| Keywords [eng] |
Long-term outcome of developmental and epileptic encephalopathies,Dravet syndrome and EEG, Dravet syndrome and long-term outcome,Menkes disease and West syndrome,West syndrome and EEG: long-term outcome, Infantile spasms to Lennox-Gastaut, West syndrome long-term outcome, Lennox-Gastaut syndrome etiology,Lennox-Gastaut and EEG,Epilepsy with myoclonic-atonic seizure, Doose syndrome clinical characteristic, Epilepsy with myoclonic-atonic seizures long-term outcome, Epileptic encephalopathy with continuous spike-waves during slow-wave sleep, Landau-Kleffner syndrome,Landau-Kleffner syndrome etiology. |
| Abstract [eng] |
This literature review focused on selected childhood-onset epilepsies and how they evolve over time until adulthood. Among different childhood-onset epilepsies, this writing focused more deeply on Dravet syndrome, West syndrome, Lennox-Gastaut syndrome, Epilepsy with myoclonic-atonic seizures (EMAS) and Landau-Kleffner syndrome. Additionally, few emerging syndrome were briefly introduced: DEE-SWAS, FIRES, EIMFS and SeLFNIE. The purpose of this review was to describe how seizures, EEG findings, cognitive function and clinical features changes with age and which factors seem to be linked to long-term outcome. Studies which was reviewed showed markedly that childhood-onset epilepsies are very heterogenous. Long-term outcome is depending on several aspects for instance etiology, age at onset, type of syndrome, seizure severity, abnormalities on EEG and response to treatment. In many cases seizure frequency tends to lower with age and some seizure types may disappear, but it is not promising necessarily good overall outcome. Cognitive, behavioral, motor or language difficulties commonly remain into adulthood. In Dravet syndrome, it is common that seizure pattern changes over time. Prolonged febrile seizures, myoclonic seizures and status epilepticus are typical in childhood, while in adulthood seizures continues in different for and tends to appear during sleep. Additionally, many adults have intellectual disability, gait problems and need support in everyday life. Even if seizures becomes less frequent, the syndrome usually remains severe. Outcome of West syndrome varies a lot depending on etiology and how quickly treatment is initiated. Early and effective treatment was linked to better developmental outcome especially in children with no underlying structural cause. However, many patients have poor long-term cognitive outcome, persisent seizures, increased mortality or later develops Lennox-Gastaut syndrome. One of the poorest long-term outcome of syndromes which was reviewed was Lennox-Gastaut syndrome. Seizure types and EEG abnormalities may change with age, however complete seizure remission is uncommon. Many patients continue to have seizures, especially tonic seizures during sleep. Additionally, most have moderate to severe intellectual disability and needs support in everyday life. Epilepsy with myoclonic-atonic seizures (EMAS) showed widest variation in outcome. Some patients become seizure free and develop normally or near to normal, while the others may have more severe course with drug-resistant seizures, non-convulsive status epilepticus and cognitive difficulties in later life. Poor outcom was linked to early onset, tonic seizures, resistance to treatment, prolonged status epilepticus and persistent EEG abnormalities. Landau-Kleffner syndrome is different in some aspects comparing to other syndromes. Seizures commonly improve or stop by adolescnece and abnormalities on EEG may also lessen, however language difficulties may remain for several years and can have major effect on school, communication and quality of life. In this syndrome the long-term burden often comes more from persistent aphasia than from seizures. Overall, this review shows that long-term outcome does not only depend on seizures alone. In many syndromes, outcome is depending also by underlying etiology and by the effect of epileptic activity on the developing brain. |
