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)

Three patients with severe motor and intellectual disabilities presented deterioration of the activities of daily living, which was revealed to be caused by prolonged non-convulsive status epilepticus (NCSE). Their condition improved by the treatment with antiepileptics. Case 1, a 4-year-old girl with profound psychomotor retardation and past history of West syndrome of unknown etiology, became unable to sit and eat orally above age of two years. EEG showed continuous generalized slow spike and wave bursts indicating NCSE. Continuous intravenous infusion of midazolam abolished EEG abnormalities of NCSE, and she regained the ability of oral feeding. Case 2, a 3-year-old boy with Angelman syndrome and past history of West syndrome, presented decreased mental response, poor oral intake and somnolence. EEG showed continuous slow spike and wave bursts, indicating NCSE. High-dose phenobarbital therapy and continuous intravenous injection of vitamin B6 were effective, and remarkably improved his psychomotor activities. Case 3, a 3-year-old boy with Lennox-Gastaut syndrome, developed decreased psychomotor activity and loss of vocalization and walking. He could not sit by himself and became nearly bed-ridden. EEG showed very frequent generalized spike and wave bursts, showing NCSE. Continuous infusion of thiopental diminished NCSE, and he could walk again. Psychomotor deterioration in patients with severe motor and intellectual disabilities may be caused by NCSE, which should not be overlooked.
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PMID:[Three cases with severe motor and intellectual disabilities presenting the severest condition caused by prolonged non-convulsive status epilepticus]. 1260 90

Status epilepticus (SP) is defined as a single seizure or recurrent seizures of over 30 min. duration, without regaining full consciousness. In approximately 50% of cases SE is related to epilepsy. The International League Against Epilepsy has recently proposed a new SE classification (2001). The purpose of this prospective study is to evaluate SE incidence in children and adolescents in relation to their age and type of epilepsy, as well as to determine usefulness of the new classification of SE. A group of 600 children and adolescents with epilepsy was observed prospectively. The inclusion criteria were: recently diagnosed epilepsy in children aged under 15 years, and long-term treatment and observations (mean = 5 years, SD = 3.2). Out of the 600 children and adolescents with epilepsy 39 (6.1%) had one or more episodes during the observation period. Two factors were correlated with SE: age at the onset of epilepsy (under 5 years), and the type of epileptic syndrome (the Lennox-Gastaut syndrome, myoclonic-astatic epilepsy, symptomatic and cryptogenic partial epilepsy, progressive myoclonic epilepsy, and the Kojevnikov syndrome). Only in a fourth of the patients the cause of SP was related to a known factor, such as e.g. infection or sudden discontinuation of anti-epileptic medication. The currently used classification of SP (by Appelton and Gibbs) was found to be more useful than the one newly proposed by ILAE, because the former is better suited to the evaluation of SE in children.
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PMID:[Status epilepticus in the course of epilepsy in children and adolescents]. 1291 Aug 29

In children, non-convulsive status epilepticus (NCSE) is rare and difficult to treat. Response to steroids and GABAergic medication is variable and often decreases with increasing duration of NCSE. We present our experience with oral ketamine, an NMDA-receptor antagonist, administered to five children with severe epilepsy (Lennox-Gastaut Syndrome, myoclonic-astatic epilepsy, progressive myoclonic epilepsy and Pseudo-Lennox Syndrome) during an episode of NCSE. Resolution of NCSE was documented in all cases clinically and electroencephalographically within 24-48 hours of starting ketamine. No significant side effects were noted.
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PMID:Oral ketamine in paediatric non-convulsive status epilepticus. 1296 77

Epileptic encephalopathies are conditions in which neurologic deterioration is attributable entirely or partly to epileptic activity. It can be due to very frequent or severe seizures and/or to subcontinuous paroxysmal interictal activity. The former mainly consists of Dravet syndrome, in which patients have seizures from the middle of the first year of life and repeat episodes of severe febrile status epilepticus and migrating partial epilepsy in infancy, in which from the first trimester of life, partial seizures affect various areas of the cortex randomly and in a subcontinuous fashion. In Rasmussen syndrome, also, epileptic activity contributes at least partly to the neurologic deterioration. Subcontinuous paroxysmal interictal activity affects newborn infants with suppression bursts, thus consisting in either Ohtahara syndrome or neonatal myoclonic encephalopathy. In infants, it is either myoclonic epilepsy of nonprogressive encephalopathy or West syndrome. In school-age children, it consists of various types of generalized seizures combined with slow spike waves of the Lennox-Gastaut syndrome, myoclonic-astatic epilepsy, and continuous spike waves in slow sleep combined with various motor or cognitive deficits including negative myoclonus, orofacial dyspraxia, Landau-Kleffner syndrome, and frontal lobe syndrome. Treatment differs for all of these syndromes. It is important to avoid potential drug-induced worsening, and valproate is preferred when a definitive diagnosis is not reached in children and especially infants.
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PMID:Epileptic encephalopathies: a brief overview. 1473 29

The treatment of status epilepticus (SE) in children with epilepsy depends on the epilepsy syndrome, in order to avoid worsening drugs such as IV barbiturates in severe myoclonic epilepsy in infancy (SMEI) (Dravet's syndrome) or IV benzodiazepam in tonic SE of Lennox-Gastaut syndrome. Intensive care procedures should not be systematical in convulsive SE (CSE) and are not indicated in non-convulsive SE (NCSE). Generalized CSE mostly involve SMEI before 3 years of age, symptomatic generalized epilepsy and partial lesional epilepsy. Treatment is an emergency and relies on IV benzodiazepines and, if necessary, IV phenytoine using plasmatic concentrations for an optimal management. The partial CSE of partial lesional epilepsy can result in focal deficit and need the same treatment as generalized CSE. NCSE consist in absence and/or myoclonic SE and are often unrecognised during a long time until EEG is performed. They mostly involve myoclonic epilepsies and can be controlled by IV benzodiazepines. The frequency of partial NCSE is underestimated, particularly in infants. Diagnosis relies on video EEG and treatment is the same as that used in partial CSE.
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PMID:[Treatment of status epilepticus in children with epilepsy]. 1547 80

We examined efficacy of continuous midazolam (MDL) infusion in seven episodes of refractory nonconvulsive status epilepticus (NCSE) in five children. Diagnosis included Lennox-Gastaut syndrome (two cases), and symptomatic generalized epilepsy, ring chromosome 20 syndrome, and epilepsy with continuous spike-waves during slow-wave sleep (one case each). One patient with Lennox-Gastaut syndrome and another with ring chromosome 20 syndrome had two episodes of NCSE. MDL was given as an initial bolus of 0.15 to 0.3 mg/kg, followed by continuous intravenous infusion at 0.1 to 0.2 mg/kg/hr. The infusion rate was increased slowly by 0.1 mg/kg/hr every 0.5 to 1.0 hr, up to 0.4 mg/kg/hr or until NCSE was controlled. The electroencephalogram, vital signs, blood pressure, and oxygen saturation were monitored during therapy. Electrical status epilepticus was abolished within a few hours in five of the seven episodes, and two patients could maintain wakefulness, oral intake, and bowel and bladder control throughout the continuous infusion. In one patient in whom NCSE recurred, it then remained uncontrolled even at a maximum dose. Serious complications such as respiratory depression or hypotension were not observed. Continuous intravenous infusion of MDL was effective and safe for NCSE in children, and can be used as firstline therapy for this condition.
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PMID:[Continuous midazolam infusion for refractory nonconvulsive status epilepticus in children]. 1616 41

The authors report a patient with Lennox-Gastaut syndrome who was a fraternal twin. The twins encountered myoclonic seizures at the age of 4 years, but the seizures in the other patient were controlled very quickly without intellectual development damage. With the disease evolving, other characteristic seizures of Lennox-Gastaut syndrome appeared and failed to be controlled by multiple antiepileptic drugs, so levetiracetam was added on. At this time, frequent partial seizures from the left occipital and posterior temporal regions occurred, which always intermixed with atypical absence seizures in a single ictal event. To control the status epilepticus, levetiracetam was withdrawn immediately, and clonazepam, midazolam, and corticotropin in turn were used. The partial seizures were gradually alleviated. The results obtained in this study suggest that there might be some correlative mechanisms between partial seizures and atypical absence seizures in a single event. There is a temporal relationship between the occurrence of partial seizures and the introduction of levetiracetam.
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PMID:Partial seizures and atypical absence seizures as a single ictal event in a patient with Lennox-Gastaut syndrome. 1898 44

For treatment of intractable epilepsies, there are no data comparing conventional adrenocorticotropic hormone and pulsatile corticoid therapy with dexamethasone. A retrospective comparison of efficacy was therefore conducted for both forms of application. Between 1989 and 2001, a series of 11 children with West syndrome and 3 with Lennox-Gastaut syndrome were treated with adrenocorticotropic hormone (group 1); between 2003 and 2006, 7 children with West syndrome, 5 with electrical status epilepticus during slow sleep, and 2 with Lennox-Gastaut syndrome were treated with pulsatile corticoid therapy (group 2). In group 1 (n = 14), 9/11 West syndrome patients became seizure free, but none with Lennox-Gastaut syndrome (0/3). In group 2 (n = 14), 4/7 West syndrome patients became seizure-free, 1/2 with Lennox-Gastaut syndrome exhibited seizure-frequency reduction, and 2/5 patients with electrical status epilepticus during slow-wave sleep exhibited significant improvement according to electroencephalograms. In West syndrome, pulsatile corticoid therapy was an effective alternative treatment to adrenocorticotropic hormone, whereas in Lennox-Gastaut syndrome in general steroids did not lead to a significant seizure reduction. In electrical status epilepticus during slow-wave sleep, treatment with pulsatile corticoid therapy seems to be effective and should be investigated in a larger group of patients.
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PMID:Adrenocorticotropic hormone versus pulsatile dexamethasone in the treatment of infantile epilepsy syndromes. 2000 58

In this review study, second-generation antiepileptic drugs (AEDs) (levetiracetam, gabapentin, topiramate, lamotrigine, zonisamide, oxcarbazepine, vigabatrin, pregabalin, rufinamide, tiagabine, lacosamide, and felbamate) and injectable AEDs (levetiracetam, lacosamide, fosphenytoin, lorazepam, and valproic acid) available in North America were compared with those available in Japan. Three second-generation AEDs (gabapentin, topiramate, and lamotrigine) were recently approved in Japan. Levetiracetam is currently under review for approval by the Japanese regulatory agency. An ideal AED would have a broad-spectrum activity to control multiple types of seizures, favorable safety profile, limited potential for drug-drug interaction, many bioequivalent formulations, long half life to allow infrequent administration, and antiepileptogenic effects that may provide a fundamental cure of epileptic patients by suppressing the development of epileptogenic network and neutralizing previously established epileptogenic foci in the brain. The second-generation AEDs have been developed to possess some of these ideal properties. All the second-generation AEDs are efficacious for the treatment of patients with partial seizures. In addition, levetiracetam, topiramate, lamotrigine, and zonisamide are effective for the treatment of patients with generalized tonic-clonic seizures, absences, myoclonic seizures, Lennox-Gastaut syndrome, and West syndrome; however, lamotrigine is not effective for the treatment of patients with myoclonic seizures. Rufinamide and felbamate are useful for the treatment of patients with Lennox-Gastaut syndrome; however owing to its serious adverse effects, including aplastic anemia and hepatic failure, felbamate is used as the last resort for the treatment of patients with intractable seizures. Vigabatrin is particularly effective for the treatment of patients with West syndrome; however, the patients need to be regularly monitored for the development of peripheral visual field defect. Gabapentin, oxcarbazepine, vigabatrin, and tiagabine are ineffective for the treatment of patients with absences and/or myoclonic seizures and may aggravate these conditions. Treatment with levetiracetam or topiramate (off-label use) is the new option for patients with refractory status epilepticus, which is characterized by downregulation of the inhibitory gamma-aminobutyric acid system, because these drugs act via different mechanisms and are rapidly titratable, especially intravenous levetiracetam. The pharmacokinetic profiles of levetiracetam, gabapentin, and pregabalin are favorable: these drugs exhibit minimal protein binding, do not undergo hepatic metabolism, are not involved in any clinically relevant drug interactions, and rarely lead to the development of serious adverse effects. In general, levetiracetam is probably the closest to being the ideal AED because of its broad-spectrum favorable pharmacokinetic profile and safety profile as well as because of the availability of its parenteral formulation. Among the injectable AEDs, fosphenytoin is a water-soluble prodrug and is used to treat patients with status epilepticus. Systemic and local side effects of this drug are fewer than those of phenytoin. Lorazepam, a benzodiazepine is used as the first-line AED for the treatment of patients with status epilepticus. The effects of this drug are more prolonged than those of diazepam. Intravenous administration of valproic acid is regarded as a new treatment option for patients with status epilepticus, because sedative and negative effects on the cardiorespiratory system of this drug are lesser than those of the traditional injectable AEDs. These novel medications will aid the improvement of the quality of life of epileptic patients through improved seizure control and reduced adverse effects.
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PMID:[Antiepileptic drugs in North America]. 2045 99

Nonconvulsive status epilepticus (NCSE) is a special epileptic state that can be more common than previously thought in children and adult patients. Currently, there is no universally accepted definition for NCSE. Early and accurate diagnosis depends on a high index of suspicion and rapid availability of electroencephalographic recording. The clinical presentation of NCSE can vary from a mild confusional state to a coma. The underlying etiology is also quite diverse. In critically ill patients, NCSE has been reported with convulsive status epilepticus (CSE), hypoxemia, acute ischemic or hemorrhagic stroke, encephalitis, or trauma. The estimated incidence of NCSE is 15% to 40% in post-CSE, 8% in subarachnoid hemorrhage, and 8% to 10% in coma. As seen in CSE, there is a bimodal distribution with NCSE in critically ill patients; children (age <1 year) and elderly appear to be at great risk. NCSE has also been reported in a number of epilepsy syndromes, such as childhood absence epilepsy, Panayiotopoulos syndrome, Lennox-Gastaut syndrome, and Dravet syndrome. However, it is difficult to determine the incidence of NCSE in an ambulatory setting because of the great variation in clinical presentation and underlying etiology. This review examines the clinical features, outcome, and treatment approach for NCSE in 2 different clinical settings, in ambulatory and critically ill patients. NCSE is reviewed in children and adults to distinguish similarities and differences in their clinical presentation.
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PMID:Nonconvulsive status epilepticus and continuous spike and slow wave of sleep in children. 2072 84


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