Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The neurological manifestations associated with acquired liver disorders of various types may present difficult diagnostic problems until the condition is far advanced. Bizarre psychological and motor disorders occur when the central nervous system is affected by liver disease. The clinical features may in some ways resemble those of Wilson's disease, but such features as remitting coma, fetor hepaticus and seizures in "cholemia," and a Kaiser-Fleischer ring in hepatolenticular degeneration help to distinguish the two conditions. The biochemical abnormalities found in all types of hepatocerebral dysfunction may be quite similar one to another. While many studies suggest that the whole problem is simply the result of brain intoxication by a substance such as ammonia, other lines of evidence indict several factors in intermediate cerebral and liver metabolism. The treatment involves use of substances which may relieve certain blocks in biochemical processes, supplementary vitamins, low protein intake and strict avoidance of all neuro- and hepatotoxins.
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PMID:Hepatocerebral dysfunction. 1356 Nov 10

Glutathione (gamma-glutamyl-cysteinyl-glycine; GSH) is the most abundant low-molecular-weight thiol, and GSH/glutathione disulfide is the major redox couple in animal cells. The synthesis of GSH from glutamate, cysteine, and glycine is catalyzed sequentially by two cytosolic enzymes, gamma-glutamylcysteine synthetase and GSH synthetase. Compelling evidence shows that GSH synthesis is regulated primarily by gamma-glutamylcysteine synthetase activity, cysteine availability, and GSH feedback inhibition. Animal and human studies demonstrate that adequate protein nutrition is crucial for the maintenance of GSH homeostasis. In addition, enteral or parenteral cystine, methionine, N-acetyl-cysteine, and L-2-oxothiazolidine-4-carboxylate are effective precursors of cysteine for tissue GSH synthesis. Glutathione plays important roles in antioxidant defense, nutrient metabolism, and regulation of cellular events (including gene expression, DNA and protein synthesis, cell proliferation and apoptosis, signal transduction, cytokine production and immune response, and protein glutathionylation). Glutathione deficiency contributes to oxidative stress, which plays a key role in aging and the pathogenesis of many diseases (including kwashiorkor, seizure, Alzheimer's disease, Parkinson's disease, liver disease, cystic fibrosis, sickle cell anemia, HIV, AIDS, cancer, heart attack, stroke, and diabetes). New knowledge of the nutritional regulation of GSH metabolism is critical for the development of effective strategies to improve health and to treat these diseases.
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PMID:Glutathione metabolism and its implications for health. 1498 35

Hepatic encephalopathy represents a reversible decrease in neurological function caused by liver disease. Overall incidence of seizures in hepatic encephalopathy varies between 2% and 33%. Non-convulsive status epilepticus may be particularly common in these patients. Psychiatric disturbances manifest as agitation, personality change, delusions, etc. Aims of seizure management include treatment of basic disease, correction of precipitant factors, imaging of head, and choice of a pharmacologically safe agent. It is important to consider non-convulsive status epilepticus and rule it out by an EEG. Absolute data for safety profile of drugs in liver disease is still not clear, as changes of pharmacokinetics make choice of drugs difficult. Free drug concentrations may be higher, making plasma concentration monitoring essential in such circumstances. A single seizure may not require therapy. However when started, antiepileptic drugs are usually discontinued early. Drugs with sedative effects are best avoided because of a risk of precipitating coma. Phenytoin and gabapentin are relatively preferred drugs; however, monitoring of drug levels is desirable. Management of agitation includes physical restraint and medication. Benzodiazepines are best avoided. Haloperidol is a safer choice in the presence of liver disease. Overall management of neuropsychiatric state aims at management of underlying pathology, the resolution of which leads to improvement in the clinical symptomatology.
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PMID:Management of agitation and convulsions in hepatic encephalopathy. 1502 57

Alpers' syndrome is a fatal neurogenetic disorder first described more than 70 years ago. It is an autosomal recessive, developmental mitochondrial DNA depletion disorder characterized by deficiency in mitochondrial DNA polymerase gamma (POLG) catalytic activity, refractory seizures, neurodegeneration, and liver disease. In two unrelated pedigrees of Alpers' syndrome, each affected child was found to carry a homozygous mutation in exon 17 of the POLG locus that led to a Glu873Stop mutation just upstream of the polymerase domain of the protein. In addition, each affected child was heterozygous for the G1681A mutation in exon 7 that led to an Ala467Thr substitution in POLG, within the linker region of the protein.
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PMID:POLG mutations associated with Alpers' syndrome and mitochondrial DNA depletion. 1512 11

This study aimed to investigate the relationship between 25-hydroxyvitamin D [25(OH)D)] and parathyroid hormone (PTH) levels in adolescent females residing in a northern climate. Concern regarding vitamin D status in this population is due to limited sunlight exposure in northern latitudes, decreased outdoor recreational activities, as well as decreased conversion in black girls from increased skin pigmentation. In this cross-sectional analysis, serum samples were assayed for 25(OH)D using competitive protein binding (CPB) assay and PTH with immuno-radiometric (RIA) procedures. Four hundred postmenarcheal females (12-18 years) residing in northeastern Ohio were recruited. Subjects were excluded if they had a history of bone, kidney, or liver disease, or used medications that affect bone. The primary goal was to determine serum 25(OH)D concentrations in relation to circulating PTH levels in a population of adolescent girls. The Spearman correlation test was used to compare PTH and 25(OH)D. Fit multiple split models were run to determine change in slope of the regression line when 25(OH)D and PTH were plotted. Analysis of variance was determined using modeled means with differences by race and season in the final model. Unadjusted mean serum 25(OH)D and PTH levels were 55.0+/-30.4 nmol/l and 39.4+/-20.6 ng/l, respectively. Blacks had lower 25(OH)D and higher PTH compared with non-blacks (P<0.0001), especially during the winter months. Decreasing 25(OH)D was inversely correlated with PTH (r=-0.314) (P<0.0001), and at concentrations of 25(OH)D < or =90 nmol/l, an increase in PTH was observed. Adolescents are at risk for decreased serum 25(OH)D concentrations, especially black girls. We found that the widely used cutoff for vitamin D deficiency is associated with increasing PTH levels and is below the inflection point for a change in the slope of the regression line. Our results support the need for further research to establish optimal vitamin D status in adolescent girls.
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PMID:Low levels of 25-hydroxy vitamin D are associated with elevated parathyroid hormone in healthy adolescent females. 1517 48

Although elevation of the levels of serum alanine aminotransferase (ALT) following liver injury is well known, confusion exists concerning skeletal muscle injury as the cause of this rise. We reviewed the records of 16 patients who had muscle necrosis without evidence of liver disease. The patients were divided into three groups: extreme exercise, polymyositis, and seizures. All patients exhibited markedly elevated creatine kinase and lactate dehydrogenase levels consistent with muscle injury. In acute cases, aspartate aminotransferase (AST) and ALT were both elevated, and the AST/ALT ratio was greater than 3, but this ratio approached 1 after a few days because of a faster decline in AST. In conclusion, this difference in half-life accounts for the comparable AST and ALT levels in our cases with chronic muscle injury.
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PMID:Serum alanine aminotransferase in skeletal muscle diseases. 1566 Apr 33

Antiepileptic drugs (AEDs) are no longer restricted to the treatment of epilepsy. These are widely used in a broad spectrum of psychiatric and neurological disorders. Liver plays a major role in the metabolism of a majority of these drugs. Hepatotoxicity is rare, but a real concern when initiating therapy. Likewise, liver disease can adversely affect the biotransformation of some of these drugs. This manuscript addresses the significance of elevated liver enzymes associated with AED use, the role of therapeutic drug monitoring, pharmacokinetics during liver disease and potential risk of hepatotoxicity.
Seizure 2006 Apr
PMID:Antiepileptic drugs and liver disease. 1644 14

Alpers syndrome was not clearly defined until the link between brain and liver disease was described. Alpers syndrome can now be clearly established as a disorder of oxidative metabolism related to mitochondrial dysfunction, and in most instances with an autosomal mode of inheritance. The symptoms and signs are discussed. The illness occurs in the first years of life with the sudden onset of intractable seizures associated with developmental delay, hypotonia, ataxia, cortical blindness, and hepatic failure, and death occurs within a short time. Treating the seizures with valproic acid can cause the rapid onset of liver failure and must be avoided. To establish a definite diagnosis, liver and muscle biopsies may be needed. The former shows bile duct proliferation with the evidence of cirrhosis, and the latter may support the involvement of the mitochondrial respiratory chain if there are ragged-red fibres. Genetic studies can show an association with mitochondrial DNA depletion and mutations in the polymerase gene. Cytochrome c oxidase deficiency has been demonstrated in some patients. Useful diagnostic tests include liver function tests, lactic acid levels in the blood and cerebrospinal fluid, electroencephalograms, computed tomography, and magnetic resonance imaging. The differential diagnosis will be from other forms of neuronal degeneration and disorders of mitochondrial function. There is no specific treatment, which must await further research into causes.
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PMID:Alpers syndrome: progressive neuronal degeneration of children with liver disease. 1710 92

The alcohol withdrawal syndrome (AWS) is a common management problem in hospital practice for neurologists, psychiatrists and general physicians alike. Although some patients have mild symptoms and may even be managed in the outpatient setting, others have more severe symptoms or a history of adverse outcomes that requires close inpatient supervision and benzodiazepine therapy. Many patients with AWS have multiple management issues (withdrawal symptoms, delirium tremens, the Wernicke-Korsakoff syndrome, seizures, depression, polysubstance abuse, electrolyte disturbances and liver disease), which requires a coordinated, multidisciplinary approach. Although AWS may be complex, careful evaluation and available treatments should ensure safe detoxification for most patients.
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PMID:The alcohol withdrawal syndrome. 1798 99

The objective of this study was to determine what contraindications to phenytoin exist in Emergency Department (ED) patients with a medical history of seizures. We conducted a retrospective chart review using ED medical records from 2005 at two network health care EDs. We identified potential patients through ICD-9 (International Classification of Diseases, Ninth Revision) codes, selected only adult patients with a prior documented history of seizures, and reviewed these charts. From 201 charts reviewed, the three most common antiepileptic drugs taken by patients were: phenytoin (38%), levetiracetam (17%), and valproic acid (15%). For absolute contraindications to phenytoin, 4.5% of seizure patients had a known hypersensitivity to phenytoin and 1.5% were pregnant; however, no pregnant patients were taking phenytoin and only 1 person with hypersensitivity to phenytoin was taking phenytoin. For relative contraindications, 6% of seizure patients had liver disease, 8% had kidney disease, 9% had alcohol use/dependence, and 16% had diabetes. However, 55% of those with liver disease, 44% with kidney disease, 77% with alcohol use/dependence, and 53% with diabetes were currently taking phenytoin. Very few seizure patients in the ED have absolute contraindications to the use of phenytoin, and most with absolute contraindications are taking other antiepileptic drugs. Conversely, a greater proportion of seizure patients have relative contraindications and many are continuing to use phenytoin.
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PMID:Contraindications to phenytoin in emergency department patients with seizures. 1832 12


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