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Sleep-related hypermotor epilepsy (SHE), previously known as nocturnal frontal lobe epilepsy, is a form of focal epilepsy characterized by seizures which arise during sleep. The seizures are most typically characterized by complex motor behaviors. It is a relatively uncommon form of epilepsy that constitutes approximately 9-13% of cases.
The epileptic seizure in the vast majority of pediatric epilepsy patients is ephemeral, and symptoms typically subside on their own after the seizure comes to an end, but some children experience what is known as a “seizure cluster," in which the first seizure is followed by a second episode approximately six hours later.
Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is an epileptic disorder that causes frequent violent seizures during sleep. These seizures often involve complex motor movements, such as hand clenching, arm raising/lowering, and knee bending. Vocalizations such as shouting, moaning, or crying are also common.
The consequence is avoidable misdiagnosis, high morbidity, and costly mismanagement. Autonomic seizures and autonomic status epilepticus as occur in Panayiotopoulos syndrome have not been described in other epileptic syndromes in that sequence though 10–20 per cent of children with the same seizure semiology may have cerebral pathology.
Thus, some children may have numerous diurnal visual seizures and only a few seizures that are exclusively nocturnal or occur on awakening. [citation needed] Frequency of seizures: If untreated, patients experience frequent and brief visual seizures (often several every day or weekly). However, propagation to other seizure manifestations, such ...
The mainstay symptoms are seizures that are frequent – occurring daily and difficult to treat with anti-seizure medications. An estimated 30% of patients with infantile epileptic spasm syndrome (formerly called West syndrome) have been reported to progress with LGS. [8] [9] Tonic seizures are the most common and present in nearly everyone ...
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%.
The onset of seizures is between the ages of 2 and 5 years of age. EEG shows regular and irregular bilaterally synchronous 2- to 3-Hz spike-waves and polyspike patterns with a 4- to 7-Hz background. 84% of affected children show normal development prior to seizures; the remainder show moderate psychomotor retardation mainly affecting speech.
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