What is Locked-in Syndrome?

Cerebromedullospinal Disconnection or Ventral Pontine Syndrome is also known as "Locked-in Syndrome" which is a form of pseudo-coma. People suffering from this disorder are awake and knowledgeable, but with the exception of their eyes, lack all motor skills. They are fully paralyzed and mute. For years past and even today, many of these patients were thought to be in a vegetative state but are actually fully awake; their dilemma is not having the ability to move or communicate. Most people with this syndrome have full cognitive functions. Specific portions of the lower brain are damaged but not the upper portions; conversely, a person in a fully vegetative state suffers damage to both the upper and lower brain. Locked-in Syndrome is a rare neurological disorder.

What causes locked-in syndrome?

There are several causes of "locked-in syndrome." A common cause is by traumatic brain injury which is referred to as intracranial injury. This is usually the result of severe automotive or motorcycle crash injuries which are usually caused by the victim not using safety devices. Another reason may be caused by a faulty circulatory system in which nutrients and blood cells cannot flow correctly and may cause non stabilized function to the brain. Nerve cell damage, specifically to the myelin sheath is another possible cause. The myelin sheath which is a dielectric or insulating nerve is responsible to speed all impulses to the brain and when damaged cannot. The most common cause is by suffering a basilar artery thrombosis. The basilar artery is designated to supply the brain with oxygen rich blood which is necessary for the brain to function properly.

Treatment for locked-in syndrome

Approximately ninety percent of all patients diagnosed with 'locked-in syndrome" will expire within four months. At present there is no standard treatment or cure. Electrical muscle stimulation (EMS) or Neuromuscular electrical stimulation (NMES) may be used to stimulate and mimic nerve action but it is not very successful. The prognosis of full or even limited recovery is extremely rare. Even if a patient's vocal cords are not paralyzed they are not able to speak because of the lack of coordination between the cords and breathing. Some patients may recover certain functions but full motion recovery is nil. Non-specific treatment is to prevent complications such as pneumonia, and pressure ulcers (bed sores).

Most often, people with this syndrome do have the ability to move their eyes and with special tracking devices may be able to operate a computer or at least blink signifying yes or no to a basic question. Due to scientific research there are some "Brain-computer interface" devices which may provide non-mechanical communication between a patient and a computer which will facilitate communication.

Conclusion

Presently at the Massachusetts General Hospital and The Natural Center for Medical Rehabilitation Research, collaborating with the NICHD (Eunice Kennedy Shriver National Institute of Child Health and Human Development) and NIDCD (National Institute on Deafness and Other Communication Disorders) are researching the implementation of the BrainGate2 Neural Interface sensor into the motor cortex. With its success it will allow people with paralysis to recover many abilities which now rely on the mobility of hands. They are now seeking volunteers for this program.

Cerebromedullospinal Disconnection is a terrible syndrome and cannot be cured, but hopefully with medical and scientific funding for research its victims will eventually be able to communicate.

Reources:

NINDS

Merk

BrainGate2