Definition:
Infantile spasms, or West syndrome, is an interictal EEG pattern termed hypsarrhythmia, and mental retardation, although the diagnosis can be made even if one of the 3 elements is missing (according to the international classification). This severe epilepsy syndrome is an age-dependent manifestation of a damaged brain.
Diagnosis:
When a baby with infantile spasms has an EEG, the doctor will usually see an unusual pattern called hypsarrhythmia when the seizures are not occurring. This chaotic, high-voltage pattern is often crucial in confirming the diagnosis
Treatment:
Compared with other forms of epilepsy, West syndrome is hard to treat. To increase the chance of successful treatment and keep down the risk of longer-lasting effects, it is very important that the condition is diagnosed as early as possible and that treatment begins straight away. There is no guarantee, however, that therapy will work even in this case.
Symptoms and Signs:
The primary manifestation may just be a minor twitch of a single limb or eye. However, there are often other linked symptoms. Many children who develop infantile spasms also have difficulties with feeding s and develop reflux. Arching backwards while feeding is a sign for infantile spasms. Many children who develop infantile spasms also have sight difficulties, and failure to focus and track visually is another sign.
Causes:
It is still unknown which bio-chemical mechanisms results to the occurrence of West syndrome. It is conjectured that it is a malfunction of the neurotransmitter function, or more exactly, a malfunction in the regulation of the GABA transmission process. Another theory being researched is a hyper-production of the Corticotropin-releasing hormone (CRH). It is possible that more than one factor is concerned. Both hypotheses are supported by the effect of certain medications used to treat infantile spasms.
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