With the evidence of a hypsarrhythmia EEG pattern, in addition to signs of clinical regression, Andrew was put on a 2-week course of ACTH. Earlier this week, Andrew had a two night/3 day hospital stay to monitor whether or not his EEG had changed.
Great news: No more hypsarrhythmia! Andrew’s EEG is still abnormal (multi-focal spikes that are low amplitude and posteriorly predominant), but this is not a surprise with Andrew’s PVL. The important thing is that his EEG has improved dramatically with ACTH treatment and he no longer has hypsarrythmia. Andrew is currently being weaned off of ACTH with a drug called prednisolone. Andrew is much happier with the prednisolone as it is orally administered and cherry flavored, while the ACTH was a twice-a-day shot. He is to be readmitted into the EMU in approximately two weeks for another extended EEG.
In general, hysparrythmia is a devastating brain wave pattern because babies and young children tend to regress and lose the developmental abilities they’ve acquired (such as head control, rolling, smiling, sitting). Some children can regain these abilities, while others never will. On the whole, Andrew is delayed in all areas. For a long time, we were thankful that his vision seemed to be unaffected. Andrew loved to watch people, visually track his mobile, and try to reach for toys if we put them in front of him. Unfortunately, the presence of hypsarrythmia has left their mark on Andrew. In addition to no longer smiling socially, he does not appear to see much at all anymore. Structurally, Andrew’s eyes are just fine. But the way that his brain processes what he sees has been greatly impaired, which is also known as cortical visual impairment (CVI). This is similar to what appears to be going on with Andrew’s hearing: his ears are fine, but his brain’s ability to process what he hears has been impaired as well. However, his hearing issues are not hyps-related, but most likely due to his initial brain injury.