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Status Epilepticus in Neonates THIEME 2 Review Article Status Epilepticus in Neonates Arushi Gahlot Saini1 Kollencheri Puthenveettil Vinayan2 1 Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Address for correspondence Kollencheri Puthenveettil Vinayan, Institute of Medical Education and Research, Chandigarh, India Division of Pediatric Neurology, Department of Neurology, Amrita 2Division of Pediatric Neurology, Department of Neurology, Institute of Medical Sciences, Cochin, 682041, Kerala, India Amrita Institute of Medical Sciences, Amrita University, Cochin, (e-mail: [email protected]). Kerala, India Int J Epilepsy 2018;5:2–8 Abstract Seizures are a reflection of acute brain injury in the neonatal period, and status epilepti- cus reflects a state of high seizure burden. Neonatal status epilepticus has been report- ed in 8 to 43% of newborns with seizures. There is no separate definition for neonatal status epilepticus, and it has been commonly defined as a continuous seizure lasting at least 30 minutes and/or a series of seizures whose total duration exceeds 50% of a given epoch. The causes of status epilepticus in the neonatal period mirror the caus- es of neonatal seizures. It is symptomatic, usually associated with hypoxic–ischemic Keywords encephalopathy, inherited metabolic disorders, infections, and cerebral hemorrhage. ► neonatal seizures Management guidelines are not separately outlined for neonatal status epilepticus and ► neonatal status are commonly derived from the recommendations for neonatal and infantile seizures. epilepticus The presence of neonatal status epilepticus significantly increases the odds for devel- ► status epilepticus opment of cerebral palsy, global developmental delay, and epilepsy later in life. Further ► antiepileptic research is needed into the role of current antiepileptic drugs in causing neuronal inju- ► brain injury ry and use of neuroprotective agents during neonatal status epilepticus. Introduction The Unique Neurobiology of a Neonatal Brain Seizures are the most common reflection of an acute brain injury in the neonatal period. The first 4 weeks of life (neona- Neonatal seizure and status epilepticus are unique as the tal period) are at the highest risk for seizures secondary to an neonatal brain differs in both structure and physiologic func- underlying predisposition of the immature brain as well as tioning from the brains of children and adults. The neona- the nature of the inciting injury. Seizures occur in 1 to 3.5 per tal brain has a distinct predilection for neuronal excitation 1,000 births.1 The neonatal seizure continuum ranges from and subsequent seizures. Several probable neurobiological isolated events to recurrent seizures and status epilepticus. mechanisms have been put forward to explain this increased Neonatal status epilepticus (NSE) reflects a high seizure bur- susceptibility: den state and is associated with worse neurologic outcomes • There is an overexpression of glutamate (the major compared with recurrent seizures.2 excitatory neurotransmitter of the central nervous sys- NSE is distinct from status epilepticus in children and tem) as well as altered expression of its N-methyl-D- adults in definition, etiopathogenesis, electroencephalograph- aspartate (NMDA) (overexpression of NR2B subunits) and ic (EEG) features, management, and outcomes. It resembles α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid neonatal seizures more than it does status epilepticus at later (AMPA) receptors (lack of GluR2 subunit).3,4 ages. However, management recommendations are currently • There is reduction in γ-aminobutyric acid (GABA) (the based on those for children and adults with some age-specific major inhibitory neurotransmitter in the central nervous modifications. We discuss here the unique features of NSE and system), its receptor expression, and alteration in its dis- discuss the current recommendations for its diagnosis and tribution (overexpression of α4 and α2 subunits compared management. with α1) leading to benzodiazepine resistance.5 published online DOI https://doi.org/ Copyright ©2018 Indian Epilepsy July 21, 2018 10.1055/s-0038-1667213. Society ISSN 2213-6320. Status Epilepticus in Neonates Saini, Vinayan 3 • There is underexpression of KCC2 (pumps Cl− ions out) and The latest ILAE report suggests classifying neonatal seizures overexpression of NKCC1 (pumps Cl− in) channels on the according to the same descriptors as other seizures, rather immature neonatal neurons resulting in a higher intra- than a separate entity.13 NSE has not been as clearly defined cellular Cl− ion concentrations. GABA-agonist medications although the 5-minute duration seems more logical as major- such as phenobarbitone and benzodiazepines (meant to ity of neonatal seizures do not last > 2 to 3 minutes.14,15 It has cause neuronal hyperpolarization in a mature brain) thus been variably defined as a “continuous seizure activity lasting result in paradoxical depolarization in the immature neu- > 30 minutes and/or a series of seizures whose total duration rons due to the net efflux of Cl− ions.6 exceeds 50% of a given epoch.”2,16 Given the worse outcomes • This Cl− ion gradient matures in a caudo-rostral direc- associated with NSE and the need for prompt treatment, a tion during development. This maturational gradient clinical definition to treat “all frequent or prolonged seizures may lead to inhibition of motor neurons in the spinal ≥ 5 minutes in duration as status epilepticus” has also been cord and brainstem while depolarizing the cortical neu- used.2,17 This variability in defining NSE has led to inconsis- rons with the use of GABA-agonists, resulting in elec- tent estimates (8–43%) of the burden of NSE in newborns with tromechanical dissociation with phenobarbitone and seizures.18–20 The challenges in defining neonatal seizures and midazolam use.7 status epilepticus are21: • In the neonatal brain, although the basic organization • The seizure semiology, clinical and EEG characteristics of the cerebral cortex remains the same as in older chil- in neonates are different compared with older children. dren, the dendritic arborization, connections, and synap- Most seizures in the neonates are either subtle, multifo- tic stabilizations are incomplete and the myelination is cal, migratory, or rarely fully generalized whereas majori- immature. This prevents rapid, well-organized spread of ty of the current literature of status epilepticus is based on epileptic discharges in the brain and absence of the clas- well-organized, generalized tonic-clonic status. sic type of generalized, tonic-clonic status epilepticus in • As the average duration of neonatal seizures ranges from neonates. Additionally, the electroclinical dissociation and 1 to 5 minutes, and solitary prolonged seizures are rare; presence of subtle nonobtrusive signs such as autonomic hence, a 30-minute definition would underdiagnose NSE.22 variations, apnea, and oculomotor or oral-buccal-lingual • Neonates with seizures and status are commonly critical- movements in neonatal seizures suggest the possibility of ly ill and encephalopathic, thus making the recognition of a subcortical generator rather the overlying cerebral cor- baseline mental status or a return to it difficult. tex as in older children and adults.1 • Nearly 80 to 90% of electrographic seizures lack a clinical • Based on serial electrographic maturational changes correlate and would not be identified without continuous during status epilepticus during the first 6 months of life, EEG.23 Further, anticonvulsant administration may ter- it has been suggested that infants older than 2 months minate clinically evident seizures whereas electrograph- start exhibiting electrographic features similar to those of ic-only seizures may persist resulting in electroclinical status epilepticus seen in older children such as presence uncoupling or dissociation. of prolonged seizures or partial seizures with one focus • Defining NSE as any electrographic recording with seizure and secondary generalization.8 However, the electro- activity noted over a certain (most commonly > 50%) pro- graphic features of NSE in infants younger than 2 months portion of the length of the recording time24 would not be are usually distinct as described above. useful and difficult to apply in resource-limited settings where routine continuous EEG monitoring is not available Definitions and interictal recording times vary. Also, there is a clear lack of evidence to support any particular threshold to The conventional definition describes status epilepticus in designate a high seizure burden as SE.25 children and adults as “any continuous seizure activity last- ing ≥30 minutes or ≥2 seizures without regaining baseline Etiology mental status in between the events.”9 An operational defi- nition of status epilepticus proposes “any continuous clinical In general, the causes of NSE mirror the causes of neonatal seizure activity lasting for ≥ 5 minutes or ≥ 2 seizures with- seizures that are most often symptomatic markers of brain out regaining baseline mental status in between.”10 Recently, injury.26 The most common cause of recurrent seizures in the the International League Against Epilepsy (ILAE) has revised newborn is hypoxic ischemic encephalopathy (50–60%).20 the definition of status epilepticus as “a condition resulting These seizures are noted within the first 12 to 24 hours of either from failure of the mechanisms responsible for seizure life, associated with encephalopathy, and are subtle and mul- termination
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