Symptoms Of Encephalopathy Research Articles

Acute necrotizing encephalopathy in children: A case report and literature review

Acute necrotizing encephalopathy (ANE) is a rare clinical-imaging syndrome with unknown etiology, characterized by acute fulminant severe encephalopathy and brain damage with multifocal symmetry. ANE has no specific clinical symptoms and signs, similar to common encephalitis or encephalopathy symptoms. The characteristic brain imaging examination is diagnostically significant. To date, no specific treatment for ANE is available and the prognosis is poor. Here, we reported a typical case of ANE in a child, and where a good outcome was achieved through combined therapy with immunoglobulin and glucocorticoids.

A case of hepatic encephalopathy in an young female due to acute hepatitis e followed by complete recovery: A rare case report

Hepatic encephalopathy is a fatal complication of both chronic liver disease and acute hepatocellular failure. Usually, in such cases, the disease has a rapid progression and fatal prognosis. We report a young female who was admitted in Medical College, Kolkata, India, in 2016 with clinical and biochemical evidence of encephalopathy and coagulopathy (hyperacute liver failure) following an acute hepatitis-like illness. Blood for hepatitis B surface antigen, anti-hepatitis C virus, IgM-hepatitis A virus, and IgM anti-Leptospira all were negative, but anti-hepatitis E virus (HEV) IgM came out to be positive (titer of 6.8 U/ml; whereas 1.1 – positive). IgM anti-HEV was tested using commercially available ELISA kits (Genelabs) using sandwich ELISA technique. The patient was aggressively managed with intravenous fluid, antibiotics, lactulose syrup and enema, and transfusion of fresh frozen plasma along with strict monitoring for other vital organ damage. Symptoms of encephalopathy completely reversed within 5–6 days, and prothrombin time and liver enzymes were normalized gradually. Although acute liver failure with high mortality is common in acute hepatitis E in pregnancy, here, in our case, it happened to a young nonpregnant female followed by complete recovery.

Lactulose decreases neuronal activation and attenuates motor behavioral deficits in hyperammonemic rats

Lactulose is a nonabsorbable disaccharide commonly used in clinical practice to treat hepatic encephalopathy. However, its effects on neuropsychiatric disorders and motor behavior have not been fully elucidated. Male Wistar rats were bile-duct ligated, and 3weeks after surgery, treated with lactulose administrated by gavage (1.43 or 3.57g/kg), once a day for seven days. Plasma levels of ammonia, aspartate aminotransferase, total bilirubin, and creatinine were quantified and histopathological analysis of the livers was performed. Locomotor activity measurements were performed in an open field. The expression of water channel aquaporin-4 was investigated and the analysis of Fos protein immunoreactivity was used to evaluate the pattern of neural activation in brain areas related to motor behavior. Bile-duct ligated rats showed hyperammonemia, loss of liver integrity and function, impaired locomotor activity, reduced aquaporin-4 protein expression, and neuronal hyperactivity. Lactulose treatment was able to reduce ammonia plasma levels, despite not having an effect on biochemical parameters of liver function, such as aspartate aminotransferase activity and total bilirubin levels, or on the cirrhotic hepatic architecture. Lactulose was also able to reduce the locomotor activity impairments and to mitigate or reverse most changes in neuronal activation. Lactulose had no effect on reduced aquaporin-4 protein expression. Our findings confirm the effectiveness of lactulose in reducing hyperammonemia and neuronal hyperactivity in brain areas related to motor behavior, reinforcing the importance of its clinical use in the treatment of the symptoms of cirrhosis-associated encephalopathy.

Nivolumab as salvage treatment in a patient with HIV-related relapsed/refractory Hodgkin lymphoma and liver failure with encephalopathy

BackgroundWe report the first case to our knowledge of a patient with relapsed/refractory classical hodgkin lymphoma and liver failure with encephalopathy along with human immunodeficiency virus/acquired immunodeficiency syndrome infection, successfully treated with nivolumab without major side effects and encouraging prolonged disease control.Case presentationIn December 2015, at the time of the patient’s progression from his Hodgkin lymphoma after fourth line treatment, he developed persistent fevers, abdominal distension, jaundice and worsening of his liver function tests. Magnetic resonance imaging of abdomen/pelvis demonstrated hepatomegaly with innumerable new liver lesions, splenomegaly with multiple splenic nodules and several new mediastinal, intraperitoneal and retroperitoneal lymphadenopathy. In accordance with the patient’s wishes before admission, and after agreement with the family, nivolumab (3 mg/kg every 2 weeks) was given. Of note, antiretroviral therapy was on hold due to liver function tests, his viral load was undectable and cluster of differentiation 4 counts were 103/uL at the time of nivolumab administration. One week after the first dose of nivolumab both his hepatic encephalopathy and constitutional symptoms started to improve, and after 2 doses, (January 2016) his LFTs were almost back to normal. After 5 months of nivolumab treatment (10 doses), restaging (computerized tomography scans of neck, chest, abdomen, pelvis) done on May 2016 showed resolution of hepatosplenomegaly with two residual small hepatic lesions, heterogeneous spleen with no splenic lesions, and stable non-enlarged retroperitoneal lymph nodes without intraabdominal lymphadenopathy; consistent with partial response.ConclusionsWe report a case of a patient with human immunodeficiency virus/acquired immunodeficiency syndrome -related relapsed/refractory classical Hodgkin lymphoma and acute liver failure with encephalopathy successfully treated with nivolumab after failing all standard therapeutic options. Unlike classic cytotoxic chemotherapy, which relies on preserved organ function to ameliorate potential severe side effects (i.e. myelosuppression), elimination of monoclonal antibodies is fairly independent of baseline renal and hepatic function since they are usually metabolized by circulating phagocytes and/or by their target antigen-expressing cell.

Hypertensive Encephalopathy: A Case of a Male Who Bit Off His Fingers

Although altered consciousness and other neurologic manifestations are frequently seen in hypertensive encephalopathy, behavioral and psychotic symptoms are rarely seen. We describe a patient with no previous psychiatric history who was admitted for hypertensive crisis. A few days after admission, his blood pressure remained uncontrolled and he started to exhibit episodes of confusion, agitation, and psychosis. During one particular episode, he overcame multiple staff members and physical restraints to bite off two of his fingers without any signs of pain. Brain computed tomography (CT) was notable for possible posterior cerebral and cerebellar edema. His confusion and agitation gradually resolved with successful blood pressure management. This is the first reported case of extreme, agitated behaviors and auditory hallucinations in a patient with hypertensive crisis.

Clinical correlates to assist with chronic traumatic encephalopathy diagnosis: Insights from a novel rodent repeat concussion model.

Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease linked to repetitive head injuries. Chronic traumatic encephalopathy symptoms include changes in mood, behavior, cognition, and motor function; however, CTE is currently diagnosed only postmortem. Using a rat model of recurrent traumatic brain injury (TBI), we demonstrate rodent deficits that predict the severity of CTE-like brain pathology. Bilateral, closed-skull, mild TBI was administered once per week to 35 wild-type rats; eight rats received two injuries (2×TBI), 27 rats received five injuries (5×TBI), and 13 rats were sham controls. To determine clinical correlates for CTE diagnosis, TBI rats were separated based on the severity of rotarod deficits and classified as "mild" or "severe" and further separated into "acute," "short," and "long" based on age at euthanasia (90, 144, and 235 days, respectively). Brain atrophy, phosphorylated tau, and inflammation were assessed. All eight 2×TBI cases had mild rotarod deficiency, 11 5×TBI cases had mild deficiency, and 16 cases had severe deficiency. In one cohort of rats, tested at approximately 235 days of age, balance, rearing, and grip strength were significantly worse in the severe group relative to both sham and mild groups. At the acute time period, cortical thinning, phosphorylated tau, and inflammation were not observed in either TBI group, whereas corpus callosum thinning was observed in both TBI groups. At later time points, atrophy, tau pathology, and inflammation were increased in mild and severe TBI groups in the cortex and corpus callosum, relative to sham controls. These injury effects were exacerbated over time in the severe TBI group in the corpus callosum. Our model of repeat mild TBI suggests that permanent deficits in specific motor function tests correlate with CTE-like brain pathology. Assessing balance and motor coordination over time may predict CTE diagnosis.

Association of Hyponatremia and Encephalopathy in Patients with Chronic Liver Disease

<p><strong>Objective</strong>: To determine the association of hyponatremia in chronic liver disease patients with presentation of hepatic encephalopathy (HE) at Isra university Hospital Hyderabad.<strong></strong></p><p><strong>Methods</strong>: This study was cross sectional and carried out at medicine and gastroenterology department of Isra university Hospital Hyderabad and MMC Mirpurkhas. Study duration was from September 2015 to March 2016. All the cases with age more than 25 years, both genders after diagnosis of chronic liver disease with sign and symptoms of Hepatic Encephalopathy were selected in this study. Blood sample for serum sodium level, was send to diagnostic laboratory of the hospital. Hyponatremia was classified as: 130–135 mEqL (mild), 125–130 mEq/L (moderate) and < 125 mEq/L (severe). Hepatic encephalopathy was categorized according to the West Haven classification (4 grades).</p><p> </p><p><strong>Results:</strong><strong> </strong>Total number of cases was 80. Mean age was 48 + 8.5 years. 55 (68.5%) patients were male. Majority of cases, 55 (68.5%) patients were infected with HCV. 40% patients had normal level of sodium, 25% had mild, 20% had moderate while 15% patients had severe hyponatremia. On the grading of encephalopathy, 30 (37.5%) patients had grade 1 encephalopathy while 25/(31.2%) patients had grade 2, 15/(18.8%) had grade 3 while 10 (12.5%) patients hade grade 4. The serum sodium levels significantly decreased with increasing severity of hepatic encephalopathy (P = 0.001).</p><p><strong>Conclusion:</strong><strong> </strong>We concluded that the hyponatremia is significantly linked with Hepatic Encephalopathy (HE) in patients having chronic liver disease (CLD).</p>

Sepsis-Associated Encephalopathy (Review)

International studies demonstrate an annual increase in the frequency and impact of sepsis in intensive care units (ICU). Cerebral dysfunction, or so-called sepsis-associated encephalopathy (SAE), is one of the earliest signs of sepsis, as well as its common complication. Up to 70% of patients with sepsis have symptoms of encephalopathy [1, 2]. A direct link between the SAE and an increased mortality rate is a major concern; more than a half of sepsis survivors experience continuous memory and concentration impairment [3—5]. Diagnosis of the cerebral dysfunction in sepsis is often difficult because of lack of specific biomarkers and frequent prescription of sedatives to critically ill patients. Clinical manifestations of SAE are diverse, may vary from simple malaise and lack of concentration to deep coma. The literature discusses probable mechanisms of formation and development of septic encephalopathy, such as oxidative stress, inflammation, mitochondrial and endothelial dysfunction, increased permeability of the blood-brain barrier, impairment of macro- and microcirculation, changes in neirotransmission, activation of microglia. The lack of clear data and consensus about the etiology and mechanisms of SAE at present does not permit predicting its development and prescribing a specific therapy. The review discusses current understanding of the causes of septic encephalopathy, methods of diagnosis, the main clinical manifestations, key mediators, and pathophysiological mechanisms. A hypothesis is proposed that poses a contribution of aromatic microbial metabolites (AMM) of phenol and indole nature (products of bacterial biodegradation of tyrosine and tryptophan) to the development of brain dysfunction. The fields of exploratory studies, which might open perspectives in the diagnosis, as well as new approaches in the treatment of SAE are outlined.

TYPHOID FEVER WITH FATAL OUTCOME IN PEOPLE WITH SEVERE TROPHOLOGICAL FAILURE IN EXTREME CONDITIONS

For purpose of clinical and morphological characterization of typhoid fever with fatal outcome was perform analysis of medical documentation as well as postmortem studies of internal tissues in 36men aged 21 to 34 years with severe body weight loss and in the conditions of combat stress in period from 1983 to 1985. The results of the study find a number of clinical and pathomorphological features of typhoid fever in patients with body weight deficiency that were in extreme conditions. Focal changes of the central nervous system were detected. The defeat of the respiratory system was characterized from the first days of the disease by bronchitis, and in the subsequent development of pneumonia, plevritis and empyema of the pleura. Majority of patients had symptoms of cardiovascular and renal failure. In died in the first week of the disease and in 2/3 parts – in a later period were combined with the symptoms of infectious-toxic encephalopathy and severe acute respiratory failure. All died had postmortem dystrophic changes of cardiomyocytes, and in patients had fatal outcome in 2–5 weeks – also focal or diffuse myocarditis. In a third of the patients who died, the disease was complicated by intestinal perforations. In a number of cases, peritonitis was a consequence of necrosis of mesenteric lymph nodes. In all patients with a fatal outcome, hemorrhagic (thrombohemorrhagic) syndrome was diagnosed. A significant proportion of patients posthumously detected signs of sepsis and other complications, many of which could be the cause of death.

Perspectives for cognitive rehabilitation of patients with diabetes mellitus

Currently, the problem of cognitive dysfunction is becoming increasingly important due to the raising demand for effective intellectual activity in modern society. One of the most significant causes of cognitive dysfunction is dismetabolic nature of the disorder, such as diabetes mellitus, which has recently been gaining prevalence. Much of the resistance of clinical symptoms of diabetic encephalopathy to conventional therapy requires a search for new approaches for solving this problem. Cognitive rehabilitation as a correctional technique has proved a positive effect in terms of the treatment of neurodegenerative diseases of different nature.This review present the ways for correction of cognitive impairment using the method of cognitive rehabilitation in patients with diabetes, its methodology, mechanisms of action and perspectives.

Tumor necrosis factor-α modifies the effects of Shiga toxin on glial cells

Shiga toxin (STX) is one of the main factors inducing hemorrhagic colitis and hemolytic-uremic syndrome (HUS) in infections with STX-producing Escherichia coli (STEC). Approximately 62% of patients with HUS showed symptoms of encephalopathy in the 2011 Japanese outbreak of STEC infections. At that time, we reported elevated serum concentrations of tumor necrosis factor (TNF)-α in patients with acute encephalopathy during the HUS phase. In the current study, we investigated whether TNF-α augments the effects of STX in glial cell lines and primary glial cells. We found that TNF-α alone or STX in combination with TNF-α activates nuclear factor-κB (NF-κB) signaling and inhibits growth of glial cells. The magnitude of the NF-κB activation and the inhibition of cell growth by the STX and TNF-α combination was greater than that obtained with TNF-α alone or STX alone. Thus, this in vitro study reveals the role of TNF-α in glial cells during STEC infections.

Being an informal caregiver for a relative with liver cirrhosis and overt hepatic encephalopathy: a phenomenological study

To explore the experiences of being an informal caregiver for a relative with liver cirrhosis and overt hepatic encephalopathy. Overt hepatic encephalopathy is a common complication in patients with liver cirrhosis. It is associated with decreased quality of life for patients, and presents a major burden for caregivers. The involvement of informal caregivers in medical care is recommended, but it has not been clearly described. An understanding of the experience of caregivers is needed to improve the support provided to them by healthcare professionals. A qualitative, interpretative, phenomenological approach was used. Twelve informal caregivers participated in qualitative interviews. The analysis followed the six steps of the interpretative phenomenological approach. Caregivers' experiences were described using five themes: (1) feeling overwhelmed by their loved one having unexplainable symptoms and behaviours; (2) learning that this and previous experiences were complications of liver disease; (3) becoming aware of the symptoms of hepatic encephalopathy; (4) having feelings of being tied down and (5) experiencing and overcoming obstacles in working with healthcare professionals. This study provides insight into caregivers' experiences and the consequences for their lives. The first occurrence of symptoms was a shock, but receiving the diagnosis was seen as an important step in understanding and learning. Caregivers provide daily assessments of their relatives' conditions, and they feel responsible for medication management. Over time, the caregivers impressively showed how they were able to incorporate their personal experiences into caregiving and to accept more accountability in managing the disease. Nurses should acknowledge caregivers as experts in caring for their loved ones. Nurses can assist caregivers in managing an episode of hepatic encephalopathy and can provide individualised interventions to ease the future burden.

Implications and benefits of the use of rifaximin for preventing episodes of hepatic encephalopathy

108 British Journal of Healthcare Management 2016 Vol 22 No 3 © 2 01 6 M A H ea lth ca re L td Encephalopathy has a wide manifestation of signs and symptoms, from non-specific personality changes to unresponsiveness and coma. Recurrences are frequent after an overt episode of encephalopathy, with a 40% recurrence rate within 6 months. In the USA, encephalopathy is the main reason for 110,000 hospitalizations annually (Stepanova et al, 2009), while there are no such figures available in the UK. The most widely used treatment for encephalopathy is lactulose, which is a laxative in liquid form. The efficacy of lactulose, however, is not universal, and many patients develop recurrent episodes of encephalopathy requiring hospitalisation on this drug. Moreover, increasing doses to control encephalopathy symptoms are associated with diarrhoea. Therefore, the treatment of patients with recurrent hepatic encephalopathy despite the use of lactulose remains an unmet clinical need. A randomised, double-blind, placebo-controlled trial has shown that rifaximin significantly reduces the episodes of recurrent encephalopathy in adult patients with two or more previous episodes of overt encephalopathy in the last 6 months as compared with placebo (Bass et al, 2010). Rifaximin, although effective, is costly with the estimated cost of a 6-month treatment being £1689.65. Recently, the National Institute for Health and Care Excellence (NICE) (2015) technology appraisal guidance TA337 addressed the use of rifaximin for preventing episodes of overt hepatic encephalopathy in patients with cirrhosis. The guidance followed 4 evidence submissions by the manufacturing company, each of which provided alternative modeling for costEmmanuel A Tsochatzis, Senior Clinical Lecturer and Honorary Consultant, UCL Institute for Liver and Digestive Health, Royal Free Hospital, London, UK Implications and benefits of the use of rifaximin for preventing episodes of hepatic encephalopathy

Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience.

Hyperammonemia is a rare, often fatal complication after transplantation. The etiology is unknown, but recognition and rapid treatment may help to improve the survival of this unusual syndrome. We present the largest case series to date of hyperammonemia after lung transplantation (LTx) and discuss a treatment protocol that has been developed at our institution. We conducted a retrospective cohort series of patients who underwent LTx between January 1, 2000, and December 31, 2013. Patients who developed hyperammonemia syndrome in the posttransplantation period, which was defined as symptoms of encephalopathy and plasma ammonia level exceeding 200 μmol/L on at least 1 occasion, were included. Data including demographics, antimicrobial and immunosuppression regimens, ammonia levels and other pertinent laboratory data, treatments administered, and outcomes were recorded. Eight of 807 lung transplant recipients developed hyperammonemia syndrome postoperatively during this time period. Median time to onset was 9.0 days, and median peak ammonia level was 370 μmol/L. All 8 patients were treated with hemodialysis, 7 of 8 patients were treated with bowel decontamination, and 5 of 8 patients were treated with nitrogen scavenging agents. Six of the 8 patients died. The incidence of hyperammonemia syndrome in LTx patients was approximately 1%. Future research is needed to determine the efficacy of treatment, including hemodialysis, bowel decontamination, antibiotics, and the use of nitrogen scavenging agents in lung recipients with hyperammonemia.

Reversible cortical blindness in a case of hepatic encephalopathy

Hepatic encephalopathy is a frequent and often fatal manifestation of chronic liver disease. The pathogenesis of hepatic encephalopathy is believed to be multifactorial including impaired blood-brain barrier function, imbalance between the excitatory and inhibitory neurotransmitters in cortex, accumulation of various toxic and false neurotransmitters, and lack of nutrients like oxygen and glucose. Signs and symptoms of hepatic encephalopathy varies and commonly ranges from personality changes, disturbed consciousness, sleep pattern alternation, intellectual deterioration, speech disturbances, asterixis to frank coma and even death. Reversible or transient cortical blindness is rare manifestation of hepatic encephalopathy. It may even precede the phase of altered consciousness in such patients. Very few similar cases have been reported worldwide. Hence, we would like to report a case of transient cortical blindness in a patient of hepatic encephalopathy.

Neurotoxic Weapons and Syndromes.

The modern era of chemical and biological warfare began in World War I with the large-scale production and use of blistering and choking agents (chlorine, phosgene and mustard gases) in the battlefield. International treaties (the 1925 Geneva Protocol, the 1975 Biological and Toxin Weapons Convention and the 1993 Chemical Weapons Convention) banned biological and chemical weapons. However, several countries are probably still engaged in their development. Hence, there is risk of these weapons being used in the future. This chapter will focus on neurotoxic weapons (e.g. nerve agents, chemical and biological neurotoxins, psychostimulants), which act specifically or preeminently on the central nervous system and/or the neuromuscular junction. Deeply affecting the function of the nervous system, these agents either have incapacitating effects or cause clusters of casualties who manifest primary symptoms of encephalopathy, seizures, muscle paralysis and respiratory failure. The neurologist should be prepared both to notice patterns of symptoms and signs that are sufficiently consistent to raise the alarm of neurotoxic attacks and to define specific therapeutic interventions. Additionally, extensive knowledge on neurotoxic syndromes should stimulate scientific research to produce more effective antidotes and antibodies (which are still lacking for most neurotoxic weapons) for rapid administration in aerosolized forms in the case of terrorist or warfare scenarios.

Diagnosis and Genetic Analysis of Glutaric Acidaemia Type I: Very rarely seen inborn error of metabolism.

Diagnosis and Genetic Analysis of Glutaric Acidaemia Type I: Very rarely seen inborn error of metabolism.

The critical role of lipopolysaccharide in the upregulation of aquaporin 4 in glial cells treated with Shiga toxin.

BackgroundIn 2011, there was an outbreak of Shiga toxin-producing Escherichia coli (STEC) infections in Japan. Approximately 62 % of patients with hemolytic-uremic syndrome also showed symptoms of encephalopathy. To determine the mechanisms of onset for encephalopathy during STEC infections, we conducted an in vitro study with glial cell lines and primary glial cells.ResultsShiga toxin 2 (Stx-2) in combination with lipopolysaccharide (LPS), or LPS alone activates nuclear factor-κB (NF-κB) signaling in glial cells. Similarly, Stx-2 in combination with LPS, or LPS alone increases expression levels of aquaporin 4 (AQP4) in glial cells. It is possible that overexpression of AQP4 results in a rapid and increased influx of osmotic water across the plasma membrane into cells, thereby inducing cell swelling and cerebral edema.ConclusionsWe have showed that a combination of Stx-2 and LPS induced apoptosis of glial cells recently. Glial cells are indispensable for cerebral homeostasis; therefore, their dysfunction and death impairs cerebral homeostasis and results in encephalopathy. We postulate that the onset of encephalopathy in STEC infections occurs when Stx-2 attacks vascular endothelial cells of the blood–brain barrier, inducing their death. Stx-2 and LPS then attack the exposed glial cells that are no longer in contact with the endothelial cells. AQP4 is overexpressed in glial cells, resulting in their swelling and adversely affecting cerebral homeostasis. Once cerebral homeostasis is affected in such a way, encephalopathy is the likely result in STEC patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s12929-015-0184-5) contains supplementary material, which is available to authorized users.

Brain extracellular levels of dimethylarginines (ADMA, SDMA) and cGMP and their modulation by exogenous S-adenosylmethionine in rats with acute liver failure

Acute liver failure (ALF) instantly evokes symptoms of hepatic encephalopathy (HE), mainly attributed to hyperammonemia. Ammonia neurotoxicity is related to disturbances in the nitric oxide (NO)/cGMP pathway. The methylated derivative of L-arginine (MDALs) - asymmetric dimethylarginine (ADMA) but not symmetrically methylated arginine (SDMA) is an endogenous inhibitor of nitric oxide synthase. Elevated blood and brain ADMA was reported in HE patients and experimental animals. The question arose whether ALF evokes changes in the incorporation of the methyl donor S-adenosylmethionine (SAM) to MDALs, and how they affect cGMP accumulation. To this end, we investigated the effect of intracortical perfusion of SAM on the brain extracellular levels of ADMA, SDMA and cGMP in rats with ALF in the thioacetamide (TAA) model. Sprague Dawley rats (250–270 g) received three i.p. injections of TAA (300 mg/kg) at 24 h intervals. Bilateral microdialysis of the prefrontal cortex was carried out 24 h after the last TAA administration. SAM at 2 mM concentration in artificial cerebrospinal fluid was infused for 40 min and then the medium was changed back. The extracellular levels of ADMA and SDMA were analyzed using positive mode electrospray LC–DMS–MS/MS and cGMP was determined with cGMP enzyme immunoassay. ALF resulted in the increased extracellular levels of ADMA (by ~800%) and SDMA (by ~250%). Moreover, in ALF rats infusion of SAM decreased the ADMA and SDMA (~30%) as compared to the basal value. It seems reasonable that an excessive amount of substrate inhibited the enzymes synthesizing MDALs. On the other hand, the cGMP level did not differ between control and TAA rats. SAM increased by ~90% cGMP only in the control group, what indicates affected NO/cGMP pathway in TAA model. The study demonstrates, that ALF modulates methylation of arginine to MDALs in a manner affecting cGMP accumulation.

Spontaneous portosystemic shunts in noncirrhotic patients presenting with encephalopathy

Portosystemic shunts (PSS) are common in patients with chronic liver disease and portal hypertension, however, their occurrence in noncirrhotic individuals is likely under appreciated. Patients may present with symptoms of hepatic encephalopathy and/or gastrointestinal bleeding and undergo extensive work up for liver disease to no avail. Often, these patients suffer for years with accruing medical expenses and numerous hospitalizations related to repeated episodes of encephalopathy. The underlying cause may go undiagnosed or misdiagnosed as occult hepatic dysfunction or even a neuropsychological disorder. In many cases, abdominal CT imaging demonstrates the abnormal portosystemic connection well before it is recognized as the cause of symptoms. In this brief report, we discuss three cases presenting with symptoms of encephalopathy and report successful management with endovascular occlusion of the portosystemic shunts.