Information on Infantile Spasms

Infantile spasms also called West syndrome. Infantile spasms are an uncommon to rare and grave form of epilepsy in infants. About 60% of the affected infants have some brain disorder or brain injury before the seizures begin, but the others have had no apparent injury and have been developing normally. Infantile spasm (IS) is a specific type of seizure seen in an epilepsy syndrome of infancy. The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal or postnatal.

Eighty-five percent of kids who increase infantile spasms do so before the age of twelve months; most end having spasms by age 5. During this period, 40 percent will have other types of seizures. It is likely that one child in every 2,200 to 4,500 children born will grow infantile spasms. More boys than girls develop infantile spasms, but the figures vary. Bio-chemical mechanisms lead to the occurrence of West syndrome. It is conjectured that it is a malfunction of the neurotransmitter function, or more precisely, a malfunction in the regulation of the GABA transmission process.

The standard pattern of infantile spasms features minor head bobbing. Eventually, the child starts to have flexor, extensor, or mixed spasms. Flexor spasms are the most common. The child's neck, arms, and legs draw towards his chest. The child frequently bends at the waist in a “jackknife" motion. Extensor spasms are the least common. The child's neck, arms, and legs stretch out. Mixed spasms, in which the child's arms and body contract and his legs stretch out, are also very common. The goals of treatment for infants with West syndrome are the best quality of life with no seizures, the fewest adverse effects from treatment, and the least number of medications.

Steroid therapy is the first treatment for infantile spasms. Antiepileptic medications, such as vigabatrin (currently not approved for use in the US) have shown some efficacy. Sabril is often used as the initial therapy because it is relatively secure and helpful. It is especially effective for children with tuberous sclerosis (a disorder associated with abnormalities involving the brain, skin, heart, and other parts of the body). If the seizures are caused by a focal brain abnormality such as a tumour or a cyst, surgery to remove the abnormality. The ketogenic diet has been employed successfully to treat a variety of seizure types.

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