Brown-Séquard syndrome

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Definition

Brown-Séquard syndrome (BSS), also known as hemisection of the spinal cord or partial spinal sensory syndrome, is a rare condition caused by an incomplete lesion of the spinal cord. This damage, most often from physical trauma, results in a contralateral (opposite side of the body) loss of sensation and temperature and ipsilateral (same side of the body) paralysis or extreme weakness.

Description

In 1849, French physiologist Charles Edouard Brown-Séquard published a document discussing the condition that now bears his name. Using information gathered through animal experimentation and human autopsies, he identified and described the hallmark signs of BSS: paralysis affecting only one side of the body (ipsilateral paralysis) and loss of sensation on the opposite side of the body.

Injury or damage to one side of the spinal cord, typically in the cervical (neck) region, results in BSS. The severity of the condition depends on the amount of damage to the spinal cord and associated neurons. The onset of symptoms may also vary depending on the cause.

Demographics

Information on the prevalence of Brown-Séquard syndrome is collected from 16 spinal cord injury centers in the United States. According to The University of Alabama's National Spinal Cord Injury Statistical Center (NSCISC), which compiles the data, approximately 11,000 spinal cord injuries (SCIs) occur each year (as of 2003). Although specific incidence is unknown, BSS is estimated to occur in 200–400 of these injuries.

The average age of a patient sustaining a spinal cord injury is 32 years, with injuries most commonly occurring in individuals between 16 and 30 years. Men account for more than 80% of reported SCIs.

Within the United States, approximately 70% of individuals with BSS are white, nearly 20% are African American, and the remaining 10% comprise other origins, according to NSCISC reports. Little data is known regarding SCIs in countries outside the United States.

Causes and symptoms

In most cases, Brown-Séquard syndrome is caused by severe physical trauma such as a puncture wound or gunshot wound, which partially severs or damages the spinal cord. Nontraumatic conditions that compress the spinal cord may also cause BSS. Examples include tumors, multiple sclerosis, epidural hematoma (swelling in the area between the brain and skull), meningitis, myelitis (spinal cord inflammation), and tuberculosis.

Physical trauma usually causes a more rapid onset of symptoms than nontraumatic conditions. The two primary symptoms of BSS are loss of sensation and paralysis. The side of the body that sustained injury typically loses touch and vibration senses. The opposite side of the body tends to lose its sense of pain and temperature. In both cases, these symptoms occur below the site of the SCI. Paralysis or muscle weakness occurs on the same side of the body as the injury.

Loss of bladder and bowel control may result, but the majority of patients will regain control. Horner syndrome, a condition resulting from damage to the sympathetic facial nerves, has also been known to develop.

Diagnosis

Brown-Séquard syndrome is diagnosed based on the patient's medical history and a physical examination. Imaging studies may be performed to isolate the extent and location of the SCI. These include magnetic resonance imaging (MRI), computed tomography (CT) scans, or x rays. Additional testing may be required for secondary conditions or symptoms.

Several neurological disorders have symptoms similar to BSS, making differential diagnosis very important, especially in those cases related to nontraumatic conditions. The incomplete lesion of the spinal cord in conjunction with the unique presentation of ipsilateral sensory loss and paralysis are key for identifying BSS.

Treatment team

The team of specialists needed to treat a patient with BSS will vary. Primary members include:

• a neurologist to evaluate brain and nerve function
• an orthopedic specialist to monitor the spine and assist with walking therapy
• a physical therapist to help regain muscle strength and walking ability
• an occupational therapist to facilitate adaptation of new physical limitations

Treatment

In cases of physical trauma, treatment begins at the accident site with proper immobilization and emergency medical care to prevent further spinal cord damage. Surgery may be required in these or nontraumatic cases to eliminate the cause, whether a bullet or a fluid-filled cyst.

Treatment of symptoms is the typical focus for this condition. Several studies have shown increased success with early administration of high-dose steroids such as corticosteroids, but this is not yet a standard practice. Other medications are prescribed as needed for secondary symptoms.

Physical therapy should begin immediately in order to maintain muscle strength and agility since most patients with BSS will regain mobility. Specialized devices, including wheelchairs or braces, may be necessary during this transition.

Recovery and rehabilitation

The recovery time for each patient depends on the extent of nerve damage and underlying cause of the syndrome. The NSCISC reports that individuals with SCIs spend an average of 16 days in the hospital and 44 days in rehabilitation. Rehabilitation may be required outside the hospital for several months or years.

Extensive physical therapy should take place immediately. Initial therapy focuses on respiratory exercises, upright positioning, and range of motion in affected muscles. Progressive therapy gradually helps the patient with the strength and control necessary to be mobile or begin walking again.

Occupational therapy is also important for helping patients return to their daily activities. This therapist provides methods for modifying everyday tasks, evaluates progress, and facilitates the necessary changes to restore independence when possible.

Clinical trials

The National Institute of Child Health and Human Development is currently conducting a clinical trial to evaluate the effectiveness of walking on a treadmill by individuals with incomplete SCIs. As of early 2004, this five-year study was in Phase II and III clinical trials and still recruiting patients. The proposed end date for the study is January 2005. For additional information contact: Andrea L. Behrman, PhD (Principal Investigator), University of Florida, "Retraining Walking after Spinal Cord Injury" (Study ID: K01HD01348); Telephone: (352) 273-6117; E-mail: abehrman@hp.ufl.edu.

Prognosis

Patients with Brown-Séquard syndrome usually have a good prognosis. The extent to which a patient recovers depends on the cause of injury and secondary conditions or complications. According to the National Organization for Rare Disorders, more than 90% of affected individuals successfully regain the ability to walk. Additional studies have found that the majority of a patient's motor skills return within the first two months after injury. The recovery period is usually two years, but will vary by patient.

Special concerns

Not all patients with BSS make a full recovery. In these instances, long-term care options need to be considered. By working with the treatment team, individuals can determine their level of activity and recognize areas where adaptation may be required. Some patients and their caregivers could benefit from psychological therapy to discuss the variety of changes that occur after traumatic injury.

Resources

BOOKS

The Official Patient's Sourcebook on Brown-Séquard Syndrome: A Revised and Updated Directory for the Internet Age. San Diego: Icon Health Publications, 2002.

PERIODICALS

Bateman, D. E., and I. Pople. "Brown-Séquard at Disney World." The Lancet 352, no. 9144 (December 12, 1998): 1902.

Lim, E., Y. S. Wong, Y. L. Lo, et al. "Traumatic Atypical Brown-Séquard Syndrome: Case Report and Literature Review." Clinical Neurology and Neurosurgy 105 (2003): 143–45.

Pollard, Matthew E., and David F. Apple. "Factors Associated with Improved Neurologic Outcomes in Patients with Incomplete Tetraplegia." Spine 28, no. 1 (January 1, 2003): 33–39.

Tattersall, Robert, and Benjamine Turner. "Brown-Séquard and His Syndrome." The Lancet 356, no. 9223 (July 1, 2000): 61.

WEBSITES

Beeson, Michael S, and Scott Wilber. "Brown-Séquard Syndrome." eMedicine. July 30, 2003 (May 20, 2004). http://www.emedicine.com/pmr/topic70.htm.

"Retraining Walking after Spinal Cord Injury." ClinicalTrials.gov. March 19, 2004 (May 20, 2004). http://www.clinicaltrails.gov/ct/show/NCT00059553?order=1.

Vandenakker, Carol. "Brown-Séquard Syndrome." eMedicine. July 29, 2002 (May 20, 2004). http://www.emedicine.com/pmr/topic17.htm.

ORGANIZATIONS

Christopher Reeve Paralysis Foundation. 500 Morris Avenue, Springfield, NJ 07081. (800) 225-0292. info@paralysis.org. http://www.christopherreeve.org.

National Organization for Rare Disorders. 55 Kenosia Avenue, P.O. Box 1968, Danbury, CT 06813. (203) 744-0100 or (800) 999-6673; Fax: (203) 798-2291. orphan@rarediseases.org. http://www.rarediseases.org.

National Spinal Cord Injury Association. 6701 Democracy Blvd. #300-9, Bethesda, MD 20817. (301) 214-4006 or (800) 962-9629; Fax: (301) 881-9817. info@spinalcord.org. http://www.spinalcord.org.

Stacey L. Chamberlin

A syndrome caused by damage to one side of the spinal cord and resulting in ipsilateral hemiparaplegia and loss of muscle and joint sensation and contralateral hemianesthesia. Also called Brown-Séquard's paralysis.

Paralysis and loss of discriminatory and joint sensation on one side of the body and of pain and temperature sensation on the other, due to a lesion involving one side of the spinal cord. Seen in primates but not so evident in domestic animals because of bilateral spinal sensory afferent pathways.

Brown-Séquard syndrome
Classification and external resources
Brown-Séquard syndrome is bottom diagram
ICD-10	G83.8
ICD-9	344.89
DiseasesDB	31117
eMedicine	emerg/70  pmr/17
MeSH	D018437

Brown-Séquard syndrome, also known as Brown-Séquard's hemiplegia and Brown-Séquard's paralysis, is a loss of sensation and motor function (paralysis and ataxia) that is caused by the lateral hemisection (cutting) of the spinal cord. Other synonyms are crossed hemiplegia, hemiparaplegic syndrome, hemiplegia et hemiparaplegia spinalis and spinal hemiparaplegia.

Contents 1 Classification 2 Diagnosis 3 Causes 4 Pathophysiology 5 Treatment 6 Epidemiology 7 History 8 References 9 External links

Classification

Any presentation of spinal injury that is an incomplete lesion can be called a partial Brown-Séquard or incomplete Brown-Séquard syndrome, so long as it has characterized by features of a motor loss on the same side of the spinal injury and loss of pain and temperature sensation on the opposite side.

Diagnosis

Magnetic resonance imaging (MRI) is the imaging of choice in spinal cord lesions.

Causes

Brown-Séquard syndrome may be caused by a spinal cord tumor, trauma (such as a gunshot wound or puncture wound to the neck or back), ischemia (obstruction of a blood vessel), or infectious or inflammatory diseases such as tuberculosis, or multiple sclerosis.

Brown-Séquard syndrome is an incomplete spinal cord lesion characterized by clinical presentation reflecting hemisection of the spinal cord (cutting the spinal cord in half on one or the other side). It is diagnosed by finding motor (muscle) paralysis on the same side as the lesion and deficits in pain and temperature sensation on the opposite side on physical exam. This is called ipsilateral (on the same side as the spinal cord lesion) hemiplegia and contralateral (on the opposite side) pain and temperature sensation deficits. The loss of sensation on the opposite side of the lesion is because these nerve fibers of the spinothalamic tract cross the spinal cord. In its pure form, it is rarely seen. Incomplete forms are also observed. The most common cause is penetrating trauma such as a gunshot wound or stab wound to the spinal cord. This may be seen most often in the cervical (neck) or thoracic spine. Other causes are tumors, bleeding episodes, tuberculosis, and multiple sclerosis.

The presentation can be progressive and incomplete. It can advance from a typical Brown-Séquard syndrome to complete paralysis. It is not always permanent, and progression or resolution depends on the severity of the original spinal cord injury and the underlying pathology that caused it in the first place.

Pathophysiology

The hemisection of the cord results in a lesion of each of the three main neural systems:

• the principal upper motor neuron pathway of the corticospinal tract
• one or both dorsal columns
• the spinothalamic tract

As a result of the injury to these three main brain pathways the patient will present with three lesions:

• The corticospinal lesion produces spastic paralysis on the same side of the body (the loss of moderation by the UMN).
• The lesion to fasciculus gracilis or fasciculus cuneatus results in ipsilateral loss of vibration and proprioception (position sense).
• The loss of the spinothalamic tract leads to pain and temperature sensation being lost from the contralateral side beginning one or two segments below the lesion.

Treatment

Treatment is directed at the pathology causing the paralysis. If it is because of trauma such as a gunshot or knife wound, there may be other life threatening conditions such as bleeding or major organ damage which should be dealt with on an emergent basis. If the syndrome is caused by a spinal fracture, this should be identified and treated appropriately. Although steroids may be used to decrease cord swelling and inflammation, the usual therapy for spinal cord injury is expectant.^{[1][2][3][4][5][6][7][8][9][10][11][12][13][14]}

Epidemiology

Brown-Séquard syndrome is rare.^[15]

History

The syndrome was first described in 1850 by the famed British / Mauritian neurologist Charles-Édouard Brown-Séquard (1817-1896), who studied the anatomy and physiology of the spinal cord. ^[16][17] Brown-Séquard was quite a controversial and eccentric figure, and is also known for self-reporting "rejuvenated sexual prowess after eating extracts of monkey testis". The response is now thought to have been a placebo effect, but apparently this was "sufficient to set the field of endocrinology off and running."^[18]

Interestingly, many nations claim him as their own, he was the son of an American sea captain and a French woman. He was born in Mauritius. He studied in the US and France and worked several years in the UK, US and France. He described this injury after observing spinal cord trauma happen to farmers while cutting sugar cane in Mauritius.

References

1. ^ Egido Herrero JA, Saldanã C, Jiménez A, Vázquez A, Varela de Seijas E, Mata P (1992). "Spontaneous cervical epidural hematoma with Brown-Séquard syndrome and spontaneous resolution. Case report". J Neurosurg Sci 36 (2): 117–9. PMID 1469473. 
2. ^ Ellger T, Schul C, Heindel W, Evers S, Ringelstein EB (June 2006). "Idiopathic spinal cord herniation causing progressive Brown-Séquard syndrome". Clin Neurol Neurosurg 108 (4): 388–91. doi:10.1016/j.clineuro.2004.07.005. PMID 16483712. 
3. ^ Finelli PF, Leopold N, Tarras S (May 1992). "Brown-Sequard syndrome and herniated cervical disc". Spine 17 (5): 598–600. PMID 1621163. 
4. ^ Hancock JB, Field EM, Gadam R (1997). "Spinal epidural hematoma progressing to Brown-Sequard syndrome: report of a case". J Emerg Med 15 (3): 309–12. doi:10.1016/S0736-4679(97)00010-3. PMID 9258779. 
5. ^ Harris P (November 2005). "Stab wound of the back causing an acute subdural haematoma and a Brown-Sequard neurological syndrome". Spinal Cord 43 (11): 678–9. doi:10.1038/sj.sc.3101765. PMID 15852056. 
6. ^ Henderson SO, Hoffner RJ (1998). "Brown-Sequard syndrome due to isolated blunt trauma". J Emerg Med 16 (6): 847–50. doi:10.1016/S0736-4679(98)00096-1. PMID 9848698. 
7. ^ Hwang W, Ralph J, Marco E, Hemphill JC (June 2003). "Incomplete Brown-Séquard syndrome after methamphetamine injection into the neck". Neurology 60 (12): 2015–6. PMID 12821761. http://www.neurology.org/cgi/pmidlookup?view=long&pmid=12821761. 
8. ^ Kraus JA, Stüper BK, Berlit P (1998). "Multiple sclerosis presenting with a Brown-Séquard syndrome". J. Neurol. Sci. 156 (1): 112–3. doi:10.1016/S0022-510X(98)00016-1. PMID 9559998. 
9. ^ Lim E, Wong YS, Lo YL, Lim SH (April 2003). "Traumatic atypical Brown-Sequard syndrome: case report and literature review". Clin Neurol Neurosurg 105 (2): 143–5. doi:10.1016/S0303-8467(03)00009-X. PMID 12691810. 
10. ^ Lipper MH, Goldstein JH, Do HM (August 1998). "Brown-Séquard syndrome of the cervical spinal cord after chiropractic manipulation". AJNR Am J Neuroradiol 19 (7): 1349–52. PMID 9726481. http://www.ajnr.org/cgi/pmidlookup?view=long&pmid=9726481. 
11. ^ Mastronardi L, Ruggeri A (January 2004). "Cervical disc herniation producing Brown-Sequard syndrome: case report". Spine 29 (2): E28–31. doi:10.1097/01.BRS.0000105984.62308.F6. PMID 14722422. 
12. ^ Miyake S, Tamaki N, Nagashima T, Kurata H, Eguchi T, Kimura H (February 1998). "Idiopathic spinal cord herniation. Report of two cases and review of the literature". J. Neurosurg. 88 (2): 331–5. PMID 9452246. 
13. ^ Rumana CS, Baskin DS (April 1996). "Brown-Sequard syndrome produced by cervical disc herniation: case report and literature review". Surg Neurol 45 (4): 359–61. doi:10.1016/0090-3019(95)00412-2. PMID 8607086. 
14. ^ Stephen AB, Stevens K, Craigen MA, Kerslake RW (October 1997). "Brown-Séquard syndrome due to traumatic brachial plexus root avulsion". Injury 28 (8): 557–8. doi:10.1016/S0020-1383(97)83474-2. PMID 9616398. 
15. ^ "Brown-Sequard Syndrome: Overview - eMedicine Emergency Medicine". http://emedicine.medscape.com/article/791539-overview. 
16. ^ synd/973 at Who Named It?
17. ^ C.-É. Brown-Séquard: De la transmission croisée des impressions sensitives par la moelle épinière. Comptes rendus de la Société de biologie, (1850)1851, 2: 33-44.
18. ^ The Practice of Neuroscience, p. 199-200, John C.M. Brust (2000).

External links

• Case studies of Brown-Séquard syndrome
• Image

v • d • e Lesions of spinal cord and brain Spinal cord/ vascular myelopathy sensory: Sensory ataxia - Tabes dorsalis motor: Motor neurone disease mixed: Brown-Séquard syndrome - cord syndrome (Posterior, Anterior, Central/Syringomyelia) - Subacute combined degeneration of spinal cord (B12) - Cauda equina syndrome Anterior spinal artery syndrome Brainstem Medulla (CN 8, 9, 10, 12) Lateral medullary syndrome/Wallenberg (PICA) - Medial medullary syndrome (ASA) Pons (CN 5, 6, 7, 8) Lateral pontine syndrome (AICA) - Millard-Gubler syndrome (lateral) - Medial pontine syndrome (basilar) - Foville's syndrome (dorsal, basilar) - Locked-In syndrome (ventral) - Internuclear ophthalmoplegia - One and a half syndrome Midbrain (CN 3, 4) Weber's syndrome (ventral peduncle, PCA) - Benedikt's syndrome (ventral tegmentum, PCA) - Parinaud's syndrome (dorsal, tumor) Other Alternating hemiplegia Cerebellum lateral (Dysmetria, Dysdiadochokinesia, Intention tremor) - medial (Cerebellar ataxia) Basal ganglia Chorea - Dystonia - Parkinson's disease Cortex ACA syndrome - MCA syndrome - PCA syndrome frontal lobe: Expressive aphasia - Abulia parietal lobe: Receptive aphasia - Hemispatial neglect - Gerstmann syndrome - Astereognosis occipital lobe: Balint's syndrome - Cortical blindness - Alexia without agraphia temporal lobe: Cortical deafness - Prosopagnosia Other Subclavian steal syndrome - Upper motor neurone lesion (Clasp-knife response) - Lower motor neurone lesion

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