Common Pediatric Epilepsy Syndromes

Abstract

Benign rolandic epilepsy (BRE), childhood idiopathic occipital epilepsy (CIOE), childhood absence epilepsy (CAE), and juvenile myoclonic epilepsy (JME) are some of the common epilepsy syndromes in the pediatric age group. Among the four, BRE is the most commonly encountered. BRE remits by age 16 years with many children requiring no treatment. Seizures in CAE also remit at the rate of approximately 80%; whereas, JME is considered a lifelong condition even with the use of antiepileptic drugs (AEDs). Neonates and infants may also present with seizures that are self-limited with no associated psychomotor disturbances. Benign familial neonatal convulsions caused by a channelopathy, and inherited in an autosomal dominant manner, have a favorable outcome with spontaneous resolution. Benign idiopathic neonatal seizures, also referred to as "fifth-day fits," are an example of another epilepsy syndrome in infants that carries a good prognosis. BRE, CIOE, benign familial neonatal convulsions, benign idiopathic neonatal seizures, and benign myoclonic epilepsy in infancy are characterized as "benign" idiopathic age-related epilepsies as they have favorable implications, no structural brain abnormality, are sensitive to AEDs, have a high remission rate, and have no associated psychomotor disturbances. However, sometimes selected patients may have associated comorbidities such as cognitive and language delay for which the term "benign" may not be appropriate.

Dynamics of sensorimotor cortex activation during absence and myoclonic seizures in a mouse model of juvenile myoclonic epilepsy

“…3,4 Determining the cerebral networks that participate in different types of generalized seizures is critical to understand their epileptogenic mechanisms 5 and for the development of potential network-specific therapies (e.g., neurostimulation). [6][7][8][9] Different types of generalized seizures associate with very distinct behavioral and electrographic manifestations, [10][11][12] observations suggesting that the epileptic brain either uses different networks to generate different seizure types or that it uses the same network but activates it differently. For example, absence seizures confer 5-20 s of behavior arrest and loss of awareness associated with spikewave discharges (SWDs), 12 whereas myoclonic seizures produce very brief (<1 s), lightening-like body jerks associated with polyspike and wave discharges.…”

“…[6][7][8][9] Different types of generalized seizures associate with very distinct behavioral and electrographic manifestations, [10][11][12] observations suggesting that the epileptic brain either uses different networks to generate different seizure types or that it uses the same network but activates it differently. For example, absence seizures confer 5-20 s of behavior arrest and loss of awareness associated with spikewave discharges (SWDs), 12 whereas myoclonic seizures produce very brief (<1 s), lightening-like body jerks associated with polyspike and wave discharges. 12 Possibly, distinct networks are activated in these two seizure types.…”

“…For example, absence seizures confer 5-20 s of behavior arrest and loss of awareness associated with spikewave discharges (SWDs), 12 whereas myoclonic seizures produce very brief (<1 s), lightening-like body jerks associated with polyspike and wave discharges. 12 Possibly, distinct networks are activated in these two seizure types. In rat models of SWDs, ictal activation is first found in the somatosensory cortex (S1) barrel fields.…”

Possibilities of early diagnosis of autism spectrum disorder, with a special attention to Asperger syndrome: A systematic literature review

“…Epi� leptic seizures (irrespective of family history) of unknown etiology may appear as early as in the second month of age. 45 Consequently, such a child may be immediately qualified as having a greater risk of ASD.…”

Polysomnographic Aspects of Sleep Architecture on Self-limited Epilepsy with Centrotemporal Spikes: A Systematic Review and Meta-analysis

“… 1 , 15 Generalized seizures can occur with variable frequency, at times following a focal-onset seizure, and status epilepticus are rare. 17 Most ictal episodes occur during sleep, of which 35% occur in the two hours preceding awakening or upon awakening. 15 Also, about half of the patients experience less than five ictal episodes during the period of active epilepsy, which is usually short (less than 8 years in 97% of patients).…”