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$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.