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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.
A spike-and-wave discharge is a regular, symmetrical, generalized EEG pattern seen particularly during absence epilepsy, also known as ‘petit mal’ epilepsy. [1] The basic mechanisms underlying these patterns are complex and involve part of the cerebral cortex, the thalamocortical network, and intrinsic neuronal mechanisms. [2]
Childhood absence epilepsy (CAE), formerly known as pyknolepsy, is an idiopathic generalized epilepsy which occurs in otherwise normal children. The age of onset is between 4–10 years with peak age between 5–7 years. Children have absence seizures which although brief (~4–20 seconds), they occur frequently, sometimes in the hundreds per ...
Juvenile myoclonic epilepsy (JME), also known as Janz syndrome or impulsive petit mal, is a form of hereditary, ... and a minority of patients have absence seizures. [6]
[3]: 458 Samuel-Auguste Tissot (1728–1797) authored Traité de l’Epilepsie, a book describing grand état (generalized tonic-clonic seizures) and petit état (absence seizures). [3]: 460 Jean-Étienne Dominique Esquirol (1772–1840) later introduced grand mal (generalized tonic-clonic seizures) and petit mal to describe these seizures.
These initial observations led to the development of two breeding colonies: [3] [4] (i) a fully inbred strain of rats, with 100% of animals displaying the EEG and behavioral characteristics of absence seizures, derived from an outbred Wistar colony and called the Genetic Absence Epilepsy Rats from Strasbourg (GAERS) (ii) a strain of non epileptic control animals selected from the same initial ...
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]
Ethosuximide, sold under the brand name Zarontin among others, is a medication used to treat absence seizures. [4] It may be used by itself or with other antiseizure medications such as valproic acid. [4] Ethosuximide is taken by mouth. [4] Ethosuximide is usually well tolerated. [5]
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