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Cortical Dysplasia

My 4 yr old son was diagnosed with seizure disorder 7/2012. Genetic and metabolic work-up turned out negative.  MRI  did show cortical dysplasia, Left Temporal Lobe. We've done monotherapy with Trileptal  and failed, and have recently added Keppra on the regimen. So far, he hasn't had his night time seizures but he has these episode in the morning where he gets startled, starts grunting,  his arms gets stif; it lasts just a few seconds so I could never get a video of it. But he does get pale, afterwards, and he also has this, almost like an excited outbursts of energy where he would be shouting then settles back to his old self. I know it's hard to know for sure, but would this be another  kind of seizure? It only happens once a day, usually after he wakes up (around 7-8am) or when he gets really sleepy (12noon or 5p), maybe 3-4x week.
Aside from the seizure, he was also diagnosed (7/2012)  as having Autism Spectrum Disorder (Pervasive Developmental Delay) and Cerebral Palsy (hypotonic). Is it normal for kids with cortical dysplasia to be having these co-morbidities? Or are these just manifestations of his cortical dysplasia? He has been getting PT, OT, Speech and Behavior Specialist since 15months. We added ABA after autism diagnosis. I have friends (with kids on the spectrum)  advising me to do just ABA (" learning to learn"); but I'm also quite hesitant to stop his other therapies, especially when he's making a lot of progress with his speech. Most of his behaviors comes out when he gets excited (hand-wringing) and frustrated- tantruming when he is unable to communicate  his needs and activities that require fine motor skills (still a work in progress). Which leads me to my final question: should I look into those intensive ABA programs (6wks/12wks) to make sure  I address these behaviors? These programs, almost always, have a very long waiting list. 
Thank you for this opportunity and your advise is much appreciated.
4 Responses
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351246 tn?1379682132
MEDICAL PROFESSIONAL
Hi!

I am sorry to hear about your son. Temporal lobe is often the focus of epilepsy. Also with presence of cortical dysplasia, the epilepsy is often intractable to treatment. Also, this can affect neuro-cognitive function, so the child may have problems with memory, intelligence, fine motor skills, etc.

Cortical dysplasia can cause focal epilepsy syndrome, which actually has an autosomal recessive characteristic with chromosomal mutation. It is characterized by focal bursts of energy, autistic traits (not autism but autistic traits like poor eye contact, poor social interaction etc), poor neurological performance, delayed milestone, poor language development and epilepsy. You son is exhibiting most of these symptoms. However, his genetic testing is clear, so you need to discuss this with your doctor. Since cortical dysplasia is associated with autism spectrum disorder like symptoms, often the child is misdiagnosed to be suffering from it. Hence, you need to discuss this possibility with your doctor. Cerebral palsy and cortical dysplasia are not related though patients with cerebral palsy may have cortical dysplasia.


The treatment modality is what you are following—a combination of drugs, physical therapy, OT, language therapy etc. In my opinion you should take up ABA as part of the treatment approach. It should not be the sole treatment as your son is responding well to other therapies. However yes, the ABA program will help address the disruptive behavior due to cortical dysplasia. The spurts of aggression can be controlled by ABA. Sometimes epilepsy with cortical dysplasia does not respond to medication and patients need surgery such as temporal lobectomy to control the seizures. Please discuss your son’s diagnosis and treatment in detail with your doctor. The diagnosis of autism should be re-assessed and may be a second opinion will help in this case.

I sincerely hope you will find this information useful. Good Luck and take care!
Helpful - 1
Avatar universal
hi
im darell..my boyfriend had a motor accident last sept.5,2013...he needs to undergo craniotomy to remove the blood clots on his left and right brain..post craniotomy i noticed that he cant control the movements of his left hand and the doctor decided to restrain him..is it a normal reaction?will it be gone in the future?he had a spinal cord injury too on his C5..according to his doctor he is not sure if he can still move his both legs but what i've noticed now there is a minimal movements on his legs..its like muscle twitching..is it normal?does it mean he can still be able to walk?he cant talk right now because he has a trache on his throat..
Helpful - 0
351246 tn?1379682132
MEDICAL PROFESSIONAL
If the left temporal lobe is his dominant lobe, then he may face memory and language impairments post surgery. However, yes, chances of remaining seizure free are pretty high.
If left temporal lobe is not his dominant lobe then prognosis in terms of memory, cognition, language will be better.
There, is nothing like a 'window period'. If he is not responding to medications and the condition either remains the same or worsens, then an earlier surgical interventions increases the chances of remaining seizure free post surgery.
However, he would need elaborate pre-surgical evaluation to see what type of surgery can be done and whether surgery will really help or not. Please discuss with his doctor. Take care!
Helpful - 0
Avatar universal
Thanks your quick response. Our neurologist did mention surgery as another option to look into if we fail Keppra (2nd drug). The efficacy of a 2nd drug working is only 10%, so I know we would definitely be making that decision down the road. What would you say the statistics are (if surgery is successful) with zero seizure outcome? He's now 4 1/2 yrs old, would you say there's a "window" of the best time to do the surgery to preserve eloquent cortex? And lastly, would surgery also improve cognitive delays?
Helpful - 0

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