Manic behavior before and/or after a seizure?

[KarenB]

Our 8 year old, who has had seizures since age 1 (with 2 long periods of no seizures), has recently developed a new thing.

Sometimes, before having a seizure, he'll get into a state where he's sort of laughing hysterically -- at anything. This can go on pretty much for an hour or more, and sometimes all day, and then that night he'll often have a seizure.

And, very commonly now, immediately after having a seizure (either tonic-clonic or what I call "scream 3 times and stare for 10 seconds") he'll sit bolt upright, look around and grin, and then try to get up and dance or run around. But it's like he's drunk -- he has no balance -- so he's falling everywhere. We have to kind of grab him and hold him for a few minutes, so he doesn't hurt himself. After about 5 minutes, he'll fall asleep, and sleep for at least a half hour (this part has always been part of his seizure recovery period, but not the manic dancing/running around part).

 

[Nakamova]

The frantic giggling may be a kind of seizure called a gelastic seizure. See: http://en.wikipedia.org/wiki/Gelastic_seizure

 

[KarenB]

Thanks...very interesting. Went to the site to take a look.

It seems that this syndrome is usually caused by a tumor, but Jon had an MRI last year (looking for tumors) and a CT scan just a few months ago -- and everything was normal (except for something to do with old damage in white matter).

And...he's had seizures for 7 years, off and on, and this didn't start manifesting itself until the past year. According to the article, the syndrome usually starts in very early childhood.


Another interesting thing -- it DOES say that the syndrome causes developmental delay and loss of cognitive function -- and we HAVE experienced that since his seizures got pretty bad (around age 5). On another thread...there was a statement that seizures don't cause brain damage, which I took exception to.

 

[Nakamova]

There are a few CWE members who experience gelastic seizures, but don't have tumors -- so that may still be a possibility for your son.

There is an association between epilepsy and mania -- both are characterized by low levels of GABA (Gamma-Aminobutyric Acid). GABA is an inhibitory neurotransmitter, so when it's in short supply, the brain tends to be overstimulated and chemically unstable. The result can be manic behaviors -- including hysteria -- as well as seizures.

 

[KarenB]

Thanks Nakamova, that is very helpful info!

Now I'm trying to wrap my head around this whole GABA thing. What causes a GABA deficiency? I read in Wikipedia "In spastic diplegia in humans, GABA absorption becomes impaired by nerves damaged from the condition's upper motor neuron lesion, which leads to hypertonia of the muscles signaled by those nerves that can no longer absorb GABA." -- however, Jon isn't spastic -- he's the opposite -- he has Down Syndrome, so has hypotonia -- altho his tone isn't bad, actually. So...in someone without Cerebral Palsy, what can cause a deficiency?

I also read that GABA is synthesized from glutamate. Is this glutamate like MSG??? And...if so...can MSG actually be HELPFUL for folks with this type of seizure? I've always heard that MSG can be a seizure trigger. But...Jon was raised in Asia (consuming MSG regularly, which didn't seem to affect him) and then when we brought him to the States, his seizures increased by about 75% -- so I've always assumed it was some change in the diet, but not sure what. He does not get MSG now, as we've eliminated all processed food, and make all his food from scratch. Am I drawing the wrong conclusions here?

 

[KarenB]

OH, and can one be tested for GABA deficiency??

 

[Nakamova]

It's incerdibly complicated, since the brain regulates itself in ways that are intricate (and not yet fully understood), ways that involve feedback loops and receptors, hormones and enzymes and electrolytes. When GABA levels are low it's not just a clear-cut deficiency -- it can be due to genetic issues with the GABA receptors, the presence of GABA inhibitors, and/or low serotonin levels. Many drugs that have a sedative effect (including anti-seizure ones) do so by targeting GABA synthesis, release and/or binding.

There are supplements that work as GABA precursors and enhancers. Glutamate is one of them, but eating more foods with glutamate can be risky because glutamate is also an excitatory (stimulating) neurotransmitter. (Glutamate is related to MSG, just as aspartate, another excitatory neurotransmitter, is related to aspartame, one reason why both those foods can sometimes be tricky for folks with seizure disorders.) It's very rare for glutamine levels to be low.

Other supplements that help with GABA or serotonin production are B6, taurine, 5-HTP, and Theanine. There are GABA-containing supplements available as well, but there are no scientific studies demonstrating their efficacy, and since GABA doesn't cross the blood brain barrier, such a supplement is less likely to reach the target receptors.

So messing with GABA isn't easy to do. In someone with Down's Syndrome, the GABA system tends to work too well, so finding ways to control seizures in someone with Down's may require a subtler approach.

 

[KarenB]

GABA and Precocious Puberty

OK, getting back to the GABA thing...I just found a study titled, "Role of GABA in the Mechanism of the Onset of Puberty in Non-Human Primates" (Int Rev Neurobiol. 2005; 71: 113–129.)

This is interesting, because not only is Jon having these giggling episodes, but he was just diagnosed with Precocious Puberty (perhaps my thread on that should be moved here to Nursery).

The article mentioned: "It is possible that the pubertal increase in gonadal steroids may sensitize neurocircuits involved in epileptic seizures, but it is also possible that there is a common mechanism of developmental deficiency, i.e., weakened GABA inhibition in the LHRH neuronal system resulting in precocious puberty and weakened GABAergic inhibition in the brain at the pubertal age resulting in epilepsy (Olsen and Avoli, 1997). Bourguignon and colleagues treated an 11-month old child who exhibited severe epileptic seizures and precocious puberty with loreclezole and vigabatrin, GABA agonists. At an earlier stage traditional treatment for epilepsy with phenobarbital was not effective in this patient. However, treatment with loreclezole followed by vigabatrin not only regressed all signs of precocious puberty, but also settled seizure attacks (Bourguignon et al., 1997)."

 

[Nakamova]

Very interesting -- worth mentioning to the docs in case loreclezole and vigabatrin would be safe to treat with/

 

[KarenB]

Well, that's the thing -- can't find much on safety or side effects of Loreclezole.

Vigabatrin's most common side effects are somnolence; and hyperactivity in children. (Presumably not both at the same time)

 

[KarenB]

I've come to believe that these super-manic episodes following a seizure are actually part of the seizure itself. In the last couple nights, he's had a tonic, visibly lasting about 10 seconds (screams and stiffens up), and then, when we think he's recovering, he'll start to relax, and then suddenly will roll over on his back, and his arms and legs will go straight up in the air (like a baby's moro reflex), and then he'll sit up, and often will lunge forward with lightening speed (we've had to move his mattress to the floor, for obvious reasons). And sometimes, he'll jump up and take off running. Last night, we thought the seizure was over, when all of a sudden he did that moro reflex thing, then with super-human speed jumped up and ran full speed right into the (closed) door. I was sure that impact would bring on a tonic/clonic. It didn't, but he has a nasty bruise on his forehead now.

 

[KarenB]

Well, yesterday,when neurologist heard about Jon running into the door (and saw the big bruise on his forehead) he went ahead and scheduled a video sleep EEG for next week. He (and the Epileptologist) both want to see what's going on with these psychotic like episodes, so want to catch them on tape while seeing what his EEG is doing while he has them.

One thing we already know (from previous EEGs) is that his seizures are almost always preceded by screaming loudly two or three or four times and a stare of horror, and then he stiffens and goes into seizure. His eegs showed the the screaming and look of fear was actually not recorded as seizure activity (so it's not like the "screech" produced when air is forced out of the lungs when a tonic or tonic/clonic starts up).

Today, I was talking to our older (grown) son on phone, was Jon was sitting on the chair with me, and Jon went into a seizure while we were talking, and our older son (who could hear on the phone but not see the seizure) said it sounded like Tourettes.