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Dravet syndrome

Orpha number ORPHA33069
Synonym(s) SMEI
Severe myoclonic epilepsy of infancy
Prevalence Unknown
Inheritance
  • Autosomal dominant
Age of onset Neonatal/infancy
ICD-10
  • G40.4
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

Dravet syndrome (DS) is one of the most severe types of childhood-onset genetic epilepsy characterized by a variety of drug-resistant seizures (including generalized tonic-clonic seizures (GTCS) and myoclonic seizures), presenting in previously healthy children, often induced by fever, and that can lead to cognitive and motor impairment.


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Detailed information

Emergency guidelines
  • EN (2008,pdf)
  • FR (2008,pdf)
  • PT (2008,pdf)
  • IT (2008,pdf)
Article for general public
  • FR (2011,pdf)
Clinical genetics review
  • EN (2011)
Disability factsheet
  • FR (2013,pdf)
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The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.