Severe myoclonic epilepsy of infancy, severe polymorphic epilepsy of infancy, borderland SMEI (SMEB), borderline SMEI, intractable childhood epilepsy with generalised tonic clonic seizures (ICEGTCS)
Pronunciation
/drəˈveɪ/
Specialty
Neurology
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] It is very difficult to treat with anticonvulsant medications. It often begins before one year of age,[1] with six months being the age that seizures, characterized by prolonged convulsions and triggered by fever, usually begin.[2]
Seizures are the most common form of DS.[2] DS is diagnosed clinically and genetic testing is recommended if there is any doubt.[2] Due to drug-refractory epilepsy in DS, many other therapies are being explored to prolong the life expectancy of patients.[3]
^ abShorvon SD, Guerrini R, Cook M, eds. (2013). Oxford textbook of epilepsy and epileptic seizures. Oxford: Oxford Univ. Press. p. 13. ISBN 978-0-19-965904-3.
^ abcMcIntosh AM, McMahon J, Dibbens LM, Iona X, Mulley JC, Scheffer IE, Berkovic SF (June 2010). "Effects of vaccination on onset and outcome of Dravet syndrome: a retrospective study". The Lancet. Neurology. 9 (6): 592–8. doi:10.1016/S1474-4422(10)70107-1. PMID 20447868. S2CID 2676315. ProQuest 314128956.
^Cite error: The named reference Couzin-Frankel_2015 was invoked but never defined (see the help page).
Dravetsyndrome (DS), previously known as severe myoclonic epilepsy of infancy (SMEI), is an autosomal dominant genetic disorder which causes a catastrophic...
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her M.D. thesis on the Lennox-Gastaut syndrome. In 1971 she was certified as psychiatrist. From 1965 to 2000, Dravet specialized in epilepsy at the Centre...
dystrophin gene. CRISPR has been used to correct for this. Similarly, for Dravetsyndrome, which is an epilepsy disorder, CRISPR has been used to correct the...
in epilepsy, by way of ion channel mutations such as those seen in Dravetsyndrome, a particularly severe form of childhood epilepsy that appears to carry...
resuscitation facilities. Stiripentol (2007). Indicated for the treatment of Dravetsyndrome. Barbiturates are drugs that act as central nervous system (CNS) depressants...
Several genetic associations have been identified, including GEFS+ and DravetSyndrome. Possible modes of inheritance for genetic predisposition to febrile...
properties; for example, it had anticonvulsant effects in a mouse model of Dravetsyndrome, a form of epilepsy. In an analysis by the University of Rhode Island...
Epidiolex for the treatment of seizures associated with Dravetsyndrome, Lennox–Gastaut syndrome, or tuberous sclerosis complex in people one year of age...
and adults with rare forms of epilepsy such as Lennox-Gastaut syndrome, Dravetsyndrome and tuberous sclerosis complex (TSC). On 31 October 2020, it was...
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cardiac channelopathies make up a key group of heart diseases. Long QT syndrome, the most common form of cardiac channelopathy, is characterized by prolonged...
medical cannabis oil as a possible treatment for pediatric epilepsy (Dravetsyndrome). In partnership with the University of British Columbia, Tilray provided...
earlier for some epilepsy and genetic syndromes where it has shown particular usefulness. These include Dravetsyndrome, infantile spasms, myoclonic-astatic...
conductance regulator (CFTR) Beta thalassaemia (β-globin) Hurler syndromeDravetsyndrome Usher syndrome Nonsense mutations in other genes may also drive dysfunction...
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