- NEW YORK - Laughter is no
joke for 7-year-old Marissa Miller. A growth in her brain regularly causes
laughing fits, known as gelastic seizures, that are completely beyond her
control.
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- "The laugh is not a normal laugh - it's an hysterical
laugh," said Gerri Miller, Michelle's mother. For 15 or 20 seconds,
"it's obvious that she's in distress. Her face goes red, her eyes
have a glazed look, and she gets really stiff."
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- Michelle's condition, a benign brain tumor known as hypothalamic
hamartoma, can also cause more serious seizures, premature puberty in babies
less than a year old and episodes of violent rage. Researchers have struggled
to find a way to treat the disease - especially as laughter remains an
enigma to science.
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- Why Do We Laugh?
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- "How laughter occurs is not completely understood,"
said Dr Samuel Berkovic of the Austin and Repatriation Medical Center in
Victoria, Australia, who authored an article on hypothalamic hamartoma
in the Feb. 22 issue of Neurology.
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- "It involves a complex relationship between the
thought processes and emotional aspects of the brain as well as physical
control of the muscles in the chest and voice box."
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- The hypothalamus, largely responsible for emotion and
behavior, plays a poorly understood role in the process. "We know
the hypothalamus is very important to the generation of laughter,"
Berkovic said. For gelastic seizure patients like Michelle, the tumor's
presence in that part of the brain results in an irresistible urge to let
loose with laughter.
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- "She says, 'I don't want to laugh " I have
to laugh,'" said Gerri Miller, who works with business presentations
in Montgomery, Ala. But not every case is the same. Although Michelle's
gelastic seizures are not accompanied by the perception of humor, some
patients report feeling "pleasant," according to Berkovic.
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- Other parts of the brain can also trigger laughter. Doctors
treating a 16-year-old epilepsy patient found that whenever they gave small
shocks to her temporal lobe, she laughed uproariously. Unlike gelastic
seizure patients, who have an irresistible compulsion to laugh, the epilepsy
patient simply found her circumstances to be unbelievably humorous.
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- "There appear to be distributed centers of laughter
throughout the brain," said Dr. Robert Provine of the University of
Maryland-Baltimore County, author of the upcoming book Laughter: A Scientific
Approach. But science hasn't figured out what happens where.
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- "We have to assemble and present the scattered evidence
that's there," he said.
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- Studying why gelastic patients are compelled to laugh
is made all the more difficult, Provine added, because we don't even really
understand laughter in healthy people " which at times can be equally
mysterious.
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- "You don't choose to laugh," he said. It's
unconsciously controlled human social behavior. For reasons we don't understand,
we often get together and go 'ha ha ha.'"
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- Constant Laughter
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- The gelastic seizures hit 6-year-old Joelle Rue about
once an hour, according to her father, Darryl. "It's a constant battle,"
he said. "She'll just start laughing, maybe even walk through it,
say things while she's doing it. Even during the night she wakes up a few
times, laughing." Doctors warned him that if left untreated, Joelle's
seizures could worsen.
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- "These tumors are known to progress into harder
seizures, where they'll fall down laughing, lose muscle and bladder control,
all the way to grand mal seizures where they're lying on the ground, unconscious,"
he said.
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- Even the milder seizures can be embarrassing in social
settings. "It's very distracting, especially as a child grows older,"
Gerri Miller said. "In kindergarten, the kids didn't notice anything
was wrong. But the older she gets the more they're aware the laughter isn't
normal. It's gotten harder every year."
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- Drugs and Surgery
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- There are no fully proven treatments for hypothalamic
hamartomas, and every case seems to respond differently to drugs and surgery.
Physicians have prescribed anti-epilepsy, anticonvulsive and antidepressant
drugs, to varying effect, and surgeons have operated to remove the tumors
all together.
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- "My 11-year-old daughter Rebecca until recently
suffered from gelastic seizures," said Craig Faulkner, who lives in
Adelaide, Australia, in an e-mail. "Three months ago, Rebecca underwent
surgery in Melbourne to remove her hypothalamic hamartoma. She was the
fifth child to undergo such surgery in the world. She has not suffered
a seizure in the 100 days since surgery."
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- The potential for a breakthrough cure has raised hope
for the Miller and Rue families, who along with Faulkner participate in
an extremely active hyperthalamic hamartoma e-mail <http://hungryminds.egroups.com/group/hhlist/info.html
newsgroup of parents who compare notes and provide encouragement.
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- "The group is the most wonderful thing to find "
there's just nobody else to talk to about [the condition]," Gerri
Miller said.
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