$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.