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Neuromyelitis optica Devic

Neuromyelitis optica Devic

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Zusammenfassung

Neuromyelitis optica Devic ist eine demyelinisierende Autoimmunerkrankung aus dem Formenkreis der multiplen Sklerose mit Befall der Sehnerven und des Rückenmarkes. Wir berichten über eine 22 Jahre alte Patientin mit einem seit einigen Monaten bestehendem langsam progredienten Visusverlust. Beide Sehnerven zeigten eine diffuse Opticusatrophie. Eine Gesichtsfeld-Untersuchung ergab eine bitemporal betonte konzentrische Einschränkung mit einem dichten Zentralskotom rechts. Zusätzlich bestanden neurologische Ausfälle mit einer spastischen Paraparese, beinbetonten Hypästhesien und Hypalgesien sowie eine diskrete Ataxie. Neuroradiologische Untersuchungen mittels Magnetresonanztomographie zeigten eine massive, kontrastmittel-speichernde Verdickung des Chiasma opticum und des rechten Sehnervs sowie längliche hyperintense Areale in der Medulla cervicalis. Trotz hochdosierter Cortisontherapie kam es zu keiner Verbesserung der ophthalmologischen und neurologischen Symptomatik.

Summary

Optic Neuromyelitis is a demyelinating disease of autoimmunological origin similar to multiple sclerosis. We report a case of a 22-year old woman with progressive visual loss in both eyes. Neuro-ophthalmic examination showed bilateral diffuse optic atrophy and bitemporal constriction of visual fields with right central scotoma. Clinical neurological investigation revealed spastic paraparesis, hypesthesia and hypalgesia of the limbs as well as ataxia. Magnetic resonance imaging of the brain showed besides enlargement of the chiasm and the right optic nerve no pathology. Spinal cord scans demonstrated hyperintense signals in the cervical medulla. There was no change in neurologic and ophthalmic state in spite of treatment with high doses of steroids.

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Literatur

  1. Aguilera AJ, Carlow TJ, Smith KJ, et al (1985) Lymphocyto-plasmapheresis in Devic’s syndrome. Transfusion 25(1): 54–56

    Article  PubMed  CAS  Google Scholar 

  2. Aimoto Y, Ito K, Morikawa F, et al (1991) Demyelinating peripheral neuropathy in Devic disease. Jpn J Psychiatry Neurol 45(4): 861–864

    PubMed  CAS  Google Scholar 

  3. Arnold TW, Myers GJ (1987) Neuromyelitis optica (Devic syndrome) in a 12-year-old male with complete recovery following steroids. Pediatr Neurol 3(5): 313–315

    Article  PubMed  CAS  Google Scholar 

  4. Barbieri F, Buscaino GA (1989) Neuromyelitis optica in the elderly. Acta Neurol Napoli 11(4): 247–251

    PubMed  CAS  Google Scholar 

  5. Barkhof F, Scheltens P, Valk J, et al (1991) Serial quantitative MR assessment of optic neuritis in a case of neuromyelitis optica, using Gadolinium-“enhanced” STIR imaging. Neuroradiology 33(1): 70–71

    Article  PubMed  CAS  Google Scholar 

  6. Breukelman AJ, Polman CH, de Siegte RG, et al (1988) Neuromyelitis optica (Devic’s syndrome): not always mutiple sclerosis. Clin Neurol Neurosurg 90(4): 357–360

    Article  PubMed  CAS  Google Scholar 

  7. Bruguier A, Ponsot G, Khouri Dagher L, et al (1981) Neuromyelitis optica or Devic disease complicating chickenpox. Arch Fr Pediatr 38(10): 763–764

    PubMed  CAS  Google Scholar 

  8. Ch’ien LT, Medeiros MO, Belluomini JJ, et al (1982) Neuromyelitis optica (Devic’s syndrome) in two sisters. Clin Electroencephalogr 13(1): 36–39

    PubMed  Google Scholar 

  9. Chusid MJ, Williamson SJ, Murphy JV, et al (1979) Neuromyelitis optica (Devic disease) following varicella infection. J Pediatr 95(5 Pt 1): 737–738

    Article  PubMed  CAS  Google Scholar 

  10. Filley CM, Sternberg PE, Norenberg MD (1984) Neuromyelitis optica in the elderly. Arch Neurol 41(6): 670–672

    PubMed  CAS  Google Scholar 

  11. Ghezzi M, Giansanti M, Malentacchi GM, et al (1987) Neuromyelitis optica in the old age: a clinico-pathological contribution. Ital J Neurol Sci 8(6): 613–616

    Article  PubMed  CAS  Google Scholar 

  12. Glaser JS (1990) Topical diagnosis: prechiasmal visual pathways. In: Tasman W, Jaeger EA (eds) Clinical ophthalmology, vol 2, chap 5, pp 44

  13. Glaser JS (1990) Topical diagnosis: the optic chiasm. In: Tasman W, Jaeger EA (eds) Clinical ophthalmology, vol 2, chap 6, pp25

  14. Guthoff R, Onken C, Triebel HJ (1991) Möglichkeiten der kernspintomographischen Herddarstellung bei demyelinisierenden Erkrankungen des orbitalen Nervus opticus. Fortschr Ophthalmol 88(1): 49–52

    PubMed  CAS  Google Scholar 

  15. Ko FJ, Chiang CH, Jong YJ, et al (1989) Neuromyelitis optica (Devic’s disease) report of one case. Acta Paediatr Sin 30(6): 428–431

    PubMed  CAS  Google Scholar 

  16. Lefkowitz D, Angelo JN (1984) Neuromyelitis optica with unusual vascular changes. Arch Neurol 41: 1103–1105

    PubMed  CAS  Google Scholar 

  17. Leonardi A, Arata L, Farinelli M, et al (1987) Cerebrospinal fluid and neuropathological study in Devic’s syndrome. Evidence of intrathecal immune activation. J Neurol Sci 81(1–3): 281–290

    Article  Google Scholar 

  18. Leys D, Petit H, Block AM, et al (1987) Devic’s optic neuromyelitis. 4 cases. Rev Neurol Paris 143(11): 722–728

    PubMed  CAS  Google Scholar 

  19. Piccolo G, Franciotta DM, Camana C, et al (1990) Devic’s neuromyelitis optica: long-term follow-up and serial CSF findings in two cases. J Neurol 237(4): 262–264

    Article  PubMed  CAS  Google Scholar 

  20. Purvin V, Herr GJ, De Myer W (1988) Chiasmal neuritis as a complication of Epstein-Barr virus infection. Arch Neurol 45: 458–460

    PubMed  CAS  Google Scholar 

  21. Ramelli GP, Deonna T, Roulet E, et al (1992) Transverse myelitis and optic neuromyelitis in children. Apropos of 3 case reports. Schweiz Rundsch Med Prax 81(20): 661–663

    PubMed  CAS  Google Scholar 

  22. Sadun AA, Yanoff M (1990) Pathology of the optic nerve. In: Duane TD, Jaeger EA (eds) Biomedical foundations of ophthalmology, vol 3, chap 16, pp 7

  23. Tashiro K, Ito K, Maruo Y, et al (1987) MR imaging of spinal cord in Devic disease. J Comput Assist Tomogr 11(3): 516–517

    Article  PubMed  CAS  Google Scholar 

  24. Waespe W, Haenny P (1987) Bitemporal hemianopia due to chiasmal optic neuritis. Neuro-Ophthalmology 7: 69

    Article  Google Scholar 

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Authors and Affiliations

  1. Universitäts-Augenklinik Graz, Auenbruggerplatz 4, A-8036, Graz, Österreich

    J. Berglöff

  2. Universitätsklinik für Radiologie, Deutschland

    G. Ranner

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  1. J. Berglöff
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  2. G. Ranner
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Berglöff, J., Ranner, G. Neuromyelitis optica Devic. Spektrum Augeheilkd 8, 142–146 (1994). https://doi.org/10.1007/BF03163923

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