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Infantile epileptic spasms syndrome (IESS) previously known as West syndrome needs the inclusion of epileptic spasms for diagnosis. [1] Epileptic spasms (also known as infantile spasms) may also occur outside of a syndrome (that is, in the absence of hypsarrhythmia and cognitive regression) - notably in association with severe brain disorders (e.g. lissencephaly).
Ohtahara syndrome (OS), also known as Early Infantile Developmental & Epileptic Encephalopathy (EIDEE) [2] is a progressive epileptic encephalopathy.The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life, [3] and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG).
Patients usually present first with seizures within the first months of life, followed by infantile spasms which progress to epileptic seizures that are largely refractory to treatment. [2] [3] Development of gross motor skills, such as sitting, standing, and walking, is severely delayed, along with restricted fine motor skills. [4]
Aicardi syndrome is a rare genetic malformation syndrome characterized by the partial or complete absence of a key structure in the brain called the corpus callosum, the presence of retinal lacunes, and epileptic seizures in the form of infantile spasms. [2]
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. [9] [10] Approximately 50% of patients receive a diagnosis of autism spectrum disorder. [8] Some patients have significant ...
Infantile spasms are treated with specific types of medication, most commonly either oral steroid, ACTH or vigabatrin. The long-term outcome is often worrisome. While infantile spasms often stop with medication, many babies will develop other seizure types over time and most will be left with intellectual disability.
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 with LGS. [10] [11] They occur most frequently during non-REM sleep (90% of the time). They initially last for a minute or less ...
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.