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Mild to severe intellectual or developmental disability is present in the majority of patients. [7] Epilepsy is present in the majority of cases, with approximately 80-98% of patients affected by seizures. [8] Truncal hypotonia and clumsy or ataxic gait are typical. [5] Behavioral and sleep problems are also common.
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 ...
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 epilepsy. [11]
Neurology. Benign Rolandic epilepsy or self-limited epilepsy with centrotemporal spikes (formerly benign childhood epilepsy with centrotemporal spikes (BECTS)) is the most common epilepsy syndrome in childhood. [1][2] Most children will outgrow the syndrome (it starts around the age of 3–13 with a peak around 8–9 years and stops around age ...
A seizure is a period of symptoms due to abnormally excessive or synchronous neuronal activity in the brain. [6] Outward effects vary from uncontrolled shaking movements involving much of the body with loss of consciousness (tonic-clonic seizure), to shaking movements involving only part of the body with variable levels of consciousness (focal seizure), to a subtle momentary loss of awareness ...
Status epilepticus (SE), or status seizure, is a medical condition consisting of a single seizure lasting more than 5 minutes, or 2 or more seizures within a 5-minute period without the person returning to normal between them. [3][1] Previous definitions used a 30-minute time limit. [2] The seizures can be of the tonic–clonic type, with a ...
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.
Neurology. Sudden unexpected death in epilepsy (SUDEP) is a fatal complication of epilepsy. [1] It is defined as the sudden and unexpected, non-traumatic and non-drowning death of a person with epilepsy, without a toxicological or anatomical cause of death detected during the post-mortem examination. [2][3] While the mechanisms underlying SUDEP ...
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