Related Experiment Videos
[Therapeutic strategy in severe encephalopathies]
1Servicio de Neuropediatría.; Hospital Clínico Universitario San Carlos, Madrid, 28040, España. jcampos@hcsc.insalud.es
Insights
Severe infantile epilepsy syndromes are often drug-resistant with poor cognitive outcomes. While some treatments show limited success in West syndrome and Lennox-Gastaut syndrome, neonatal myoclonic encephalopathies and Dravet syndrome have limited therapeutic viability.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neuroscience
Context:
- Severe epileptic syndromes in infancy present significant challenges due to drug resistance and poor prognosis.
- These conditions, including neonatal myoclonic encephalopathies, West syndrome, and Lennox-Gastaut syndrome, require precise electroclinical differentiation.
- Understanding the nuances of these syndromes is crucial for developing effective management strategies.
Purpose:
- To review the electroclinical semiology and therapeutic strategies for severe infantile epileptic syndromes.
- To evaluate the efficacy of current treatments, including pharmacological and surgical options.
- To differentiate between various infantile epilepsy syndromes and their respective prognoses.
Summary:
- Neonatal myoclonic encephalopathies and severe myoclonic epilepsy of childhood show limited therapeutic response.
- West syndrome and Lennox-Gastaut syndrome exhibit variable treatment outcomes, influenced by etiology.
- Cryptogenic forms of West and Lennox-Gastaut syndromes offer better seizure control (20-30%) but with only 5-9% achieving normal intelligence.
Impact:
- Highlights the limited efficacy of current treatments for certain severe infantile epilepsies.
- Emphasizes the importance of etiology in predicting treatment success for West and Lennox-Gastaut syndromes.
- Underscores the poor cognitive outcomes even with seizure control in these challenging pediatric epilepsy conditions.
Introduction:
The severe epileptic syndromes of infancy are dependent on age, symptomatic or cryptogenic aetiology and are drug-resistant so that overall prognosis control of epilepsy and adequate cognition is poor so that some authors consider it to be catastrophic. All may be identified and differentiated on electroclinical criteria.
Development:
We study the neonatal myoclonic encephalopathies in the two clinical forms described by Aicardi and Ohtahara, West s syndrome (infantile spasms), severe myoclonic epilepsy of Dravet-Dalla Bernardina and the Lennox-Gastaut syndrome, briefly describing their electroclinical semiology and therapeutic strategies using the range of available drugs, together with other medical and surgical therapeutic options.
Conclusions:
Treatment of severe epileptic encephalopathies of childhood is useless/not viable in neonatal myoclonic encephalopathies and severe myoclonic epilepsy of childhood, whilst in the West and Lennox-Gastaut syndromes the results depend on the aetiology, and are better in the cryptogenic types with control of 20% and 30% of the cases but with normal intelligence in only 5% and 9%.