How Often Do Infantile Spasms Occur – Infantile spasms often called West Syndrome, are a rare special type of seizure with both focal and generalized features. Infantile spasms usually begin in the first year of life (over 90% of cases begin before 12 months of life), typically starting between three and six months of age, but some children may experience spasms as early as one month and appear as brief stiffening movements that last a to two seconds each. The attacks often resemble a sudden bending forward of the body with stiffening of the arms and legs, lasting 1-2 seconds; some children arch their backs when they stretch their arms and legs. Infantile spasms may involve the whole body or only the head or part of the face. Spasms typically recur a few seconds apart in what is called a cluster, and the infant usually appears to recover or relax between each spasm. Clusters of spasms often occur after awakening from sleep and often occur in multiple clusters and hundreds of attacks per day.
Infantile spasms are characterized by epileptic spasms, which consist of massive myoclonic jerks of the body, which may be extensor or flexor (or both) in nature. Infantile spasms are often accompanied by developmental problems and a characteristic interictal brain wave pattern on electroencephalography (EEG) testing called hypsarrhythmia. Most children, but not all, will have EEG measurements of hypsarrhythmia. When all three features are present, the term “West syndrome” is commonly used. West syndrome is characterized by an electroclinical triad of (1) epileptic spasms, (2) hypsarrhythmia on EEG examination, and (3) developmental stagnation or regression. West syndrome is considered catastrophic because of the frequent sequelae of global neurodevelopmental delay, significant intellectual disability, and medically refractory epilepsy.
How Often Do Infantile Spasms Occur
Infantile spasms (West syndrome) usually stop by the age of five, but may be replaced by other types of seizures. Many underlying conditions, such as birth defects, metabolic disorders, and genetic disorders can give rise to infantile spasms, making it important to identify the underlying cause. In some children, no cause can be found.
Long Term Weekly Adrenocorticotropic Hormone Therapy For Relapsed Infantile Spasms
. About 1,200 children in the United States are diagnosed with infantile spasms each year. Infantile spasms are slightly more common in males, accounting for about 60% of cases, and a family history exists in 3% to 6% of cases
Infantile spasms are so uncommon that most pediatricians will see only one or two cases of infantile spasms in all their years of practice. Also, infantile spasms often resemble common disorders such as a normal startle reflex, colic or reflux.
It is very important to recognize that a child has infantile spasms as soon as they start because
Unfortunately, children who develop infantile spasms are at high risk for developmental disabilities and autism, but some children will do well if treated early. Because the spells can be subtle, diagnosis can be delayed for weeks or months
What Parents Should Know About Infantile Spasms
. However, using the revised classification based on the 2001 International League Against Epilepsy report, this epileptic syndrome can be classified as: Level I (ictal phenomenology): clinical spasms, flexor, extensor, mixed or subtle; Level II (type of epileptic seizure): epileptic spasms; Level III (epilepsy syndrome): infantile spasm syndrome
Determining the cause of infantile spasms is very important because it affects treatment and prognosis. After careful evaluation, the underlying cause can be identified in more than 70% of cases
. There are dozens of disorders known to cause infantile spasms. When neurologists are able to identify the cause, it is labeled “symptomatic.” It is important that an appropriate diagnostic evaluation be performed on each child. A diagnosis is important because it leads to specific treatment that can improve the long-term developmental outcome. In fact, some children with infantile spasms can eventually lead normal lives, but only if they are diagnosed and treated correctly.
The goal of treating infantile spasms is for the seizures to stop and the EEG to improve (hypsarrhythmia should disappear). Standard first-line treatments for infantile spasms include several forms of hormone therapy (including adrenocorticotrophic hormone [ACTH] or prednisone) or the anti-seizure drug vigabatrin (GABA aminotransferase inhibitor)
Deep Bench: Infantile Spasms Are Rare But Dangerous
. These treatments are highly effective but have serious side effects and should be administered in consultation with a pediatric neurologist. Some children have spasms as a result of brain lesions, and surgical removal of these lesions can result in improvement. When standard treatments do not lead to improvement, other options such as the ketogenic diet and anti-seizure medications are considered. Regardless of the specific treatment chosen, it is critical to begin treatment as soon as possible. Ongoing infantile spasms (and hypsarrhythmias) have the potential to adversely affect all aspects of brain development.
To make it clear what infantile spasms look like, please watch these videos of a child with infantile spasms.
. His description is as accurate today as it was then. It is a remarkable report because Dr. West described his own son and he asked for help. The following is a quote from Dr. West’s report describing his son:
Like Dr. West’s son Many children with infantile spasms appear normal until the spasms begin. It seems he wasn’t worried when he first saw the spasms because they didn’t seem serious. Like Dr. West often tells parents today that they weren’t worried at first. But as the spasms become more apparent, they realize this is something serious. Dr. West then described what happened to his son over the next few months.
Infantile Spasms (west Syndrome): Update And Resources For Pediatricians And Providers To Share With Parents
The spasms usually become more pronounced, as they did in this case. Sometimes they stay subtle, maybe just a brief nod, but nothing else. Dr. West’s son had what are called “flexor spasms,” where the baby’s head goes forward and the legs come up. The arms normally flare out from the body as the head and body come forward. Sometimes spasms occur with the head thrown back and the legs straightening. These are called “extensor spasms.” Each spasm episode lasts only a second or two, but they often come in clusters. Although the spasms can occur as a single jerk, clusters are more common and often occur upon waking in the morning or after a nap. When Dr. West wrote about his son, he had had the spasms for several months, and the effect on his development was evident, as Dr. West wrote:
Unfortunately for Dr. For West’s son, there were no effective treatments available in 1841, so he had the worst possible outcome. In addition to not developing mentally, he also appeared to be very weak and was not even able to control his head when sitting.
The prognosis for children with infantile spasms is largely dependent on the underlying cause. The intellectual prognosis of children with infantile spasms is generally poor because many babies with infantile spasms have neurological impairment before the onset of spasms. Children who have rapid onset of treatment, normal development prior to infantile spasms, and no identifiable cause may do well. Infantile spasms usually resolve by mid-childhood, but more than half of children with infantile spasms will develop other types of seizures, such as Lennox-Gastaut syndrome, an epileptic disorder from later childhood. In addition, children with infantile spasms have a higher risk of autism. Shorter duration between the onset of infantile spasms and initiation of standard therapy appears to lead to an improved outcome; therefore, early recognition of the seizures and early treatment are essential.
The spontaneous remission rate of infantile spasms in limited natural history studies is 30%. Although clinical spasms and the typical EEG pattern disappear by age 3 to 4 years, up to 60% of children with infantile spasms develop other types of seizures. The long-term prognosis of infantile spasms in terms of neurodevelopmental outcomes and development of other types of seizures remains dismal, although most studies reported better neurodevelopmental outcomes in idiopathic or cryptogenic cases.
Seizure Initiation In Infantile Spasms Vs. Focal Seizures: Proposed Common Cellular Mechanisms
The United Kingdom Infantile Spasms Study reported on the long-term outcomes of 77 patients with infantile spasms at 4 years and noted that while there was no significant difference between patients treated with either hormone therapy or vigabatrin in outcomes for either neurodevelopmental delay or epilepsy, patients with cryptogenic infantile spasms who received hormone therapy had higher mean Vineland Adaptive Behavioral Scale scores
. A decrease of 3.9 points in mean Vineland Adaptive Behavioral Scale score was also observed for each increase in the category of lead time duration after controlling for the effects of treatment and etiology
The 2012 updated guideline recommended a shorter delay time to treat infantile spasms with either hormone therapy or vigabatrin to possibly improve long-term developmental outcomes
These data emphasize the importance of prompt diagnosis and acute treatment of children with infantile spasms to improve their long-term outcomes.
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From Finland have followed 214 infantile spasm patients for 20-35 years. She has compiled the best long-term follow-up studies of these patients. In her series, almost 1/3 of the patients died during the follow-up period; many in the first 3 years of life. Many of the 24 patients who died at age 3 died from complications of ACTH treatment. During
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