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20-September-2008 10:08:30 - Devic's disease Devic's disease Classification and external resources ICD-10 G36.0 ICD-9 341.0 DiseasesDB 29470 MeSH D009471 Devic's disease, also known as Devic's syndrome or neuromyelitis optica NMO, is an autoimmune, inflammatory disorder in which a person's own immune system attacks the optic nerves and spinal cord. This produces an inflammation of the optic nerve optic neuritis and the spinal cord myelitis. Although inflammation may also affect the brain, the lesions are different from those observed in the related condition multiple sclerosis.1 Spinal cord lesions lead to varying degrees of weakness or paralysis in the legs or arms, loss of sensation, and/or bladder and bowel dysfunction.2 Devic's disease is a rare disorder which resembles multiple sclerosis MS in several ways, but requires a different course of treatment for optimal results.2 NMO has also been suggested to be a variant form of acute disseminated encephalomyelitis.34 The likely target of the autoimmune attack at least in some patients with NMO has been identified. The target is a protein of the nervous system cells called aquaporin 4.5 Contents 1 Symptoms 2 Mechanism 3 Diagnosis 3.1 Variants 4 Treatment 4.1 Attacks 4.2 Secondary prevention 5 Prognosis 6 Epidemiology 7 History 8 See also 9 References 10 External links Symptoms The main symptoms of Devic's disease are loss of vision and spinal cord function. As for other etiologies of optic neuritis, the visual impairment usually manifests as decreased visual acuity, although visual field defects, or loss of color vision may occur in isolation or prior to formal loss of acuity. Spinal cord dysfunction can lead to muscle weakness, reduced sensation, or loss of bladder and bowel control.6 The typical patient has an acute and severe spastic weakness of the legs paraparesis or all four limbs tetraparesis with sensory signs, often accompanied by loss of bladder control. Mechanism Devic's disease is similar to MS in that the body's immune system attacks the myelin surrounding nerve cells. Unlike standard MS, the attacks are not believed to be mediated by the immune system's T cells but rather by antibodies called NMO-IgG. These antibodies target a protein called aquaporin 4 in the cell membranes of astrocytes which acts as a channel for the transport of water across the cell membrane. 2 Aquaporin 4 is found in the processes of the astrocytes that surround the blood-brain barrier, a system responsible for preventing substances in the blood from crossing into the brain. The blood-brain barrier is weakened in Devic's disease, but it is currently unknown how the NMO-IgG immune response leads to demyelination. Most research into the pathology of Devic's disease has focused on the spinal cord. The damage in the spinal cord can range from inflammatory demyelination to necrotic damage of the white and grey matter. The inflammatory lesions in Devic's disease have been classified as type II lesions complement mediated demyelinization, but they differ from MS pattern II lesions in their prominent perivascular distribution. Therefore, the pattern of inflammation is often quite distinct from that seen in MS. 27 Diagnosis The Mayo Clinic proposed a revised set of criteria for diagnosis of Devic's disease in 2006. The new guidelines for diagnosis require two absolute criteria plus at least two of three supportive criteria being:8 Absolute criteria: Optic neuritis Acute myelitis Supportive criteria: Brain MRI not meeting criteria for MS at disease onset Spinal cord MRI with contiguous T2-weighted signal abnormality extending over 3 or more vertebral segments, indicating a relatively large lesion in the spinal cord NMO-IgG seropositive status. The NMO-IgG test checks the existence of antibodies against the aquaporin 4 antigen. Variants After the development of the NMO-IgG test, the spectrum of disorders that comprise Devic's disease was expanded. The Devic's disease spectrum is now believed to consist of: Standard Devic's disease, according to the diagnostic criteria described above Limited forms of Devic's disease, such as single or recurrent events of longitudinally extensive myelitis, and bilateral simultaneous or recurrent optic neuritis Asian optic-spinal MS. This variant can present CNS involvement like MS9 Longitudinally extensive myelitis or optic neuritis associated with systemic auto-immune disease Optic neuritis or myelitis associated with lesions in specific brain areas such as the hypothalamus, periventricular nucleus, and brainstem10 Whether Devic's disease is a distinct disease or part of the wide spectrum of multiple sclerosis is debated. Devic's disease differs in that it usually has more severe sequelae after an acute episode than in MS, MS infrequently presents as transverse myelitis, and oligoclonal bands in the CSF, as well as white matter lesions on brain MRI, are uncommon in Devic's disease but occur in over 90% of MS patients11. Recently it has been found that antiviral immune response distinguishes multiple sclerosis and neuromyelitis optica.12 Devic's disease has been associated with many systemic diseases, based on anecdoctal evidence of some Devic's disease patients with a comorbid condition. Such conditions include: collagen vascular diseases, autoantibody syndromes, infections with varicella-zoster virus, Epstein-Barr virus, and HIV, and exposure to clioquinol and antituberculosis drugs.13 Treatment Currently, there is no cure for Devic's disease, but symptoms can be treated. Some patients recover, but many are left with impairment of vision and limbs, which can be severe. Attacks Attacks are treated with short courses of high dosage intravenous corticosteroids such as methylprednisolone IV. When attacks progress or do not respond to corticosteroid treatment, plasmapheresis can be an effective treatment.10 Clinical trials for these treatments contain very small numbers, and most are uncontrolled. Secondary prevention No controlled trials have established the effectiveness of treatments for the prevention of attacks. Many clinicians agree that long term immunosuppression is required to reduce the frequency and severity of attacks, while others argue the exact opposite14. Commonly used immunosuppresant treatments include azathioprine Imuran plus prednisone, mycophenolate mofetil plus prednisone, Rituximab, Mitoxantrone, intravenous immunoglobulin IVIG, and Cyclophosphamide.1510 The monoclonal antibody rituximab is under study.16 In 2007, Devic's disease was reported to be responsive to glatiramer acetate17 and to low-dose corticosteroids.18 Prognosis Normally, there is some measure of improvement in a few weeks, but residual signs and disability may persist, sometimes severely. The disease can be monophasic, i.e. a single episode with permanent remission. However, at least 85% of patients have a relapsing form of the disease with repeated attacks of transverse myelitis and/or optic neuritis. In patients with the monophasic form the transverse myelitis and optic neuritis occur simultaneously or within days of each other. On the other hand, patients with the relapsing form are more likely to have weeks or months between the initial attacks and to have better motor recovery after the initial transverse myelitis event. Relapses usually occur early with about 55% of patients having a relapse in the first year and 90% in the first 5 years.2 Unlike MS, Devic's disease rarely has a secondary progressive phase in which patients have increasing neurologic decline between attacks without remission. Instead, disabilities arise from the acute attacks.2 Approximately 20% of patients with monophasic Devic's disease have permanent visual loss and 30% have permanent paralysis in one or more legs. Among patients with relapsing Devic's disease, 50% have paralysis or blindness within 5 years. In some patients 33% in one study, transverse myelitis in the cervical spinal cord resulted in respiratory failure and subsequent death. However, the spectrum of Devic's disease has widened due to improved diagnostic criteria, and the options for treatment have improved; as a result, researchers believe that these estimates will be lowered.2 Epidemiology The prevalence and incidence of Devic's disease has not been established partly because the disease is underrecognized and often confused with MS.2 Devic's disease is more common in women than men, with women comprising over 2/3 of patients and more than 80% of those with the relapsing form of the disease. 2 Devic's disease is more common in Asiatic people than Caucasians. In fact, Asian optic-spinal MS which constitutes 30% of the cases of MS in Japan has been suggested to be identical to Devic's diseaseDifferences between optic-spinal and classic MS in Japanese patients. In the indigenous populations of tropical and subtropical regions, MS is rare, but when it appears it often takes the form of optic-spinal MS.19 The majority of Devic's disease patients have no affected relatives, and it is generally regarded as a non-familial condition.2 History In 1870, Sir Thomas Clifford Allbutt first reported an association between myelitis and an optic nerve disorder. In 1894, Eugène Devic and his PhD. student Fernand Gault described 16 patients who had lost vision in one or both eyes and within weeks developed severe spastic weakness of the limbs, loss of sensation and often bladder control. They recognized that these symptoms were the result of inflammation of the optic nerve and spinal cord, respectively.20 Similar instances of optic neuritis and myelitis were reported, and many believed it constituted a distinct clinical entity. However, some patients had pathology in other parts of the brain, a feature which was more suggestive of acute disseminated encephalomyelitis ADEM or MS. In 2004 the Mayo Clinic identified the aquaporin 4 protein as the target of the disease and developed a test to aid in the diagnosis of Devic's disease by detection of an antibody, NMO-IgG, in the blood.5 Some patients with NMO may be seronegative for NMO-IgG whilst some patients with NMO-IgG may still not fulfill clinical criteria for NMO thus serological testing is now an important part of the diagnostic procedure and seropositive and seronegative cases are described in a manner similar to myasthenia gravis. According to the Mayo Clinic report, this was the first time that a molecular target had been identified for a type of demyelinating inflammatory disease.21 See also Multiple sclerosis Multiple sclerosis borderline References ^ Pittock SJ, Weinshenker BG, Lucchinetti CF, Wingerchuk DM, Corboy JR, Lennon VA 2006. Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression. Arch. Neurol. 63 7: 964-8. doi:10.1001/archneur.63.7.964. PMID 16831965. ^ a b c d e f g h i j Wingerchuk, DM 2006. Neuromyelitis optica. The International MS Journal 13: 42-50. PMID 16635421. ^ Lennon VA, Wingerchuk DM, Kryzer TJ, et al 2004. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 364 9451: 2106-12. doi:10.1016/S0140-67360417551-X. PMID 15589308. ^ Pittock SJ, Lennon VA, Krecke K, Wingerchuk DM, Lucchinetti CF, Weinshenker BG 2006. Brain abnormalities in neuromyelitis optica. Arch. Neurol. 63 3: 390-6. doi:10.1001/archneur.63.3.390. PMID 16533966. ^ a b Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR 2005. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J. Exp. Med. 202 4: 473-7. doi:10.1084/jem.20050304. PMID 16087714. ^ Mayo Clinic. Devic's Disease Symptoms. Retrieved on 2008-03-11. ^ Lucchinetti CF, Mandler RN, McGavern D, et al 2002. A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica. Brain 125 Pt 7: 1450-61. doi:10.1093/brain/awf151. PMID 12076996. ^ Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG 2006. Revised diagnostic criteria for neuromyelitis optica. Neurology 66 10: 1485-9. doi:10.1212/01.wnl.0000216139.44259.74. PMID 16717206. ^ Li Y, Xie P, Lv F, et al 2008. Brain magnetic resonance imaging abnormalities in neuromyelitis optica. Acta Neurol. Scand. 0: 080331054152017. doi:10.1111/j.1600-0404.2008.01012.x. PMID 18384459. ^ a b c Wingerchuk, Dean 2006. Neuromyelitis Optica Devic's Syndrome. 2006 Rare Neuroimmunologic Disorders Symposium. Retrieved on 2007-01-05. ^ Pearce JM 2005. Neuromyelitis optica abstract. Spinal Cord 43 11: 631-4. doi:10.1038/sj.sc.3101758. PMID 15968305. ^ Jarius S, Franciotta D, Bergamaschi R et al 2008. Polyspecific, antiviral immune response distinguishes multiple sclerosis and neuromyelitis optica. J Neurol Neurosurg Psychiatry.. doi:10.1136/jnnp.2007.133330. PMID 18270237. ^ Cree BA, Goodin DS, Hauser SL 2002. Neuromyelitis optica. Seminars in neurology 22 2: 105-22. doi:10.1055/s-2002-36534. PMID 12524556. ^ Poser CM, Brinar VV 2007. Disseminated encephalomyelitis and multiple sclerosis: two different diseases - a critical review. Acta Neurol. Scand. 116 4: 201-6. doi:10.1111/j.1600-0404.2007.00902.x. PMID 17824894. ^ Weinstock-Guttman B, Ramanathan M, Lincoff N, et al 2006. Study of mitoxantrone for the treatment of recurrent neuromyelitis optica Devic disease. Arch. Neurol. 63 7: 957-63. doi:10.1001/archneur.63.7.957. PMID 16831964. ^ Matiello M, Jacob A, Wingerchuk DM, Weinshenker BG 2007. Neuromyelitis optica. Curr. Opin. Neurol. 20 3: 255-60. doi:10.1097/WCO.0b013e32814f1c6b. PMID 17495617. ^ Gartzen K, Limmroth V, Putzki N 2007. Relapsing neuromyelitis optica responsive to glatiramer acetate treatment. Eur. J. Neurol. 14 6: e12-3. doi:10.1111/j.1468-1331.2007.01807.x. PMID 17539924. ^ Watanabe S, Misu T, Miyazawa I, et al 2007. Low-dose corticosteroids reduce relapses in neuromyelitis optica: a retrospective analysis. Multiple Sclerosis 13: 968. doi:10.1177/1352458507077189. PMID 17623727. ^ Cabre P, Signate A, Olindo S, et al 2005. Role of return migration in the emergence of multiple sclerosis in the French West Indies. Brain 128 Pt 12: 2899-910. doi:10.1093/brain/awh624. PMID 16183661. ^ T. Jock Murray 2005. Multiple Sclerosis: The History of a Disease. New York: Demos Medical Publishing. ISBN 1888799803. ^ Devic's Disease Research - Mayo Clinic. Retrieved on 2007-11-22. External links Devic's Disease Neuromyelitis Optica at myelitis.org Devics Disease - Information and treatment options at Mayo Clinic The Walton centre for Neurology and Neurosurgery, Liverpool, United Kingdom, information for patients and physicians - at NHS EDMUS, European Database for Multiple Sclerosis at edmus.org NMO / Devic's NHSDirect - Documentation on the UK National Health Service Database for Medical staff and Patients attending the UK for treatment, it also links to the database gathering data for Mayo Clinic USA and Walton Centre UK - added by MND pp: Dr. Anu Jacob v d e Multiple sclerosis Signs and symptoms Ataxia · Depression · Diplopia · Dysarthria · Dysphagia · Fatigue · Incontinence · Neurological fatigue · Nystagmus · Optic neuritis · Pain · Uhthoff's phenomenon Diagnosis and evolution following McDonald criteria · EDSS Investigation Pathophysiology · Experimental autoimmune encephalomyelitis Treatment Interferon · Glatiramer acetate · Mitoxantrone · Natalizumab · Therapies under investigation Borderline forms Acute disseminated encephalomyelitis · Balo concentric sclerosis · Devic's disease · Guillain-Barré syndrome · Marburg multiple sclerosis · Schilder's disease Other List of people with multiple sclerosis · List of multiple sclerosis organizations v d e Pathology of the nervous system, primarily CNS G00-G47, 320-349 Inflammatory Meningitis Arachnoiditis - Encephalitis - Myelitis - Encephalomyelitis Acute disseminated - Tropical spastic paraparesis - Cavernous sinus thrombosis Systemic atrophies Huntington's disease - Spinocerebellar ataxia Friedreich's ataxia, Ataxia telangiectasia, Herary spastic paraplegia - Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio-Londe - MND ALS, PMA, PBP, PP, PLS Extrapyramidal and movement disorders Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia/Dyskinesia Spasmodic torticollis, Meige's, Blepharospasm - Essential tremor - Myoclonus - Lafora - Chorea Choreoathetosis - Restless legs - Stiff person Other degenerative/ demyelinating diseases dementia: Alzheimer's - Pick's - Dementia with Lewy bodies - Frontotemporal lobar degeneration mitochondrial disease: Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis - Marchiafava-Bignami disease - CAMFAK syndrome - Alpers' Seizure/epilepsy Focal - Generalised - Status epilepticus - Myoclonic epilepsy Headache Migraine Familial hemiplegic - Cluster - Vascular - Tension Vascular Transient ischemic attack Amaurosis fugax, Transient global amnesia Cerebrovascular disease MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke Sleep disorders Insomnia - Hypersomnia - Sleep apnea Obstructive, Ondine's curse - Narcolepsy - Cataplexy - Kleine-Levin - Circadian rhythm sleep - Delayed sleep phase - Advanced sleep phase Intracranial hypertension Hydrocephalus Normal pressure - Idiopathic intracranial hypertension Other encephalopathy Brain herniation - Cerebral edema - Reye's Other spinal cord disease Syringomyelia - Syringobulbia - Morvan's syndrome - Spinal cord compression Retrieved from http://en..org/wiki/Devic%27s_disease Categories: Multiple sclerosis | Autoimmune diseases | Neurological disorders | Neurology | Rare diseases Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Français Polski This page was last modified on 20 July 2008, at 14:59
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