Cortical dysplasia

[Amy Thibodeau]

My neice sho just turned 2 years old has global cortial dysplasia, migrational anomaly.  The doctors have said they do not know what her outcome will be as far as schooling, independance, etc due to the fact that they don't know much at all about the disorder.  She is involved with our Birth to Three program which includes, occupational, speech and physical therapy, etc.  She has improved a great deal.  She can crawl very well, scale furniture, take steps with help, say a couple words when she feels like it (mainly baby babble).  She has an amazing memory and an incredible love of books.  She also loves to be very involved with all...much like any average 2 year old.  She just can't walk or talk much at all.
She originally had lazy eye, which is improved.  She also was very sensitive to certain noises and textures.  She has since greatly improved.  I believe she is effected in the insular cortex.  All of her tests, i.e. EEG, Genetics, chromosomes, etc have all been amazingly normal.  The only thing that showed anything was the MRI (as above).  She has no evidence of seizure activity as of yet.  She does tend to "zone out", however can quickly be brought back without difficulty.
My questions are...Is there any treatment?  Will she ever develop seizure activity?  Is there any hope for her to go to school with other children of her age other than special education?  Is there a chance of her being independant at some time in her life?  Any information will be greatly appreciated as I have a third appointment with a third neurologist.  This appointment is at Yale New Haven Children's Hospital.  I would love to enlighten the doctors here since none of them seem to be able to give me any answers to my questions.  
Thank you for your time.  Amy Thibodeau

[CCF Neuro[P] MD, RPS]

Dear Ms Thibodeau:

God has never told his people not to be active and proactive in the things of life.  We are to be planners, workers, store up grain for the day of drought, etc.  It is only when we place ourselves on the throne or ignore His teachings that we get ourselves in trouble.  That is my play on things and that is why I tell parents to do all they can for their children.

CCF Neuro MD

[CCF Neuro[P] MD, RPS]

Dear Ms. Thibodeau:

There is no way to tell if she will develop speech, she will let you know by actually talking.  As she has delayed speech presently, likely her endpoint will be somewhat blunted.  But, again, no one knows.  The range of dysplasia is difficult to gauage as most use MRI.  So, depending on the resolution of the MRI scan the neuroradiologist gives a best guess.  The better the MRI the better the guess.  It depends on whether it is global dysplasia, only partial cortical dysplasia and the location of the dysplasia etc.  Improvement though is very dependent on the input by the parents and therapist.  The more imput the better the improvement.  It sounds like your niece will improve but the degree is dependent on her help and God's grace.  I can't even venture a guess since I have no way to gauge her by exam, studies, etc.  I know you want an answer but it is impossible.  The ability to communicate takes on various forms, such as pointing to objects of desire.  If she is pointing to objects then this is good.  If she only get frustrated by not getting the object of her desire without letting anyone know what the object is, then her ability to communicate is more blunted.  Sign language or computer directed communication sometimes helps.  However, this is very child dependent.  A speech evalutation by a good (very good) speech therapist will answer your question.  As far as outcome, I try and tell parents to hope for the best but to plan for the worst.  Usually, the result is somewhere in-between.

Sincerely,

CCF Neuro MD

[Amy Thibodeau]

Thank you for your very quick response to my initial question.  I do, however have just a few more things to ask.  
1.  Do you know if there will in fact be any improvement in Alicia's speech after working with the speech therapy program?  
2.  How much improvement have you seen in children with Cortical dysplasia ( I know every child is different and ther are many different degrees of cortical dysplasia).  Actually the doctors have said that she is on the better side of cortical dysplasia.  Knowing that I am just curious to just have some idea.
3.  My sister has actually wondered if at some point teaching sign language to her would be beneficial since she uses her hands so well.  Any thoughts on that?  Alicia is very anxious to communicate and seems to get pretty upset at times if she can't get her point across (like most babies).  It is as if She has her thoughts and wants to share them but they get jumbled in their travels to her mouth.  She actually changes the tone her baby babble when she is trying to communicate, such as when she is wondering what something is she ends her babble with sounds that resemble a question.
I don't know if what the possibilities for a child are at any end of the cortical dysplasia scale.  We have no idea if we should hope she walks and talks and goes to school or if we should start planning for a lifetime of constant care and therapy.
Thank you again,
Amy Thibodeau

[Amy Thibodeau]

Thank you so much for your honesty.  In Alicia's case it is global and from what the neurologist's say she is on the better end.  It actually took quite some time for her pedi to even acknowledge that there was a problem to this degree (he is a very good pedi).  As many doctors she saw nobody guest it was even close to this kind of situation because she was so much on the "better end".  She just seemed slightly slow.  The therapy she is getting is great though, with each day she is doing something better every day.  She just seems to be climbing in one area at a time.  All of our homes have turned into mini pysical therapy homes as well as speech and occupation therapy as well.  We jump thrugh hoops to gain her interest and hold it just to get a little higher on the cortical dysplasia latter.  She is doing well and we feel very blessed just to have her, no matter the outcome.  We too feel as though it is in God's hands but to sit by and allow everything just happen without our help will not work.  I firmly believe that God helps those who help themselves.
Thank you again for all of your help and honesty.
Amy Thibodeau

[CCF Neuro[P] MD, RPS]

Dear Ms. Thibodeau:

Sorry to hear about the cortical dysplasia in you niece.  The causes of cortical dysplasia are unclear at present.  A great many of patients with cortical dysplasia have epilepsy.  However, it must be said that since we look at almost all children with epilepsy on MRI for dysplasia, the population is very dominant with epilepsy.  No one has done a general population of people to see how many people with cortical dysplasia do not have epilepsy.  However, most of us think that cortical dysplasia often leads to epilepsy.  Whether your niece will eventually have epilepsy is unknown.  The good news is that at this point in her life, she doesn't seem to have seizures and epilepsy.  As you probably already know from watching your niece, that she is delayed.  At two years she should be walking quite well on her own, talking with about 50 - 100 words, etc.  How delayed is also not certain, because each child is so different.  With all the added therapy, she is getting what she needs.  Actually, the more the better.  The goal is to optimize her potential and allow her to be her very best at all facets of life.  Child have such a marvelous way of compensating that we try and not predict outcome.  If anyone tells you that they can say for certain that she will be really delayed or normal cognitively at this point is probably giving you false information.

Sorry I probably didn't help you much.

Sincerely,

CCF Neuro MD