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Dravet syndrome (DS), previously known as severe myoclonic epilepsy of infancy (SMEI), is an autosomal dominant genetic disorder which causes a catastrophic form of epilepsy, with prolonged seizures that are often triggered by hot temperatures or fever. [1]
8 Μαΐ 2023 · Myoclonic epilepsy of infancy (MEI) is a rare self-limited epileptic syndrome characterized by brief myoclonic seizures in previously healthy and developmentally normal children with onset in the first three years of life.
3 Ιουν 2022 · Myoclonic seizures are a fairly common seizure type, especially in people with juvenile myoclonic epilepsy (JME). In JME, myoclonic seizures are one of the main seizure types. JME is a common form of childhood generalized (genetic) epilepsy. JME makes up about 5% to 10% of all epilepsy cases.
Dravet syndrome, previously known as Severe Myoclonic Epilepsy of Infancy (SMEI), is an intractable developmental and epileptic encephalopathy that begins in infancy and proceeds with accumulating morbidity that significantly impacts individuals throughout their lifetime.
8 Σεπ 2023 · Several epilepsy syndromes cause myoclonic seizures. Together, they are often known as progressive myoclonic epilepsy. They have several features in common, including:
The term “myoclonic seizure” is used if myoclonus results from abnormal brain activity. In epilepsy, myoclonic seizures usually cause quick jerking movements on both sides of the body at the same time. They occur in a variety of epilepsy syndromes that have different characteristics: Juvenile Myoclonic Epilepsy
Severe Myoclonic Epilepsy in infancy (SMEI, or Dravet syndrome) is a drug-resistant epilepsy that occurs in the first year of life of previously healthy children. The main clinical features are prolonged and repeated febrile and afebrile generalized or unilateral convulsive seizures.