[Epileptic encephalopathies in infancy. How do we treat them? Does the aetiology influence the response to treatment?]

Abstract

INTRODUCTION:

Resistance to treatments is a common feature of Ohtahara, Aicardi, West and Dravet syndromes, as well as malignant migrating epilepsy in infancy.

AIMS:

To update the therapeutic management and to analyse whether the aetiology somehow determines the treatment.

DEVELOPMENT:

Convulsive seizures in the first year of life may be due to a potentially treatable aetiology, which makes it essential to carry out a complete evaluation so as to be able to begin, as early as possible, the most suitable and the non-specific symptomatic treatments to control the seizures, which prevents or minimises their deleterious effects. Metabolic disease must be ruled out and it is also essential to try a therapeutic regimen of vitamins and cofactors, as well as antiepileptic drugs. In Ohtahara and Aicardi syndromes, the first-order treatment is phenobarbital and phenytoin, and the most commonly used second-order drugs are midazolam, levetiracetam, lidocaine and valproate. In West's syndrome, the first-order treatment consists of adrenocorticotropic hormone and vigabatrine for the case of tuberous sclerosis; if there is no response, other pharmaceuticals, a ketogenic diet and surgery must be considered. For Dravet's syndrome, the main treatment consists in valproate with clobazam and stiripentol, and as the second order, other drugs and a ketogenic diet should be considered. In epilepsy with migrating seizures, the most effective treatment is with bromides, stiripentol, clonazepam and levetiracetam.

CONCLUSIONS:

Today there is little consensus on the therapeutic approach to be able to establish specific indications. The aetiology has an influence on the treatment, both in cases in which a curative treatment exists (metabolic diseases) and in the symptomatic management with antiepileptic drugs or other treatments (ketogenic diet or surgery).

• Authors: S Roldan
• Journal: Revista de neurologia (Rev Neurol) Vol. 64 Issue s03 Pg. S71-S75 (May 17 2017) ISSN: 1576-6578 [Electronic] Spain
• Vernacular Title: Encefalopatias epilepticas del lactante. Como las tratamos? Influye la etiologia en la respuesta al tratamiento?
• PMID: 28524224 (Publication Type: Journal Article, Review)

Chemical References

• Anticonvulsants
• Vitamin B 6
• Adrenocorticotropic Hormone

Topics

• Adrenocorticotropic Hormone (therapeutic use)
• Aicardi Syndrome (drug therapy)
• Anticonvulsants (therapeutic use)
• Combined Modality Therapy
• Diet, Ketogenic
• Disease Management
• Drug Resistant Epilepsy (drug therapy, etiology, therapy)
• Epilepsies, Myoclonic (drug therapy)
• Humans
• Infant
• Infant, Newborn
• Neurosurgical Procedures
• Spasms, Infantile (drug therapy, therapy)
• Treatment Outcome
• Tuberous Sclerosis (drug therapy, surgery)
• Vitamin B 6 (therapeutic use)