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.

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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.

Preventing Locked-In Syndrome

Especially in younger people, under 45 years of age, the events that lead to locked-in syndrome occur over several days or even several weeks. Maybe there's some kind of accident that causes what seems at first to be neck strain. There could be a strange headache after having hair washed. There could have been dull pain after swinging a golf club or a hockey mallet or a tennis racket. There could have been persistent pain after lifting a heavy object.

Maybe there is headache. The kind of headache associated with the vascular events that lead to locked-in syndrome tends to be at the base of the head, although any kind of persistent headache, in the same part of the head each time, coupled with pain at the base of the skull, is consistent with these events.

In older people, locked-in syndrome is typically a consequence of stroke. The chronic conditions that lead to stroke can be much more insiduous, harder to detect. Many years of uncontrolled high blood pressure, especially when readings range 180/130 and higher, stresses arteries all over the body, including the brain. The linings of the arteries in the brain can be prone to dissection, peeling away so that the brainstem, which is served by blood vessels arranged in a loop. However, even if injury or chronic blood vessel disease has caused the catastrophic event that leads to locked-in syndrome, at least for the first few hours, it's possible to save the brainstem.

Anytime there is sudden

• Loss of speech,
• Loss of movement on one side of the body,
• Loss of vision, especially loss of vision on just one side of the body, or
• Sudden loss of consciousness,

it is important to get to a well-equipped emergency room right away. It is often possible for doctors to restore circulation with intra-arterial or intravenous (IV) recombinant tissue-type plasminogen activator (rt-PA), or "clot busters". These drugs restore the flow of blood in the brain, but they should be given in the first 90 minutes after the clot forms, and they cannot be given more than seven hours after the clot forms. If you are already on blood thinning medications, such as Plavix (clopidrogel), usually you cannot be given these kinds of drugs, but you are also less likely to have a large blood clot form.

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Of course, if you are the person experiencing these symptoms, you may not be able to call for help. The American Heart Association and the American Stroke Association recommend that everyone learn how to respond to possible strokes by memorizing FAST:

• F: Face drooping. Does one side of the face droop or is it numb?
• A: Arm weakness. Is one arm weak or numb?
• S: Speech difficulty. Is the speech slurred or incoherent?
• T: Time to call 911 when someone is experiencing these symptoms.

Getting a friend, a coworker, or a loved one to treatment fast can make a huge difference, sometimes even preventing locked-in syndrome after a brainstem stroke.