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$Unique_ID{BRK03544}
$Pretitle{}
$Title{Brown-Sequard Syndrome}
$Subject{Brown-Sequard Syndrome BSS Partial Spinal Sensory Syndrome
Hemisection of the Spinal Cord Motor Neuron Disease Progressive Spinal
Muscular Atrophy Primary Lateral Sclerosis Stroke}
$Volume{}
$Log{}
Copyright (C) 1993 National Organization for Rare Disorders, Inc.
950:
Brown-Sequard Syndrome
** IMPORTANT **
It is possible that the main title of the article (Brown-Sequard
Syndrome) is not the name you expected. Please check the SYNONYM listing to
find the alternate names and disorder subdivisions covered by this article.
Synonyms
BSS
Partial Spinal Sensory Syndrome
Hemisection of the Spinal Cord
Information on the following diseases can be found in the Related
Disorders section of this report:
Motor Neuron Disease
Progressive Spinal Muscular Atrophy
Primary Lateral Sclerosis
Stroke
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.
Brown-Sequard Syndrome is a rare spinal motor disease that affects one
side of the spinal cord. It is usually caused by an injury to the neck or
spinal area. Often the patient has received some type of puncture wound to
the neck or back which caused symptoms to appear.
Symptoms
Symptoms of Brown-Sequard Syndrome usually appear after the patient
experienced a trauma to the neck or back. First symptoms are usually loss of
the sensations of pain and temperature often below the area of the trauma.
There may also be loss of bladder and bowel function. Paralysis on the same
side as that of the wound often occurs. Paralysis may be permanent if
diagnosis is delayed.
Causes
The exact cause of Brown-Sequard Syndrome is often unknown. The symptoms
usually result from an injury by a knife or gunshot to the spine or neck. In
some cases, however, the syndrome has been caused by viral or bacterial
disease. Often spinal disorders such as cervical spondylosis, arachnoid cyst
or epidural hematoma may also be causes of the Brown-Sequard Syndrome. Blunt
trauma, such as occurs in a fall or automobile accident, may also rarely
cause the Brown-Sequard Syndrome disorder.
Affected Population
Brown-Sequard Syndrome is a vary rare disorder that affects males and females
in equal numbers. Approximately five hundred cases have been reported to
date.
Related Disorders
Symptoms of the following disorders can be similar to those of Brown-Sequard
Syndrome. Comparisons may be useful for a differential diagnosis:
Motor Neuron Disease is a degeneration of motor neurons. Motor neurons
control the behavior of muscles. Motor Neuron Disease may affect the upper
motor neurons, which control the signals from the brain to the spinal cord,
or the lower motor neurons which lead from the spinal cord to the muscles of
the body. (For more information on this disorder, choose "Motor Neuron
Disease" as your search term in the Rare Disease Database).
Progressive Spinal Muscular Atrophy is a slowly progressive Motor Neuron
Disease. Muscle weakness and wasting may begin in the hands and eventually
affect the arms, shoulders, legs, and the rest of the body. Muscle twitching
may occur in the limbs and tongue. (For more information on this disorder,
choose "Motor Neuron Disease" as your search term in the Rare Disease
Database).
Primary Lateral Sclerosis affects adults. It is characterized by
progressive degeneration of the upper motor neurons. Difficulty in speech
and swallowing, semi or complete paralysis of the legs and/or arms, and
muscle twitching and spasticity may occur. (For more information on this
disorder, choose "Motor Neuron Disease" as your search term in the Rare
Disease Database).
Stroke is one of the most common neurological conditions affecting the
central nervous system. Stroke is caused by a blockage of blood flow to part
of the brain. The may happen because of a blood clot or because of the
bursting of an aneurysm in the brain.
Therapies: Standard
Treatment of Brown-Sequard Syndrome may involve drugs that control muscle
symptoms. Baclofen is often prescribed to relieve spasticity, quinine for
cramping, diazepam for muscular contractions, and pyridostigmine to improve
nerve-to-muscle message transmission.
Devices that help the patient continue daily activities such as braces,
hand splits, limb supports, or a wheelchair are important. Various other aids
may be necessary if the patient has difficulty breathing or swallowing. Other
treatment is symptomatic and supportive.
Therapies: Investigational
Scientists are conducting extensive ongoing research on Motor Neuron Disease
in the areas of nerve growth factors, axonal transport, and androgen
receptors in motor neurons. DNA/RNA changes, and metabolic studies of the
neuromuscular junction are also ongoing. Several drug studies are underway
to learn if pharmaceuticals may slow the progression of motor neuron
disorders. Regeneron Pharmaceuticals is testing an orphan product for the
treatment of motor neuron diseases. The chemical name is human ciliary
neurotrophic factor, recombinant.
The orphan drug Insulin-Like Growth Factor-1 (Myotrophin) is being
investigated as a treatment for patients with Motor Neuron Diseases. The
drug is made by Cephalon, Inc.
More study is needed to determine the longterm safety and effectiveness
of these drugs as treatments for this type of disorder.
This disease entry is based upon medical information available through
June 1993. 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 Brown-Sequard Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812
(203) 746-6518
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
Amyotrophic Lateral Sclerosis Association
21021 Ventura Blvd., Suite 321
Woodland Hills, CA 91364
(800) 782-4747
(818) 990-2151
For information about Motor Neuron Disease that occurs during childhood:
Families of Spinal Muscular Atrophy
P.O. Box 1465
Highland Park, IL 60035
(708) 432-5551
References
PRINCIPLES OF NEUROLOGY, 4th Ed.; Raymond D. Adams, M.D. and Maurice Victor,
M.D., Editors; McGraw-Hill Information Services Company, 1989. Pp. 50, 129-
30, 722, 740, 743.
BLUNT CERVICAL SPINE BROWN-SEQUARD INJURY. A REPORT OF THREE CASES.,
D.W. Oller, et al.; Am Surg, June, 1991, (issue 57 (6)). Pp. 361-365.
AN UNUSUAL PRESENTATION OF BROWN-SEQUARD SYNDROME., R.D. Herr, et al.;
Ann Emerg Med, November, 1987, (issue 16 (11)). Pp. 1285-1288.
BROWN-SEQUARD SYNDROME FOLLOWING CERVICAL SPINE COMPRESSION FRACTURE.,
P.J. Zorn, Del Med J, September, 1991, (issue (9)). Pp. 549-553.
BROWN SEQUARD SYNDROME CAUSED BY BORRELIA BURGDORFERI., P. Berlit, et
al.; Eur Neurol, 1991, (issue 31 (1)). Pp. 18-20.
BROWN-SEQUARD SYNDROME ASSOCIATED WITH POSTTRAUMATIC CERVICAL EPIDURAL
HEMATOMA: CASE REPORT AND REVIEW OF THE LITERATURE., G.M. Zupruk, et al.;
Neurosurgery, August, 1989, (issue 25 (2)). Pp. 278-280.