The EEG shown above was taken in a 6 month old child who is brought in because of recurrent motor spasms. You observe a video taken by the parents which shows symmetric movements starting as sudden brief contractions of muscle groups lasting a second followed by a longer tonic phase lasting 2-10 seconds. Some involve sudden flexion of the neck, trunk, arms, and legs; others involve extension of the neck and trunk with abduction of the legs. Occasionally the eyes show nystagmus. Changes in respiratory pattern occur commonly. The parents say the attacks occur in clusters up to a peak, then decline until they stop. When you consult the chart, you also note neurodevelopmental delay.
Challenge: What syndrome is described here?
Image shown under Fair Use.
Infantile spasms, aka West Syndrome. A rather sad diagnosis.
ReplyDeletebeautifully done
ReplyDelete-
Wild Wild West
This is West Syndrome, a triad of infantile spasms, arrest of psychomotor development, and hypsarhythmia (the EEG finding).
Sources: UpToDate; pediatrics.georgetown.edu.