HELP JME

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Can anyone tell me how JME differs from progressive myoclonic epilepsy. How can you confirm diagnosis. Also does JME usually include tonic -clonic seizures as my daughter is only having eyelid flutter plus occasional(4-6x/day) myoclonus-only brief jump(like when someone says 'boo' behind you!)

Comments

RE: HELP JME

Submitted by cstrain on Sun, 2005-01-02 - 20:31

hi deborah

I have JME, i've never heard of progressive JME. But, it took about 23 yrs for me to be diagnosed with JME, although i had all the classic symptoms.

my memory is bad because of the depekene. it isn't because of my epilepsy i do not think. my myclonic jerks are at their worst in the morning, if i am sick or stressed, or if i'm very tired. i have tonic clonic or grand mal seizures every so often (at most once per year), though none in the past 2 yrs. i have photosensitivity as well.

so you may be noticing a change in your daughter from the medications. i wouldn't worry much about it being the progressive JME, unless there is something more. if u have any questions about living with JME, please feel free to ask

cassandra

hi deborah

I have JME, i've never heard of progressive JME. But, it took about 23 yrs for me to be diagnosed with JME, although i had all the classic symptoms.

my memory is bad because of the depekene. it isn't because of my epilepsy i do not think. my myclonic jerks are at their worst in the morning, if i am sick or stressed, or if i'm very tired. i have tonic clonic or grand mal seizures every so often (at most once per year), though none in the past 2 yrs. i have photosensitivity as well.

so you may be noticing a change in your daughter from the medications. i wouldn't worry much about it being the progressive JME, unless there is something more. if u have any questions about living with JME, please feel free to ask

cassandra

RE: RE: HELP JME

Submitted by deborah on Mon, 2005-01-03 - 02:30

thank you everyone for your very detailed and personal responses.there are lots of things for us to follow up there.you all sound very positive although I'm sure there are days when you feel down.... just want you to know that you are being a huge help/support. Thanks Deborah.

RE: HELP JME

Submitted by mexican_fire on Sun, 2005-01-02 - 21:05

Yes, JME involves TCs. Usually in the early morning hours, or right after you wake up.

JME is a syndrome of Myoclonic and TC seizures with typical on-set at 12-18 years of age, but it can start as early as 8 and as late as 35.

It is a primary generalized epilepsy. It doesn't go away, it is there forever, and meds can never be reduced, because people with JME are at a very high risk for the return of seizures within 48 hours of being removed from the meds.

The characteristic symptom is sudden, mild-to-moderate jerks of the shoulder and arms that occur shortly after awakening. Consciousness is not disturbed during these seizures.

Jerks also occur when the person is falling asleep or during their sleep, (not the same as a hypnic jerk that occurs as a non-epileptic person is falling asleep). They may occur at any time as well.

Absence seizures occur in about 35% of the people with it, and TCs occur 90% of the time, with Myoclonics occuring all the time.

Absence seizures are hard to detect, and therefore may go un-noticed for a long time.

JME usually responds WELL to Depakote, and not much else.

More common in females than in males.

EEG usually shows a bilateral, symmetric, synchronous, and diffuse polyspike-and-slow wave complexes with a ferquency of 4-6 Hz.

Progressive Myoclonic Epilepsy (PME) encompasses a group of disorders in which myoclonus is a major component. In addition, the patients typically have TCs, or clonic seizures, mental deterioration culminating in dementia, and a neurologic syndrome that almost always includes cerebellar dysfunction.

In addition to cerebellar dsyfunction, common neurologic deficits involve visual, pyramidal, extrapyramidal systems and partial seizures that originate in the occipital region.

Conditions commonly seen with PME incluse Unverrict-Lundborg Disease, Sialidosis, Gaugher's Disease, Niceroid Lipofuscinosis.

PME is usually progressive wtih relentless deterioration of neurologic functions, and with increasing severity of myoclonus and seizures. Almost all people with PME are severely ataxic, demented, wheel chair bound, or bed-ridden.

The EEG findings are quite a bit different, and rare, as well.

I have a sub-type of TLE in my LTL, and abnormalities in the anterior portion on the EEG pattern, I also have Juvenile Absence Epilepsy and Myoclonic seizures that I was born with.

The TLE has only been in the last 4 years, and the Juvenile Absence Epilepsy started in the second grade, and never went away. It isn't supposed to start that early, but mine did.

I also had nocturnal TCs a a child.

I am currently being treated with 1000 mgs of Depakote and 3600 mgs of Neurontin.

I have also been on 7 other drugs, and none of them did anything to control this mess I have.

However, I am of above average intelligence, far from being gifted, but in the mid to upper range of normal, and have no mental retardations. I was not born with developmental delays, even though I had seizures, I was not born slow or stupid, and I can tell you that I am very grateful for who I am, even if the epilepsy has sinice screwed me up.

I have to wear a helmet on the days that my Myoclonics are bad. I have had my feet pulled out from under and I will go crashing to the ground, and get bruised up. I will drop things or throw things, if I get a jerk in my arms, and have broken alot of stuff.

I have 5 seizure types total between these two syndromes.

For the TLE, I have epileptic aura, SPS, CPS and SGTC.

For the JAE, I have Absence, and Primary Generalized TCs.

Then I have the Myoclonics I was born with.

I am also photosensitive.

Nancy

Yes, JME involves TCs.  Usually in the early morning hours, or right after you wake up.JME is a syndrome of Myoclonic and TC seizures with typical on-set at 12-18 years of age, but it can start as early as 8 and as late as 35.It is a primary generalized epilepsy.  It doesn't go away, it is there forever, and meds can never be reduced, because people with JME are at a very high risk for the return of seizures within 48 hours of being removed from the meds.The characteristic symptom is sudden, mild-to-moderate jerks of the shoulder and arms that occur shortly after awakening.  Consciousness is not disturbed during these seizures.Jerks also occur when the person is falling asleep or during their sleep, (not the same as a hypnic jerk that occurs as a non-epileptic person is falling asleep).  They may occur at any time as well.Absence seizures occur in about 35% of the people with it, and TCs occur 90% of the time, with Myoclonics occuring all the time.Absence seizures are hard to detect, and therefore may go un-noticed for a long time.JME usually responds WELL to Depakote, and not much else.More common in females than in males.EEG usually shows a bilateral, symmetric, synchronous, and diffuse polyspike-and-slow wave complexes with a ferquency of 4-6 Hz.Progressive Myoclonic Epilepsy (PME) encompasses a group of disorders in which myoclonus is a major component.   In addition, the patients typically have TCs, or clonic seizures, mental deterioration culminating in dementia, and a neurologic syndrome that almost always includes cerebellar dysfunction.  In addition to cerebellar dsyfunction, common neurologic deficits involve visual, pyramidal, extrapyramidal systems and partial seizures that originate in the occipital region.Conditions commonly seen with PME incluse Unverrict-Lundborg Disease, Sialidosis, Gaugher's Disease, Niceroid Lipofuscinosis.PME is usually progressive wtih relentless deterioration of neurologic functions, and with increasing severity of myoclonus and seizures.  Almost all people with PME are severely ataxic, demented, wheel chair bound, or bed-ridden.The EEG findings are quite a bit different, and rare, as well.I have a sub-type of TLE in my LTL, and abnormalities in the anterior portion on the EEG pattern, I also have Juvenile Absence Epilepsy and Myoclonic seizures that I was born with.The TLE has only been in the last 4 years, and the Juvenile Absence Epilepsy started in the second grade, and never went away.  It isn't supposed to start that early, but mine did.I also had nocturnal TCs a a child.I am currently being treated with 1000 mgs of Depakote and 3600 mgs of Neurontin.I have also been on 7 other drugs, and none of them did anything to control this mess I have.However, I am of above average intelligence, far from being gifted, but in the mid to upper range of normal, and have no mental retardations.  I was not born with developmental delays, even though I had seizures, I was not born slow or stupid, and I can tell you that I am very grateful for who I am, even if the epilepsy has sinice screwed me up.I have to wear a helmet on the days that my Myoclonics are bad.  I have had my feet pulled out from under and I will go crashing to the ground, and get bruised up.  I will drop things or throw things, if I get a jerk in my arms, and have broken alot of stuff.I have 5 seizure types total between these two syndromes.For the TLE, I have epileptic aura, SPS, CPS and SGTC.For the JAE, I have Absence, and Primary Generalized TCs.Then I have the Myoclonics I was born with.I am also photosensitive.Nancy