$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.