Adult ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hepatic Encephalopathy ; diagnosis ; etiology ; psychology ; Humans ; Intelligence Tests ; Male ; Middle Aged ; Neuropsychological Tests

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.
Aged ; Brain/diagnostic imaging ; Electroencephalography ; Female ; Hepatic Encephalopathy/*diagnosis ; Humans ; Magnetic Resonance Imaging ; Pedigree ; Telangiectasia, Hereditary Hemorrhagic/*diagnosis ; Tomography, X-Ray Computed ; Vascular Malformations/etiology

BACKGROUND/AIMS: Minimal hepatic encephalopthy in patients with clinically asymptomatic chronic progressive liver disease may have adverse effects on daily activity. We evaluated the differences in the cognitive function of patients with chronic hepatitis and liver cirrhosis group according to the Child-Pugh classification. METHODS: We enrolled 61 consecutive chronic liver disease patients. We used the following study instruments: visual continuous performance test, a spatial memory test, the Wisconsin card-sorting test chosen from Neuroscan and STIM system (Study of the Usefulness of Computerized Neuropsychological Test, Neurosoft company, New York, NY, USA), a global-local processing test and an electroencephalogram (EEG). RESULTS: A significant correlation was found between neurologic abnormalities and the degree of liver disease. The result of the neuropsychological test and the EEG showed that cognitive function decreased according to the severity of chronic liver disease, especially in liver cirrhosis. Cirrhotic patients, especially in Child-Pugh C group, exhibited selective deficits in complex attention and fine motor skills as well as visual spatial perception, with preservation of memory. CONCLUSIONS: The STIM and EEG are simple, subjective and reproducible methods and may be used as early detection methods of minimal hepatic encephalopthy.
Adult ; Chronic Disease ; Cognition ; *Electroencephalography ; Female ; Hepatic Encephalopathy/*diagnosis/etiology ; Hepatitis, Viral, Human/complications ; Humans ; Liver Cirrhosis/complications ; Male ; Middle Aged ; *Neuropsychological Tests

OBJECTIVETo identify the risk factors of early post-TIPS hepatic encephalopathy (HE) and the long-time survival of patients with or without early post-TIPS HE.

METHODSConsecutive cirrhotic patients who underwent TIPS for variceal rebleeding or refractory ascites in our center from January 2003 to December 2008 were included in this study. More than 60 clinical characteristics were enrolled in univariate analysis and logistic regression analysis to define the risk factors of HE in 3 months after TIPS procedure (early post-TIPS HE). The long-time survival of patients with or without early post-TIPS HE was compared by Cox regression with several covariates.

RESULTSAccording to our inclusion criteria, 190 patients were included. The median follow-up was 30.5 months. Lower serum concentration of fibrinogen and higher Child-Pugh score were the independent risk factors for suffering early post-TIPS HE. Patients without early post-TIPS HE after TIPS showed better prognosis than those with early post-TIPS HE after TIPS (P = 0.044).

CONCLUSIONPatients with lower serum fibrinogen and higher Child-Pugh score before TIPS might be more probably attacked by early post-TIPS HE which indicated worse long-term survival.

Adult ; Female ; Fibrinogen ; analysis ; Follow-Up Studies ; Hepatic Encephalopathy ; diagnosis ; etiology ; Humans ; Male ; Middle Aged ; Portasystemic Shunt, Transjugular Intrahepatic ; adverse effects ; Prognosis ; Risk Factors

Ascites, hepatic encephalopathy and variceal hemorrhage are three major complications of portal hypertension. The diagnostic evaluation of ascites involves an assessment of its etiology by determining the serum-ascites albumin gradient and the exclusion of spontaneous bacterial peritonitis. Ascites is primarily related to an inability to excrete an adequate amount of sodium into urine, leading to a positive sodium balance. Sodium restriction and diuretic therapy are keys of ascites control. But, with the case of refractory ascites, large volume paracentesis and transjugular portosystemic shunts are required. In hepatorenal syndrome, splanchnic vasodilatation with reduction in effective arterial volume causes intense renal vasoconstriction. Splanchnic and/or peripheral vasoconstrictors with albumin infusion, and renal replacement therapy are only bridging therapy. Liver transplantation is the only definitive modality of improving the long term prognosis.
Anti-Bacterial Agents/therapeutic use ; Ascites/complications/*diagnosis/therapy ; Bacterial Infections/*diagnosis ; Hepatic Encephalopathy/complications ; Hepatorenal Syndrome/complications/*diagnosis/therapy ; Humans ; Hypertension, Portal/*complications ; Liver Transplantation ; Peritonitis/*diagnosis/drug therapy/etiology ; Serum Albumin/administration & dosage

Adult ; Female ; Hepatic Encephalopathy ; diagnosis ; etiology ; therapy ; Hepatitis B, Chronic ; complications ; therapy ; Humans ; Liver Failure, Acute ; diagnosis ; therapy ; Liver, Artificial ; Male ; Middle Aged ; Multiple Organ Failure ; complications ; therapy ; Prognosis ; Sorption Detoxification

Cryptococcus neoformans, an encapsulated fungus, is an important opportunistic pathogen that can cause meningitis in immunocompromised patients. Since patients with cryptococcemia have high mortality, it is essential to make an early diagnosis and promptly initiate antifungal therapy. However, it is often very difficult to differentiate between cryptococcal meningitis and hepatic encephalopathy in patients with liver cirrhosis, and there is delay in making the diagnosis. Therefore, these patients have a particularly grave prognosis and consequently many patients die before culture results become available. In one study, starting antifungal therapy within 48 hours of the blood culture was associated with improved survival, but patients with liver cirrhosis were significantly less likely to receive antifungal therapy within 48 hours compared to those without liver cirrhosis. Recently, the authors experience a case of a 68-year-old woman with liver cirrhosis who presented with fever and a drowsy mental status. She had a previous history of having been admitted for infection-associated hepatic encephlopathy. Cryptococcal meningitis and cryptococcemia were diagnosed by spinal puncture and culture of cerebrospinal fluid. In spite of adequate treatment, the patient developed multi-system organ failure and eventually expired. Herein, we report a case of cryptococcal meningitis mimicking hepatic encephalopathy in a patient with liver cirrhosis.
Aged, 80 and over ; Brain/radiography ; Cryptococcus/isolation & purification ; Female ; Hepatic Encephalopathy/complications/*diagnosis ; Hepatitis C, Chronic/complications/pathology ; Humans ; Liver Cirrhosis/etiology/pathology ; Meningitis, Cryptococcal/complications/*diagnosis/microbiology ; Tomography, X-Ray Computed

BACKGROUNDBalanced adjustment of the portal vein shunt volume during a transjugular intrahepatic portosystemic shunt (TIPS) is critical for maintaining liver perfusion and decreasing the incidence of liver insufficiency. A stent-graft is proved to be superior to a bare metal stent (BMS) for the construction of a TIPS. However, the clinical results of the combination application of stents and stent-grafts have not been determined. This study aimed to compare the technique of using a combination of stents and stent-grafts with using a single stent-graft to construct a TIPS.

METHODSFrom April 2011 to November 2014, a total of fifty patients were randomly assigned to a stents-combination group (Group I, n = 28) or a stent-graft group (Group II, n = 22). Primary patency rates were calculated. Clinical data, including the technical success rate, bleeding control results, incidence of encephalopathy, liver function preservation, and survival rate, were assessed.

RESULTSTechnically, the success rate was 100% for both groups. The primary patency rates at 1, 2, and 3 years for Group I were 96%, 84%, and 77%, respectively; for Group II, they were 90%, 90%, and 78%, respectively. The survival rates at 1, 2, and 3 years for Group I were 79%, 74%, and 68%, respectively; for Group II, they were 82%, 82%, and 74%, respectively. The incidence of hepatic encephalopathy was 14.3% for Group I and 13.6% for Group II. The Child-Pugh score in Group I was stable at the end of the follow-up but had significantly increased in Group II (t = -2.474, P = 0.022).

CONCLUSIONSThe construction of a TIPS with either the single stent-graft or BMS/stent-graft combination is effective for controlling variceal bleeding. The BMS/stent-graft combination technique is superior to the stent-graft technique in terms of hepatic function preservation indicated by the Child-Pugh score. However, considering the clinical results of the TIPS, the two techniques are comparable in their primary shunt patency, incidence of encephalopathy and patient survival during the long-term follow-up.

Aged ; Female ; Hepatic Encephalopathy ; diagnosis ; etiology ; Humans ; Hypertension, Portal ; complications ; mortality ; surgery ; Male ; Middle Aged ; Polytetrafluoroethylene ; Portal Vein ; surgery ; Portasystemic Shunt, Transjugular Intrahepatic ; adverse effects ; methods ; Postoperative Complications ; Prospective Studies ; Stents ; Treatment Outcome

Adult ; Aged ; Aged, 80 and over ; Female ; Follow-Up Studies ; Hepatic Encephalopathy ; blood ; diagnosis ; etiology ; Humans ; Insulin-Like Growth Factor Binding Protein 1 ; Insulin-Like Growth Factor Binding Proteins ; blood ; Liver Cirrhosis ; complications ; Male ; Middle Aged ; Neuropsychological Tests ; Pregnancy Proteins ; blood

We report on a 55-year-old man with alcoholic liver cirrhosis who presented with status epilepticus. Laboratory analysis showed markedly elevated blood ammonia. Brain magnetic resonance imaging (MRI) showed widespread cortical signal changes with restricted diffusion, involving both temporo-fronto-parietal cortex, while the perirolandic regions and occipital cortex were uniquely spared. A follow-up brain MRI demonstrated diffuse cortical atrophy with increased signals on T1-weighted images in both the basal ganglia and temporal lobe cortex, representing cortical laminar necrosis. We suggest that the brain lesions, in our case, represent a consequence of toxic effect of ammonia.
Ammonia/blood ; Atrophy/pathology ; Brain Diseases/blood/*diagnosis/*etiology ; Hepatic Encephalopathy/*complications ; Humans ; Liver Cirrhosis, Alcoholic/*complications ; Magnetic Resonance Imaging/*methods ; Male ; Middle Aged ; Necrosis/pathology ; Status Epilepticus/pathology