Angelman syndrome
Angelman syndrome (AS) is a neurodevelopmental disorder characterized by severe intellectual and developmental disability, sleep disturbance, seizures, jerky movements (especially hand-flapping), frequent laughter or smiling, and usually a happy demeanor.
AS is a classic example of genomic imprinting in that it is caused by deletion or inactivation of genes on the maternally inherited chromosome 15 while the paternal copy, which may be of normal sequence, is imprinted and therefore silenced. The sister syndrome, Prader–Willi syndrome, is caused by a similar loss of paternally inherited genes and maternal imprinting.
AS is named after a British pediatrician, Harry Angelman, who first described the syndrome in 1965. An older, alternative term for AS, "happy puppet syndrome", is generally considered pejorative and stigmatizing so it is no longer the accepted term. People with AS are sometimes referred to as "angels", both because of the syndrome's name and because of their youthful, happy appearance.
Podcasts:
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by 10,000 Maniacs
You Happy Puppet
by: 10,000 Maniacs[ music: Robert Buck/words: Natalie Merchant ]
How did they teach you to be just a happy puppet dancing on a string?
How did you learn everything that comes along with slavish funnery?
Tell me something, if the world is so insane,
is it making you sane again to let another man tug at the thread that pulls up your nodding head?
How did they teach you to be just a happy puppet dancing on a string?
How do you manage to live inside this tiny stage you can't leave?
Tell me something, if the world is so insane,
is it making you sane again to let another man tug at the thread that pulls up your nodding head?
A dullard strung on the wire.
When the master's gone you hang there with your eyes and your limbs so lifeless.
How did they teach you to be just a happy puppet dancing on a string?
How do you manage to speak, your mouth a frozen grin?
A dullard strung on the wire.
When the master's gone you hang there with your eyes and your limbs so lifeless.
Tell me something, if the world is so insane,
is it making you sane again to let another man tug at the thread that pulls up your empty wooden head?
Your hollow head, your marble eyes, your wooden hands and your metal jaw pins
all wait in limbo for the man who knows how to move you this way.