Dravet syndrome, 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. It is very difficult to treat with anticonvulsant medications. It often begins before 1 year of age, with 6 months being the age that seizures, char­ac­ter­ized by prolonged convulsions and triggered by fever, usually begin. Signs and symptoms
Dravet syndrome has been characterized by prolonged febrile and non-febrile seizures within the first year of a childs life. This disease progresses to other seizure types like myoclonic and partial seizures, psychomotor delay, and ataxia. It is characterized by cognitive impairment, behavioural disorders, and motor deficits. Behavioural deficits often include hyperactivity and impulsiveness, and in more rare cases, autistic-like behaviours. 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, as the disease is not very observable when symptoms first appear. 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.
However, conditions associated with ulegyria, such as epilepsy and cerebral palsy, can be treated using the appropriate treatment. For instance, seizures caused by epilepsy, due to the presence of ulegyria in the occipital lobe, can be controlled using antiepileptic drugs in some patients. In other patients, such as those who suffer from ulegyria in the posterior cortex, drugs are not effective and surgery of the area causing epilepsy is needed. These treatments treat only the conditions but have no effect on the condition of ulegyria itself. == References ==