About Cerebral Palsy Polymicrogyria is the condition which results in the diagnosis of cerebral palsy (CP). Ben has mixed tone CP. This means his trunk is loose (hypotonic) and his limbs are tight (hypertonic). His legs, particularly, have spacticity. This means his muscles have grown so tight that he cannot straighten his legs. So, Ben's CP can be characterized as bilateral Spastic Cerebral Palsy.
Ben also has a right side weakness. His right hand and right leg are not as functional as his left side. So most of the hard work Ben puts into moving is finding compensation for a weaker right side. Ben has worked very hard to get most of his right hand out of the fisted position. Although he cannot spread his fingers wide on that hand (his thumb is often stuck in the adducted position still), he has learned how to use this hand as a helper-hand with his left hand being dominant. More About Polymicrogyria Polymicrogyria (PMG) is a disorder of neuronal migration resulting in structurally abnormal cerebral hemispheres. This means that the neurons had not made it to their appropriate places in the brain while it was forming in utero. Ben's brain has too many (poly), smaller (micro) folds (gyri) which constitutes PMG.
Most children with polymicrogyria have some degree of global developmental disabilities or delays, seizures, feeding difficulties, respiratory problems, motor dysfunction and intellectual disability. It is difficult to make a predictable prognosis for children with the diagnosis of PMG because each child is very unique in their presentation of this disorder. And, the part of the brain affected by the PMG determines the severity of the symptoms (think bilateral as opposed to unilateral, or affecting several lobes of the brain instead of only one). The form of PMG that Benjamin has is called bilateral frontoparietal polymicrogyria, meaning it affect both sides of his brain in the frontal and parietal lobes.
In Ben's case, all of the known-to-date genetic testing has been done, and a genetic link has not yet been determined as the cause of his PMG. PMG can result from several different causes that are both genetic and non-genetic in nature.
My pregnancy with Ben was wonderful, and I had not experienced anything distressing or troublesome that would have alerted us to a problem. It was a typical pregnancy and safe delivery. Everything was perfect, and Ben and I experienced a beautiful natural birth. It wasn't until Ben was about 2 months old that we noticed he was not hitting his milestones. An MRI at 5 months of age confirmed the presence of PMG.
There is a wonderful website called PMG Awareness (www.pmgawareness.org) which provides a very comprehensive overview on polymicrogyria. It also provides valuable resources, research and information about PMG and other related disorders.
About Bilateral Congenital Cataracts When Ben was 2 days old, the cataracts were discovered and we were immediately seen by a pediatric ophthalmologist. Ben had surgery at one week old to remove the cataract (and lens) in his left eye, and Ben still could not see out of that eye. Two weeks later, we began inserting a special contact lens in his left eye so that he may have vision. He has been wearing a contact lens ever since - without it, he would not have sight. Ben also wears glasses, and they have helped his vision improve even more.
The cataract in Ben's right eye is still there. It is not impeding his vision in this eye, so there is no need to remove it. He is under close watch by a pediatric ophthalmologist.
Ben's vision is quite functional. Although he had a degree of cortical vision impairment (CVI) when he was much younger, he has outgrown it and has come to use his vision in a very positive way.
Thank you all for your interest. We are gratefully and humbly blessed to be his parents.
Why Selective Percutaneous Myofascial Lengthening (SPML)? Our brain sends messages to our muscles to work when we want to move. The brain tells the muscle to fire up when we want to, say, flex our foot. With people who have Spastic Cerebral Palsy (CP) like Ben, the brain doesn't stop sending messages to contract the muscle. So much so, that the muscle is in a constant state of spasm, causing tightness. The affected body part then has a limited range of motion.
This is why Ben walked on his tip toes and with a crouched gait. His hamstring, gastrocnemius (calf), and adductor (inner thigh) muscles are so tight that he cannot stand upright with flat feet, let alone walk with a regular gait or balance.
SPML lengthens the fascia - the fibers that surround the muscles - of his hamstrings, gastrocs, and adductors. This is not a surgery - therefore it does not cause scar tissue, and there are NO RESTRICTIONS after surgery. He can start therapy right away to keep these muscles supple and loose.
This specific procedure is only performed by two doctors in the US. One is Dr. Roy Nuzzo located in New Jersey. This surgery is not covered under Ben's insurance plan, and it comes with a substantial price tag. Ben had SPML in July 2019 with Dr. Nuzzo.
And it was through the support of our community via the fundraisers we had that we were able to provide Ben with this life-changing surgery.
As for the constant messages being sent from his brain to his muscles - there are more invasive surgeries for that, to be tackled at a later point in time. For now, SPML is the first step in helping to get Ben more range of motion so he can walk and have greater independence.
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