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Dravet syndrome
Dravet syndrome, also known as severe myoclonic epilepsy of infancy (SMEI), is a type of epilepsy with seizures that are often triggered by hot temperatures or fever.〔 It often begins around six months of age.
==Signs and symptoms==
Dravet syndrome has been characterized by prolonged febrile and non-febrile seizures within the first year of a child’s life. This disease progress to other seizure types like myoclonic and partial seizures, psychomotor delay, and ataxia. it is characterized by cognitive impairment, behavioral disorders, and motor deficits. Behavioral deficits often include hyperactivity and impulsiveness, and in more rare cases, autistic-like behaviors.〔 Dravet syndrome is also associated with sleep disorders including somnolence and insomnia.〔 The seizures experienced by people with Dravet syndrome become worse as the patient ages since the disease is not very predictable when first diagnosed.〔 This coupled with the range of severity differing between each individual diagnosed and the resistance of these seizures to drugs has made it challenging to develop treatments.〔
Dravet syndrome appears during the first year of life, often beginning around six months of age with frequent febrile seizures (fever-related seizures). Children with Dravet syndrome typically experience a lagged development of language and motor skills, hyperactivity and sleep difficulties, chronic infection, growth and balance issues, and difficulty relating to others. The effects of this disorder do not diminish over time, and children diagnosed with Dravet syndrome require fully committed caretakers with tremendous patience and the ability to closely monitor them.〔Miller, I.O. and Sotero de Menezes. M.A. SCN1A-Related Seizure Disorders. 2007 Nov 29 (2014 May 15 ). GeneReviews® (). Seattle (WA): University of Washington, Seattle; 1993-2015. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1318/〕
Febrile seizures are divided into two categories known as simple and complex. A febrile seizure would be categorized as complex if it has occurred within 24 hours of another seizure or if it lasts longer than 15 minutes. A febrile seizure lasting less than 15 minutes would be considered simple. Sometimes modest hypothermic stressors like physical exertion or a hot bath can provoke seizures in affected individuals.〔 However, it goes without saying that any seizure at all- whether generalized or partial, absence or tonic/clonic- that goes on uninterrupted for much longer than 2 to 5 minutes, without a resumption of postictal (more normal; recovery-type; after-seizure) consciousness, should be treated as a medical emergency, since uninterrupted seizures can lead to status epilepticus (SE) - a state of unremitting seizures that can be difficult to stop, and eventually potentially fatal, if not soon treated.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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