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Brown Séquard Syndrome

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 1993, 2003, 2005

Synonyms of Brown Séquard Syndrome

Disorder Subdivisions

General Discussion

Brown-Séquard syndrome is a rare spinal disorder that results from an injury to one side of the spinal cord in which the spinal cord is damaged but is not severed completely. It is usually caused by an injury to the spine in the region of the neck or back. In many cases, affected individuals have received some type of puncture wound in the neck or in the back that damages the spine and causes symptoms to appear.

Characteristically, the affected person loses the sense of touch, vibrations and/or position in three dimensions below the level of the injury (hemiparalysis or asymmetric paresis). The sensory loss is particularly strong on the same side (ipsilateral) as the injury to the spine. These sensations are accompanied by a loss of the sense of pain and of temperature (hypalgesia) on the side of the body opposite (contralateral) to the side at which the injury was sustained.

Symptoms

Symptoms of Brown-Séquard syndrome usually appear after an affected individual experiences 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 control. Weakness and degeneration (atrophy) of muscles in the affected area may occur. Paralysis on the same side as that of the wound often occurs. Paralysis may be permanent if diagnosis is delayed.

Individuals with this syndrome have a good chance of recovering a large measure of function. More than 90% of affected individuals recover bladder and bowel control, and the ability to walk. Most affected individuals regain some strength in their legs and most will regain functional walking ability.

Causes

This syndrome is often a consequence of a traumatic injury by a knife or gunshot to the spine or neck. In many cases, however, it is caused by, or is the result of, other spinal disorders such as cervical spondylosis, arachnoid cyst or epidural hematomas. Brown-Séquard syndrome may also accompany bacterial or viral infections. Blunt traumas, such as occur in a fall or automobile accident, on rare occasions may be the cause of the Brown-Séquard syndrome.

The medical literature cites, as causing or being associated with BSS, the following conditions: lateral curvature of the spine (kyphosis), Chiari I malformation, methamphetamine injection in the neck, multiple sclerosis, spinal epidural hematoma, intramedullary spinal cord tumor, and myeloschisis. Among the infectious or inflammatory causes cited are: meningitis, empyema, herpes zoster, herpes simplex, myelitis, and tuberculosis.

Affected Populations

Brown-Séquard syndrome is a rare disorder that affects males and females in equal numbers. More than 500 cases have been reported to date. The incidence of Brown-Séquard syndrome has been estimated to be 2% of all traumatic spinal cord injuries. The annual incidence of all forms of spinal cord injury is estimated to be 30-40 per 1,000,000 people.

Related Disorders

Symptoms of the following disorders can be similar to those of Brown-Séquard 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.

Standard Therapies

There is no specific treatment for individuals with Brown-Séquard syndrome. In most instances, treatment focuses on the underlying cause of the disorder. Treatment may involve drugs that control muscle symptoms, and there is some dispute as to whether high-dose steroid administration is effective.

Devices that help an affected individual 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.

Investigational Therapies

The National Institute of Child Health and Human Development (NICHD) is sponsoring a Phase II and Phase III clinical trial designed to improve walking ability after spinal cord injury. Patients with Brown-Sequard syndrome may be eligible to participate in the study.

It is well known that incomplete spinal cord injury often makes walking very difficult. A group of physical therapists are trying to determine the effect of treadmill speed on spinal cord function and walking performance.

Recently, evidence has been building to contradict the conventional wisdom that recovery of nerve function following spinal cord injury was not possible. It has been shown that nerve circuits (neuronal circuits) may reorganize by strengthening previously inactive connections and circuits. Sensory information related to movement is used to improve treadmill and overground walking.

About 16 persons will participate in the study, which is based at the University of Florida in Gainesville.

For further information contact:
Andea L. Behrman, PhD
Principal Investigator
University of Florida
Tel: 352-273-6117
e-mail: abehrman@hp.ufl.edu or

Michelle L. Woodbury, OTR, MA
e-mail: mwoodbury@hp.ufl.edu
Study ID number is: K01HD01348

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov.

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Brown Séquard Syndrome Resources

Organizations:

References

TEXTBOOKS
Winchester P. Brown-Séquard Syndrome. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:520.

Rowland LP, ed. Merritt's Neurology. 10th ed. Lippincott Williams & Wilkins. Philadelphia, PA. 2000:416-23.

Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:2382.

Adams, RD, et al., eds. Principles of Neurology. 6th ed. New York, NY: McGraw-Hill, Companies; 1997:162.

REVIEW ARTICLES
Iyer RV, Coutinho C, Lye RH. Spontaneous spinal cord herniation. Br J Neurosurg. 2002;16:507-10.

Massicotte EM, Montanera W, Ross Fleming JF, et al. Idiopathic spinal cord herniation: report of eight cases and review of the literature. Soine. 2002;27:E233-41.

Antich PA, Sanjuan AC, Girvent FM, et al. High cervical disc herniation and Brown-Séquard syndrome. A case report and review of the literature. J Bone Joint Surg Br. 1999;81:462-63.

JOURNAL ARTICLES
Abe M. Incomplete Brown-Séquard syndrome caused by cervical kyphosis secondary to neurofibromatosis: report of a case. J Orthop Sci. 2003;8:602-06.

Lim E, Wong YS, Lo YL, et al. Traumatic atypical Brown-Séquard syndrome: case report and literature review. Clin Neurol Neurosurg. 2003;105:143-45.

Pollard ME, Apple DF. Factors associated with improved neurologic outcomes in patients with incomplete tetraplegia. Spine. 2003;28:33-39.

Cellerini M, Bayon S, Scazzeri F, et al. Idiopathic spinal cord herniation: a treatable cause of Brown-Séquard syndrome. Acta Neurochir (Wien). 2002;144:321-25.

Sakakibara R, Hattori T, Uchiyama T, et al. Urinary dysfunction in Brown-Séquard syndrome. Neurourol Urodyn. 2001;20:661-67.

McCarron MO, Flynn PA, Pang KA, et al. Traumatic Brown-Séquard-plus syndrome. Arch Neurol. 2001;58:1470-72.

FROM THE INTERNET
NINDS Brown-Séquard Syndrome Information Page. Reviewed 2-25-2003. 2pp.
www.nih.gov/health_and_medical/disorders/brown-sequard.htm?format=printable

NINDS Spinal Cord Injury Information Page. Reviewed 7-01-2001.
www.nih.gov/health_and_medical/disorders/sci.htm?format=printable

Beeson MS. Brown-Séquard Syndrome. emedicine. Last Updated: July 30, 2003. 7pp
www.emedicine.com/emerg/topic70.htm

Wheeless' Textbook of Orthopaedics. Brown-Séquard syndrome. nd.
www.ortho-u.net/o11/979.htm - Brown-Sequard syndrome: 1p.
www.ortho-u.net/o11/973.htm - Incomplete Spinal Cord Lesion: 2pp.
www.ortho-u.net/o6/181.htm - Primary Spinal Cord Neoplasia: 1p.

Brown-Séquard Syndrome. Med Help International. nd. 2pp.
www.medhelp.org/perl6/neuro/archive/7996.html

Report last updated: 2008/04/25 00:00:00 GMT+0