Locked-In Syndrome: Losing Movement, But Not Love

Bob Viellete, now age 62,  was the managing editor of a smalltown newspaper in Connecticut, the Waterbury America-Republican. He has been compared to the Jimmy Stewart character in the movie It's a Wonderful Life. A pillar of his church, the father figure everyone depended on at his job, a fixture of every community charitable campaign, and a devoted husband and father to three girls, Viellete had reached the point in life when he had his kids out of college and he had thought he might pay off all his bills, slow down, and travel a bit.

Instead, he spends his days sitting motionless in a chair. He communicates by blinking at letters called out by his wife, Bonnie. Once the pianist for every community fundraiser, always working for free, he listens to big band music and Duke Ellington, but his hands no longer move. However, his mind is still working. "The world is deceived by ornament," he recently signalled his wife, quoting Shakespeare.

Viellete's life as he knew it ended April 8, 2006. He had played a concert at the town library. He had carried his equipment out to the car, but never came back. He remembered trying to honk the car horn to get help, but by the time he was found, he had had a stroke, and would never move again. He had locked-in syndrome.

What Is Locked-In Syndrome?

Locked-in syndrome, sometimes abbreviated as LIS, is sometimes described as the closest thing to be buried alive. Locked-in syndrome tends to strike younger victims, in their thirties, forties, and fifties, rather than the very old. Older victims of this kind of stroke usually die.

Usually locked-in syndrome is caused by a blood clot in the basilar artery. This is the blood vessel that provides blood and oxygen to the pons, the brainstem, at the base of the brain. Locked-in syndrome can also result from a dissection, a kind of "peeling" away of the basilar artery from the tissues around it, caused by atherosclerosis at the end of the artery.  It can be a consequence of migraine, and from manipulation of the neck by chiropractors or in sports injuries. A few cases have occurred from getting a shampoo. The hairdresser failed to cushion the neck while tilting the head back into the hair washing sink, and the sides of the artery tore.

In this condition, the base of the brain that makes a "bridge" to the rest of the body is damaged, but the rest of the brain is intact. Sometimes minimal motor functions are maintained. It may be possilbe to blink, making slow and tedious communication possible. It may be possible to lift a finger, or even to smile. Sometimes there is no motion at all.

READ ALS: The Disease Behind The Ice Bucket Challenge

What Does It Feel Like To Be Locked In?

Surprisingly, many people with locked-in syndrome who are eventually able to communicate do not want to be euthanised. The body is locked out of the brain's control, but the brain also is spared many of the inputs that cause emotional distress. There is total dependence on caretakers, but there is total freedom to think and feel as one wants. No one, of course, would ever want to live locked in if it could be prevented, and sometimes it can be. No one wants to live with the consequences of a brain stem (vertebrobasilar) stroke.