MacKenzie and the diagnosis of Epilepsy


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JME vs. FLE

Well, as of mid-March 2002, we found out that MacKenzie definitely does NOT have JME. Dr. C is thinking posterior frontal lobe E or anterior temporal lobe E. She has had a battery of testing done (see her journal)... but we still are unsure. As this is still really good information, it will be left :)

This is long guys!!

Since I am just learning more about frontal lobe epilepsy myself, I will try my best to explain why Dr. C thinks that we would want MacKenzie to have JME vs. FLE.

With FLE (frontal lobe epilepsy) and Dr. C's comment about surgeries, hopefully this will explain, to both you and me (!!) why he said it plural: Patients with medically intractable epilepsy should be considered for resective epilepsy surgery. If resective surgery is not possible, other surgical options include corpus callosotomy, multiple subpial transections, or the vagal nerve stimulator.

Resective surgery - Less successful than temporal lobe surgery; 20-50% of patients become seizure-free
In some series, lesional frontal lobe epilepsy is associated with a better surgical outcome than nonlesional epilepsy. (MacKenzie has no lesion) In general, prognosis is best if the lesion can be completely resected along with the adjacent cortex, if it is a part of the epileptogenic zone. Usefulness of resecting areas of interictal spiking is controversial.
Intraoperative electrocorticography has prognostic significance, especially if spikes are continuous or nearly so, as is often the case when cortical dysplasia is present. In these instances, absence of post-resection epileptiform activity is a strong predictor of a favorable outcome.
Potential complications, in addition to the risks of cranial surgery, include motor weakness and behavioral changes.
Corpus callosotomy: Aimed at prevention of bilateral synchrony, thus preventing seizure generalization.

Partial seizures that do not generalize often do not improve and may worsen.

With the advent of improved surgical techniques, this procedure is rarely done for well-defined frontal lobe epilepsy (I'm not sure if MacKenzie's is well-defined... well, we aren't even sure if it is FLE!).

Multiple subpial transection

In this technique multiple vertical transections are created, thus interrupting the pathways for horizontal ictal spread, while preserving projection fibers important for function.

Performed in some centers, often in conjunction with resection, for epileptogenic zones that overlap with eloquent cortex.

As well, it is most likely that FLE will not be outgrown. (I really feel like I am being a naysayer here, but this is just the facts that I have read and were told to me ) Most of MacKenzie's seizures, which are myoclonic, are at night. She has 45 minutes to 3 hours worth of activity EVERY NIGHT and has for the past 10 months. It was better with Keppra, but she had bad behavior (which now we realize may be related to the frontal lobe seizure activity) plus she had day-time myoclonic and atonic seizures on it. Mac also has generalized convulsions, 17 to date, most of which have occured in the waking hours between 5-8 a.m.

With JME, there is a certain criteria that has to be met to get that diagnosis. As I mentioned before with the PET scan showing normal, but also this is typically an adolescent onset between ages 8-12. Mac had her first seizure at 5 years, 4 months. Dr. C said it is not typical, but it has happened (to have an earlier than adolescent onset). JME is apparently easier than FLE to control with medication, and I believe he said that 80% of people tend to 'outgrow' it. JME does not require surgeries either, as it is typically controlled with meds. But as I have been doing my research, I am reading that JME typically requires lifelong treatment, that the overall progonosis is good. "Prognosis:


Seizure control tends to be excellent with relative low doses of the appropriate anticonvulsants (eg, valproic acid). It appears that the severity of JME seizures decreases in adulthood and senescence. Whether JME is outgrown at a later age (i.e.: >60) as compared to other primary generalized epilepsies is unknown. However, in one of the author's experience (JEC), older relatives of people with JME that have a history of seizures are often untreated and rarely have seizures. An epidemiological study is needed to settle this question.
Risk of recurrence is high (>80%) if anticonvulsants are withdrawn, even after many years of complete seizure control with low doses of appropriate medications.
Life-long treatment is usually necessary. However, treating a patient older than 60 years with this condition is rare. Whether JME spontaneously remits after a particular age is uncertain."

I found a great link with some FLE info:
Frontal Lobe Epilepsy info

As well, JME: Juvenile Myoclonic Epilepsy





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