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Juvenile Myoclonic Epilepsy (JME), also referred to as Janz Syndrome and Impulsive Petit Mal, is a form of epilepsy that is characterized by absence, Myoclonic, and Generalized Tonic-Clonic Seizures. This epilepsy variant is marked by its idiopathic and hereditary characteristics, as well as its generalization across seizures.
The pathophysiology of absence seizures has been linked to oscillatory thalamic-cortical potentials, calcium currents, and the interaction of GABAergic neurons. It seems clear that the pathophysiology of absence seizures differs from other epilepsies which may, in part, explain the unique efficacy of ethosuximide in this syndrome.
Syndromes are characterized into 4 groups based on epilepsy type: [1] a. Generalized onset epilepsy syndromes. These epilepsy syndromes have only generalized-onset seizures and include both the idiopathic generalized epilepsies (specifically childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy and epilepsy with generalized tonic- clonic seizures alone), as well as ...
The seizures can be of the tonic–clonic type, with a regular pattern of contraction and extension of the arms and legs, or of types that do not involve contractions, such as absence seizures or complex partial seizures. [1] Status epilepticus is a life-threatening medical emergency, particularly if treatment is delayed. [1]
Jeavons syndrome is a type of epilepsy. It is one of the most distinctive reflex syndromes of idiopathic generalized epilepsy characterized by the triad of eyelid myoclonia with and without absences , eye-closure-induced seizures, EEG paroxysms, or both, and photosensitivity .
There are three principal seizure types which may occur in JME: myoclonus, generalized tonic–clonic seizures and absence seizures. Approximately one-third of patients have all three seizure types. [13] The majority of patients (58.2%) have frequent myoclonic jerks, [13] with some sources stating that all patients with JME have myoclonic ...
This rare epilepsy has a wide age range of presentation (from the first year of life through the early teens). This epilepsy is characterized by absence seizures concurrent with myoclonic jerks, typically occurring several times daily. The genetics of this disorder have not been delineated. Seizures from this disorder often cease within 5 years.
Myoclonic seizures: seizures with rapid, brief contractions of muscles. Atonic seizures: seizures with a sudden loss of muscle tone, often resulting in sudden collapse. These are also called drop seizures or astatic seizures. Absence seizures: a generalized seizure characterized by staring off and occasionally some orofacial automatisms.
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