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)

The severe refractory type of status epilepticus is very rare in the pediatric population. Eight children with the severe refractory type of status epilepticus owing to presumed encephalitis are described. The age at the onset of status epilepticus of the eight study children ranged between 2.5 and 15 years. Seven of the eight children presented with fever several days prior to the onset of seizures. A comprehensive clinical and laboratory investigation failed to delineate a cause for their seizures. Burst suppression coma was induced by pentothal, midazolam, propofol, or ketamine in all of the children. The mean duration of anesthesia was 28 days (range 4-62 days), but the seizures persisted in spite of repeated burst suppression cycles in all of them. Two children died. Four of the surviving children continued to suffer from seizures, and cognitive sequelae were present throughout follow-up in four children. In summary, the severe refractory type of status epilepticus of the acute symptomatic type owing to relatively mild encephalitis carries a high mortality rate and poor morbidity in terms of seizures and cognition at follow-up.
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PMID:Severe refractory status epilepticus owing to presumed encephalitis. 1583 6

The pathophysiological mechanisms that cause spontaneous seizures following status epilepticus are largely unknown. Erosion of inhibition is regarded as an important pathophysiological hallmark of ongoing status epilepticus. Therefore, we investigated if loss of inhibitory functions also plays an important role in the development of spontaneous seizures after status epilepticus. Furthermore, we analyzed possible changes in excitation that might contribute to epileptogenesis. Finally, neuronal cell loss in the dentate gyrus granule cell layer was analyzed. In rats, inhibition and excitation in the dentate gyrus were monitored 1, 4, and 8 weeks after electrically induced self-sustaining status epilepticus (SSSE). Control animals had electrodes implanted either without subsequent stimulation or with stimulation but under barbiturate anesthesia, neither of which resulted in subsequent spontaneous seizures or impairment of inhibition. Following SSSE 80% of animals developed seizures after 8 weeks. A pronounced impairment of inhibition 1 week after SSSE was followed by gradual recovery over 8 weeks. In the dentate gyrus, cell damage was highly variable most likely explaining the heterogeneity of changes in excitatory parameters. Loss of GABAergic inhibition in the dentate gyrus may facilitate initiation of epileptogenesis but impaired inhibition is not required for the process of epileptogenesis to be maintained.
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PMID:Transient loss of inhibition precedes spontaneous seizures after experimental status epilepticus. 1583 71

The patient with status epilepticus has continuous or rapidly repeating seizures. Generalised convulsive status epilepticus (GCSE) is the most common form of the disorder and is a life-threatening condition that requires prompt medical management. Status epilepticus that does not respond to first-line benzodiazepines (lorazepam or diazepam) or to second-line antiepileptic drugs (phenytoin/fosphenytoin, phenobarbital or valproate) is usually considered refractory and requires more aggressive treatment. The optimal treatment of refractory GCSE has not been defined, but patients should be treated in an intensive care unit, as artificial ventilation and haemodynamic support are required. Invasive haemodynamic monitoring is often necessary and EEG monitoring is essential. The drug treatment of refractory GCSE involves general anaesthesia with continuous intravenous anaesthetics given in doses that abolish all clinical and electrographic epileptic activity, often requiring sedation to the point of burst suppression on the EEG. Barbiturate anaesthetics, pentobarbital in the US and thiopental sodium in Europe and Australia, are the most frequently used agents and are highly effective for refractory GCSE both in children and adults. Indeed, they remain the only way to stop seizure activity with certainty in severely refractory cases. Other options are midazolam for adults and children and propofol for adults only.Regardless of the drug selected, intravenous fluids and vasopressors are usually required to treat hypotension. Once seizures have been controlled for 12-24 hours, continuous intravenous therapy should be gradually tapered off if the drug being administered is midazolam or propofol. Gradual tapering is probably not necessary with pentobarbital or thiopental sodium. Continuous EEG monitoring is required during high-dose treatment and while therapy is gradually withdrawn. During withdrawal of anaesthetic therapy, intravenous phenytoin/fosphenytoin or valproate should be continued (these agents having been administered during earlier phases of GCSE) to ensure an adequate baseline of antiepileptic medication so as to prevent the recurrence of status epilepticus. If additional medication is needed, the most appropriate antiepileptic drugs are gabapentin for focal seizures and levetiracetam and topiramate for all seizure types, as these drugs can be started at high doses with a low risk of idiosyncratic reactions. Even with current best practice, mortality in patients who experience refractory GCSE is about 50% and only the minority return to their premorbid functional baseline. Therefore, new treatment options are urgently needed. The ideal new drug for refractory GCSE would be one that has the ability to stop seizures more effectively and safely than current drugs, and that has neuroprotective properties to prevent the brain damage and neurological morbidity caused by GCSE.
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PMID:Refractory generalised convulsive status epilepticus : a guide to treatment. 1614 91

Prolonged sedation in the pediatric intensive care unit may be difficult because of tolerance, drug dependence and withdrawal, drug interactions and unwanted drug effects. We present three patients sedated with isoflurane via the Anesthetic Conserving Device, AnaConDa. AnaConDa is a modified heat and moisture exchanger that allows evaporation and delivery of inhalational anesthetics without an anesthesia machine, vaporizer or adapted ventilator. Two patients with abdominal complications and prolonged sedation for mechanical ventilation were converted to isoflurane sedation for several days. The third patient with refractory status epilepticus received isoflurane to treat epileptiform electroencephalogram activity. Patients weighing 40 and 30 kg were treated with AnaConDa placed at the Y-piece, while the patient weighing 20 kg was treated with AnaConDa in the inspiratory limb of the respiratory circuit. Adequate sedation was achieved with endtidal isoflurane concentration of 0.3-0.4%, while antiepileptic effect was achieved at a higher dose, 0.9%. Intravenous sedatives could be reduced or discontinued during isoflurane sedation. Inhaled sedation of isoflurane with AnaConDa was effective in these patients. It may provide an alternative in difficult cases needing prolonged sedation and should be evaluated further.
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PMID:Three cases of PICU sedation with isoflurane delivered by the 'AnaConDa'. 1617 17

A previously healthy 32-year-old man visited our clinic with a 5-week history of involuntary movement. Examination demonstrated continuous myoclonic jerks in the left elbow. Two days later, he developed generalized convulsion. Electroencephalography demonstrated small spikes over the right central region. Thus, we made a diagnosis of epilepsia partialis continua (EPC) with a secondary generalization. On admission, serum electrolytes, glucose and ceruloplasmin levels, and amino acid analysis were unremarkable. HIV serology was negative. Anticardiolipin, anti-MPO ANCA, and anti-Hu antibodies were negative. The cerebrospinal fluid (CSF) showed 151/microl lymphocytes and 23/microl polymorphs, 70 mg/dl of glucose, and 61 mg/dl of protein. Autoantibodies against the glutamate receptor subunits epsilon-2 and delta-2 were detected in the serum and CSF. Cranial MRI was unremarkable. Treatment with acyclovir and high-dose methylprednisolone failed to halt the jerks. Two weeks after admission, he developed status epilepticus, which necessitated general anesthesia. Intravenous immunoglobulin infusion gave no beneficial effects. Two months after the onset of the status epilepticus, his convulsions were controlled with zonisamide, clobazam, and carbamazepine. While he had no motor dysfunctions, he had loss of spontaneity and memory impairment. This report suggests that EPC might be the initial symptom of subacute encephalitis with a possible autoimmune mechanism.
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PMID:[Subacute encephalitis with anti-glutamate receptor antibodies presented with epilepsia partialis continua]. 1618 Jul 13

The incidence of convulsive status epilepticus in children is approximately 20-50/100,000/year, and is an emergency requiring prompt medical intervention. Prolonged seizures lasting over 5 min are unlikely to stop spontaneously, and time-to-treatment influences treatment response. Prolonged seizures should thus be treated as early status epilepticus. Mortality and morbidity increase significantly with the length of ongoing seizure activity, especially after 60 min. Benzodiazepines remain the first-line drug therapy due to their rapid onset of action. Recent studies imply that buccal midazolam is more effective and easier to administer than rectal diazepam. Phenytoin/fosphenytoin and phenobarbital administered intravenously remain the second-line treatments of choice, whilst barbiturates and midazolam as intravenous anesthetics are used for third-line treatment. Electroencephalogram monitoring is essential to evaluate the electrophysiologic treatment response and depth of anesthesia, especially in refractory status epilepticus. In the future, more individualized protocols and pathways are needed in order to optimize treatment responses. Randomized clinical trials are needed to evaluate new treatment protocols, which should not only stop the seizures more effectively but also be safer and include some neuroprotective elements to halt the cascade of neuronal injury and minimize the risk for neurologic morbidity caused by the convulsive status epilepticus.
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PMID:Pharmacologic management of convulsive status epilepticus in childhood. 1627 35

As in Clark and Prout's classic work, we identify three phases of generalised convulsive status epilepticus, which we call impending, established, and subtle. We review physiological and subcellular changes that might play a part in the transition from single seizures to status epilepticus and in the development of time-dependent pharmacoresistance. We review the principles underlying the treatment of status epilepticus and suggest that prehospital treatment is beneficial, that therapeutic drugs should be used in rapid sequence according to a defined protocol, and that refractory status epilepticus should be treated with general anaesthesia. We comment on our preference for drugs with a short elimination half-life and discuss some therapeutic choices.
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PMID:Status epilepticus: pathophysiology and management in adults. 1648 80

A 37-year-old man in a status epilepticus due to meningitis was admitted to Intensive Care because of respiratory insufficiency. Spinal fluid culture yielded Streptococcus salivarius. Despite extensive diagnostics, the source of this bacterium could not be found. However, the patient had recently undergone spinal anaesthesia for surgery on a toe ulcer, from which other bacteria were cultured. The patient died 2 weeks after admission with a picture of multiple organ failure. Bacterial meningitis following spinal anaesthesia may be the result of impairment of the blood-brain barrier due to a sudden drop of spinal fluid pressure during the puncture, or of the introduction of bacteria from the hair follicles or from a haematoma caused by the needle or the introducer. Hygienic measures and a proper technique when performing regional anaesthesia are important in preventing the dissemination of bacteria.
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PMID:[Bacterial meningitis following spinal anaesthesia]. 1668 97

An 84-year-old Asian woman with hypertension and chronic renal failure was evaluated for incoherent speech, followed by intermittent interruptions of consciousness, and then status epilepticus after ingesting one star fruit (Averrhoa carambola) each day for 3 days. Conventional first-line anticonvulsants and hemodialysis were administered without significant control of the patient's seizures. Treatment was started with propofol, an intravenous agent that induces anesthesia with rapid onset and elimination from the central nervous system; this resulted in complete control of the seizures. Propofol may be an effective alternative when dialysis and conventional first-line anticonvulsants are unsuccessful in treating the symptoms of neurotoxicity.
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PMID:Management of star fruit-induced neurotoxicity and seizures in a patient with chronic renal failure. 1650 56

We studied 26 patients belonging to 20 families with a disorder caused by mutations in the POLG gene. The patients were homozygous for 1399 G/A or 2243 G/C (giving the amino acid changes A467T and W748S, respectively) or compound heterozygotes for these two mutations. Irrespective of genotype, the patients exhibited a progressive neurological disorder usually starting in their teens and characterized by epilepsy, headache, ataxia, neuropathy, myoclonus and late onset ophthalmoplegia. However, major differences in survival were seen depending on genotype, with compound heterozygotes having a significantly shorter survival time than patients homozygous either for the A467T or W748S (P = 0.006). Epilepsy occurred in 22 of the 26 patients and in the majority of these there was an occipital EEG focus. Episodes of both generalized and focal motor status epilepticus were common and highly resistant to treatment, even with generalized anaesthesia. Status epilepticus was the recorded cause of death in 9 of 11 patients. Liver failure was the sole cause of death in two patients and evolved terminally in six others, all but one of whom were being treated with sodium valproate. Two patients underwent liver transplantation, but only one survived. Delayed psychomotor development and subsequent cognitive decline also occurs. This study demonstrates the clinical spectrum of a disorder that combines features of Alpers' syndrome and a later onset mitochondrial spinocerebellar ataxia with epilepsy and headache. Patients with this disorder are at high risk of death from status epilepticus and from liver failure, if exposed to sodium valproate. Each mutation appears capable of producing a disorder that is recessively inherited, although we also find evidence in one patient suggesting that heterozygotes may manifest. Compound heterozygotes have a significantly more severe phenotype raising the possibility of a dominant negative effect.
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PMID:The spectrum of clinical disease caused by the A467T and W748S POLG mutations: a study of 26 cases. 1743 11


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