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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]
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. If the abnormal brain wave activity is persistent and results from ongoing seizures, then a diagnosis of myoclonic epilepsy may be ...
The sudden jerks and twitching of the body can often be so severe that it can cause a small child to fall. A myoclonic seizure (myo "muscle", clonic "jerk") is a sudden involuntary contraction of muscle groups. The muscle jerks consist of symmetric, mostly generalized jerks, localized in the arms and in the shoulders and also simultaneously ...
Atypical absence seizures occur with a less sudden impairment in awareness, often accompanied by a gradual head, limb, or truncal slumping. [6] Myoclonic absence seizures occur with myoclonic jerks of arms and shoulders. [6] Absence with eyelid myoclonia seizures occur with 4-6 per second eyelid myoclonic jerks and upward eye movement. [6]
Therefore, in the early stages of PME the symptoms and EEG may appear like Generalized epilepsy, Juvenile myoclonic epilepsy, benign childhood myoclonic epilepsy, and Huntington's disease. [3] It is crucial for ensure initial treatment is appropriate to measure how the condition progresses. Incorrect treatment can also result in wrong PME ...
ii. Juvenile myoclonic epilepsy (JME) presents in developmentally normal teens and young adults with myoclonic seizures, that often occur in the morning and can be triggered by sleep deprivation or flashing lights. The majority (over 90%) also will develop generalized tonic-clonic seizures, which are often heralded by a cluster of myoclonic jerks.