Infantile spasms refer to subtle seizure activity in a baby, and not normal shivering due to being exposed to a cold draft or suddenly surprised. In fact, the appearance of infantile spasms actually indicates a type of epilepsy is present. The physical symptoms are also very specific. The infant usually arches his or her back or doubles over, while the arms and legs become rigid. A baby may experience hundreds of infantile spasms in a single day, most often right after nursing or upon waking from a nap. Also known as West Syndrome, this condition most commonly occurs between the ages of four and eight months of age, and seizures may continue until the age of five.
While the exact cause of infantile spasms cannot always be determined, they are generally due to an 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. In addition, while many children cease having these seizures as they mature, many others will unfortunately 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, jackknife convulsion syndrome, generalized flexion epilepsy syndrome, eclampsia nutans, and several other names. However, infantile spasms is now considered the preferred medical term.
Diagnosis of this disorder takes a few primary factors into consideration, most notably age at onset, positioning while seizing, the number of seizure clusters observed, and EEG abnormalities. The latter is cause for close scrutiny, since the presence of hypsarrhythmia, or erratic EGG patterns, is a key indicator of infantile spasms. In addition, the child’s medical history will be checked for any previous incidence 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 the majority of cases typically consists of the use of corticosteroids, such as prednisone. In some cases, adrenocorticotropic hormone (ACTH) may be administered, sometimes in combination with the drug vigabatrin. Several anti-epileptic drugs medications 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 necessary to remove them.