Dravet syndrome, previously 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 is treated with anticonvulsant medications. It often begins around six months of age.
- Myoclonic epilepsy refers to a family of epilepsies that present with myoclonus. When myoclonic jerks are occasionally associated with abnormal brain wave activity, it can be categorized as myoclonic seizure.
- These myoclonic twitches, jerks, or seizures are usually caused by sudden muscle contractions (positive myoclonus) or brief lapses of contraction (negative myoclonus). The most common circumstance under which they occur is while falling asleep (hypnic jerk).
Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy that typically becomes apparent during infancy or early childhood. Affected children experience several different types of seizures most commonly atonic, tonic and atypical absence seizures.
- An atonic seizure (also called drop seizure, akinetic seizure or drop attack) is a type of seizure that consists of partial or complete loss of muscle tone that is caused by temporary alterations in brain function. These seizures are brief – usually less than fifteen seconds.
- Tonic seizures have been divided into four types:
*Axial tonic seizures begin with a tonic contraction of the neck muscles, leading to fixation of the head in an erect position, widely opened eyes, and jaw clenching or mouth opening. Contraction of the respiratory and abdominal muscles often follows and may lead to a high-pitched cry and brief periods of apnea.
*Tonic axorhizomelic seizures begin with a sequence similar to the axial type, but then the tonic contractions extend to the proximal musculature of the upper limbs, elevating the shoulders and abducting the arms.
*In global tonic seizures, the tonic contractions extend to the periphery of the limbs. The arms are pulled upward to a semiflexed position in front of the head, and the fists are clenched, producing a body position similar to that of a child defending himself against a facial blow. The lower extremities also can be involved, leading to a fall if the child is in a standing position.
*Asymmetric tonic seizures vary from a slight rotation of the head to a tonic contraction of all the musculature of one side of the body.
- An atypical absence seizure has less abrupt onset and offset of loss of awareness than typical absence seizures. They are often associated with other features such as loss of muscle tone of the head, trunk or limbs (often a gradual slump) and subtle myoclonic jerks.
Tuberous sclerosis complex is a genetic disorder characterized by the growth of numerous noncancerous (benign) tumors in many parts of the body. These tumors can occur in the skin, brain, kidneys, and other organs, in some cases leading to significant health problems.
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