$Unique_ID{BRK03604} $Pretitle{} $Title{Chronic Inflammatory Demyelinating Polyneuropathy} $Subject{Chronic Inflammatory Demyelinating Polyneuropathy CIDP Charcot-Marie-Tooth Disease Dejerine-Sottas Disease Friedreich's Ataxia Guillain-Barre Syndrome Multiple Sclerosis} $Volume{} $Log{} Copyright (C) 1992 National Organization for Rare Disorders, Inc. 903: Chronic Inflammatory Demyelinating Polyneuropathy ** IMPORTANT ** It is possible that the main title of the article (Chronic Inflammatory Demyelinating Polyneuropathy) is not the name you expected. Please check the SYNONYMS listing to find the alternate name and disorder subdivisions covered by this article. Synonyms CIDP Information on the following diseases can be found in the Related Disorders section of this report: Charcot-Marie-Tooth Disease Dejerine-Sottas Disease Friedreich's Ataxia Guillain-Barre Syndrome Multiple Sclerosis 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. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare disorder in which there is swelling of nerve roots and destruction of the covering (myelin sheath) over the nerves. This causes weakness, paralysis and/or impairment in motor function, especially of the limbs. Sensory loss may also be present causing numbness, tingling, or prickling. The motor and sensory impairments are usually symmetrical (on both sides of the body), and the degree of severity can vary. The course of CIDP may also vary. Some patients may follow a slow steady pattern of symptoms while others may have symptoms that wax and wane with the most severe symptoms occuring after many months or a year or more. One feature that distinguishes this disorder from other similar disorders is that there is typically no preceding viral infection at least three months prior to the appearance of the disorder, and no family history of other similar disorders or polyneuropathy. Symptoms Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a disorder in which there is swelling of nerve roots destroying the covering (myelin sheath) of nerves. Typically there has been no preceding viral infection for at least three months prior to the appearance of CIDP. Nerve signals become altered causing impairment in motor function and/or abnormal or loss of sensation. The course of CIDP as well as the severity of impairment can vary from patient to patient. In some patients the disorder may progress slowly while others may have symptoms that worsen, get better, then reoccur. In some people the disorder becomes more obvious and/or severe as it progresses over many months. Symptoms of CIDP may be: burning, numbness and/or tingling of the hands and feet or arms and legs; weakened or absent reflexes; facial weakness; weak arms and/or legs; paralysis of the arms and/or legs; weakness of the muscles between the ribs; respiratory problems; loss of feeling; and/or difficulty in walking. Causes The exact cause of Chronic Inflammatory Demyelinating Polyneuropathy is not known. It is thought that a defect in the immune system may be the cause but this has not yet been proven. Autoimmune disorders occur when the body's natural defenses (antibodies, lymphocytes, etc.), against invading organisms suddenly begin to attack perfectly healthy tissue. The cause of autoimmune disorders is unknown. Affected Population Chronic Inflammatory Demyelinating Polyneuropathy is a very rare disorder that can affect any age group and the onset of the disorder may begin during any decade of life. Related Disorders Symptoms of the following disorders can be similar to those of Chronic Inflammatory Demyelinating Polyneuropathy. Comparisons may be useful for a differential diagnosis: Charcot-Marie-Tooth Disease is a hereditary neurological disorder, characterized by weakness and atrophy primarily, in the legs. Disappearance of the fatty shield surrounding the peripheral nerves (segmental demyelination), and associated degeneration of part of the nerve cells, characterize this disorder. Initial symptoms may appear between middle childhood and the age of thirty. Charcot-Marie-Tooth Disease may be inherited as an autosomal dominant, autosomal recessive or X-linked trait. (For more information on this disorder, choose "Charcot-Marie-Tooth Disease" as your search term in the Rare Disease Database). Dejerine-Sottas Disease is a hereditary neurological disorder which progressively affects mobility. Peripheral nerves become enlarged or thickened causing an irregular progression of muscle weakness. Pain, weakness, numbness, and a tingling, prickling or burning sensation can occur in the legs of patients with this disorder. Dejerine-Sottas Disease is inherited as an autosomal dominant trait. (For more information on this disorder, choose "Dejerine-Sottas Disease" as your search term in the Rare Disease Database). Friedreich's Ataxia is a hereditary neuromuscular syndrome characterized by slow degenerative changes of the spinal chord and brain. Dysfunction of the central nervous system affects coordination of the muscles in the limbs. Speech can be affected and numbness or weakness of the arms and legs may develop. Various transitional and overlapping forms of Friedreich's Ataxia can occur. In a few cases, spontaneous remission may occur which can last five to ten years or sometimes longer. This disorder usually begins in childhood or the teen years. (For more information on this disorder, choose "Freidreich's Ataxia" as your search term in the Rare Disease Database). Guillain-Barre Syndrome occurs when the body's defense system attacks the nerves. Weakness and paralysis of the muscles of the legs, arms, and other parts of the body along with abnormal sensations may occur. This disorder is very similar to Chronic Inflammatory Demyelinating Polyneuropathy but the course of the disorder begins quickly, is intense and then slows down becoming stationary with a slow recovery. Unlike CIDP, patients with Guillain-Barre Syndrome rarely have signs of sensory loss. About half of the patients with Guillain-Barre Syndrome have had a viral infection prior the disorder. The disease has also occurred following seemingly unrelated events, i.e., swine flu injections, surgery, insect stings, or a disorder of the nervous system and skin (porphyria). (For more information on this disorder, choose "Guillain-Barre Syndrome" as your search term in the Rare Disease Database). Multiple Sclerosis is a chronic disease of the brain and spinal cord (central nervous system) which may be progressive, relapsing and remitting, or stable. The pathology of MS consists of small lesions called plaques that may form randomly throughout the brain and spinal cord. These patches prevent proper transmission of nervous system signals and thus result in a variety of neurological symptoms. (For more information on this disorder choose "Multiple Sclerosis" as your search term in the Rare Disease Database). Therapies: Standard Chronic Inflammatory Demyelinating Polyneuropathy can be difficult to diagnose. The symptoms must be present for at least a month and typically there is no preceding ailment. Extensive destruction of the covering (myelin sheath) of peripheral nerves is present. The reflexes of the tendons may be absent or reduced and the progression of the disorder may vary from a slow, gradual onset eventually waxing and waning to a course where the disorder becomes more obvious and severe as it progresses. Motor and sensory loss is present in patients with CIDP and there typically is no family history of a related disease. Glucocorticoid drugs have had some affect in treating patients with CIDP but often the side affects deter long-term therapy. Intravenous Immunoglobulin (IVIG) is often used as a treatment for Chronic Inflammatory Demyelinating Polyneuropathy. IVIG can enhance the immune system. Very high doses are usually used for initial treatment of CIDP. Other drugs which alter the immune system such as Azathioprine and Cyclophosphamide have been beneficial to some patients. Therapies: Investigational Plasmapheresis may be of benefit in some cases of CIDP. This procedure 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. This therapy is still under investigation to analyze long-term side effects and effectiveness. More research is needed before plasmapheresis can be recommended for use in all but the most severe cases of CIDP. This disease entry is based upon medical information available through April 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 Chronic Inflammatory Demyelinating Polyneuropathy, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 References CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 2262. HIGH-DOSE INTRAVENOUS HUMAN IMMUNOGLOBULIN IN CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY: J.M. Faed, FRCPA, et al.; Neurology (1989, issue 39). Pp.422-25. CONSENSUS STATEMENT-NIH CONSENSUS DEVELOPMENT CONFERENCE: CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY: May 21-23, 1990, Volume 8, Number 5. THE MAYO CLINIC EXPERIENCE WITH PLASMA EXCHANGE IN CHRONIC INFLAMMATORY- DEMYELINATING POLYNEUROPATHY: P.J. Dyck, et al.; Prog Clin Biol Res (1982, issue 106). Pp. 197-204.