Infantile spasms, also known as West syndrome, is a rare and severe form of epilepsy that affects infants. This condition is often associated with brain disorders or injuries, although it can also occur in infants who have been developing normally. This article provides a detailed overview of infantile spasms, including its causes, symptoms, and treatment options.
Infantile spasms (IS) are a specific type of seizure that is part of an epilepsy syndrome that occurs during infancy. This syndrome is age-related, typically occurring between the third and twelfth month of life, with the majority of cases manifesting around the fifth month. The cause of this syndrome is often linked to an organic brain dysfunction, which could have originated prenatally, perinatally, or postnatally.
Approximately 85% of children who develop infantile spasms do so before the age of twelve months, and most stop having spasms by age 5. During this period, 40% of these children will experience other types of seizures. It is estimated that one child in every 2,200 to 4,500 children born will develop infantile spasms. The condition is more common in boys than girls, although the exact figures vary. The occurrence of West syndrome is believed to be due to a malfunction in the neurotransmitter function, specifically in the regulation of the GABA transmission process.
The typical pattern of infantile spasms involves minor head bobbing. Over time, the child may begin to experience flexor, extensor, or mixed spasms. Flexor spasms, which are the most common, involve the child's neck, arms, and legs drawing towards their chest, often bending at the waist in a "jackknife" motion. Extensor spasms, which are less common, involve the child's neck, arms, and legs stretching out. Mixed spasms, where the child's arms and body contract while their legs stretch out, are also common. The primary goal of treatment for infants with West syndrome is to provide the best quality of life possible, with no seizures, minimal adverse effects from treatment, and the least number of medications.
Steroid therapy is often the first line of 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 safe and effective. It is particularly effective for children with tuberous sclerosis, a disorder associated with abnormalities in the brain, skin, heart, and other parts of the body. If the seizures are caused by a focal brain abnormality, such as a tumor or a cyst, surgery may be performed to remove the abnormality. The ketogenic diet has also been successfully used to treat a variety of seizure types.
For more information on infantile spasms, visit the Epilepsy Foundation or the National Institute of Neurological Disorders and Stroke.
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