Locked-In Syndrome: Trapped in Your Own Body

Locked-in syndrome (LIS), also known as pseudocoma or cerebromedullospinal disconnection, is a condition in which an individual is conscious and aware of their surroundings but are unable to communicate or move in any manner because all voluntary muscles in the body are paralyzed (with the exception of the eyes and vocal cords) and aphonia occurs (inability to speak).

Consciousness, but with the inability to move or communicate: essentially, trapped in one’s own body.

[When one’s eyes are paralyzed as well, the condition is called Total Locked-in Syndrome.]

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Some of the causes of LIS are:

  1. Amyotrophic Lateral Sclerosis, a.k.a. Lou Gehrig’s Disease (well publicized by the Ice Bucket Challenge)
  2. Multiple Sclerosis
  3. Direct brain damage to the brainstem/pons via stroke, etc.

Basically, in every cognitive aspect, the patient is fully functioning and is fine.  There’s no damage to the upper brain, such as in patients with a persistent or permanent vegetative state, and only certain parts of the brainstem and lower brain are damaged.

However, they have almost no means of expressing or conveying these cognitions to anything outside of their own minds..

..the only methods to accomplish this so far are through blinking and moving the eyes (usually unaffected by the paralysis).

 

So if vocal cords are usually unaffected by LIS, why can’t the patients talk?

Although they are conscious and aware, LIS patients can’t coordinate their voice and breathing– which means voluntary sounds, such as speech, cannot be achieved.

This is because the brainstem, more specifically the pons, is responsible for conveying information to other areas of the brain via nerve fibers.  When this area is damage, the inter-bodily communication is cut off.

Damage to one simple part of the brain– and in a split second, all means of communicating your cognitions to the outside world are crippled.

Sadly, motor functioning and control rarely recover due to the permanent natures of the common causes of LIS, and mortality rates in the first 6 months following diagnosis have been reported to be as high as 85%.

To date, there is no cure and a limited amount of treatment options available for patients (due to the relatively low incidence rate).

 

What do you guys think? Did you know about LIS being a side effect of ALS before?
Let us know your thoughts in the comment section below.


Bauer, G. and Gerstenbrand, F. and Rumpl, E. (1979). “Varieties of the locked-in syndrome”. Journal of Neurology 221 (2): 77–91.

Bruno MA, Schnakers C, Damas F, et al. (October 2009). “Locked-in syndrome in children: report of five cases and review of the literature”. Pediatr. Neurol. 41 (4): 237–46.

Fager, Susan; Beukelman, Karantounis, Jakobs (2006). “Use of safe-laser access technology to increase head movements in persons with severe motor impairments: a series of case reports”.Augmentative and Alternative Communication 22 (3): 222–29.

“‘Locked-In’ Patients Can Follow Their Noses”. Science Mag. 26 Jul 2010. Retrieved 27 Jul 2010.

Plum; Posner, JB (1966), The diagnosis of stupor and coma, Philadelphia, PA, USA: FA Davis, 197 pp.

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