Seizure disorders in infancy and childhood
Related Experiment Videos
Insights
Childhood epilepsy prognosis has improved, with an 81.7% remission rate observed over 10 years. Age-dependent epileptic encephalopathy (ADEE) shows developmental links between early infantile epileptic encephalopathy (EIEE), West syndrome, and Lennox syndrome.
Area of Science:
- Pediatric Neurology
- Epileptology
- Developmental Neuroscience
Background:
- Childhood epilepsy prognosis has significantly improved due to advancements in epileptology and new antiepileptic drugs.
- Long-term observations indicate a high remission rate for childhood epilepsy, though some syndromes remain intractable.
Purpose of the Study:
- To describe the long-term prognosis of childhood epilepsy.
- To investigate the developmental aspects of age-dependent epileptic encephalopathy (ADEE).
- To outline the concept and categorization of ADEE, focusing on early-infantile epileptic encephalopathy with suppression-burst (EIEE).
Main Methods:
- A 10-year longitudinal observation of epileptic children.
- Long-term follow-up study of early-infantile epileptic encephalopathy (EIEE) cases.
- Analysis of clinical seizure patterns and EEG evolution.
Main Results:
- An 81.7% remission rate for childhood epilepsy was observed over three years.
- West syndrome and Lennox syndrome were identified as having a high proportion of intractable cases.
- Six of 10 EIEE cases evolved into West syndrome, and two transitioned to Lennox syndrome, demonstrating a developmental continuum.
Conclusions:
- There is a close relationship and developmental progression among EIEE, West syndrome, and Lennox syndrome (types of ADEE).
- Developmental aspects are crucial for understanding the progression of these epileptic encephalopathies.
- Improved antiepileptic drugs have enhanced the overall prognosis for childhood epilepsy.
Abstract:
Of various topics concerning convulsive disorders in children, long-term prognosis of childhood epilepsy and developmental aspects of age-dependent epileptic encephalopathy (ADEE) were described. Recent progress in epileptology and introduction of effective antiepileptic drugs has allowed marked improvement in the prognosis of epilepsy. According to our long-term observation of epileptic children over 10 years, the remission rate for over three years was as high as 81.7%. Intractable cases were notably high in the West and Lennox syndromes. One of the main targets of child epileptology is ADEE, i.e. the West and Lennox syndromes in addition to early-infantile epileptic encephalopathy with suppression-burst (EIEE). The concept and categorization of ADEE were outlined with special reference to the developmental aspects of EIEE by a long-term follow-up study. Six of 10 cases of EIEE evolved into the West syndrome at two to six months of age, and two cases showed further transition to the Lennox syndrome at one year one month and three years one month of age. In accordance with the evolutional change in clinical seizure pattern, EEG showed an evolutional change from suppression-burst to hypsarhythmia and further development to diffuse slow spike-waves. These facts suggest a close relationship among these three types of ADEE and the significance of developmental aspects in the study of epilepsy.