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

The high fat, low carbohydrate, low protein ketogenic diet (KD) has been used to control refractory epilepsy in children since 1920, although its mechanism of action is unknown. Previous animal studies have shown that the KD can increase acute seizure threshold, but the effect of the KD on the process of epileptogenesis has not been studied. We tested the effect of an experimental KD on epileptogenesis in adult rats using the kainic acid (KA) model. P54 rats underwent KA-induced status epilepticus, followed by assignment to a control diet or a KD consisting of (by weight), 14% protein, 70% fat and no carbohydrate. KD-fed animals tolerated the diet and maintained ketosis. KD-fed rats had significantly fewer and briefer spontaneous recurrent seizures and less supragranular mossy fiber sprouting, although the degree of hippocampal pyramidal cell damage was similar in both groups. These results provide the first evidence that the KD retards epileptogenesis in an experimental model.
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PMID:Ketogenic diet reduces spontaneous seizures and mossy fiber sprouting in the kainic acid model. 1038 Sep 73

Prolonged status epilepticus (SE) can be refractory to conventional interventions, with high rates of subsequent morbidity and mortality. A high fat, low protein, low carbohydrate ketogenic diet (KD) has been used successfully to treat intractable epilepsy. However, its possible role in prolonged SE has not been well described. We report successful use of the KD in two adult patients with prolonged nonconvulsive SE (NCSE) refractory to multiple other interventions. Our observations suggest induction of ketosis may be a novel strategy to safely and effectively treat status in adults even after weeks to months of refractory seizures. Although there are few data regarding the use of the ketogenic diet in the treatment of adult epilepsy syndromes, it may be an option for the treatment of adults with refractory, prolonged SE.
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PMID:The ketogenic diet in treatment of two adults with prolonged nonconvulsive status epilepticus. 1984 31

Refractory status epilepticus (RSE), defined as status epilepticus that fails to respond to the acute administration of two antiepileptic medications, occurs in approximately a third of patients with status epilepticus, and is associated with increased hospital length of stay, mortality, and functional disability. Common presentations include: (1) generalized convulsive status epilepticus or complex partial status epilepticus that continue despite initial therapies; (2) stupor or coma following a generalized convulsive or complex partial seizure; or (3) stupor or coma following brain surgery or acute brain injury. When status epilepticus continues or recurs 24 hours or more after the initiation of anesthetic therapy, or recurs on the reduction or withdrawal of anesthesia, it is termed super RSE. Published evidence on optimal management of RSE consists largely of case reports or small series. The mainstay of treatment is the administration of anesthetic agents titrated to electrographic seizure control. Adjunctive therapies include hypothermia and immunosuppression and less commonly, surgery, electrical stimulation therapies, and induction of ketosis. Patients with cardiopulmonary complications and prolonged duration of drug-induced coma tend to have worse post-treatment functional outcomes. However, significant improvement over time can occur in survivors, and thus treatment is justified even in patients who require prolonged anesthetic coma. The strongest predictors of outcome are duration of anesthetic coma, etiology, and development of cardiopulmonary complications.
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PMID:Refractory status epilepticus: new insights in presentation, treatment, and outcome. 2333 20

Refractory status epilepticus carries significant morbidity and mortality. Recent reports have promoted the use of the ketogenic diet as an effective treatment for refractory status epilepticus. We describe our recent experience with instituting the ketogenic diet for 4 critically ill children in refractory status epilepticus, ranging in age from 9 weeks to 13.5 years after failure of traditional treatment. The ketogenic diet allowed these patients to be weaned off continuous infusions of anesthetics without recurrence of status epilepticus, though delayed ketosis and persistently elevated glucose measurements posed special challenges to effective initiation, and none experienced complete seizure cessation. The ease of sustaining myocardial function with fatty acid energy substrates compares favorably over the myocardial toxicity posed by anesthetic doses of barbiturates and contributes to the safety profile of the ketogenic diet. The ketogenic diet can be implemented successfully and safely for the treatment of refractory status epilepticus in pediatric patients.
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PMID:The ketogenic diet as broad-spectrum treatment for super-refractory pediatric status epilepticus: challenges in implementation in the pediatric and neonatal intensive care units. 2446 15

Background: Febrile infection-related epilepsy syndrome (FIRES) is a fatal epileptic encephalopathy associated with super-refractory status epilepticus (SRSE). Several treatment strategies have been proposed for this condition although the clinical outcomes are poor. Huge efforts from neurointensivists have been focused on identifying the characteristics of FIRES and treatment to reduce the mortality associated with this condition. However, the role of ketogenic diet (KD) in FIRES is not fully understood. Methods: We performed a retrospective review of patients who met the diagnostic criteria of FIRES, SRSE, and were treated with KD between 2015 and 2018 at the Department of Pediatrics, Xiangya Hospital of Central South University. The following data were recorded: demographic features, clinical presentation, anticonvulsant treatment, timing and duration of KD and follow-up information. Electroencephalography recordings were reviewed and analyzed. Results: Seven patients with FIRES were put on KD (5 via enteral route, and 2 via intravenous line) for SRSE in the PICU. The median age was 8. Four patients were male and 3 were female. Although patients underwent treatment with a median of 4 antiepileptic drugs and 2 anesthetic agents, the status epilepticus (SE) persisted for 7-31 days before KD initiation. After KD initiation, all patients achieved ketosis and SE disappeared within an average of 5 days (IQR 3.5), although there were minor side effects. In 6 patients, a unique pattern was identified in the EEG recording at the peak period. After initiation of KD, the number of seizures reduced, the duration of seizure shortened, the background recovered and sleep architecture normalized in the EEG recordings. The early initiation of KD (at the onset of SE) in the acute phase of patients decreased the mRS score in the subsequent period (p = 0.012, r = 0.866). Conclusions: The characteristic EEG pattern in the acute phase promoted timely diagnosis of FIRES. Our data suggest that KD may be a safe and promising therapy for FIRES with SRSE, and that early initiation of KD produces a favorable prognosis. Therefore, KD should be applied earlier in the course of FIRES. Intravenous KD can be an effective alternative route of administration for patients who may not take KD enterally.
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PMID:Ketogenic Diet as a Treatment for Super-Refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome. 3110 38