Dear Mary:
I am sorry to hear about your son. I have not heard of PFAPA before? But febrile convulsions are very common, however we do not usually treat febrile convulsions with medications. From what your telling me, your son has been having afebrile (without fever) seizures that occur out of sleep. I think you need to have him evaluated with your pediatric neurologist. I would make sure that his level of depakote or valproate is appropriate. I think he might have changed to focal epilepsy and would have a look at his EEG once again and maybe a MRI. I am sorry that your son is displaying a new seizure type.
Sincerely,
CCF Neuro MD
Thank you for your reply. We just got back the results of a recent 24 hr EEG(done about a month ago). They are increasing his Depakote and will be doing another MRI soon. Below is the EEG that I have a few questions about:
EEG Descrpition: The background showed an intermix of predominantly theta/delta activity intermixed with the same alpha/beta frequencies. Runs of anterior dominant delta activity, some with sharp waves intermixed, were intermittently present throughout this tracing. At other points, clear spike and sharp wave discharges were present. Some of the spike discharges phase reversed in the F4/C4 region. Although this was an inconsistent finding, many of the discharges were diffusely distributed. Photic stimulation failed to activate epileptiform discharges.
ClNICAL EVENTS: Several head drops were captured. Ten of these were reviewed. The typical clinical manifestations consisted of a head drop with the patient's arms up tonically for approximately one second and then was back to baseline. With seizure #3, the patient was slow to responcd although all others appeared to be similar to the initial with normal recovery.
EEG ICTAL CORRELATES: Each of the head drops was associated with diffuse spike and wave discharges lasting approximately one to two seconds. Some of these were followed by anterior dominant activity that was described above.
CLINICAL INTERPRETATION: This record provides evidence for a high spike wave index. There are several possibly subclinical seizures that occur during play and sleep. These are manifest with anterior dominant spike or sharp wave followed by slow wave activity. On some occasions, phase reversals in the right frontal regions were present. This requires consideration of a structural lesion in the right frontal region although the dominant activity was one of generalized anterior dominant spike and wave discharges. These patterns are consistent with that described in myoclonic astatic epilepsy of Doose although the background activity does appear to be slower than would be expected for a primary generalized epilpesy. Nevertheless, such finding have been descirbed with myoclonic astatic epilepsy of Doose.
I understand most of it, but my questions are these: What exactly are phase reversals and what do they possibly signify? What are some of the possible reasons/implications of the background slowing? (Just a note: in 2 of the last 3 EEG's he's had there was also mention of mild background slowing) Any other interpretations/comments are welcome.
Mary
Dear Mary:
I am sorry, but it does seem that your son has epilepsy. I am not sure why the problem with the posterior rhythm having some delta/theta as at his age there can be posterior slowing of youth that fits this exact description. But, not seeing it I can only assume this is what the rhythm they are talking about. Phase reversal, depending on the montage of the EEG, usually mean that the spike is either maximal or minimum at those electrodes. So, likely the maximum is at the frontal central region on the right. This would fit his clinical symptoms. Although Droose described a type of "myoclonic variant", I have never heard it called astastic myoclonic epilepsy of Droose. There is usually myoclonic activity that is followed by atonia (loss of muscle tone). There are brief absence seizures in 62%, tonic-clonic seizures in 75%. During the seizure event there is bursts of generalized 3/4 Hertz spike or multiple spike wave complexes. There are theta rhythms at 4 to 7 Hertz maximum over the parietal region. The good news is that at least half of the patients enter into remission of seizure activity. So, it does sound like your son may have this type of myoclonic epilepsy.
I hope that I helped alittle.
Sincerely,
CCF Neuro MD
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