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. 1996 Apr;60(4):382–387. doi: 10.1136/jnnp.60.4.382

Clinical, CSF, and MRI findings in Devic's neuromyelitis optica.

J I O'Riordan 1, H L Gallagher 1, A J Thompson 1, R S Howard 1, D P Kingsley 1, E J Thompson 1, W I McDonald 1, D H Miller 1
PMCID: PMC1073888  PMID: 8774400

Abstract

OBJECTIVES: Since Devic's original description of neuromyelitis optica in 1894 there has been much debate regarding its aetiology. A specific cause has been identified in a minority of cases but in most the question has arisen whether or not Devic's neuromyelitis optica is a variant of multiple sclerosis. This study was undertaken to help clarify this issue. METHODS: Neuromyelitis optica was defined as (1) a severe transverse myelitis; (2) an acute unilateral or bilateral optic neuropathy; (3) no clinical involvement beyond the spinal cord or optic nerves, and (4) a monophasic or multiphasic illness. The clinical and autoantibody status was documented. Patients underwent CSF examination and MRI of brain and spinal cord. RESULTS: Twelve patients, with a mean age of presentation of 35.1 years, were seen. Eleven were women; vision was reduced to counting fingers or worse in 10 patients and seven became confined to a wheelchair. Examination of CSF showed local synthesis of oligoclonal bands in only two patients and a neutrophil pleocytosis in two. A possible aetiology was identified in five: a specific connective tissue disorder (two), pulmonary tuberculosis (one), and possible acute disseminated encephalomyelitis (two). Six had non-specific increases in various autoantibodies. Eleven patients underwent MRI of the brain and spinal cord. In 10 there were diffuse abnormalities involving cervical and thoracic cords with extensive swelling in the acute phase. Brain MRI was normal in five; in five there were multiple deep white matter lesions, and one patient had minor age related changes. CONCLUSION: It is proposed that Devic's neuromyelitis optica is a distinctive disorder with some clinical, CSF, and MRI features different from those found in classic multiple sclerosis. In most cases a specific aetiology is not identified, but an immunological mechanism of tissue damage seems likely.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. April R. S., Vansonnenberg E. A case of neuromyelitis optica (Devic's syndrome) in systemic lupus erythematosus. Clinicopathologic report and review of the literature. Neurology. 1976 Nov;26(11):1066–1070. doi: 10.1212/wnl.26.11.1066. [DOI] [PubMed] [Google Scholar]
  2. Campi A., Filippi M., Comi G., Martinelli V., Baratti C., Rovaris M., Scotti G. Acute transverse myelopathy: spinal and cranial MR study with clinical follow-up. AJNR Am J Neuroradiol. 1995 Jan;16(1):115–123. [PMC free article] [PubMed] [Google Scholar]
  3. Compston A. Risk factors for multiple sclerosis: race or place? J Neurol Neurosurg Psychiatry. 1990 Oct;53(10):821–823. doi: 10.1136/jnnp.53.10.821. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Fazekas F., Offenbacher H., Schmidt R., Strasser-Fuchs S. MRI of neuromyelitis optica: evidence for a distinct entity. J Neurol Neurosurg Psychiatry. 1994 Sep;57(9):1140–1142. doi: 10.1136/jnnp.57.9.1140. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Francis D. A., Brown A., Miller D. H., Wiles C. M., Bennett E. D., Leigh N. MRI appearances of the CNS manifestations of Mycoplasma pneumoniae: a report of two cases. J Neurol. 1988 Sep;235(7):441–443. doi: 10.1007/BF00314492. [DOI] [PubMed] [Google Scholar]
  6. Fukazawa T., Hamada T., Tashiro K., Moriwaka F., Yanagihara T. Acute transverse myelopathy in multiple sclerosis. J Neurol Sci. 1990 Dec;100(1-2):217–222. doi: 10.1016/0022-510x(90)90036-m. [DOI] [PubMed] [Google Scholar]
  7. Goldman M., Herode A., Borenstein S., Zanen A. Optic neuritis, transverse myelitis, and anti-DNA antibodies nine years after thymectomy for myasthenia gravis. Arthritis Rheum. 1984 Jun;27(6):701–703. doi: 10.1002/art.1780270616. [DOI] [PubMed] [Google Scholar]
  8. Hughes R. A., Mair W. G. Acute necrotic myelopathy with pulmonary tuberculosis. Brain. 1977 Jun;100(2):223–238. doi: 10.1093/brain/100.2.223. [DOI] [PubMed] [Google Scholar]
  9. Kesselring J., Miller D. H., Robb S. A., Kendall B. E., Moseley I. F., Kingsley D., du Boulay E. P., McDonald W. I. Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis. Brain. 1990 Apr;113(Pt 2):291–302. doi: 10.1093/brain/113.2.291. [DOI] [PubMed] [Google Scholar]
  10. Kidd D., Thorpe J. W., Thompson A. J., Kendall B. E., Moseley I. F., MacManus D. G., McDonald W. I., Miller D. H. Spinal cord MRI using multi-array coils and fast spin echo. II. Findings in multiple sclerosis. Neurology. 1993 Dec;43(12):2632–2637. doi: 10.1212/wnl.43.12.2632. [DOI] [PubMed] [Google Scholar]
  11. Kinney E. L., Berdoff R. L., Rao N. S., Fox L. M. Devic's syndrome and systemic lupus erythematosus: a case report with necropsy. Arch Neurol. 1979 Oct;36(10):643–644. doi: 10.1001/archneur.1979.00500460077012. [DOI] [PubMed] [Google Scholar]
  12. Leonardi A., Arata L., Farinelli M., Cocito L., Schenone A., Tabaton M., Mancardi G. L. Cerebrospinal fluid and neuropathological study in Devic's syndrome. Evidence of intrathecal immune activation. J Neurol Sci. 1987 Dec;82(1-3):281–290. doi: 10.1016/0022-510x(87)90024-4. [DOI] [PubMed] [Google Scholar]
  13. Mandler R. N., Davis L. E., Jeffery D. R., Kornfeld M. Devic's neuromyelitis optica: a clinicopathological study of 8 patients. Ann Neurol. 1993 Aug;34(2):162–168. doi: 10.1002/ana.410340211. [DOI] [PubMed] [Google Scholar]
  14. Miller D. H., McDonald W. I., Blumhardt L. D., du Boulay G. H., Halliday A. M., Johnson G., Kendall B. E., Kingsley D. P., MacManus D. G., Moseley I. F. Magnetic resonance imaging in isolated noncompressive spinal cord syndromes. Ann Neurol. 1987 Dec;22(6):714–723. doi: 10.1002/ana.410220607. [DOI] [PubMed] [Google Scholar]
  15. Motomura S., Tabira T., Kuroiwa Y. A clinical comparative study of multiple sclerosis and neuro-Behçet's syndrome. J Neurol Neurosurg Psychiatry. 1980 Mar;43(3):210–213. doi: 10.1136/jnnp.43.3.210. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Ortiz de Zárate J. C., Tamaroff L., Sica R. E., Rodriguez J. A. Neuromyelitis optica versus subacute necrotic myelitis. II. Anatomical study of two cases. J Neurol Neurosurg Psychiatry. 1968 Dec;31(6):641–645. doi: 10.1136/jnnp.31.6.641. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Paty D. W., Oger J. J., Kastrukoff L. F., Hashimoto S. A., Hooge J. P., Eisen A. A., Eisen K. A., Purves S. J., Low M. D., Brandejs V. MRI in the diagnosis of MS: a prospective study with comparison of clinical evaluation, evoked potentials, oligoclonal banding, and CT. Neurology. 1988 Feb;38(2):180–185. doi: 10.1212/wnl.38.2.180. [DOI] [PubMed] [Google Scholar]
  18. STANSBURY F. C. Neuromyelitis optica; presentation of five cases, with pathologic study, and review of literature. Arch Ophthal. 1949 Oct;42(4):465–501. [PubMed] [Google Scholar]
  19. Silber M. H., Willcox P. A., Bowen R. M., Unger A. Neuromyelitis optica (Devic's syndrome) and pulmonary tuberculosis. Neurology. 1990 Jun;40(6):934–938. doi: 10.1212/wnl.40.6.934. [DOI] [PubMed] [Google Scholar]
  20. Swingler R. J., Compston D. The distribution of multiple sclerosis in the United Kingdom. J Neurol Neurosurg Psychiatry. 1986 Oct;49(10):1115–1124. doi: 10.1136/jnnp.49.10.1115. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Tashiro K., Ito K., Maruo Y., Homma S., Yamada T., Fujiki N., Moriwaka F. MR imaging of spinal cord in Devic disease. J Comput Assist Tomogr. 1987 May-Jun;11(3):516–517. doi: 10.1097/00004728-198705000-00027. [DOI] [PubMed] [Google Scholar]
  22. Thompson A. J., Kermode A. G., MacManus D. G., Kendall B. E., Kingsley D. P., Moseley I. F., McDonald W. I. Patterns of disease activity in multiple sclerosis: clinical and magnetic resonance imaging study. BMJ. 1990 Mar 10;300(6725):631–634. doi: 10.1136/bmj.300.6725.631. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Tola M. R., Granieri E., Caniatti L., Paolino E., Monetti C., Dovigo L., Scolozzi R., De Bastiani P., Carreras M. Systemic lupus erythematosus presenting with neurological disorders. J Neurol. 1992 Feb;239(2):61–64. doi: 10.1007/BF00862972. [DOI] [PubMed] [Google Scholar]
  24. Trojano M., Avolio C., Manzari C., Calò A., De Robertis F., Serio G., Livrea P. Multivariate analysis of predictive factors of multiple sclerosis course with a validated method to assess clinical events. J Neurol Neurosurg Psychiatry. 1995 Mar;58(3):300–306. doi: 10.1136/jnnp.58.3.300. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Willoughby E. W., Grochowski E., Li D. K., Oger J., Kastrukoff L. F., Paty D. W. Serial magnetic resonance scanning in multiple sclerosis: a second prospective study in relapsing patients. Ann Neurol. 1989 Jan;25(1):43–49. doi: 10.1002/ana.410250107. [DOI] [PubMed] [Google Scholar]
  26. al-Deeb S. M., Yaqub B. A., Khoja W. O. Devic's neuromyelitis optica and varicella. J Neurol. 1993 Jul;240(7):450–451. [PubMed] [Google Scholar]

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