Epidemiology of Severe Myoclonic Epilepsy of Infancy

  title={Epidemiology of Severe Myoclonic Epilepsy of Infancy},
  author={Daniel L. Hurst},
  • D. Hurst
  • Published 1 August 1990
  • Medicine
  • Epilepsia
Summary: Severe myoclonic epilepsy of infancy (SMEI) is a newly recognized epileptic syndrome. It is characterized by multiple febrile seizures, often prolonged, subsequent development of uncontrollable mixed‐myoclonic seizures, and, eventually, psychomotor retardation. Drugs for myoclonic epilepsy–valproate (VPA), the suximides, and the benzodiazepines–have been shown to be useful in SMEI. Among children with seizures in the National Institute of Neurological and Communicative Disorders and… 
Severe Myoclonic Epilepsy in Infancy – Adult Phenotype with Bradykinesia, Hypomimia, and Perseverative Behavior: Report of Five Cases
The motor and behavioural symptoms are discussed in the context of a possibly underlying frontal lobe/mesofrontal and cerebellar dysfunction and may be fit to define the adult phenotype of SMEI beyond seizure/epilepsy criteria.
Severe Myoclonic Epilepsy of Infants (Dravet Syndrome): Natural History and Neuropsychological Findings
The data suggest that SMEI can be considered as a prototype of an epileptic encephalopathy, and language capacities were better preserved than visuospatial functions, and behavior improved.
Dravet syndrome (severe myoclonic epilepsy in infancy).
It is crucial to diagnose this epilepsy soon after its onset in order to prescribe the most appropriate treatment, as there are no well-established correlations between genotype and phenotype.
Dravet syndrome
Dravet syndrome is a rare disorder characterized by an early, severe, generalized, epileptic encephalopathy followed by different types of seizures (either focal or generalized), which are typically resistant to antiepileptic drugs.
Early diagnosis of severe myoclonic epilepsy in infancy
Of 329 epileptic patients referred in a six year period with the first seizure occurring in the first year of life, 20 met the following criteria: generalized seizures excluding infantile spasms,
"Severe myoclonic epilepsy in infancy". Relevance for the clinician of severe epilepsy starting in infancy.
Treatment of severe myoclonic epilepsy in infancy has changed, and a simple combination of two major anti-epileptic drugs (sodium valproate and topiramate) and a strict acute seizure treatment significantly improve the quality of life for patients.
Update on Dravet syndrome
  • A. Covanis
  • Medicine
    Developmental medicine and child neurology
  • 2011
The eponymous term ‘Dravet syndrome (DS)’ was introduced by the ILAE task force in 2001, 2006 and the latest Commission Report in 2010 to include all groups of epilepsy patients.
Epileptic Encephalopathies with Myoclonic Seizures in Infants and Children (Severe Myoclonic Epilepsy and Myoclonic-Astatic Epilepsy)
  • R. Guerrini, J. Aicardi
  • Medicine
    Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
  • 2003
A number of cases of myoclonic epilepsies in infancy and early childhood, however, remain unclassified, and intermediate forms between the different syndromes exist, and differentiation is often difficult and may require extensive neurophysiologic studies.
Severe Myoclonic Epilepsy in Infancy: Toward an Optimal Treatment
Severe myoclonic epilepsy in infancy, or Dravet syndrome, is one of the catastrophic epilepsy syndromes. In the past, treatment was mainly based on valproate and phenobarbital. Recently, some of the
Dravet Syndrome: An Overview
Dravet syndrome (DS), also known as severe myoclonic epilepsy of infancy (SMEI), is one of the rare early childhood intractable epileptic encephalopathies associated with pleomorphic seizure


Severe myoclonic epilepsy of infancy.
  • D. Hurst
  • Medicine
    Pediatric neurology
  • 1987
Seizure control can be achieved in SMEI with aggressive use of drugs which are beneficial for myoclonic seizures, and five of 7 patients are doing significantly better than the literature would suggest is possible.
Epilepsie myoclonique grave de la premiere annee
The evolution is characterized by the persistence of fits and the appearance of severe language disorder and light cerebellar and pyramidal signs and the nosological problems of this severe infant myoclonic epilepsy.
The myoclonic epilepsies of childhood.
ARELATIVELY important proportion of the epilepsies that occur during the first 5 to 8 years of life include frequently repeated seizures characterized clinically by brief muscular contraction or loss
Predictors of epilepsy in children who have experienced febrile seizures.
Epilepsy developed in children whose neurologic or developmental status was suspect or abnormal before any seizure and whose first seizure was complex (longer than 15 minutes, multiple or focal) epilepsy developed at a rate 18 times higher than in children with no febrile seizures.
Classifications of Epileptic Syndromes: Advantages and Limitations for Evaluation of Childhood Epileptic Syndromes in Clinical Practice
Summary: The advantages and limitations of the two most recent International League Against Epilepsy classifications of the epilepsies and epileptic syndromes have been assessed after examining the
Age at onset of seizures in young children
Age at onset of seizures in young children and its relationship to factors such as prior neurological status and neurological outcome were examined. Of 52,360 children, 39,270 of whom were followed
Proposal for Classification of Epilepsies and Epileptic Syndromes
  • Medicine
  • 1985
The purpose of the ICE is to supplement the International Classification of Epileptic Seizures, the revised form of which was accepted by the General Assembly of the ILAE in September, 1981.
Age at onset of seizUrrs in young children
  • Ann Neurol
  • 1984
eds. Advances in epileprology: Xlllth epilepsy inrernarional symposium
  • 1982