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Treatment of seizure emergencies: convulsive and non-convulsive status epilepticus.

Status epilepticus (SE), defined as recurrent epileptic seizures without complete recovery between seizures, is one of the most serious manifestations of epilepsy. Generalized convulsive status epilepticus (GCSE) is the most common and most life-threatening form of SE, and aging increases the mortality risk. In a recent study of treatment of GCSE, 226 of 518 evaluable patients (43.6%) were of age 65 or older. In the 157 elderly patients with overt GCSE, phenobarbital was successful as first-line treatment in 71.4%, lorazepam in 63%, diazepam and phenytoin in 53.3%, and phenytoin alone in 41.5%. Phenobarbital and lorazepam were more successful than phenytoin alone. In the 69 elderly patients with subtle GCSE, success as the first treatment was 30.8% for phenobarbital, 14.3% for lorazepam, 11.8% for phenytoin, and 7.7% for diazepam and phenytoin. Overall, the results were similar to those reported for the entire study. Lorazepam, because of ease of use, is probably the best drug for the initial treatment of overt GCSE in the elderly; phenobarbital may be the best drug for subtle GCSE in this group, but more data are needed. The term "nonconvulsive SE" has been used to include complex partial SE and absence SE - both of which present as an "epileptic twilight state" - and SE in comatose patients. The diagnosis can be challenging, particularly in the elderly, as overlapping clinical features and electroencephalogram patterns can be seen in SE and in a variety of encephalopathic conditions. There is a suggestion that aggressive treatment of elderly patients with nonconvulsive SE may worsen prognosis. Clearly, there is a need for more data to better understand management of elderly patients with both convulsive and nonconvulsive SE.

Aged↗

New human sodium/glucose cotransporter gene (KST1): identification, characterization, and mutation analysis in ICCA (infantile convulsions and choreoathetosis) and BFIC (benign familial infantile convulsions) families.

Cotransporters represent a major class of proteins that make use of ion gradients to drive active transport of substrate into cells. A new human gene, KST1, encoding a member of the sodium/glucose cotransporter family, was identified onto human chromosome 16p12-p11. This genomic region contains a major gene responsible for a syndrome of infantile convulsions and paroxysmal dyskinesia (ICCA syndrome), inherited as an autosomal dominant trait, as well as for benign familial infantile convulsions (BFIC). The entire coding sequence of the human KST1 gene was determined using a combination of methods including in silico comparison of its rabbit orthologous DNA complementary to RNA (cDNA) to the corresponding human genomic sequences, reverse transcription-polymerase chain reaction on human brain RNA, 5' and 3' rapid amplification of cDNA ends. The gene is divided into 16 exons and the predicted protein of 675 amino acids contains 14 transmembrane domains. It shares significant homology to the sodium-glucose transporter 1 cotransporter proteins. An alternatively spliced transcript resulting from the skipping of exon 6 led to a predicted protein lacking the 4th transmembrane domain. As ion transporters are good candidates for a large variety of human diseases, including paroxysmal disorders, a mutation search was performed in four families with ICCA or BFIC syndromes. No pathogenic mutation was found, although several polymorphic variants with amino acids exchanges were identified. Due to its broad expression in human tissues, the human KST1 gene could be involved in several other diseases mapped to human chromosome 16p12-p11.

Amino Acid Sequence↗

Amino acid metabolism in the brain with convulsive disorders. Part 3: Free amino acid patterns in cerebrospinal fluid in infants and children with convulsive disorders.

Free amino acid patterns of cerebrospinal fluid in infants and children with various types of convulsive disorders were compared with those in age-matched normal subjects. The total free amino levels in Lennox syndrome were higher than the normal values, and those in infantile spasms controlled by ACTH were higher than those in uncontrolled infantile spasms. Although the levels of only one or two amino acids in tonic-clonic seizure, focal seizure and febrile seizure were higher or lower than those of the controls, the levels of 8 amino acids in infantile spasms were lower and those of 10 amino acids in Lennox syndrome were generally higher compared to the controls. Among amino acids in CSF of children with tonic-clonic seizure, infantile spasms or Lennox syndrome, only the ornithine level was commonly lower than that of the controls. After the treatment, in tonic-clonic seizure, the levels of taurine, asparagine and glycine were increased, and in infantile spasms, those of asparagine, glutamine, glycine, alanine, phenylalanine, lysine and arginine were increased while that of taurine was decreased. These results suggest that each type of convulsive disorder shows the specific amino acid pattern, and the effects of anticonvulsants may be partially understood through the changes of the free amino acid patterns in the brain.

Adolescent↗

Postoperative convulsions due to iophendylate (Myodil). Report of a case and review of the causes of postoperative convulsions.

A previously fit 58-year-old man underwent lumbar laminectomy following a myelogram. Postoperatively convulsions occurred. He was successfully treated and made a complete recovery. Large quantities of intracranial iophendylate (Myodil) were seen on postoperative skull X-rays. In the absence of other likely causes, a diagnosis of iophendylate-induced encephalopathy was made. A review of the aetiology of convulsions in the intra- and postoperative period is presented.

Anesthetics↗

[A case with mild subdural hematoma presenting with a transient cluster of convulsions--problems concerning differentiation from benign infantile convulsion and benign complex partial epilepsies in infancy].

A 1-month-old girl showed frequent partial seizures of sudden onset. Continuous spikes were observed in left central area during the ictal period, although interictal EEG showed neither epileptiform discharges nor focal signs. Optimal seizures control was obtained with intravenous administration of diazepam and subsequent oral administration of phenobarbital. She showed neither abnormal physical and neurological signs after seizures nor sequelae. CT examination on two days after the onset of seizures showed no obvious abnormal high density area. However, MRI examination on 10 days after the onset showed subdural hematoma in the left front-temporal area and right occipital area. Except for MRI findings, this case may be diagnosed as benign infantile convulsion or benign complex partial epilepsies in infancy. This case suggested that serial neuroradiological examinations were recommended for prospective studies about benign infantile convulsion and benign complex partial epilepsies in infancy.

Acute Disease↗

[Characteristics of drugs used in the treatment of acute convulsions and convulsive status].

OBJECTIVE: To evaluate the pharmacokinetic and pharmacodynamic characteristics of the drugs used in the treatment of acute seizures and status epilepticus. DEVELOPMENT: After emphasizing the clinical importance of seizure episodes and the pathophysiology of status epilepticus, we describe the ideal characteristics of the drugs used for their treatment. Subsequently we analyze the pharmacokinetic characteristics, efficacy and toxicity of phenobarbitone, phenytoin, diazepam and valproate given parenterally. Although the efficacy of the four drugs is similar when given parenterally, fewer side-effects are seen with valproate. CONCLUSIONS: Parenteral valproate should be included in the normal guidelines for the treatment of convulsions. In this paper we include the recommended measures for the treatment of acute seizures and status epilepticus in childhood and suggest the development of similar measures for adult patients.

Anticonvulsants↗