Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemosorption was performed in 8 patients with a resistant status epilepticus and serial epileptic attacks; arrest of the attacks correlated with significantly faster and stabler normalization of the circulating levels of immune complexes and of the phagocytic activity of neutrophils as compared to the same parameters in patients receiving a course of conventional anticonvulsive and dehydration therapy without hemosorption. The data obtained point to the advisability of the inclusion of hemosorption into a multiple modality treatment of patients with a persistent grand mal status and serial epileptic attacks. High circulating levels of immune complexes in the blood serum may be considered as indication for hemosorption.
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PMID:[Hemosorption in status epilepticus and serial epileptic seizures]. 363 Apr 93

BACKGROUND--Acute dehydration with hypochloronatremic metabolic alkalosis is a classical complication of cystic fibrosis of the pancreas. Its progressive development as a revealing manifestation of the disease is rare as is its appearance in newborns. Case n. 1.--A 13 month-old girl was admitted because of status epilepticus. She was severely dehydrated (20% weight loss) but had normal diuresis. Investigations showed metabolic alkalosis, hypochloronatremia and hypokalemia. All the manifestations disappeared within 5 days with treatment but three sweat tests were abnormal. Case n. 2.--A 7 month-old girl was admitted because she suffered from progressive loss of weight (10%); she was dehydrated and had metabolic alkalosis plus hypochloronatremia, but her diuresis was normal. Two sweat tests were abnormal. Case n. 3.--A 4.5 month-old boy was admitted because he suffered from severe (12%) weight loss. His diuresis was normal despite dehydration; metabolic alkalosis and hypochloronatremia were found. Two subsequent sweat tests were abnormal. Case n. 4.--A 3 day-old girl was admitted suffering from meconium ileus. Two initial mechanism analysis for protein were abnormal. She had a cardiorespiratory arrest on the 13th day of life, when she had lost 14% of her birth weight; investigations showed metabolic alkalosis, hyponatremia (83 mEq/l), hypochloremia (45 mEq/l); kalemia was 5.9 mEq/l. Peritoneal dialysis was needed to correct hydroelectrolytic changes. A sweat test performed on the 26th day of life was also abnormal. Investigations performed during the periods of dehydration, and repeated later, showed transient functional kidney failure. None of the four patients had any respiratory or gastrointestinal clinical manifestations of cystic fibrosis. CONCLUSION--Metabolic alkalosis with hypochloronatremia plus progressive, severe dehydration in infants whose diuresis is paradoxically normal must be followed by examination for cystic fibrosis.
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PMID:[Cystic fibrosis revealed by dehydration with hypochloronatremic alkalosis in 3 infants and a neonate]. 808 18

A case is reported of a duodenal perforation by a Kimray-Greenfield filter hook in a 66-year-old female patient. This device had been inserted four years before, after a pulmonary embolism. The patient presented with epigastric pain, vomiting and extracellular dehydration with renal failure. A plain abdominal film showed the filter to be tilted 15 degrees to the left, with an opening 28 mm wide. Various investigations were carried out, none of which providing a satisfactory diagnosis. Steroid treatment (1 mg.kg-1 x day-1 of prednisone) was started before admission to intensive care. Only at that time gastroduodenoscopy showed on of the filter's hooks jutting through the duodenal wall. This perforation was located in the posterior wall of the third part of the duodenum, and was associated with an ulcer of the mucosa facing this hook. The diagnosis was confirmed by an abdominal CT scan. The hook was cut and the perforation sealed off during a first laparotomy. Twenty-six days later, the patient developed intestinal obstruction due to a haematoma of the jejunal wall. She later had a cerebrovascular accident, with status epilepticus and deep coma. She died four months after her admission. The late complications of vena caval filters are discussed. The position of these devices should be regularly checked by a plain abdominal film. Abdominal CT scanning is a useful investigation for the diagnosis of intra and extravascular complications.
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PMID:[Duodenal perforations by the hooks of a Kimray-Greenfield filter]. 833 71

Although midazolam has been proposed for the treatment of a variety of conditions such as anxiety, dyspnoea, hiccups and status epilepticus, terminal agitation is the only condition where its use is based on a reasonably large number of published clinical studies. A causal approach is generally recommended. Whenever possible, the aetiological condition (pain, fever, constipation, etc.) should be corrected. Such general measures as ensuring a peaceful, familiar environment, and the use of a night light, fluid therapy to counteract dehydration, and antipyretics for fever are beneficial. When symptomatic treatment is needed, drugs with little anticholinergic effect are to be recommended. The use of benzodiazepines as single drug treatment may exacerbate the condition. Haloperidol or risperidone (which has fewer side effects) are recommended. If the agitation is marked, a common strategy is to add lorazepam. Chlormethiazole is an alternative. Subcutaneous midazolam should be reserved for refractory cases. Attention should be paid to dosage, reduced doses being given to the elderly, patients on opioid medication, and patients with impaired liver or renal function. Overdosage may induce deep sedation, and result in carbon dioxide retention and subsequently heart failure and pulmonary oedema which may be fatal.
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PMID:[Midazolam (Dormicum) in terminal anxiety and agitation. The last choice alternative in palliative care]. 1035 70

We report a case of a 2-year-old boy with Down syndrome complicated by Moyamoya syndrome who developed extensive multiple cerebral infarction after status epilepticus on the third day of a measles infection. MR angiography revealed the occlusion of the terminal portion of the bilateral internal carotid artery and the basilar artery. Fever, dehydration, activation of coagulation associated with the infection, relatively decreased cerebral blood flow during status epilepticus, and central nervous invasion of the measles virus may have played a role in the development of the infarction. Moyamoya like cerebrovascular lesions occur more often and is more aggressive in children with Down syndrome than in the general pediatric population, and their clinical presentation is always the infarction type. In patients with Down syndrome complicated by Moyamoya syndrome, an early diagnosis and prevention of infarction are important.
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PMID:[A case of Down syndrome with moyamoya syndrome presenting extensive multiple cerebral infarction during measles infection]. 1065 50

We used single nucleotide polymorphism (SNP) microarrays to investigate the cause of a symptomatic epilepsy syndrome in a group of seven distantly related Old Order Mennonite children. Autozygosity mapping was inconclusive, but closer inspection of the data followed by formal SNP copy number analyses showed that all affected patients had homozygous deletions of a single SNP (rs721575) and their parents were hemizygous for this marker. The deleted SNP marked a larger deletion encompassing exons 9-13 of LYK5, which encodes STE20-related adaptor protein, a pseudokinase necessary for proper localization and function of serine/threonine kinase 11 (a.k.a. LKB1). Homozygous LYK5 deletions were associated with polyhydramnios, preterm labour and distinctive craniofacial features. Affected children had large heads, infantile-onset intractable multifocal seizures and severe psychomotor retardation. We designated this condition PMSE syndrome (polyhydramnios, megalencephaly and symptomatic epilepsy). Thirty-eight percent (N = 16) of affected children died during childhood (ages 7 months to 6 years) from medical complications of the disorder, which included status epilepticus, congestive heart failure due to atrial septal defect and hypernatremic dehydration due to diabetes insipidus. A single post-mortem neuropathological study revealed megalencephaly, ventriculomegaly, cytomegaly and extensive vacuolization and astrocytosis of white matter. There was abundant anti-phospho-ribosomal S6 labelling of large cells within the frontal cortex, basal ganglia, hippocampus and spinal cord, consistent with constitutive activation of the mammalian target of rapamycin (mTOR) signalling pathway in brain.
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PMID:Polyhydramnios, megalencephaly and symptomatic epilepsy caused by a homozygous 7-kilobase deletion in LYK5. 1752 5

Hypernatremic dehydration is a rare but serious clinical condition in newborns and small infants. It is usually caused by diarrhea, improperly prepared infant formula, decreased fluid intake, or exclusive breastfeeding. Symptoms are usually masked until neurological symptoms occur. We report two infants who presented with fever and hypernatremic dehydration caused by concentrating infant formula to alleviate symptoms of constipation, and careless formula preparation due to confusion over spoon sizes, respectively. In the first case, status epilepticus occurred during early treatment, despite close serum sodium monitoring, though the infant was asymptomatic and thriving 4 years after discharge, with no identified neurodevelopmental deficits. The course of treatment was smooth in the second case, and no neurological complications developed. The practice of concentrating infant formula to relieve symptoms of constipation, although temporarily effective, is hazardous to newborns or young infants and can cause hypernatremic dehydration. Spoon sizes supplied with commercial infant formulas (30 mL/spoonful or 60 mL/spoonful) should be unified to avoid mistakes during preparation, especially by inexperienced and teenage mothers.
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PMID:Hypernatremic dehydration due to concentrated infant formula: report of two cases. 1945 82

Seizure activity can alter GABA transporter and osmoprotective gene expression, which may be involved in the pathogenesis of epilepsy. However, the response of the betaine/GABA transporter (BGT1) is unknown. The goal of the present study was to compare the expression of BGT1 mRNA to that of other osmoprotective genes and GABA transporters following status epilepticus (SE). The possible contributory role of dehydration and inflammation was also investigated because both have been shown to be involved in the regulation of GABA transporter and/or osmoprotective gene expression. BGT1 mRNA was increased 24 h post-SE, as were osmoprotective genes. BGT1 was decreased 72 h and 4 weeks post-SE, as were the GABA transporter mRNAs. The mRNA values for osmoprotective genes following 24-h water withdrawal were significantly lower than the values obtained 24 h post-SE despite similarities in their plasma osmolality values. BGT1 mRNA was not altered by lipopolysaccharide-induced inflammation while the transcription factor tonicity-responsive enhancer binding protein and the GABA transporters 1 and 3 were. These results suggest that neither plasma osmolality nor inflammation fully account for the changes seen in BGT1 mRNA expression post-SE. However, it is evident that BGT1 mRNA expression is altered by SE and displays a temporal pattern with similarities to both GABA and osmolyte transporters. Further investigation of BGT1 regulation in the brain is warranted.
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PMID:Hippocampal betaine/GABA transporter mRNA expression is not regulated by inflammation or dehydration post-status epilepticus. 2121 32

The risk factors for cerebral sinus venous thrombosis include dehydration, infection, and anemia. The clinical presentation in children of venous strokes associated with cerebral venous thrombosis is variable and may include seizures. Acute management should focus on the treatment of the primary cause and anticoagulation or antiplatelet therapy if needed. Early recognition and targeted treatment is important because survivors are at increased risk for long-term neurologic complications. We report a case of a 4-year-old girl who presented with status epilepticus and was subsequently found to have a cerebral venous sinus thrombosis in the transverse and sigmoid sinus, with venous infarction in the temporal lobe. Laboratory results were significant for a microcytic anemia caused by excessive milk intake. Although iron deficiency anemia is a common pediatric disorder, this uncommon presentation demonstrates the potential for neurologic complications secondary to anemia, as well as the need for a high index of suspicion in order to identify venous stroke as a cause in children who present to the emergency department with seizures.
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PMID:Venous stroke and status epilepticus due to milk-induced anemia in a child. 2551 78

Cerebral salt wasting syndrome can occur in children with encephalitis. Clinicians should be aware of hyponatremia in patients who develop polyuria with the signs of dehydration and deteriorated consciousness. Furthermore, patients who present with status epilepticus or who are suspected to have high intracranial pressure may have an increased risk of cerebral salt wasting syndrome.
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PMID:Mycoplasma pneumoniae-associated encephalitis complicated by cerebral salt wasting syndrome. 2915 80


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