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Published online by Cambridge University Press:  21 November 2024

James Dolbow
Affiliation:
University Hospitals Cleveland Medical Center
Neel Fotedar
Affiliation:
University Hospitals Cleveland Medical Center
Joshua Edmondson
Affiliation:
University Hospital Cleveland Medical Centter
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Summary

Dravet syndrome is a type of developmental epileptic encephalopathy (DEE), which typically begins in the first year of life with a febrile hemi-clonic seizure. This later progresses to more frequent, prolonged afebrile seizure types including focal and generalized clonic, myoclonic, and atypical absence seizures that are refractory to treatment. Within a year of seizure onset, developmental delay becomes evident and the child eventually shows profound cognitive and behavioral deficits. Because of frequent convulsive seizures, the patients with Dravet syndrome have a much higher risk of sudden unexpected death in epilepsy (SUDEP) at ~9.3 per 1000 patient-years, which is almost the same as adults with drug-resistant epilepsy. In fact, the most common cause of death in Dravet syndrome is SUDEP.

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The Neurology Riddle Book
150 Common and Rare Neurological Diseases in Riddle Form
, pp. 101 - 104
Publisher: Cambridge University Press
Print publication year: 2024

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  • 33
  • James Dolbow, University Hospitals Cleveland Medical Center, Neel Fotedar, University Hospitals Cleveland Medical Center, Joshua Edmondson, University Hospital Cleveland Medical Centter
  • Book: The Neurology Riddle Book
  • Online publication: 21 November 2024
  • Chapter DOI: https://doi.org/10.1017/9781009527378.035
Available formats
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Save book to Dropbox

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  • 33
  • James Dolbow, University Hospitals Cleveland Medical Center, Neel Fotedar, University Hospitals Cleveland Medical Center, Joshua Edmondson, University Hospital Cleveland Medical Centter
  • Book: The Neurology Riddle Book
  • Online publication: 21 November 2024
  • Chapter DOI: https://doi.org/10.1017/9781009527378.035
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • 33
  • James Dolbow, University Hospitals Cleveland Medical Center, Neel Fotedar, University Hospitals Cleveland Medical Center, Joshua Edmondson, University Hospital Cleveland Medical Centter
  • Book: The Neurology Riddle Book
  • Online publication: 21 November 2024
  • Chapter DOI: https://doi.org/10.1017/9781009527378.035
Available formats
×