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$Unique_ID{BRK04261}
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$Title{Thalamic Syndrome (Dejerine-Roussy)}
$Subject{Thalamic Syndrome Dejerine-Roussy Dejerine-Roussy Syndrome Posterior
Thalamic Syndrome Retrolenticular Syndrome Thalamic Hyperesthetic Anesthesia
Thalamic Pain Syndrome Reflex Sympathetic Dystrophy Syndrome RSDS
Guillain-Barre Syndrome Carpal Tunnel Syndrome }
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Copyright (C) 1990 National Organization for Rare Disorders, Inc.

796:
Thalamic Syndrome (Dejerine-Roussy)

** IMPORTANT **
It is possible that the main title of the article (Thalamic Syndrome
(Dejerine-Roussy)) is not the name you expected.  Please check the SYNONYM
listing to find the alternate names and disorder subdivisions covered by this
article.

Synonyms

     Dejerine-Roussy Syndrome
     Posterior Thalamic Syndrome
     Retrolenticular Syndrome
     Thalamic Hyperesthetic Anesthesia
     Thalamic Pain Syndrome

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

     Reflex Sympathetic Dystrophy Syndrome (RSDS)
     Guillain-Barre Syndrome
     Carpal Tunnel Syndrome

General Discussion

** REMINDER **
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.

Thalamic Syndrome (Dejerine-Roussy) is a rare neurological disorder which
occurs as a result of damage to the thalamus (a part of the brain which can
affect sensation).  Primary symptoms are pain and loss of sensation usually
in the face, arm, or leg.

Symptoms

Thalamic Syndrome (Dejerine-Roussy) is characterized by pain and loss of
sensation.  Some of the body may be affected, or one half of the body,
usually the face, arm, or leg.  All types of sensations can be affected
including touch, pain, and awareness of temperature.  There may also be pain
in the affected part of the body which may increase with stimulation.  For
example, being touched, or being exposed to cold temperatures may cause
spontaneous pain.  Taste may be affected; food and drink may have an unusual
or different flavor.  A slow tremor of the hand, arm, foot, or leg which
increases when the patient attempts to voluntarily move the limb (intention
tremor) may be apparent.  Hand spasms may also occur.  Mild muscular weakness
or partial paralysis limited to one side of the body (hemiparesis) may be
another symptom.

The patient may experience a disagreeable sensation or strong pain in
response to touch which, under normal conditions, would not be uncomfortable
(hyperpathia).  He or she may, at first, have trouble feeling a touch
sensation (dysesthesia) and the threshold for pain may be raised; however,
once the level of pain that he or she can accept is exceeded, there is an
over-reaction to the pain (hyperresponsiveness).  The patient may not have a
sense of what position the affected part of the body is in.  Emotional
over-reactions may occur.

Causes

Thalamic Syndrome (Dejerine-Roussy) stems from damage to the thalamus,
usually appearing 4 to 6 weeks after the damage has occurred.  Symptoms
appear when there is loss of nerve cells in the back part of the thalamus.
The thalamus is a part of the brain that acts as a coordinating center for
nerve impulses from all the senses, relaying them to appropriate areas in the
rest of the brain where they are then consciously perceived.  The loss of
nerve cells may be due to blockage in blood circulation caused by a blood
clot, or by an abnormal particle such as an air bubble circulating in the
blood (embolus).  It may also be due to a small lesion or a tumor in the
thalamus.

When one side of the thalamus is damaged, some or all of the opposite
side of the body (face, arm, leg) may be abnormally sensitive to all types of
stimuli such as touch (contralateral hypersensitivity).  This may cause pain
in the areas where there is a loss of sensation (anesthesia dolorosa).

Affected Population

Thalamic Syndrome (Dejerine-Roussy) is a rare disorder that affects males and
females in equal numbers.

Related Disorders

Reflex Sympathetic Dystrophy Syndrome (RSDS) is a term encompassing a
group of chronic pain syndromes.  Symptoms include severe pain and
alternating constriction and dilation of blood vessels after trauma, often
minor in nature.  Other cases of RSDS can begin without apparent cause.
Symptoms can become chronic if treatment is not begun as soon as possible
after diagnosis.  However, diagnosis and treatment are difficult due to the
wide variety of body areas which can be affected.  Also, RSDS can easily be
misdiagnosed as a nerve injury which is characterized by similar painful
symptoms.  (For more information on this disorder, choose "RSDS" as your
search term in the Rare Disease Database).

Guillain-Barre Syndrome occurs when the body's defense system against
disease (e.g., antibodies or lymphocytes) attacks the nerves, damaging the
nerve's myelin and axon.  Nerve signals are delayed and altered, causing
weakness and paralysis of the muscles of the legs, arms, and other parts of
the body along with abnormal sensations.  (For more information on this
disorder, choose "Guillain-Barre" as your search term in the Rare Disease
Database).

Major symptoms of Carpal Tunnel Syndrome include a sensation of numbness,
tingling, burning and/or slight pain in the hand and wrist.  This sensation
can be temporary at first, later becoming chronic.  It can cause patients to
awaken during the night.  Left untreated, muscle atrophy in the hand may
develop.  Symptoms may become worse with activities that require wrist
flexing or prolonged gripping such as hammering or driving for long periods
of time.  Carpal Tunnel Syndrome is a very prevalent disorder that can be
treated through weight loss, hand splints or surgery.  (For more information
on this disorder, choose "Carpal Tunnel" as your search term in the Rare
Disease Database).

Therapies:  Standard

In Thalamic Syndrome (Dejerine-Roussy) surgical lesions can interrupt the
sensory pathway of the brain and may help decrease the pain without affecting
sensory ability.  Patients may be able to experience touch without feeling
pain or discomfort after surgical treatment.

Therapies:  Investigational

This disease entry is based upon medical information available through
November 1990.  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 Thalamic Syndrome (Dejerine-Roussy Syndrome), please
contact:

     National Organization for Rare Disorders
     P.O. Box 8923
     New Fairfield, CT 06812-1783
     [phone]

     NIH/National Institute of Neurological Disorders & Stroke (NINDS)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]
     [phone]

     International Tremor Foundation
     360 W. Superior St.
     Chicago, IL  60610
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 18th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1988.  Pp. 2128-2129.

BRAIN GLUCOSE METABOLISM IN THALAMIC SYNDROME.  E. C. Laterre, et al.; J
Neurol Neurosurg Psychiatry (March 1988; issue 51 (3)).  Pp. 427-428.

THALAMIC PAIN SYNDROME OF DEJERINE-ROUSSY.  F.  Mauguiere and J. E
Desmedt; Arch Neurol (December 1988; issue 45 (12)).  Pp. 1312-1320.