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Has anyone's child been diagnosed with Progressive Myoclonic

Epilepsy? This may be premature but Mallorie may have this type of

epilepsy. She is being scheduled for a 24 hr. video EEG and MRI. She

will also see a metabolic specialist in February. From what I have

read this is a progressivly degenerative type of epilepsy with a poor

prognosis. We just saw another neurologist who thinks that Mallorie

did not fit the profile of infantile spasms and that she probably has

PME. She is 3 yrs. old. and started having seizures at 10 months.

Thanks for any feedback.

(Mallorie's mom)

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What are the "signs" of PME - how is the diagnosis made? We are still

waiting on a diagnosis for our son. They said they werent willing

to diagnose for a full year to see how things progress.

Jenn

joeycmiller wrote:

Has anyone's child been diagnosed with

Progressive Myoclonic

Epilepsy? This may be premature but Mallorie may have this type

of

epilepsy. She is being scheduled for a 24 hr. video EEG and MRI.

She

will also see a metabolic specialist in February. From what I have

read this is a progressivly degenerative type of epilepsy with

a poor

prognosis. We just saw another neurologist who thinks that Mallorie

did not fit the profile of infantile spasms and that she probably

has

PME. She is 3 yrs. old. and started having seizures at 10 months.

Thanks for any feedback.

(Mallorie's mom)

"The Ketogenic Diet....a realistic treatment option, NOT

just a last resort!"

List is for parent to parent support only.

It is important to get medical advice from a professional keto team!

Subscribe: ketogenic-subscribe

Unsubscribe: ketogenic-unsubscribe

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We suspected this at one stage with , he had a muscle biopsy, which I think is the most definite way of diagnosing PME. They looked for 'ragged red fibres', which in 's case was negative. Other indicators they checked were increased lactate or pyruvate in the spinal fluid, and a full enzyme assay was sent away to Adelaide.

Hill

Re: PME?

What are the "signs" of PME - how is the diagnosis made? We are still waiting on a diagnosis for our son. They said they werent willing to diagnose for a full year to see how things progress. Jenn joeycmiller wrote: Has anyone's child been diagnosed with Progressive Myoclonic Epilepsy? This may be premature but Mallorie may have this type of epilepsy. She is being scheduled for a 24 hr. video EEG and MRI. She will also see a metabolic specialist in February. From what I have read this is a progressivly degenerative type of epilepsy with a poor prognosis. We just saw another neurologist who thinks that Mallorie did not fit the profile of infantile spasms and that she probably has PME. She is 3 yrs. old. and started having seizures at 10 months. Thanks for any feedback. (Mallorie's mom) "The Ketogenic Diet....a realistic treatment option, NOT just a last resort!" List is for parent to parent support only. It is important to get medical advice from a professional keto team! Subscribe: ketogenic-subscribe Unsubscribe: ketogenic-unsubscribe

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We are going to see the genetics people on Jan 4th and they have talked

to me before about a muscle biopsy. Can this be done on the diet

- and the other tests that genetics does? Does any of this interfere

with the diet? How painful is this for Mike? Is there a long recouperation

period? Does he need to stay in the hospital for any length of time?

Jenn

richard & susan hill wrote:

We

suspected this at one stage with , he had a muscle biopsy, which

I think is the most definite way of diagnosing PME. They looked for 'ragged

red fibres', which in 's case was negative. Other indicators they

checked were increased lactate or pyruvate in the spinal fluid, and a full

enzyme assay was sent away to Adelaide. Hill

Re: PME?

What are the "signs" of PME - how is the diagnosis made? We

are still waiting on a diagnosis for our son. They said they werent

willing to diagnose for a full year to see how things progress.

Jenn

joeycmiller wrote:

Has anyone's child been diagnosed with

Progressive Myoclonic

Epilepsy? This may be premature but Mallorie may have this type

of

epilepsy. She is being scheduled for a 24 hr. video EEG and MRI.

She

will also see a metabolic specialist in February. From what I have

read this is a progressivly degenerative type of epilepsy with

a poor

prognosis. We just saw another neurologist who thinks that Mallorie

did not fit the profile of infantile spasms and that she probably

has

PME. She is 3 yrs. old. and started having seizures at 10 months.

Thanks for any feedback.

(Mallorie's mom)

"The Ketogenic Diet....a realistic treatment option, NOT

just a last resort!"

List is for parent to parent support only.

It is important to get medical advice from a professional keto team!

Subscribe: ketogenic-subscribe

Unsubscribe: ketogenic-unsubscribe

Link to comment
Share on other sites

We are going to see the genetics people on Jan 4th and they have talked

to me before about a muscle biopsy. Can this be done on the diet

- and the other tests that genetics does? Does any of this interfere

with the diet? How painful is this for Mike? Is there a long recouperation

period? Does he need to stay in the hospital for any length of time?

Jenn

richard & susan hill wrote:

We

suspected this at one stage with , he had a muscle biopsy, which

I think is the most definite way of diagnosing PME. They looked for 'ragged

red fibres', which in 's case was negative. Other indicators they

checked were increased lactate or pyruvate in the spinal fluid, and a full

enzyme assay was sent away to Adelaide.

Hill

Re: PME?

What are the "signs" of PME - how is the diagnosis made? We

are still waiting on a diagnosis for our son. They said they werent

willing to diagnose for a full year to see how things progress.

Jenn

joeycmiller wrote:

Has anyone's child been diagnosed with

Progressive Myoclonic

Epilepsy? This may be premature but Mallorie may have this type

of

epilepsy. She is being scheduled for a 24 hr. video EEG and MRI.

She

will also see a metabolic specialist in February. From what I have

read this is a progressivly degenerative type of epilepsy with

a poor

prognosis. We just saw another neurologist who thinks that Mallorie

did not fit the profile of infantile spasms and that she probably

has

PME. She is 3 yrs. old. and started having seizures at 10 months.

Thanks for any feedback.

(Mallorie's mom)

"The Ketogenic Diet....a realistic treatment option, NOT

just a last resort!"

List is for parent to parent support only.

It is important to get medical advice from a professional keto team!

Subscribe: ketogenic-subscribe

Unsubscribe: ketogenic-unsubscribe

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Share on other sites

Jenn,

's biopsy was in Mar this year, he wasn't on the diet then, but I can't see there would be any problem. He was given a general anaesthetic, ie; gas, and had a muscle and rectal biopsy, and a repeat spinal tap. Whole thing only took about 40 mins, only thing you may have to look at is the timing if Mike has a general anaesthetic, due to the standard pre op starvation period, ie, time it so that there is not too long a delay where he cannot eat, as this could upset ketosis, suppose it depends on the child. 's ketones drop when he doesn't eat for a long period, others rise, I think it depends if they have any excess body 'fat' to eat into to produce ketones.

We were told he wouldn't know anything about the rectal biopsy, but that the muscle one may hurt a bit for a day or two. Well as with everything with , the opposite happened, he woke up crying, saying his bottom hurt, turns out there was a complication, and we ended up back in hospital a week later with a repeat op needed to repair the tissue. The muscle one was a breeze, in fact he asked what the plaster was for that afternoon, didn't feel a thing. He has a thin scar about a cm long on his thigh, but scars are not a new thing with , having had drop attacks for a year or more. We can now add some nasty chicken pox ones to the list as well!

was only in hospital for one night, but only as a precaution due to the risk of increased seizures with the anaesthetic, again, some kids seizures respond well to the anaesthetic! So apart from the rectal complication, no, not traumatic at all, he was walking around that day, albeit with a sore behind!

The results took about 3 months to come back, but a lot of this delay was due to our lab in Ak. I think the norm is about 6 weeks.

had these tests as an elimination process rather than a suspicion of PME. His spinal tap and enzyme assay had previously come back normal, so they were not really expecting to find anything. We were concerned about his worsening ataxia, drooling, motor skills etc, even tho' he seemed relatively well preserved cognitively. In hindsight, all the suspicious signs were attributable to the high med levels, and frequent seizure activity.

Having said all of this, no negative test like this is apparently 100% accurate, we still cannot wipe the possibility of a degenerative disease completely for a while to come yet. has been having seizures for approx 18 months, but his predominant seizure type only became myoclonic after 5+ months. Again, in hindsight, this coincided with the introduction of lamictal into his 'cocktail', I have long harboured the suspicion that this drug aggravates the myoclonics in .

I have to say that the period leading up to the biopsy, and the long wait for the results was possibly our darkest period since his illness began. I think I had learnt too much about the potential diagnoses and prognoses, depending on what the results were to show, sometimes ignorance can be bliss, especially if the suspicions turn out to be unfounded.

Hill.

P.S, have enzyme blood tests and spinal taps already been done, as these usually precede a biopsy. We have a very well respected metabolic specialist at our hospital now, is the person you are consulting with trained in this field as well as genetics? Why are thy suspecting PME, there are other seizure disorders with myoclonics as one of the predominant seizure types, eg, has a tentative diagnosis of Myoclonic Astatic Epilepsy of Early Childhood

Re: PME? What are the "signs" of PME - how is the diagnosis made? We are still waiting on a diagnosis for our son. They said they werent willing to diagnose for a full year to see how things progress. Jenn joeycmiller wrote: Has anyone's child been diagnosed with Progressive Myoclonic Epilepsy? This may be premature but Mallorie may have this type of epilepsy. She is being scheduled for a 24 hr. video EEG and MRI. She will also see a metabolic specialist in February. From what I have read this is a progressivly degenerative type of epilepsy with a poor prognosis. We just saw another neurologist who thinks that Mallorie did not fit the profile of infantile spasms and that she probably has PME. She is 3 yrs. old. and started having seizures at 10 months. Thanks for any feedback. (Mallorie's mom) "The Ketogenic Diet....a realistic treatment option, NOT just a last resort!" List is for parent to parent support only. It is important to get medical advice from a professional keto team! Subscribe: ketogenic-subscribe Unsubscribe: ketogenic-unsubscribe

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