Surgery ends ‘Joker’ seizures

When she was having her seizures -- sometimes more than 30 times a day -- Anastasia Lagala’s face locked into a smiling grimace as her limbs flailed, and an eerie laugh came from her throat.

“The Joker face,” her physician, Dr. Steven Schneider of Schneider Children’s Hospital, called it.

But since recent surgery, the 3-year-old girl with a rare form of epilepsy has been seizure-free.

For the past year, Anastasia suffered from gelastic epilepsy, which is marked by numerous seizures, each lasting about 10 seconds.

“It was very frightening at first, when we didn’t know what was going on,” her mother, Jessica Anderson, 23, said Thursday at a news conference at the hospital.

Anderson, an insurance coordinator, said she was especially alarmed by the noises coming from her daughter, whom everyone calls Anna.

“She was smiling and grinding and laughing.

“It sounded very scary,” she said.

The seizures were triggered by a benign tumor on Anastasia’s hypothalamus, in the center of her brain.

Only about 30 cases of the condition, called hypothalamic hamartoma, are diagnosed worldwide each year, according to the hospital.

Left untreated, the condition can lead to severe retardation.

By early March, Anastasia was having 20 to 40 seizures a day, and her sweet disposition turned sour as she kicked and screamed her way through tantrums.

The condition does not respond to medication, and people seeking treatment typically travel to a specialist in Australia or to a neurological institute in Phoenix.

After seeing the child, Schneider, co-chief of the hospital’s pediatric neurosurgery unit, decided to study the surgical techniques and perform the operation much closer to home.

On March 30, he removed a grape-sized tumor by splitting the lobes of Anastasia’s brain and using a special navigation system, much like a car’s GPS, to maneuver around the tissue.

“The tumor, it’s very hard to get to,” Schneider said.

Since the surgery, Anastasia hasn’t had another seizure. It’s too soon to tell whether she will have any developmental issues, he said.

Schneider, who already has fielded inquiries from other people with the condition, said he was happy with the outcome.

“I’m very pleased. I’m very excited,” he said.

Anastasia was in a feisty mood Thursday, looking forward to chicken nuggets and a day outdoors.

“I want to go to the park,” she announced to her father, Peter Lagala, 24, a personal trainer, who stood beside her, holding her pink Dora the Explorer backpack.

Lagala smiled. “Everything’s good,” he said as he hoisted his daughter and held her close.