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Antiepileptic Drugs in Children
Published in Stanley R. Resor, Henn Kutt, The Medical Treatment of Epilepsy, 2020
W. Edwin Dodson, James D. Reggin
Benzodiazepines are used most frequently in pediatric epilepsy to treat status epilepticus and severe epilepsies, especially the encephalopathie epilepsies—West syndrome and Lennox-Gastaut syndrome (8). Although they are effective in absence seizures, the development of tolerance to the antiepileptic effect plus the high incidence of sedative and adverse behavioral effects render them generally unsatisfactory in benign epilepsies such as childhood absence epilepsy. Furthermore, the occurrence of withdrawal seizures makes them tedious to discontinue. Rectally administered diazepam (DZP) is effective in preventing recurrent febrile seizures. Studies that are currently underway are testing the possibility that orally administered benzodiazepines might also be effective in this situation (see Chapter 14). Although not established by controlled studies, intermittently administered benzodiazepines are helpful in treating patients who have infiequent but severe clusters of seizures (see Chapter 37). When possible, it is best to avoid chronic treatment with benzodiazepines because of the many problems associated with their chronic administration.
Seizures
Published in Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan, Diagnosing and Treating Common Problems in Paediatrics, 2017
Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan
Childhood absence epilepsy is a form of idiopathic generalised epilepsy and account for more than 10% of childhood epilepsy. A family history is often present. Seizures start between the ages of 4 and 10 years, with a peak incidence at 4–7 years. Typical absence seizures are characterised by sudden impairment of consciousness, the child stares blankly and is non-responsive. There may be minor motor phenomena, including jerking of eyelids or subtle hands automatism. Episodes are brief, typically lasting 10 seconds. The child has no recollection of the event and there is no post-ictal phase, with the child quickly resuming normal activities. Up to 100 seizures can occur a day, with typically 20 seizures occurring daily. School performance may be adversely affected. Absence seizures must be distinguished from focal seizures. Focal seizures are often longer in duration, may be associated with an aura, have more complex automatisms and are followed by a post-ictal phase.
Didi
Published in Walter J. Hendelman, Peter Humphreys, Christopher R. Skinner, The Integrated Nervous System, 2017
Walter J. Hendelman, Peter Humphreys, Christopher R. Skinner
Childhood absence epilepsy is a specific epilepsy syndrome whose diagnostic criteria are outlined in the International League Against Epilepsy classification system of epileptic disorders (see Table 11.3). Childhood absence epilepsy is now known to be a genetic disorder, as are the other non-localization-related ‘idiopathic’ epilepsy syndromes listed in Table 11.3. In the case of childhood and juvenile-onset absence epilepsy, a growing list of gene mutations has been described. To date, the majority of gene mutations implicated in absence epilepsy have either been in subunits of GABA receptors or components of calcium channels (those interested are directed to the review article by Yalçin in the chapter reading list).
Non-convulsive status epilepticus in Sotos syndrome: rare first presentation in a rare syndrome
Published in International Journal of Neuroscience, 2023
Christine Ragaie, Nirmeen A Kishk, Amani M. Nawito, Alshimaa S. Othman, Rehab Magdy
Routine laboratory evaluation was done and showed no abnormality. The electroencephalogram (EEG) was done as soon as possible and showed SSW & polyspike slow waves, fluctuating in frequency between 1.5-2 Hertz (Hz), lasting through all the period of recording (30 minutes) & corresponding to altered consciousness, staring & eyelid fluttering (Figure 1A and B). Diagnosis of NCSE was made according to the modified Salzburg criteria (Leitinger et al. 2015). Intravenous levetiracetam was given (20 mg/kg), after which the patient regained his baseline consciousness. The mother reported history of similar episode 3 weeks earlier lasting for 2 hours & resolving spontaneously without medical intervention. She also reported history of single episode of febrile seizures at age of 1.5 years, but no history of other seizure types. Pre-, intra- & immediate post-natal history were unremarkable, apart from polyhydramnios during pregnancy. Family history was significant for childhood absence epilepsy at his younger sister, since the age of 8 years, currently controlled on valproate monotherapy (1000 mg/day).
Pediatric epilepsy surgery: the earlier the better
Published in Expert Review of Neurotherapeutics, 2018
Kees P. J. Braun, J. Helen Cross
In some pediatric epilepsy syndromes, such as childhood absence epilepsy and benign epilepsy with centrotemporal spikes, spontaneous ‘cure’ may come with time; after a certain age, seizures do not recur and AEDs can be safely and permanently discontinued. Children who are considered surgical candidates, however, by definition suffer from a – presumed – lesional form of focal epilepsy. When the MRI of a child with nonsyndromic epilepsy is abnormal, the chance of reaching spontaneous complete remission, defined as being at least 5 years seizure free and medication free, is only 32.7% [10]. The long-term seizure outcome of children with a single seizure and a brain lesion – who, according to previous definitions would in the past not necessarily have been classified as having epilepsy – is less known. Even when children reach early seizure control after start of AEDs, there is no guarantee for permanent success of drug treatment. Over 40% of children with intractable focal epilepsy met the criteria for intractability only 3 or more years after diagnosis, and 76% of them had shown a prior remission of at least 1 year [11]. The only independent factor predicting enduring intractability in newly diagnosed childhood epilepsy was a neuroimaging abnormality [12]. The chance of reaching seizure freedom without surgery in children with early intractability and abnormal neuroimaging was only 8.6% [12]. In other words; a child with focal epilepsy and a documented lesional cause – by definition a likely surgical candidate – has a low chance of spontaneous and permanent cure, particularly if the epilepsy proves resistant to medication from the outset.
How to select the appropriate pharmacotherapy for absence seizures in children
Published in Expert Opinion on Pharmacotherapy, 2018
Chiara Mazzocchetti, Pasquale Striano, Alberto Verrotti
Childhood absence epilepsy (CAE) is an age-dependent, genetic form of generalized nonmotor epilepsy, characterized by frequent brief staring episodes. This epilepsy syndrome accounts for approximately 10–17% of all childhood-onset epilepsies though it has an excellent prognosis. Nevertheless, up to 20% of patients which present do not go into remission despite the adequate availability of drug therapies [1].