What does IS look like?
IS can be mistaken for another condition or seen as not harmful, but knowing what to look for, followed by prompt diagnosis and appropriate treatment are critical for a child’s best developmental outcome. We thank the Infantile Spasms Project for sharing these videos.
VIDEO #1: In this first example, we see an infant with a cluster of individual spasms. Each spasm is less than one second long and heralded by sudden change in behavior with a look of surprise, wide opening of the eyes, brief stare, and elevation/extension of both arms. In between each spasm, the infant appears to be fine. This is very typical of infantile spasms.
VIDEO #2: Next, we see a 6 month old boy who similarly exhibits a cluster of spasms. Of particular note, each spasm is characterized by more vigorous extension of the right arm, in comparison to the left. This suggests that the seizures (spasms) originate in the left brain. This was confirmed by neurodiagnostic testing, and this boy underwent a successful hemispherectomy after several medications were unsuccessful. You can read more about his story at evanstauff.com.
VIDEO #3: The infant in this video experiences a cluster of spasms that is slightly different. Although each spasm in the cluster is brief, and similarly characterized by a sudden arrest of behavior with extension of both arms, we see that the spasms provoke an emotional reaction with crying—which momentarily stops with each spasm. This is fairly common and many children with infantile spasms have clusters accompanied by crying or fear. In contrast, some children experience unexpected laughter or happiness. The reasons for these emotional phenomena are not entirely clear but suggest that spasms often affect regions the brain responsible for emotion.
VIDEO #4: The next video shows an infant with fairly typical infantile spasms, with a vigorous head drop and simultaneous lifting of both arms. There is no clear asymmetry in the appearance of the spasms. Compare this video with the following VIDEO 5 – the same patient.
VIDEO #5: This shows the same infant presented in VIDEO #4. This video was recorded 10 weeks after the first video, following a course of therapy and partial improvement. Notice that the spasms are now “focal” and manifest with a much milder head-drop, and lifting of just the left arm. This suggests that the right brain is the source of the seizures (spasms). This example demonstrates how it is important not to assume that symmetric spasms exclude the possibility of a “focal” source of seizures.
We share this video, produced by the UK Infantile Spasms Trust and developed to raise public awareness of this rare but potentially devastating disease of infants. Find out more at ukinfantilespasmstrust.org