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Lennox–Gastaut syndrome (LGS) is a complex, rare, and severe childhood-onset epilepsy syndrome. It is characterized by multiple and concurrent seizure types including tonic seizure, cognitive dysfunction, and slow spike waves on electroencephalogram (EEG), which are very abnormal. [1] Typically, it presents in children aged 3–5 years and ...
Progressive Myoclonic Epilepsies (PME) are a rare group of inherited neurodegenerative diseases characterized by myoclonus, resistance to treatment, and neurological deterioration. [2] The cause of PME depends largely on the type of PME. Most PMEs are caused by autosomal dominant or recessive and mitochondrial mutations. [2]
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 ] It is very difficult to treat with anticonvulsant medications.
Deaths. 140,000 (2021) [ 9 ] Epilepsy is a group of non-communicable neurological disorders characterized by recurrent epileptic seizures. [ 10 ] An epileptic seizure is the clinical manifestation of an abnormal, excessive, and synchronized electrical discharge in the neurons. [ 1 ] The occurrence of two or more unprovoked seizures defines ...
Febrile infection-related epilepsy syndrome (FIRES), is onset of severe seizures (status epilepticus) following a febrile illness in someone who was previously healthy. [1] The seizures may initially be focal; however, often become tonic-clonic. [4] Complications often include intellectual disability, behavioral problems, and ongoing seizures ...
Unverricht–Lundborg disease. Unverricht–Lundborg disease (abbreviated ULD or EPM1) is the most common form of an uncommon group of genetic epilepsy disorders called the progressive myoclonus epilepsies. [1] It is caused due to a mutation in the cystatin B gene (CSTB). [2] The disease is named after Heinrich Unverricht, who first described ...
Status epilepticus is a life-threatening medical emergency, particularly if treatment is delayed. [1] Status epilepticus may occur in those with a history of epilepsy as well as those with an underlying problem of the brain. [2] These underlying brain problems may include trauma, infections, or strokes, among others.
Juvenile myoclonic epilepsy (JME), also known as Janz syndrome or impulsive petit mal, is a form of hereditary, idiopathic generalized epilepsy, [1] representing 5–10% of all epilepsy cases. [2][3][4] Typically it first presents between the ages of 12 and 18 with myoclonic seizures (brief, involuntary, single or multiple episodes of muscle ...
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