Infantile spasms are a type of seizure activity in infants that indicate the presence of epilepsy. The physical symptoms include arching of the back and stiffening of the arms and legs. The condition, also known as West syndrome, usually occurs between four and eight months of age and can continue until age five. The cause is often due to impaired development of the nervous system, but can also be caused by genetic or metabolic disorders or neurological damage. Treatment varies but often includes corticosteroids or antiepileptic drugs.
Infantile spasms refer to subtle seizure activity in an infant and not the normal shivering from exposure to a cold current or sudden surprise. Indeed, the appearance of infantile spasms actually indicates that a type of epilepsy is present. The physical symptoms are also very specific. The child usually arches his back or doubles over, while his arms and legs stiffen. A baby can experience hundreds of infantile spasms in just one day, most often right after feeding or waking up from a nap. Also known as West syndrome, this condition most commonly occurs between four and eight months of age, and seizures can continue until age five.
Although it is not always possible to determine the exact cause of infantile spasms, they are usually due to impaired development of the nervous system during the first year of life. However, this scenario can be driven by other factors, such as genetic disorders, metabolic disorders and neurological damage caused by an injury sustained during birth or even during the neonatal period. Also, while many children stop having these seizures as they mature, many more will sadly develop other problems later on, such as Lennox-Gastaut syndrome, a type of epilepsy.
There are a number of cross-terms commonly used in association with this condition other than West syndrome. For example, this disorder has been referred to as Blitz-Nick-Salaam-Krämpfe, switchblade-knife syndrome, flexion generalized epilepsy syndrome, eclampsia nutans, and many other names. However, infantile spasms is now considered the preferred medical term.
The diagnosis of this disorder takes into consideration a few primary factors, in particular age of onset, positioning during the seizure, number of seizure clusters observed, and EEG abnormalities. The latter is cause for close scrutiny, as the presence of hypsarrhythmia, or irregular EGG patterns, is a key indicator of infantile spasms. Also, the child’s medical history will be checked for any previous incidences of problems known to be associated with this disorder, such as hypoxic ischemic encephalopathy. Finally, various neurological tests, imaging scans, and metabolic tests usually follow.
Treatment varies with each child. However, treatment in most cases typically consists of the use of corticosteroids, such as prednisone. In some cases, adrenocorticotropic hormone (ACTH) may be given, sometimes in combination with the drug vigabatrin. Several antiepileptic drugs are also used to treat infantile spasms, including topiramate and lamotrigine. If the disorder is found to be caused by lesions in the brain, surgery may be needed to remove them.
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