Locked-In Syndrome

Medical Meanderings 28 October 2009

The Horror ©

“A corpse with living eyes...”
- Alexander Dumas, “The Count of Monte Cristo”

Your eyes flutter open and slowly adjust. You hear voices in the distance, and something beeping in a regular rhythm. You try to sit up. No go. Okay, okay, you tell yourself--take it slow. To your right, just inside your peripheral vision, is a glowing monitor with colorful, squiggly lines and numbers, and a clicking box with a bag of water hanging above it. Toward your feet but above you, a television flickers. Directly in front of your face, a corrugated plastic tube seems to emerge from your face and stretch away somewhere. To your left, a sliding glass door beyond which uniformed men and women move briskly about. The answer comes: you’re in a hospital.

Again, you try to sit up without success or even movement. You try to raise your head. Nothing. Move your hand. Nothing. You try to call for help, to get someone’s attention. No movement, no sound. Then you realize you can blink, but you cannot move your eyes. You hear intermittent hissing of air through the tube which you now realize is in your throat, and the beeping of the monitor.

Soon, an older man in a long white coat and a young woman in bright scrubs enter your room. “Sad thing,” says the nurse. What happened? you want to say. “No obvious stroke on the CT scan, yet,” answers the doctor, “but sometimes big strokes can take awhile to show up.” A stroke? you think silently. I had a stroke? I don’t remember. “Well, at least the heart, liver, kidneys...everything seems healthy,” says the nurse. That sounds like good news, you think. “Yes, true. Hopefully some good will come of this,” the doctor answers. “I’ll go speak to the family about organ donation.”

What?! you want to scream. No, I’m here! But you cannot speak. Your eyelids can flutter, but you cannot speak, nor move, nor make a sound. You have indeed suffered a small stroke, but to a very important area of your brain. As a result, you have a rare neurological syndrome appropriately called locked-in syndrome.

Locked-in syndrome was first described in 1966. It is a state in which the connections between the higher regions of the brain (thinking and language) are severed from the regions, called the midbrain and pons, that carry impulses to the spinal cord. The result of this devastating injury is a patient who is fully conscious and awake, but unable to speak or move. It is vital that physician differentiate between locked-in syndrome and more common brain injury states such as coma (in which the patient is unmoving and unconscious) and persistent vegetative state (in which the patient can move but is unconscious). If not, an awake, thinking patient may end up an unwilling organ donor.

This Halloween, though, no need to fear that one day you will wake up unable to move or speak--appearing, essentially, to be brain dead. It wouldn’t, it couldn’t happen to you...right?