Dravet syndrome: clinical features

Evidence-based neurology checklist on dravet syndrome: clinical features: Epidemiology This is an infantile onset epileptic encephalopathy It causes severe myoclonic epilepsy of infancy There is a family history of epilepsy in 25% of cases Pertussis vaccination may provoke an earlier onset by…

Epidemiology

  • This is an infantile onset epileptic encephalopathy
  • It causes severe myoclonic epilepsy of infancy
  • There is a family history of epilepsy in 25% of cases
  • Pertussis vaccination may provoke an earlier onset by causing fever 

Types

Onset features

Developmental features

Speech and language impairments

Seizure types

Seizure features

Sleep disorders

Other features

Dravet syndrome in adulthood

Acronym

References

  1. Millichap JJ, Koh S, Laux LC, Nordli DR. Dravet syndrome: when to suspect the diagnosis. Neurology 2009; 73:e59-e62.
  2. van Dam VS, Korff CM. Dravet syndrome: an update. Schweiz Arch Neurol Psychiatr 2013; 164:153-157.
  3. Dravet C. The core Dravet syndrome. Epilepsia 2011; 52(Suppl 2): 3-9.
  4. Carvill GL, Weckchuysen S, McMahon JM, et al. GABRA1 and STXBP1: novel genetic causes of Dravet syndrome. Neurology 2014; 82:1245-1253.
  5. Cetica V, Chiari S, Mei D, et al. Clinical and genetic factors predicting Dravet syndrome in infants with SCN1A mutations. Neurology 2017; 88:1037-1044. 
  6. And 12 more. Subscribe to see the full list

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