Dravet Syndrome (severe myoclonic epilepsy of infancy)
• Presentation:
- Age: year of age
* Seizure types: starts with prolonged febrile seizures (status epilepticus), generalized tonic clonic seizures (GTCS), atypical absence and complex partial seizures
• Diagnosis:
- Genetic testing
• Causes:
- Genetic causes
- Associated problems:
* Status epilepticus
* SUDEP
* Autism
* Movement disorders
* Upper respiratory infections
* Sleep problems
* Problems with nutrition and growth
• Prognosis:
- Seizures stabilize by age four . 85% survive to adulthood.
- Before seizures and then regression with developmental delay (???)
* Risk of intellectual disability
* Risk of persistent epilepsy into adulthood
* Risk of death
• Treatment: difficult to control.