After telling the nurse all about Rayden's new development (seizure) since we were last seen, she went straight to ask Dr. El-Dairi what she wanted to do next. She did not want to dilate Rayden's eyes. She wanted pictures of his optic nerves but this time they did it without any flashy lights. Rayden did very well and of course his optic nerves looked the same-scarred but not swollen. When Dr. Brian White, Neurologist working with Dr. El-Dairi, entered the room he immediately asked about our eventful time. He inquired about what the doctors at UNC told me as to WHY Rayden had a seizure. I informed him that they just told me he had epilepsy because they could not find a clinical explanation as to why he had the seizures. He said "that's one way of putting it, but epilepsy always has an explanation." He asked me to go through the events that led up to Rayden's seizure and everything that happened afterwards. He said the description I gave him was slam dunk the description of a seizure. He said that the UNC team did a very good job of going through all the things that could lead to a "one time seizure" but he believes there are clear reasons as to why Rayden had this seizure. He then inquired about all the MRI's Rayden has had in the past. (Apparently he had just spent quite some time going over Rayden's MRI history) We already knew that Rayden had a Chiari II malformation that is now corrected. He has always had, and will continue to have, large ventricles. He never developed a corpus callosum or a septum pellucidum. Dr. Brian White then proceeded to tell me that he has
polymicrogyria. This very clearly explains why he had the seizures. He says seizures come from the surface of the brain. He explained that the brain was like a bowl of thick spaghetti that folds on itself. If the 'noodles' are too thick, too thin, too many or not enough folds, folds too close together or far apart; these are all abnormalities of the brain. In Rayden's case, his brain is folded too much. There are tons of folds in a single area instead of just a handful of folds.
Polymicrogyria, literally means too many (poly-) small (micro-) folds (gyria) in the surface of the brain. This is very clearly a reason for seizures. Common problems associated with PMG in general are: swallowing and speech difficulties, reflux, seizures of varying degrees (about 90% are affected with seizures at some point in their lives), developmental delays, lack of muscle coordination, impaired cognition of varying degrees and cerebral palsy. Many cases are mild enough to go undiagnosed and children are labeled as "developmentally delayed" or having a "seizure disorder". Dr. White agreed that Rayden needs to be on both medications that UNC placed him on. He says Rayden's brain is kind of two fold. He has the areas of his brain that didn't form correctly when he was developing. He has the areas that changed due to the chiari malformation and pulling everything down. Some of the areas of polymicrogyria are all over his brain. And of course he has the changes from the pressure of the hydrocephalus. They believe all of these things are causing him to have a vision issue. He said he noticed that Rayden's visual acuity is much better when they only give him one letter instead of an entire line of letters. They see that often in patients that have
cortical visual impairment. (Cortical meaning the surface of the brain.) The eye receives the message and sends it through multiple paths through the brain to the occipital lobe. If you have an issue with these paths or the occipital lobe then the brain has problems interpreting what you see. In Rayden's case he has both, a malformation of many areas including the occipital lobe, as well as an injury to them. So the processing of his vision is impaired. There's no structural problem with his eyes, he can see 20/40. The problem lies within his brain. At his age he can mostly just fill in the blanks but some things will be harder for him to process (letters, numbers, written print). They believe his vision will improve with time. Dr. El-Dairi went into an example of Dr. Oliver Sacks who has a cortical processing disorder where he cannot recognize faces although his brain operates on a superior level. You can have parts of your brain that do not operate well on one level but be super well on other levels. This is what we are seeing with Rayden. He has reactions that are way more than what we expect from him in certain areas and very low in other things. She recommends taking Rayden to a neural behavioral psychologist to do testing. They can identify his strengths and weaknesses giving us strategies on how to improve weakness and recognize strengths to target his education. She wants to stagger his appointments with his neurology team so that he is getting some kind of test done every six months whether it be MRI, or optic nerve photos. She concluded that she does not believe he has a central scotoma (what she originally thought). She firmly believes, after seeing his MRI, that his issues come from his abnormalities of his occipital lobe not his vision.
Rayden lost his first tooth today. We didn't even know it was loose so we couldn't give him any warning. We were riding home from school when Rayden yelled "My tooth fell out!" I turned around to see his little hands dripping blood and a tiny tooth in his fingers. Of course, he started crying. I'm not sure if it was the blood, if he was scared because his tooth fell out, or if he thought something was terribly wrong. We were almost home so I just told him not to drop it and that it was perfectly fine. When we arrived home I cleaned up the blood and explained that he was going to get a 'big boy' tooth in it's place. His mood quickly changed. He was excited and couldn't wait to tell everyone. 
