EEG

[Mary]

I have several questions.  First I will start with a little history:
Last November(1999) our 4 year old son had a grand mal(tonic
clonic)seizure which lasted about 15 minutes. He was taken to
the hospital and spent 3 days there where he had an EEG done,
cat scan and several blood tests(CBC, Toxicology(drug testing like PCP, Cocaine etc), and Chemistry(Co2, calcium, magnesium, glucose, etc.). He was then referred to a pediatric neurologist and was diagnosed as having primary generalized epilepsy based on the abnormal EEG(cat scan, MRI and all other tests came back normal). He was immediately put on Depakote 125mg twice a day and carnitor which is to counteract the side effects of the depakote. He was a very healthy child up until the beginning of last year(shortly after he turned 3) when he also started having a fever around once a month(he did NOT have a fever when he had his seizure). He has had 8 of these types of fevers since last June when we started keeping track of them(we believe there may have been a few more before that, but were not keeping track then).  He has had coughs & colds in the past---not too many though, but they are different than these fevers.  The fever lasts for 2-3 days, goes up to around 104 degrees and there are no other symptoms with it. We were referred to a specialized general pediatrician and then an infectious disease doctor for this(because our family doctor could find nothing that was causing the fevers) and was diagnosed as having Periodic Fever Syndrome or PFAPA and they feel that the fevers and Epilepsy are not related. He has had one more grand mal seizures(during his last fever when it spiked to 105), but that one only lasted a minute. He also has what we call "starts" of seizures(myoclonic?) where his head and upper body will suddenly jerk forward like he is going to fall, but then it is like he catches himself. He has had any where from 0-50 of these a week---towards the higher end when has a fever of course.  The cause of his Epilepsy is unknown and they originally said he may grow out of it, but now his neuro doesn't believe he will because of the jerks(she just also recently upped his does to 125mg in am and 250 mg pm because of these jerks). There is also no known cause for PFAPA and it is also something that they say he should grow out of.    He has had several blood and urine tests done during his fevers.  I believe these were all just standard tests(checking glucose, protein, bacteria levels in the urine and doing CBC of the blood) and I believe they have all come back relatively normal except for elevated WBC(usually around 13,000) and elevated monocytes I believe.  With his last fever, the doctor also ran some other blood tests.  I believe they were for Parvovirus, Immunoglobulin and CMV and those were all normal.  
This is his EEG report:
EEG Description: The background appears symmetric and consists of an intermix of theta, alpha and beta activity. A posterior dominant rhythm was intermittenlty present of 5Hz. The patient frequently rubbed his eyes and had lots of movement and muscle artifact. Eye movement artifact was frequent. There were severel clinical seizures observed where the patient was blinking. These were associated with anterior dominant, 2.5Hz spike and wave discharges that were diffuse in nature.
Clinical Interpretation: This record shows mild background slowing that is possibly a postictal phenomenon. Much more importantly, however, there were multiple clinical seizures manifest with eyelid blinking that were associated with anterior dominant, diffuse spike and wave discharges that would be suggestive of a primary generalized epilepsy.
Just a little background on this. This EEG was done at 10:00 pm, approximately 9 hours after our sons first seizure(15 minute tonic-clonic). He was very stressed and over tired at this point and did not want any more doctors, nurses, etc poking him anymore. So as you can imagine he was screaming the whole time they were connecting the wires. It's been a few months since this eeg so I don't remember all the details, but I know I was laying next to him during most of the EEG trying to talk him into going to sleep because of course they wanted him to sleep for part of it. I don't believe he ever actually went to sleep. He tried hard to keep his eyes closed so I'm a little confused on how they interpreted that because of how they state that there were "several clinical seizures observed where the patient was blinking". Does the EEG show that the blinking was a seizure or could it still just have been blinking? Any interpretation or explaination of this EEG report would be greatly appreciated. Specifically what these two statements mean: "A posterior dominant rhythm was intermittenlty present of 5Hz & These were associated with anterior dominant, 2.5Hz spike and wave discharges that were diffuse in nature". He had a sleep deprived EEG approximately six weeks later which I am awaiting a copy of. His neuro said the second EEG was much better, but that was all that was said about it.

Our questions are these:  Are there other tests(blood or otherwise) that should be done?  Can the two still be related.  Could allergies of some sort play a role in all of this?  He is a very bright and happy child and we want to make sure that he has the best chance of staying that way.  
Thank you in advance.


Mary & Randy

[CCF Neuro[P] MD, RPS]

Dear Mary:

Sorry to hear about your son.  I will assume that your son is a completely normal boy and developing normally, with the exception of the fevers.

The EEG is worrisome, even if it was performed post ictal. The slow background might be the result of the ictal event, but the spike wave formations are indicative of epilepsy.  The slow 2.5 hertz frontal or anterior waves are also very worrisome.  Now, I need to tell you that reading the EEG is somewhat subjective and without reading it myself I can only speculate if I was reading what you are telling me.

The abnormalities of the first EEG are likely present on the second EEG or the neurologist would have told you (?).  The symptoms of short myoclonic like movements is also not a good indicator.  Valproate is a good medication for these type of seizures.  

Allergies would not have anything to do with his seizure events.  The many seizure events, myoclonic, occurring is troubling.  Myoclonic epilepsy in a 4 year old may signal a progressive epilepsy.  There are several conditions that might give rise to a progressive condition, Unverricht-Lundborg disease, sialidosisl, Gaucher's disease, mitochondrial disease (this would also induce high fevers of unknown etiology), Lafora's disease, and neuronal ceroid lipofuscinosis.  I think I would ask to rule out these diseases.

Sincerely,

CCF Neuro MD

[Mary]

Thank you for your quick response.  Yes, you were right to assume that he is developing normally(physically & mentally).  He has been a very healthy boy up until this last year and in a way still is except for the fevers and seizures.  What do you mean that the "The slow 2.5 hertz frontal or anterior waves are also very worrisome." and what does it indicate?  I also forgot to ask if any type of deficiency could be the cause of all of this or if a metabolic disorder could cause this.  Thanks again.
Mary & Randy

[Mary]

Sorry, I just looked up some of the conditions you mentioned and realized that some of them are metabolic disorders.  However, after reading about some of them I wonder if he would show signs of some sort of deterioration or other symptoms besided the fevers and seizures(He also has a slight heart murmur(detected recently) and some constipation at times which I forgot to mention, but an EKG was done and they don't feel that is anything to worry about.  The constipation has been mentioned to the doctors, but they don't seem too concerned about that and it is not something that is constant and is taken care of by natural methods(eating more fruits, karo syrup, etc)).  
Mary & Randy

[CCF Neuro[P] MD, RPS]

Dear Mary and Randy:

The difficulty with trying to correlate a disease with symptoms over the internet is not being able to see the child and the work up.  The slow anterior waves are worrisome, and likely are not a post-ictal response.  That is why I mentioned the diseases associated with progressive myoclonus seizures.  The workup and time will tell.  Just a note: mitochondrial diseases are associated with myoclonic seizures, constipation, kidney abnormalities (RTA), eye problems (retinal and eye muscles), hypotonia, muscle weakness (usually episodic), cardic problems and autonomic dysfunction.  Some or all of these symtpoms may be present.

CCF Neuro MD

[Mary---Thanks]

Thank you for your responses.  We will wait and watch now.  We will try to discuss some of these things with our son's neuro.  She does not seem too interested in finding any causes, however.  She seems to just want to medicate, which is why we have turned to the internet to look for causes.  We may try to get a referral  to a new neuro depending on how the next few visits go.  We do not want to wait until our son goes drastically down hill.  Again thank you.  Your responses have been much appreciated.
Mary & Randy

[CCF Neuro[P] MD, RPS]

your welcome

CCF Neuro MD

[Mary--second EEG]

I finally received a copy of the second EEG and was wondering if you could respond to it since you were very helpful of the first.  It was done approximately six weeks after the first one.  It states:  
The background rhythm during awake is 6-7 Hz, moderately well organized and develped, bilaterally synchronous and symmetrical.  Photic stimulation produced no change.  Hyperventilation was associated with brief bursts of generalized, polyspike and wave without symptoms.  Occasional generalized bursts of spikes were noted, and spike and wave and polyspike and wave were seen with drowsiness and sleep.  None of these were clinically asymptomatic.  During sleep, several muscle and jerks and twitches were noted without any EEG abnormalities to accompany.  

CLINICAL INTERPRETATION:  This EEG is abnormal due to the presence of interictal activity consistent with seizure disorder on treatment.  

How does this compare to the first in terms of does it still show the same type of Epilespy or seizures?  Also what is the difference between "spike and wave" vs "polyspike and wave" and what does interictal mean?
Thanks,
Mary & Randy

[CCF Neuro[P] MD, RPS]

Dear Mary and Randy:

I do not get the same feeling about the intrepretation of the EEG as what was written.  One can see spike and wave formations upon going into and out of sleep in a youngster.  It is also normal in some people to see burst of polyspikes with hyperventilation in a young child or even an adult.  I would get a second opinion from a pediatric epileptologist before I would conclude that the EEG is abnormal.  That is my opinion.

CCF Neuro MD

[Mary]

Thanks again!

[CCF Neuro[P] MD. RPS]

your welcome.

CCF Neuro MD