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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). [2] IESS is an epileptic encephalopathy, a childhood epilepsy syndrome arising during infancy. [3]
What are infantile spasms? The spasms are actually seizures. They are a form of epilepsy that tends to occur between the first 4 and 8 months of a baby’s life, according to the National ...
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.
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).
It is an abnormal interictal pattern, consisting of high amplitude and irregular waves and spikes in a background of chaotic and disorganized activity seen on electroencephalogram (EEG), and frequently encountered in infants diagnosed with infantile spasms, although it can be found in other conditions such as tuberous sclerosis.
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]
A simple febrile seizure is generalized, occurs singularly, and lasts less than 15 minutes. [19] A complex febrile seizure can be focused in an area of the body, occur more than once, and lasts for more than 15 minutes. [19] Febrile seizures affect 2–4% of children in the United States and Western Europe, it is the most common childhood ...
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