People with locked-in syndrome are fully conscious and aware, but cannot move or communicate verbally because of complete paralysis of almost all of their voluntary muscles, the result of a lesion in the lower brain or brain stem.
Also known as pseudocoma, locked-in syndrome usually leaves eye muscles unaffected, allowing a person to communicate with eye movements or blinking.
In a study being presented Thursday at the Canadian Stroke Congress in Montreal, researchers followed 25 locked-in stroke patients over a period of 20 years and found many enjoyed greater autonomy as a result of various types of adaptive technology.
"These patients can achieve a remarkable level of independence in communication and mobility with the use of rehabilitation and technological help," said researcher Dr. Nicole Beaudoin, chief of rehabilitation at the Institut de readaptation Gingras-Lindsay de Montreal.
"They have a strong will to live and we help improve the quality of their lives," Beaudoin said.
Most patients in the study were trained to use some form of technology that could enhance their independence.
Forty per cent were able to use a joystick activated by the movements of the head to drive a wheelchair. Patients with incomplete locked-in syndrome, or LIS, who had regained motor function in one hand through rehabilitation, were more likely to use a joystick, the authors say.
The study found one-quarter of patients were able to master a head mouse emulator, a small device worn on the forehead, to access a computer.
Other locked-in stroke patients, who have less movement, require more sophisticated computerized interfaces to activate a scanning system, which permits combined computer-supported communication and safe wheelchair control.
"In spite of considerable motor disabilities, many patients achieved a high level of autonomy to communicate and to control a motorized wheelchair," said Beaudoin.
Although LIS patients are immobile for the most part, they are sometimes left with the ability to make a one- or two-degree movement of the head, twitch a finger or toe, as well as perform eye movements.
Even such minimal functions can open up their world in many ways, Beaudoin said.
Some patients learned to operate a motorized wheelchair or control a mouse to access a computer with just the slightest head movement. Others could use eye-tracking technology to access an interface with words or symbols, allowing them to communicate.
One patient has a switch under one of her toes, with which she can write, communicate, send emails and play computer games. It also allows her words to be translated into sound.
Researchers reported on another patient, who spent a year on his back staring at the ceiling, because no one realized initially that he could communicate with his eyes. With technology, the man can now relate his needs to caregivers.
Because most patients with locked-in syndrome are unable to move, they are often thought to be in a vegetative state and unaware of their surroundings.
"Often it is the family that is around (the patient) who will have the idea and say 'Perhaps he understands,'" said Louise De Serres, an occupational therapist at the rehabilitation institute who led the study with Beaudoin. "It's really important to determine a way to (get them to) say yes and no. And often it will be with the eyes."
De Serres said only a fraction of one per cent of people who suffer a stroke will be left with locked-in syndrome. And in some cases, a person will regain muscle function over time. Three of the patients in the study are now able to walk, though most recover only tiny movements in one part of the body, such as a finger or toe.
Even regaining that much movement can allow a patient to press a switch that operates a motorized wheelchair or a computer interface of some kind, she said from Montreal.
Ongoing advances in technology can greatly improve the lives of many people following a stroke, including those with LIS, said Ian Joiner of the Heart and Stroke Foundation.
"These results underscore how rehabilitation can have a huge positive impact, even when the outcomes appear very grave at first."
De Serres said the goal of rehabilitation specialists is to maximize quality of life for patients, who can live for decades following a debilitating stroke.
"They are really an inspiration for us because they find a way to take the good part of life," she said.
Perhaps the best-known public figure with locked-in syndrome was Parisian journalist Jean-Dominique Bauby, who had a massive stroke in December 1995 that left him fully paralyzed except for the ability to control his left eyelid. By blinking this eye, he painstakingly dictated one alphabetic character at a time to write his memoir, "The Diving Bell and the Butterfly."
Bauby died of pneumonia three days after the book was published in March 1997. A critically acclaimed 2007 film version of "The Diving Bell and the Butterfly" starred Mathieu Amalric as Bauby.