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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 ...
Progressive myoclonus epilepsy (PME) is a group of diseases characterized by myoclonus, epileptic seizures, tonic–clonic seizures, and other serious symptoms such as trouble walking or speaking. These rare disorders often get worse over time and can be fatal.
Myoclonic seizures are brief jerks of limbs or body lasting milliseconds. [6] Tonic seizures are abrupt increases in muscle tone greater than 2 seconds in duration. [6] Clonic seizures occur as rhythmic body jerks. [7] Myoclonic-atonic seizures begins with one or more jerks (myoclonic phase) followed by a loss of muscle tone (atonic phase). [6]
Myoclonic astatic epilepsy (MAE), also known as myoclonic atonic epilepsy or Doose syndrome, and renamed "Epilepsy with myoclonic-atonic seizures" in the ILAE 2017 classification, is a generalized idiopathic epilepsy. It is characterized by the development of myoclonic seizures and/or myoclonic astatic seizures. Some of the common monogenic ...
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]
The majority of patients (58.2%) have frequent myoclonic jerks, [13] with some sources stating that all patients with JME have myoclonic seizures. [10] Generalized tonic–clonic seizures are less common [13] but still reported in 85–90%. [10] Absence seizures are believed to be least common, with an estimated prevalence between 10% and 40%.
MEAK is a form of progressive myoclonus epilepsy that typically begins between the ages of 3 and 15 years (the average of onset is 10 years). The first symptoms may include ataxia and myoclonus (unsteadiness and difficulty coordinating movements), along with generalized tonic-clonic ("grand mal") seizures.
Generalized seizures can take the form of myoclonic jerks, absences, or generalized tonic-clonic seizures. [2] Myoclonic jerks are the most common generalized seizures seen among reflex seizures and can be located in the limbs, trunk, or in specific regions of the body (e.g., in the muscles of the jaw or the eyelids).
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