Infantile Spasms

Infantile Spasms (also known as West Syndrome, Generalized Flexion Epilepsy, Jackknife Convulsions, Salaam Spasms, Massive Myoclonia, and Infantile Myoclonic Encephalopathy) is a condition that begins in infancy. Babies with this condition show abnormal, jerking bodily movements called spasms; hence the name, Infantile Spasms. Children tend to show signs of the condition before they reach one year of age, often displaying symptoms at around four to eight months.



The cause of Infantile Spasms is now always known. However, the most common known cause of Infantile Spasms is a lack of oxygen to the baby during delivery (known as birth asphyxia, perinatal

asphyxia, or birth hypoxia). In some cases, the brain damage caused by this lack of oxygen could have been prevented if doctors had taken proper steps during delivery to ensure the baby was receiving enough oxygen. As a result, many children who have Infantile Spasms are victims of medical malpractice.

While some children who have Infantile Spasms recover completely, others will have serious problems that will require a lifetime of care. According to the National Institutes of Health, more than half the children with West Syndrome will go on to show signs of other kinds of seizures, like Lennox-Gastaut Syndrome.

The exact number of children suffering from Infantile Spasms is not known, but according to the Tuberous Sclerosis Alliance, it affects around one out of every two thousand babies. Infantile Spasms usually begin between one and twelve months of age and usually go away on their own, even if left untreated, by the age of two to four years old.

The spasms affect a child’s head, body, arms and legs. Spasms commonly occur in grouped clusters of as many as one hundred at a time. These movements may be small and barely noticeable like twitches, or large movements of the arms, legs, and body, followed by stiffening

of those parts or wrenching of the back and/or neck, often called retrocollis or opisthotonus. Infants may have dozens of clusters and several hundred spasms per day. The intensity and/or the number of the spasms may increase, decrease, or stop altogether over time. The spasms tend to occur after feeding or when the baby is tired, usually just after waking up or just before going to sleep. They rarely occur during sleep. Pathognomonic/pathognomic EEG tests of children suffering from Infantile Spasms exhibit a brain wave pattern called hypsarrhythmia.

Doctors can sometimes find the cause of Infantile Spasms and sometimes they can’t: origins of the condition can be prenatal, perinatal, or postnatal (occurring before, during, or shortly after birth). Because of this, doctors have divided patients with Infantile Spasms in two groups.

The first group consists of patients in whom an underlying problem can be identified (also called the symptomatic group). The kinds of problems that can be found in this group are many. As was mentioned earlier, the most common cause is a lack of oxygen to the baby during delivery. Other causes include such things as mothers who had infections during pregnancy, mothers who had been exposed to certain chemicals during pregnancy, and babies who have abnormal brain development for other reasons.

The second group includes those patients in whom no specific underlying problem can be found (also called the cryptogenic or idiopathic group) after different medical tests (blood tests, spinal taps, CAT scans, etc.) are done looking for a cause.

It is important to divide the groups this way, because children in the first category (symptomatic) almost always have problems for the rest of their lives (epilepsy, cerebral palsy, mental retardation), while patients in the second category (cryptogenic) often make a complete recovery.

The two most common treatments are medicines called Prednisone and ACTH (Adrenocorticotropic Hormone). These are related to the extent that both have something to do with cortisone, a chemical normally made in the adrenal glands of the body. Prednisone is a pill form of cortisone. ACTH is a hormone normally made in the brain, but which can also be given as a medicine to stimulate the adrenal glands to make extra cortisone.

Cortisone has been shown in many cases to stop the spasms. Most doctors think that controlling the spasms with early treatment with Prednisone and ACTH improves the chances of these patients developing normally.

Another medicine used to control Infantile Spasms is the epilepsy (anticonvulsive) medication Topiramate

If your baby has been diagnosed with Infantile Spasms, you may have a medical malpractice claim against healthcare providers who caused this injury. The malpractice claim is for the harm your child has suffered, and for the cost and care and treatment for your child in the future.

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