Search

West syndrome is a severe form of epilepsy with onset in infancy and early childhood. The characteristic triad described includes infantile spasms (IS), an abnormal electroencephalogram (EEG) pattern referred to as hypsarrhythmia, and developmental regression. It comprises 2% of childhood epilepsies. Etiologically, West syndrome can be classified into symptomatic with known etiology and cryptogenic with unknown etiology. Hypsarrhythmia is defined as chaotic, asynchronous, disorganized slow background activity with high-voltage spike activity. Burst-suppression pattern is a rarely described pattern of modified hypsarrhythmia, which denotes severe disease. Hereby, we describe the case of a female infant who presented with IS; EEG showed a modified hypsarrhythmia pattern in the form of burst suppression, and magnetic resonance imaging brain showed complete corpus callosal agenesis. She was initiated on injection of adrenocorticotropic hormone and showed marked improvement in epileptic spasms.

West syndrome is a severe form of epilepsy with onset in infancy and early childhood. The characteristic triad described includes infantile spasms (IS), an abnormal electroencephalogram (EEG) pattern referred to as hypsarrhythmia, and developmental regression. It comprises 2% of childhood epilepsies. Etiologically, West syndrome can be classified into symptomatic with known etiology and cryptogenic with unknown etiology. Hypsarrhythmia is defined as chaotic, asynchronous, disorganized slow background activity with high-voltage spike activity. Burst-suppression pattern is a rarely described pattern of modified hypsarrhythmia, which denotes severe disease. Hereby, we describe the case of a female infant who presented with IS; EEG showed a modified hypsarrhythmia pattern in the form of burst suppression, and magnetic resonance imaging brain showed complete corpus callosal agenesis. She was initiated on injection of adrenocorticotropic hormone and showed marked improvement in epileptic spasms. [ABSTRACT FROM AUTHOR]

Nonketotic hyperglycinemia is a rare inborn error of glycine metabolism characterized by a severe metabolic encephalopathy with drug-resistant seizures. Here, we report the outcome of nonketotic hyperglycinemia in a cohort of patients diagnosed and followed-up at a tertiary care reference center in Lebanon, between 2000 and 2014. Eight out of 12 patients with nonketotic hyperglycinemia were retrospectively reviewed. The remainders were excluded for incomplete data. The majority of cases presented with seizures and hypsarrhythmia or burst suppression patterns. Half of the patients died. Survival varied between 7 days and 18 years. Seizures remained unresponsive with poor outcome, despite standard supportive care and antiepileptic therapy; however, two patients were responsive to ketogenic diet and one of them became seizure-free. Scarce data on the outcome of nonketotic hyperglycinemia patients from the Middle East and North Africa region are available. The ketogenic diet, in combination with standard therapies, appears to be effective in controlling the seizures in this devastating disorder. Larger multicenter studies are still needed to establish the role of the ketogenic diet in nonketotic hyperglycinemia. [ABSTRACT FROM AUTHOR]

We here report an unusual case of a term neonate who presented with myoclonic seizures and lactic acidemia at 30 hours of life, who was later on diagnosed as a case of pyridoxine dependant epilepsy. Pyridoxine dependant epilepsy is one of the rare but easily treatable causes of refractory neonatal seizures. Failure to diagnose and treat this condition early would result in devastating neurodevelopmental outcomes for the surviving newborn.

• Status epilepticus is a neurological emergency that requires timely administration of appropriate medications. • Ketamine effect of noncompetitive NMDA receptor antagonism was augmented by co-administering perampanel acting at AMPA receptors. • Data is limited on the use of perampanel in humans. • This combination could provide if validated, a novel approach to treating status epilepticus. Status epilepticus (SE) continues to be a challenging neurological emergency with high morbidity and mortality. During treatment, different regimens are practiced encompassing all known seizure termination mechanisms. To our knowledge, this is the first case series report describing EEG patterns and clinical outcomes in patients treated with ketamine and perampanel (PER) concomitantly. To assess clinical and electrographic outcomes in patients receiving dual antiglutamatergic therapy in SE. Twenty-one out of twenty five patients were treated with ketamine, and four patients with ketamine were associated with PER. In the ketamine plus PER group, three out of four patients had convulsive SE, and one had non-convulsive status epilepticus (NCSE), whereas eight patients in the ketamine group had NCSE. The incidence of beta pattern appearance on EEG after starting patients on ketamine and PER was achieved in all four patients (100%) compared to (61.9%) in the other group. A burst suppression pattern was recorded in 75% of patients treated with ketamine and PER, in comparison to 28.5% of patients in patients treated with a different regimen. The time to resolution of SE was significantly shorter in the ketamine group (median 24 (24–64) h vs. 6 (05–144) h p > 0.05). Moreover, the average number of days on IV anesthetic was slightly lower in a patient treated with PER concomitantly. In terms of morbidity, the average increase in mRS was also lower in the ketamine and PER group, although it was not statistically significant. Dual anti-glutamatergic therapy could provide a favorable approach to treating SE, which yet needs to be further investigated through larger randomized control studies. [ABSTRACT FROM AUTHOR]

Objective To develop a computational method to detect and quantify burst suppression patterns (BSP) in the EEGs of critical care patients. A multi-center validation study was performed to assess the detection performance of the method. Methods The fully automatic method scans the EEG for discontinuous patterns and shows detected BSP and quantitative information on a trending display in real-time. The method is designed to work without setting any patient specific parameters and to be insensitive to EEG artifacts and periodic patterns. For validation a total of 3982 h of EEG from 88 patients were analyzed from three centers. Each EEG was annotated by two reviewers to assess the detection performance and the inter-rater agreement. Results Average inter-rater agreement between pairs of reviewers was κ = 0.69. On average 22% of the review segments included BSP. An average sensitivity of 90% and a specificity of 84% were measured on the consensus annotations of two reviewers. More than 95% of the periodic patterns in the EEGs were correctly suppressed. Conclusion A fully automatic method to detect burst suppression patterns was assessed in a multi-center study. The method showed high sensitivity and specificity. Significance Clinically applicable burst suppression detection method validated in a large multi-center study. [ABSTRACT FROM AUTHOR]

Most of the metabolic disorders can lead to neurologic disease, including neonatal epileptic encephalopathy. Sometimes, neonatal seizures may be the first sign of inborn errors of metabolism, a fact that makes them much more difficult to diagnose. Thus, a high index of suspicion is required. Distinctively, however, epilepsy secondary to inborn errors of metabolism is usually associated with other neurologic symptoms, such as microcephaly, hypotonia, or hypertonia. After exclusion of acquired causes such as structural or infectious causes, inborn errors of metabolism should always be considered in the differential diagnosis. Among the most common metabolic causes are disorders of neurotransmitter metabolism, disorders of energy production, and synthetic or catabolic disorders associated with brain malformation, dysfunction and degeneration. In most cases, epilepsy secondary to inherited metabolic disorders is characterized by polymorphic clinical and electroencephalographic features, such that they are difficult to classify into precise epileptic syndromes. However, specific types of seizures, such as myoclonic seizures, or distinctive electroencephalographic patterns, such as suppression burst patterns, epileptic syndrome or early myoclonic encephalopathy, may suggest a specific metabolic disease. Timely diagnosis and early treatment may improve the prognosis of these disorders. An early diagnosis of inborn errors of metabolism is crucial from the point of view of a favorable long-term outcome, because some of them can be treated effectively with dietary restriction or supplementation. In addition, all are inherited disorders that may recur in future pregnancies, and therefore their diagnosis allows for appropriate genetic counseling. The treatment of epileptic encephalopathies due to inborn errors of metabolism generally relies on the use of one or more anticonvulsant medications along with evaluation and management of any underlying etiology. Certain rare vitamin-responsive inborn errors of metabolism, for example, may present as neonatal encephalopathy with anticonvulsant-resistant seizures. Therefore, the clinicians caring for newborns with epileptic encephalopathy should consider these particular disorders early in the hospital course. In this review, the importance of considering inborn errors of metabolism in the differential diagnosis of epileptic encephalopathies is emphasized, and an overview of the diagnoses and treatments of inborn errors of metabolism most commonly associated with neonatal epileptic encephalopathy is given. [ABSTRACT FROM AUTHOR]

Tranexamic acid (TXA) is an antifibrinolytic agent that is commonly used in cardiac, gynecologic, and obstetric surgeries. Inadvertent intrathecal injection of the TXA may lead to serious side effects including myoclonus, seizure, and ventricular fibrillation. So far, the proconvulsive properties of TXA and its mechanism of action are poorly understood. Medical error leads to serious adverse effects that can be attributed to similar appearance of ampoules, location of ampoules, and incorrect labeling of prefilled syringes. We herein report a case of refractory status epilepticus after accidental intrathecal injection of TXA. The patient was treated with thiopentone infusions until achieving a burst suppression pattern on electroencephalogram along with other antiepileptic drugs. Subsequently, the patient recovered completely without any neurological deficit. To the best of our knowledge, this is the first case of refractory status epilepticus after inadvertent intrathecal injection of TXA. Such catastrophic complications can be avoided by vigilance, correct labeling of syringes and ampoules, double checking medications prior to administration, and preventing manufacturing of vials of different drugs with similar appearance. [ABSTRACT FROM AUTHOR]