Epilepsy can show itself in many ways, including as episodes of giggling and laughing. An expert discusses the case of a nine-year old boy with such seizures, the danger they posed, and the novel way he was treated.
- Dr. Aria Fallah, Assistant Professor of Neurosurgery, UCLA Geffen School of Medicine and Mattel Children’s Hospital
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Nancy Benson: Nine-year old Justin Cho of Los Angeles was one of those kids who giggle a lot. Usually, it’s simply the mark of a happy child. But Justin’s father, Robert, says something about his son’s laughter wasn’t quite right. It seemed to be prompted more by the time of day than something funny.
Justin Cho: We always thought that it was a sign for him to tell us somehow his body somehow was telling us that it’s time for him to go to sleep. He would also giggle in the middle of the night anywhere from twelve midnight to let’s say five in the morning while he’s sleeping. The scary part of it all is my son tells me that he’s had more than two seizures. We just never knew about it.
Benson: “Seizures” is the operative word, because Justin’s giggling fits were the mark of a rare disorder—gelastic (jel-as-tick) epilepsy.
Fallah: It actually took a few years for the family to realize that this giggling is abnormal, especially when it would happen right before he went to sleep or when he’d wake up or especially in situations that were generally not episodes that you would giggle to or laugh to. That’s when they started realizing there was something that’s wrong.
Benson: That’s Dr. Aria Fallah, assistant professor of neurosurgery at UCLAs Geffin School of Medicine and Mattel Children’s Hospital in Los Angeles. He says the source of Justin’s giggles was a benign tumor deep inside the brain, known as a hypothalamic hamartoma (hy-po-thal-am-ick ham-ar-toe-mah). Several others hospitals were unable to diagnose it.
Fallah: This is quite rare. Hypothalamic hamartoma is a quite rare lesion that most physicians will probably not seen across their entire career. But we do see this from time to time especially being in the big epilepsy center here at UCLA. Hypothalamic hamartoma, the difficulty is in the diagnosis, but it has a very classic recognizable clinical manifestation, which is the gelastic epilepsy, what we call laughing seizures, giggling seizures. This is the most classic way that it can present. However, it can sometimes present as a full on seizure or large seizure what we call a tonic-clonic seizure. These are types of seizures that you usually see in the movies where the arms and legs are shaking and a patient may be on the ground with the whole body convulsing. But most commonly it presents as just laughing epilepsy.
Benson: Since laughter is the main symptom—or at least the first one—a delay in diagnosis is nothing unusual. But Fallah says laughter is often just the start of problems when such a brain lesion occurs. It can end up being very serious.
Fallah: Gelastic epilepsy if left untreated can be associated with developmental delay, with behavioral issues. A hypothalamic mass itself can cause endochronilogical issues. If the mass is really large it can cause visual deficits. More importantly seizures that are untreated always have the potential to even cause a life-threatening problem. For example, if a child is swimming and has a seizure there’s a possibility of drowning.
Benson: Anti-seizure drugs are typically ineffective against gelastic epilepsy, so surgery is really the only treatment. But Fallah says hypothalamic hamartomas are found in just about the worst possible place to reach in the brain. So the way doctors used to have to do surgery was a lot less than perfect.
Fallah: This is one of the most challenging areas of the brain to get to. It’s deep in the brain right in the center of the brain and the most critical structures of the brain wrap right around it. Generally the way to approach these in the brain would be done through open brain surgery, which requires very large skin incision, a large part of the skull needs to be elevated and a very long tedious operation for us to very carefully dissect down to the center of the brain and then physically remove the hypothalamic hamartoma. Essentially what happens is a lot of collateral damage that occurs just from us having to approach this lesion. So for several hours of the operation you’re spending a considerable time and effort to try to get down to where the hypothalamic hematoma is. Those are all the structures that you’re putting at risk due to either retraction injury or direct injury just from surgical manipulation.
Benson: And recovery from all that used to be long and arduous.
Fallah: For these types of operations I’d say usually a child would be in the hospital for at least a week. After that it would take about six weeks or so for the child to get back to where he was before. And that’s obviously provided there’s no neurological deficits or side effects that happened from an operation like this.
Benson: Fortunately, brain surgery is improving quickly. Fallah and his team were able to use a different approach for Justin… threading a thin fiber optic strand to the site of the lesion.
Fallah: We used a technology that’s already available to make lesions in the brain or to destroy tissue in the brain, which is called laser thermal ablation therapy. It uses heat energy to what I call cook the tissue or destroy the local tissue without injuring the rest of the brain. So what we did is we used GPS-like technology to very carefully insert this small laser probe which is the size or thickness of a spaghetti noodle deep inside the brain right into the center of the hypothalamus and destroy this lesion from the inside out without causing any collateral damage to the rest of the brain. We’re able to do those through a very small incision that requires just a single stitch at the end.
Benson: With this advance, recovery becomes in credibly short. More like nonexistent.
Fallah: Soon as we woke Justin up from general anesthesia he didn’t have any pain, he didn’t really have any symptoms or side effects, and he was ready to essentially go out the door on day one. We kept him around for an extra day just because the technology is relatively newer just to make sure that everything else is okay. But there really wasn’t much recovery time required for this operation. He’s not had single seizure since the episode. I don’t expect he’ll have any other seizures. We usually wait until at least a year out until we are confident that he has been completely cured, but as you can imagine going from several a day to now it’s been well over six months he’s not had a single episode and it’s quite likely that he’s never going to have another seizure again.
Benson: Fallah says surgery can be more complicated when the lesion is very large. Sometimes more than one laser fiber is needed, and occasionally the mass is so large that laser isn’t an option. So in spite of its name, giggling epilepsy is nothing to trifle with. Our production director is Sean Waldron. I’m Nancy Benson.