Demyelination in my child

[Tiggy]

Hi, I initially posted under child behavioural health as I felt my child wasn't progressing normally and the dr there advised me to post here. My son's history is quite complex but I'll try my best to keep it as short as poss.

My nine year old son had a left temporal lobectomy in November for removal of an area of abnormality including ganglioglioma/DNET and cortical dysplasia. He was having intractable severe seizures since the age of 5 but until this age developed normally and was a bright boy. Since his operation he is having severe memory problems and does not seem to be developing as a normal nine year old. He is very emotional - cires at the least little thing and just seems very immature for his age. His reading/writing ability is badly affected - he has an intentional tremor and struggles with his handwriting. He attends hospital school at the moment - he would not cope in mainstream school.

Following his surgery the biopsy showed "striking demyelination" and so he is to have further MRI scans and further investigations. He is still having seizures although they are different to his complex partial and grand mal he was having pre-surgery - now they seem to affect his gastro-intestinal tract - he gets a strong aura of nausea, belches alot and has a headache. His legs just go floppy and go from under him, he cannot support his weight.  He had this only last night but he did not lose consciousness, he just said his legs felt "weird" and had to lie down - his legs went floppy along with his right arm. He also seems to be full of bruises but a full blood count was okay although his platelets were high. The bruises do tend to be on the right side of his body.  Other than this he is bothered by a constant itch down the back of his thighs/buttock area. There is definitely no rash and it comes and goes.

I would really appreciate your thoughts on the "striking demyelination" and his symptoms at present.  I am quite prepared for the worst as any searches I have done on demyelination brings up leukodystrophy and MS mainly.  He is also having visual problems with reduced visual field - he is having further follow up on that too.  Many thanks.

[CCF Neuro[P]-M.D.-RPS]

Dear Tiggy:

I am very sorry to hear about the troubles your son is having.  I am involved in many children with epilepsy surgery.  Where did the surgery take place?  What were you told about the outcome?  How extensive was the malformation of cortical developemnt?  We do as many pediatric surgery cases at the Cleveland Clinic so I am very familiar with what your son is going through.  It sounds like the surgery was not completely successful and that is not uncommon if the area of dysplasia was extensive and they could not completely remove all the area of dysplasia.  Was that the case.  Most of the symptoms that you describe are suggestive of continued seizure activity.  What did the presurgical MRI of the brain show?  If it did not show extensive demyelination (should have been very visable on the T2 weighted images and FLAIR sequences) then I would not place too much weight on what you have been told.  It is likely that they are describing the tissue they resected for the epilepsy surgery.  

Your son received his surgery at a fairly young age.  He should be able to tranfer language to the other hemisphere and likely he shifted language to some extent outside the area of dysplasia.  We see this constantly in our surgical candidates who have extensive malformations of cortical development within the dominant hemisphere.  What you have to balance is the recovery, the surgery, the time it takes to learn (now that his dominant languages areas have been changed) with what he had to begin with, such as the seizures and any developmental delay from the malformation.  Things take time and likely he just needs alittle more help and encouragement than the average boy who hasn't had a big surgery on his brain.  Hang in there and give your son plenty of encouragement and love.

Sincerely,

CCF Neuro MD

[Tiggy]

Hi and many thanks for your reply which was very informative and reassuring.

I am in England and my son had his surgery at the Walton Centre for Neurology and Neurosurgery in Liverpool. His neurosurgeon was Mr Paul May who was fantastic! We thought the whole of the abnormal area had been removed as they took the whole of the temporal lobe (which was hard and shrivelled). However, we always knew further seizures may be an option as he was showing separate discharges from the right temporal lobe on his EEG before the surgery (we had hoped this might be a mirroring). The biopsy mentioned focal cortical dysplasia - does this mean it was just in one area?  I believe the dysembryoplastic neuroepithelial tumour area is quite rare and this is what showed up on the pre-surgery MRI - no mention was made then of any demyelination, this just showed on the biopsy of the lobe that was removed.

Although I am disappointed that he is still showing signs of seizures, I am relieved that it is not MS or leukodystrophy. As I mentioned earlier, he is still to have further MRI scans and investigations on the rest of his brain but as long as he is happy in himself and functioning okay (even with his memory problems etc) then I am happy. The seizures are something we have learned to deal with. His tremor I feel may be due to his quite high dose of Lamictal that he is still on, hopefully it is not due to demyelination in the rest of his brain.  Many thanks again for your help and advice, you do a great job! Best wishes, Tiggy.