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$Unique_ID{BRK03660}
$Pretitle{}
$Title{Devic Disease}
$Subject{Devic Disease Neuromyelitis Optica Optic Neuromyelitis
Opthalmoneuromyelitis Optic Neuroencephalomyelopathy Opticomyelitis Devic
Syndrome Retrobulbar Neuropathy Acute Transverse Myelitis Guillain-Barre
Syndrome Acute Disseminated Encephalomyelitis (Postinfectious Encephalitis)
Multiple Sclerosis Systemic Lupus Erythematosus}
$Volume{}
$Log{}
Copyright (C) 1987, 1988, 1989 National Organization for Rare Disorders,
Inc.
479:
Devic Disease
** IMPORTANT **
It is possible the main title of the article (Devic Disease) is not the
name you expected. Please check the SYNONYMS listing on the next page to
find alternate names, disorder subdivisions, and related disorders covered by
this article.
Synonyms
Neuromyelitis Optica
Optic Neuromyelitis
Opthalmoneuromyelitis
Optic Neuroencephalomyelopathy
Opticomyelitis
Devic Syndrome
Retrobulbar Neuropathy
Information on the following diseases can be found in the Related
Disorders section of this report:
Acute Transverse Myelitis
Guillain-Barre Syndrome
Acute Disseminated Encephalomyelitis (Postinfectious Encephalitis)
Multiple Sclerosis
Systemic Lupus Erythematosus
General Discussion
** IMPORTANT **
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.
Devic Disease is a rare nerve disorder characterized by loss
(demyelination) of the fatty sheath surrounding the optic nerve and nerves in
the spinal cord. This disorder can begin at any age after puberty. An
initial phase consisting of a slight fever, sore throat and/or head cold
often occurs. Loss of clear vision is accompanied by mild paralysis (usually
of the lower limbs) and loss of bladder and bowel control. Devic Disease can
occur spontaneously, or in conjunction with Multiple Sclerosis or Systemic
Lupus Erythematosus. (For more information on these disorders, choose "MS"
and "Lupus" your search terms in the Rare Disease Database).
Symptoms
Devic Disease is initially marked by a slight fever, sore throat, and/or head
cold. Inflammation, loss (demyelination) of the fatty tissue sheath
surrounding nerves, and softening and swelling of the optic nerve leads to
pain inside the eye and eventually, loss of clear vision. Initially, only
one eye may be affected although later both eyes become involved. Following
this, spinal cord abnormalities associated with mild paralysis (paraparesis)
of the lower limbs and loss of bowel and bladder control tend to develop.
Deep tendon reflexes are diminished or absent and variable sensory loss
occurs. However, paralysis often improves with time, and partial recovery of
vision difficulties usually occurs.
Causes
The exact cause of Devic Disease is not known. Cases of this disorder may
occur spontaneously, usually following a fever, or they may occur in
conjunction with Multiple Sclerosis or Systemic Lupus Erythematosus. (For
more information on these disorders, choose "MS" and "Lupus" as your search
terms in the Rare Disease Database). Some researchers believe Devic Disease
may be an autoimmune disorder, or that it may be genetic. Autoimmune
disorders occur when the body's natural defenses against disease or invading
organisms (such as bacteria), for unknown reasons, suddenly begin to attack
healthy tissue. In this case, these defenses may attack healthy fatty tissue
surrounding the optic and spinal nerves.
Affected Population
Devic Disease affects males and females in equal numbers, and may occur as a
feature of Multiple Sclerosis or Systemic Lupus Erythematosus. In some
cases, it may affect patients following a disease which included fever as a
symptom.
Related Disorders
Symptoms of the following disorders can be similar to those of Devic Disease.
Comparisons may be useful for a differential diagnosis:
Acute Transverse Myelitis is an acute inflammation, with softening of the
spinal cord. Spinal nerves of a limited lengthwise section of the spinal
cord are affected by obstruction of blood vessels, swelling, cellular loss or
infiltration, and loss of the fatty tissue around the nerves (demyelination).
Guillain-Barre Syndrome (Acute Idiopathic Polyneuritis) is an autoimmune
disorder which occurs when the body's defense system attacks the nerves,
damaging the nerve's fatty sheath (myelin) and axis cylinder (axon). Nerve
signals are delayed and altered, causing weakness and paralysis of the
muscles of the legs, arms, and other parts of the body along with abnormal
sensations. Fischer's Syndrome (a form of polyneuroradiculitis marked by
ophthalmoplegia, ataxia and arreflexia) and Chronic Idiopathic Polyneuritis
(chronic inflammation of groups of spinal nerve cells with an unknown origin)
are two very rare forms of the disorder. (For more information on this
disorder, choose "Guillain-Barre Syndrome" as your search term in the Rare
Disease Database).
Acute Disseminated Encephalomyelitis (Postinfectious Encephalitis) is a
central nervous system disorder characterized by inflammation of the brain
and spinal cord caused by damage to the fatty sheath surrounding the nerves.
This can occur spontaneously, but usually follows a viral infection or
inoculation such as a bacterial or viral vaccine.
The following disorders may precede the development of Devic Disease.
They can be useful in identifying an underlying cause of some forms of this
disorder:
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
form randomly throughout the brain and spinal cord. These plaques are due to
loss of the fatty sheath surrounding nerves and prevents proper transmission
of nervous system signals and thus result in a variety of neurological
symptoms. Most patients with MS have a near normal life span. Symptoms
often include visual difficulties as well as speech impairment, abnormal skin
sensations or numbness, gait disturbance, and difficulties with bladder and
bowel function. In a small number of cases, Devic Disease has occurred as a
complication of MS. (For more information on this disorder, choose "MS" as
your search term in the Rare Disease Database).
Systemic Lupus Erythematosus (also known as Lupus) is an inflammatory
connective tissue disease that can affect many parts of the body including
the joints, skin and internal organs. Lupus is a disease of the body's
immune system, most often striking young women between the ages of fifteen
and thirty-five years. (For more information on this disorder, choose
"Lupus" as your search term in the Rare Disease Database).
Therapies: Standard
Early treatment of Devic Disease using ACTH hormone or corticosteroid drugs
usually controls inflammation of the optic nerve and spine. Other treatment
is symptomatic and supportive.
Therapies: Investigational
Lymphocytoplasmapheresis is being investigated as a possible treatment for
patients with Devic Disease. 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 or lymph cells are
separated from plasma. The new plasma is then transfused into the patient.
This therapy is still under investigation to analyze side effects and
effectiveness. More research is needed before lymphocytoplasmapheresis can
be recommended for use in all but the most severe cases of Devic Disease.
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 Devic Disease, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Eye Institute (NEI)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5248
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
References
LYMPHOCYTAPLASMAPHERESIS IN DEVIC'S SYNDROME: A.J. Aguilera, et al.;
Transfusion (January-February 1985, issue 25(1)). Pp. 54-56.
DEVIC'S SYNDROME AND SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE REPORT WITH
NECROPSY: E.L. Kinney, et al.; Arch Neurol (October 1979, issue 36 (10)).
Pp. 643-644.
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown
and Co., 1987. Pp. 2246.