$Unique_ID{BRK03793} $Pretitle{} $Title{Guillain-Barre Syndrome} $Subject{Guillain-Barre Syndrome Acute Idiopathic Polyneuritis Acute Immune-Mediation Polyneuritis AIMP Ascending Paralysis Post-Infective Polyneuritis Kussmaul-Landry Paralysis also known as Landry Paralysis GBS Landry Ascending Paralysis Miller-Fischer Syndrome also known as Fischer's Syndrome and Acute Disseminated Encephalomyeloradiculopathy Chronic Guillain-Barre Syndrome also known as Chronic Idiopathic Polyneuritis or CIP, Relapsing Guillain-Barre Syndrome, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Chronic Relapsing Polyneuropathy, Polyneuropathy and Polyradiculoneuropathy Neuropathy, Peripheral Fischer Syndrome Chronic Idiopathic Polyneuritis} $Volume{} $Log{} Copyright (C) 1985, 1987, 1988, 1990, 1992 National Organization for Rare Disorders, Inc. 40: Guillain-Barre Syndrome ** IMPORTANT ** It is possible that the main title of the article (Guillain-Barre Syndrome) is not the name you expected. Please check the SYNONYMS listing to find the alternate name and disorder subdivisions covered by this article. Synonyms Acute Idiopathic Polyneuritis Acute Immune-Mediation Polyneuritis AIMP Ascending Paralysis Post-Infective Polyneuritis Kussmaul-Landry Paralysis also known as Landry Paralysis GBS Landry Ascending Paralysis Disorder Subdivisions: Miller-Fischer Syndrome also known as Fischer's Syndrome and Acute Disseminated Encephalomyeloradiculopathy Chronic Guillain-Barre Syndrome also known as Chronic Idiopathic Polyneuritis or CIP, Relapsing Guillain-Barre Syndrome, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, Chronic Relapsing Polyneuropathy, Polyneuropathy and Polyradiculoneuropathy Information on the following diseases can be found in the Related Disorders section of this report: Neuropathy, Peripheral Fischer Syndrome Chronic Idiopathic Polyneuritis 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. Guillain-Barre Syndrome (acute idiopathic polyneuritis) is a very rare, rapidly progressive disorder causing inflammation of the nerves (polyneuritis) and paralysis. Although the precise cause of Guillain-Barre Syndrome is unknown, a viral or respiratory infection precedes the onset of the syndrome in about half of the cases. This has led to the theory that Guillain-Barre Syndrome may be an autoimmune disease (caused by the body's own immune system). Damage to the covering of nerve cells (myelin) and nerve axons (the extension of the nerve cell that conducts impulses away from the nerve cell body) results in delayed nerve signal transmission. There is a corresponding weakness in the muscles that are supplied with nerve impulses (innervated) by the affected nerves. The following subdivisions are recognized: Miller-Fischer syndrome (Fischer syndrome, acute disseminated encephalomyeloradiculopathy); chronic Guillain-Barre Syndrome (chronic idiopathic polyneuritis); relapsing Guillain-Barre Syndrome; chronic inflammatory demyelinating polyradiculoneuropathy; chronic relapsing polyneuropathy; polyneuropathy; and polyradiculoneuropathy. Symptoms Guillain-Barre Syndrome is a very rare, rapidly progressive disorder that affects the nervous system. This disorder is characterized by ascending polyneuritis (inflammation of nerves) causing paralysis that usually begins in the feet and progresses upwards to the trunk and arms. Numbness, tingling and/or "pins and needles" (paresthesias) begin in the feet. This is generally followed by weakness and paralysis of the legs. Eventually the torso, upper limbs, and face are affected. The symptoms of Guillain-Barre Syndrome progress over hours to weeks. Deep tendon reflexes may be lost; most notably the ankle jerk reflex. Other symptoms may include fever, paralysis of the lips, tongue, mouth and voice box (bulbar palsy), and an increase in cerebrospinal fluid protein levels. The neurological symptoms of Guillain-Barre Syndrome affect both sides of the body (bilateral) and directly reflect the portion of the nervous system that is involved in an inflammatory process. Sensory nerve damage produces numbness and tingling in the feet, hands, gums and face. The face may appear pouchy and lopsided. In the acute stage Guillain-Barre Syndrome may cause difficulty breathing or swallowing food. Involvement of the autonomic nervous system (part of the nervous system that regulates vital functions) may cause an abnormally rapid heart beat (sinus tachycardia) or abnormally low heart rate (bradycardia), high blood pressure (hypertension) and/or a sudden drop in blood pressure upon arising from a bed or chair (postural hypotension). Other symptoms may include changes in body temperature, vision, bladder function and blood chemistries. The symptoms experienced by every person with Guillain-Barre Syndrome vary widely. Paralysis generally peaks in less than 10 days, although in rare cases it may continue to progress for months. Recovery begins after the condition has stabilized and this may take 6 months to 2 years. A favorable prognosis for Guillain-Barre Syndrome generally correlates with speedy recovery. Causes Guillain-Barre Syndrome is a very rare, rapidly progressive disorder that affects the nervous system. This disorder is thought to be caused by an autoimmune mechanism following a viral infection. Autoimmune disorders are caused when the body's natural defenses against invading organisms (antibodies, lymphocytes, etc.) mistakenly attack healthy tissue. Many viral infections have been identified as preceding the onset of Guillain-Barre Syndrome. These can include the common cold, viral hepatitis or mononucleosis. Guillain-Barre Syndrome has also been observed following surgery, porphyria (a group of inherited metabolic disorders), an insect sting, Lyme Disease and swine flu inoculations. Affected Population Guillain-Barre Syndrome affects approximately 1 or 2 adults in 100,000 in the United States. Men and women are affected in equal numbers. Related Disorders Symptoms of the following disorders can be similar to those of Guillain-Barre Syndrome. Comparisons may be useful for a differential diagnosis: Peripheral Neuropathy is a disorder of the nervous system characterized by sensory, motor, reflex and vasomotor (nerve and muscles that control the blood vessels) symptoms. Degenerative changes in the nerve fibers occur. The symptoms may involve a single nerve (mononeuropathy) or many nerves (symmetric polyneuropathy, polyneuritis, multiple peripheral neuritis). Symptoms may include muscle weakness and muscular pain. Generally there is a tingling feeling, numbness or a burning sensation in the affected area (paresthesias). The symptoms of Peripheral Polyneuropathy are frequently confused with the early symptoms of Guillain-Barre Syndrome. Peripheral Polyneuropathy usually affects both sides of the body equally and has a wide range of symptoms. It may be caused by malnutrition, poisoning or other underlying diseases such as diabetes or other metabolic disorders. (For more information on this disorder, choose "Peripheral Neuropathy" as your search term in the Rare Disease Database). Fischer Syndrome is a rare subtype of Guillain-Barre Syndrome that commonly follows an upper respiratory tract infection. Men are predominantly affected. A generalized weakness occurs and is particularly severe in the muscles of the eyes (ocular). This may cause impaired vision. Involvement of the muscles of the face and neck may lead to impaired speech and a sagging look to the face. Deep tendon reflexes may be lost and an awkward, unsteady gait (ataxia) is common. (For more information on this disorder, choose "Fisher" as your search term in the Rare Disease Database.) Chronic Idiopathic Polyneuritis produces symptoms that are indistinguishable from those of Guillain-Barre Syndrome with the exception that the eyes and face are involved in only 15 percent of cases. The course of Chronic Idiopathic Polyneuritis is unpredictable. Symptoms tend to progress over 6 to 12 months and then subside for a period of time before recurring. (For more information on this disorder, choose "Chronic Idiopathic Polyneuritis" as your search term in the Rare Disease Database.) Therapies: Standard The debilitating effects of Guillain-Barre Syndrome vary greatly and usually reach a plateau from which recovery begins. Relapses of this disorder rarely occur. Muscular strength is first regained in the upper body. Recovery from Guillain-Barre Syndrome can take from 6 months to over 2 years. More rapid recovery patterns are associated with a diminished chance of long-term disabilities. Various forms of treatment have proven effective for some people with Guillain-Barre Syndrome. Physical therapy may help restore muscle strength as recovery begins. Therapy must be customized for each patient. Respiratory assistance may be needed by hospitalized patients during the acute stage of this disorder. Corticosteroid drugs (for chronic Guillain-Barre Syndrome) and plasmapheresis may be prescribed. Plasmapheresis is a method for removing unwanted substances (toxins, metabolic substances and plasma parts) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient's plasma is then replaced with other human plasma and the blood is transfused back into the patient. Plasmapheresis is recommended only for Guillain-Barre patients with severe weakness at the time of the first plasma exchange (e.g., those unable to walk). The benefits of plasmapheresis are clearest when treatment is started within two weeks after symptoms begin. This procedure can reduce motor weakness and reduce time spent on a respirator, sometimes enabling patients to leave the hospital sooner. It is unclear whether patients able to walk without support will benefit from plasmapheresis. Researchers have found that patients who respond best to plasmapheresis are the younger patients who also have a larger number of muscle fibers that contract in response to an electric nerve stimulus. Age and electrical stimulation of the median nerve at the wrist and the peroneal nerve at the ankle may predict the success of treatment with plasmapheresis. Over fifty percent of people with Guillain-Barre Syndrome typically resume full and active lives. Approximately 5 to 15 percent of affected people experience significant long-term disabilities, and 35 to 45 percent experience mild long-term difficulties such as tingling and muscle aches. Only 1 to 5 percent die of complications of Guillain-Barre Syndrome. Fischer Syndrome and Chronic Idiopathic Polyneuritis are similarly treated with corticosteroid drugs and/or plasmapheresis. Therapies: Investigational Research is ongoing to investigate possible treatments for Guillain-Barre Syndrome. Several studies suggest that high-dose intravenous immunoglobulin may be beneficial for some people with severe cases of Guillain-Barre Syndrome. For information on plasmapheresis as a treatment for Guillain-Barre Syndrome, your physician can contact: The Johns Hopkins University Hospital Plasmapheresis Center 601 North Broadway Baltimore, Maryland 20205 This disease entry is based upon medical information available through October 1992. 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 Guillain-Barre Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Guillain-Barre Syndrome Support Group International P.O. Box 262 Lynnewood, PA 19096 (215) 667-0131 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 References THE MERCK MANUAL 15th ed. R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 1446. CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 471, 1855, 2181. PRINCIPLES OF NEUROLOGY, 4th Ed.; Raymond D. Adams, M.D. and Maurice Victor, M.D., Editors; McGraw-Hill Information Services Company, 1989. Pp. 1035-1039. AUTOIMMUNE DISEASE AND THE NERVOUS SYSTEM. BIOCHEMICAL, MOLECULAR, AND CLINICAL UPDATE, J.E. Graves et al.; West J Med (Jun 1992; 156(6)): Pp. 639- 646.