Occipital Lobe Seizures in 7 month old

[nancy]

My daughter has been diagnosed with partial complex seizures coming from the right occipital region based on an EEG.  She had a seizure during the EEG (coming form right occipital region spread to left o.r.) and the EEG also showed recurrent right occipital spike and sharp wave activity.  Background: Normal birth. At 4 mos. she was not focusing or tracking objects.  She had an ERG and a MRI both normal.  Then at 5 mos. her vision seemed improved and we thought it was just delayed visual maturation.  At 6 mos, she began to have seizures - her pupils contract and her eyes flutter around and she often turns to the left as if looking at something.  She seems alert during the seizures (continues to try to play and grap things) but they occur many times a day (up to 25 that we have know of).  The Neurologist started her on Tegretol but she continues to have seizures.  Her latest Tegretol level is 6.2.  Other information:  She has unusual hair and skin pigmentation.  Her hair is dark on top with the rest of her hair mostly blond/white (with random dark spots).  She also has a triangle area on her forehead which is lighter pigmentation.  She does not roll over yet although she can roll to her side and arch.  Otherwise she seem developmentally normal - loves to sit up and passes things from hand to hand.  My questions: 1)Any chance she will outgrow the seizures? 2)Can occipital lobe epilepsy be controlled with medication - if not what?  3) Are there any other test/actions we should take? 4)Could the hair pigmentation indicate any known condition? (I know of Waardenburg Syndrome but she seems to hear OK) 5) Should we worry at this point about her development?   It all seems like such a mystery I deeply appreciate any information or comments you may have. Thank you!

[CCF Neuro[P] MD, RPS]

Dear Nancy:

Sorry to hear about your daughter.  I am assuming that she had EEG changes both with her eyes open and closed?  First, various epileptologist have different values for therapeutic levels of tegratol.  We at the Cleveland Clinic like the range of 8 - 12 for tegratol and we would push the dose until at least 12 or side effects to see if tegratol would be beneficial.  I would assume that some decrease in seizure frequency has occurred on the tegratol.  Second, assuming that the EEG changes were both with eyes open and closed, then we know that some occipital lobe epilepsies are difficult to control.  

By what your telling me, your daughter seems alittle behind developmentally.  This is likely, at least in part, due to her seizure activity.  Was the MRI of high resolution (3D acquired)?  If the MRI was a routine MRI then it might have missed subtle migrational defects.  You might want to repeat the MRI if the acquistion was not optimal for neuronal migration detection.  

Usually with the Waardenberg syndrome, there would be hearing loss but also eye color differences between eyes.  It is usually a tuft of hair that is in a warlock type pattern.  The widespread color change is not Waardenberg-like.  So, I would agree with you.  The hypopigmentation may just be a congenital thing but the high resolution MRI should detect any possible neuronal migration problem.

It is always difficult to say if your daughter will outgrow her epilepsy.  Some children do and some don't, alot depends on the type of epilepsy and family history.  If the EEG was normal with your daughter's eyes closed then I would say there is a chance that she would outgrow her epilepsy.  If the EEG was abnormal regardless of eye closure then I would think that the chances are much less likely.

Let us know about the tegratol and other medications your neurologist tries and the outcome.

Sincerely,

CCF Neuro MD

[nancy]

Thank you for your reply.  I am not sure about the MRI but the Dr. wants to have another one done in January. What is neuronal migration?  As for the EEG, I am not sure about eyes open and closed (asleep?).  The EEG report does say "during sleep stage II...some sharply contoured waveforms are seen over the right occipital regions."  Her uncle also has severe grand mal seizures.  Is it normal for it to take a long time (months?) to find the right medication to control seizures?  Thank you for your input.  You provided me with additional information and questions to ask at our next appointment.

[CCF Neuro[P] MD, RPS]

Dear Nancy:

As the brain develops, neurons migrate to their proper position.  When they do not migrate properly, neurons are not in their proper position and can give rise to areas of dysfunction.  One of the dysfunctions is seizure activity.  Unless one looks for neuronal migration abnormalities with high resolution MRI (3D acquistion) one will miss the more subtle migrational abnormalities.  I do not know what contour waves are, we call epileptiform waves: sharp or spikes.  These latter terms are the ones used by EEG criteria for epileptiform waves.  Contours may or may not mean anything.  I would ask your neurologist about this.  Because if there were no epileptiform waves with your daughter's eyes closed (asleep or just closed voluntarily) then the diagnosis may change into one more favorable to ceasing as she ages.

CCF Neuro MD