$Unique_ID{BRK03944}
$Pretitle{}
$Title{Locked-In Syndrome}
$Subject{Locked-In Syndrome De-Efferented State Cerebromedullospinal
Disconnection Pseudocoma Akinetic Mutism Quadriplegia Reye Syndrome with Total
Paralysis Spinal Cord Injuries }
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Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.

472:
Locked-In Syndrome

** IMPORTANT **
It is possible the main title of the article (Locked-In Syndrome) is not
the name you expected.  Please check the SYNONYMS listing on the next page to
find alternate names, disorder subdivisions, and related disorders covered by
this article.

Synonyms

     De-Efferented State
     Cerebromedullospinal Disconnection
     Pseudocoma

Information on the following disorders may be found in the Related
Disorders section of this report:

     Akinetic Mutism
     Quadriplegia
     Reye Syndrome with Total Paralysis
     Spinal Cord Injuries

General Discussion

** IMPORTANT **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.

Locked-In Syndrome is characterized by complete paralysis except for
voluntary eye movements.  It is usually caused by lesions in the nerve
centers which control muscle contractions, or a blood clot that blocks
circulation of oxygen to the brain stem.

Symptoms

In the Locked-In Syndrome, all voluntary muscles controlling movement are
paralyzed, except for those performing eye and eyelid movements.  Patients
with Locked-In Syndrome are conscious, but unable to speak.  However, they
can learn to communicate through an eye blink code.

Causes

Locked-In Syndrome is caused by lesions cutting across corticospinal and
corticobulbar nerve tracts, which cut off all motor nerves except for those
to the eye muscles.  Tissue death may also occur on both sides of the body
caused by lack of oxygen circulated to the internal capsule of the brain.  In
some cases the circulation may be blocked by a blood clot in a blood vessel
in the brain stem.  If circulation can be restored the patient's condition
may improve.

Affected Population

Locked-In Syndrome is a very rare disorder.  It affects males and females in
equal numbers.

Related Disorders

Symptoms of the following disorders may resemble those of Locked-In Syndrome.
Comparisons can be useful for a differential diagnosis:

Akinetic Mutism is a disorder characterized by the patient appearing to
be in an awake state with open eyes, but without communication.  Immobility
occurs as a result of lesions causing bilateral frontal lobe damage or
destruction of the reticular activating system of the brain.  The response of
muscles to painful stimuli is poor.

Quadriplegia (quadriparesis; tetraplegia) is a term denoting paralysis of
all four limbs.  Spinal cord injury is one of the more common causes of
Quadriplegia.

Reye Syndrome is a combination of acute brain disease (encephalopathy)
and fatty degeneration of the abdominal organs, which tends to follow some
acute virus infections such as flu or chicken pox, combined with certain
precipitating agents such as aspirin.  Seizures may occur.  A complete
recovery is possible.  However, permanent brain damage, ranging from a slight
decrease in I.Q. to total paralysis, may occur.  (For more information on
this disorder, choose "Reye" as your search term in the Rare Diease
Database.)

Spinal Cord Injury can be caused by trauma to the spine.  Symptoms
include retention of urine, possibly incontinence, reflex spasm below the
site of the injury, and/or paralysis with sensory loss.

Therapies:  Standard

Functional neuromuscular stimulation may help activate paralyzed muscles.
Several devices to facilitate communication for people who cannot speak are
on the market.  Other treatment is symptomatic and supportive.

Therapies:  Investigational

This disease entry is based upon medical information available through April
1989.  Since NORD's resources are limited, it is not possible to keep every
entry in the Rare Disease Database completely current and accurate.  Please
check with the agencies listed in the Resources section for the most current
information about this disorder.

Resources

For more information on Locked-In Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     NIH/National Institute of Neurological Disorders & Stroke (NINDS)
     9000 Rockville Pike
     Bethesda, MD  20892
     (301) 496-5751
     (800) 352-9424

References

LOWER EXTREMITY FUNCTIONAL NEUROMUSCULAR STIMULATION IN CASES OF SPINAL CORD
INJURY:  G.R. Cybulski, et al.; Neurosurgery (July 1984:  issue 15(1)).  Pp.
132-146.

ADAPTIVE EQUIPMENT FOR C6 QUADRIPLEGIA:  AN APPROACH TO EFFECTIVE, SIMPLE,
AND INEXPENSIVE DEVICES:  J.R. Basford, et al.; Archives Phys Med Rehabil
(December 1985:  issue 66(12)).  Pp. 829-831.

RECOVERY FROM LOCKED-IN SYNDROME AFTER POSTTRAUMATIC BILATERAL DISTAL
VERTEBRAL ARTERY OCCLUSION:  J.M. Cabezudo, et al.; Surg Neurol (February
1986:  issue 25(2)).  Pp. 185-190.