivo e humor em pacientes em lista de espera de transplante de fígado e suas relações com modelo para doença hepática e fase terminal

Objective: To investigate the links between depression and cognitive functioning in patients with Hepatitis C and other chronic liver diseases with and without the use of alcohol on the waiting list for liver transplantation and their associations with the MELD classification. Method: 40 patients were evaluated on a waiting list for liver transplant by a battery of neuropsychological tests, depression scales and interview at the Liver Transplant Service, of the Hospital das Clínicas University of São Paulo Medical School. Results: After splitting the sample according to the education, the results showed statistical significance in the comparisons between groups of MELD > 15 and <15 in the following functions: estimated IQ, visual-spatial delayed recall and recognition as part of episodic memory and short term memory. Conclusion: These findings, usually found in hepatic encephalopathy, corroborated with the literature and emphasized the need to investigate in more detail the cognitive functions of these patients in order to facilitate the adoption of different conducts.

Objetivo: Investigar as relações entre depressão e funcionamento cognitivo em pacientes portadores de hepatite C e demais doenças hepáticas crônicas com e sem uso de álcool em fila de espera para transplante hepático e suas relações com a classificação MELD. Método: Foram avaliados 40 pacientes em lista de espera para transplante hepático por bateria de testes neuropsicológicos, escalas de depressão e entrevista no Serviço de Transplante do Fígado do HC-FMUSP. Resultados: Após divisão da amostra por escolaridade os resultados mostraram significância estatística nas comparações entre grupos de MELD > 15 e <15 nas funções: QI estimado, memória episódica de evocação tardia e de reconhecimento visuo-espacial e memória de curto prazo. Conclusão: As dificuldades encontradas, comuns ao quadro de encefalopatia hepática, corroboram a literatura pesquisada e enfatizam a necessidade de se investigar de maneira mais detalhada o funcionamento cognitivo destes pacientes, uma vez que diferentes condutas podem ser adotadas.

Detecção de encefalopatia hepática mínima através de testes neuropsicológicos e neurofisiológicos e o papel da amônia no seu diagnóstico / Minimal hepatic encephalopathy detection by neuropsychological and neurophysiological methods and the role of ammonia for its diagnosis

CONTEXTO: A encefalopatia hepática mínima vem sendo sistematicamente investigada em pacientes com cirrose hepática. Entretanto, existem controvérsias quanto aos melhores métodos, bem como o papel da amônia para seu diagnóstico. OBJETIVO: Avaliar a frequência de encefalopatia hepática mínima diagnosticada através de testes neuropsicológicos e neurofisiológicos em cirróticos, bem como os possíveis fatores de risco para esta condição, incluindo o papel da concentração arterial de amônia em seu diagnóstico. MÉTODOS: Indivíduos com cirrose hepática foram avaliados através do teste de conexão numérica partes A e B (TCN-A e TCN-B) e potencial evocado relacionado a eventos (P300). O diagnóstico de encefalopatia hepática mínima foi feito quando da presença de anormalidade no P300 e em, pelo menos, um dos testes neuropsicológicos. As concentrações arteriais de amônia, a escolaridade e a gravidade da cirrose hepática também foram avaliadas em todos. RESULTADOS: Foram avaliados 48 pacientes cirróticos, com média de idade 50 ± 8 anos, sendo 79 por cento do sexo masculino. As principais causas foram a alcoólica e a viral. O P300 foi anormal em 75 por cento dos casos e o TCN-A e TCN-B anormais em 58 por cento e 65 por cento dos casos, respectivamente. Os resultados do TCN-B foram influenciados pela escolaridade. A frequência de encefalopatia hepática mínima foi de 50 por cento. A concentração arterial de amônia não foi significantemente maior em pacientes com diagnóstico de encefalopatia hepática mínima (195 ± 152 mmol/L versus 148 ± 146 mmol/L; P>0,05). Não houve diferença significante entre os grupos com e sem encefalopatia hepática mínima quanto às demais variáveis estudadas. CONCLUSÃO:A encefalopatia hepática mínima é condição frequente em pacientes com cirrose hepática. A concentração arterial de amônia não parece ter papel importante no seu diagnóstico.

CONTEXT: Minimal hepatic encephalopathy has been systematically investigated in cirrhotic patients. Although, there are controversies regarding the best methods as well as the role of ammonia for its diagnosis. OBJECTIVE: To evaluate the frequency of minimal hepatic encephalopathy diagnosed by neuropsychological and neurophysiological methods in cirrhotic patients, as well as possible associated risk factors for this condition, including the role of arterial ammonia concentrations for its diagnosis. METHODS: Cirrhotic patients were evaluated by the number connection test parts A and B (NCT-A and NCT-B), and auditory evoked-related potentials (P300). Minimal hepatic encephalopathy was diagnosed by the presence of abnormal P300 and in unless one of the performed neuropsychologic tests. Arterial ammonia concentration, scholarity and cirrhosis severity accessed by Child-Pugh classification were evaluated in all. RESULTS: Forty-eight cirrhotic patients were evaluated, with median age 50 ± 8 years old, 79 percent male. The main etiologies were alcoholic and viral. The P300 was abnormal in 75 percent of cases, while NCT-A and NCT-B were abnormal in 58 percent and 65 percent, respectively. The NCT-B results were influenced by scholarity. The minimal hepatic encephalopathy frequency was 50 percent. Arterial ammonia concentration was not significantly increased in minimal hepatic encephalopathy diagnosed patients (195 ± 152 mmol/L versus 148 ± 146 mmol/L; P>0,05). There was no difference between groups with or without minimal hepatic encephalopathy in the other studied variables. CONCLUSION: Minimal hepatic encephalopathy is a frequent condition in cirrhotic patients. The arterial ammonia concentration does not play a major role in its diagnosis.

Avaliação dos potenciais evocados relacionados a eventos (ERP-P300) em pacientes com cirrose hepática sem encefalopatia / Evaluation of the event-related potential (ERP-P300) in patients with hepatic cirrhosis without encephalopathy

RACIONAL: Na cirrose hepática ocorrem alterações na estrutura do fígado, levando à perda das funções do órgão, com conseqüências neuropsiquiátricas, como disfunções cognitivas. Um dos meios mais efetivos para avaliar objetivamente as funções cognitivas é medir a atividade eletrofisiológica do sistema nervoso central através do potencial evocado relacionado a eventos (ERP-P300). OBJETIVO: Estudar a utilidade do potencial evocado relacionado a eventos (ERP), para avaliar distúrbios cognitivos em pacientes com cirrose hepática e como auxiliar no diagnóstico da encefalopatia hepática mínima. MÉTODO: Foram selecionados 50 pacientes, diagnosticados com cirrose hepática sem sinais clínicos de encefalopatia hepática e 35 voluntários saudáveis de idades e sexo semelhantes. Em todos os pacientes foram realizados exames clínico-neurológico e laboratoriais. Para identificação do prejuízo cognitivo foi utilizado o ERP-P300 e obtida a média da latência da onda P300. RESULTADOS: Houve diferença significativa entre as médias da latência do ERP-P300 do grupo cirrótico e controle. CONCLUSÃO: A realização do ERP-P300 é simples, depende de fatores controláveis de variação e tem fácil reprodutibilidade, podendo ser útil para o rastreio de distúrbios cognitivos em pacientes com cirrose e auxiliar no diagnóstico de encefalopatia hepática mínima.

BACKGROUND: In hepatic cirrhosis structural liver alterations occur leading to the loss of the organ functions with neuro-psychiatric consequence, as cognitive dysfunctions. One of the most effective ways of objectively evaluating cognition is to measure electrophysiological activity in the central nervous system trough event-related potentials (ERP-P300). AIM: To assess the value of the event-related potential (ERP) in order to determine cognitive disturbances in patients with liver cirrhosis and to assist in the diagnosis of minimal hepatic encephalopathy. METHODS: Fifty patients with liver cirrhosis were selected, without clinical symptoms of hepatic encephalopathy and 35 healthy volunteers, matched by sex and age. The patients were submitted to clinical-neurological and laboratorial examination. The ERP-P300 was performed by the two groups to determine cognitive disturbances. RESULTS: The study showed significant differences between the ERP-P300 latency averages of the two groups. CONCLUSION: The ERP-P300 is simple to use and depends on controllable variables. It is also easy to reproduce and, when properly used, can be useful both to determine cognitive disturbances in patients with hepatic cirrhosis and to assist in minimal hepatic encephalopathy diagnosis.

Minimal hepatic encephalopathy: time to recognise and treat.

Minimal hepatic encephalopathy represents a part of the spectrum of hepatic encephalopathy and is the mildest form. While patients with hepatic encephalopathy have impaired intellectual functioning, personality changes, altered levels of consciousness, and neuromuscular dysfunction, patients with minimal hepatic encephalopathy have no recognisable clinical symptoms of hepatic encephalopathy but have mild cognitive and psychomotor deficits. The prevalence of minimal hepatic encephalopathy has been reported to vary between 30% and 84% in patients with liver cirrhosis and is higher in patients with poor liver function. The diagnosis is usually made by neuropsychological and/or neurophysiological testing in cirrhotic patients who are otherwise normal on neurological examination. Minimal hepatic encephalopathy is a clinically significant disorder that impairs the health-related quality of life, predicts the development of overt encephalopathy and is probably associated with a poor prognosis. Thus screening all patients with cirrhosis for minimal hepatic encephalopathy using psychometric testing is recommended. Pharmacologic therapy is recommended for patients diagnosed with minimal hepatic encephalopathy. The pathogenesis of minimal hepatic encephalopathy is considered similar to that of overt hepatic encephalopathy and ammonia plays a key role. Thus ammonia lowering agents such as lactulose, L-ornithine and L-aspartate that have good safety profiles are recommended. Future studies will better define the role of probiotics, levocarnitine and sodium benzoate.