El trastorno del espectro de la neuromielitis óptica es una rara enfermedad sistémica autoinmune, que a veces se asocia a otros trastornos autoinmunes incluyendo el síndrome de Sjögren. Las manifestaciones neurológicas ocurren en 20 a 25% de los casos diagnosticados de síndrome de Sjögren. Sin embargo, menos del 5% de los pacientes con este síndrome debutan con sintomatología neurológica. Se presenta el caso de una mujer adulta mayor con anticuerpos positivos para aquaporina-4 (neuromielitis óptica-IgG) y síndrome de Sjögren que debuta con mielitis longitudinal extensa.

Authors: Carlos Alva Díaz [1,2,3], Luis Andamayo Villalba[1,2], Nicanor Mori[1,4], Jésica Janet Ventura Chilón[1,2], Roberto Romero[1]

Affiliation:
[1] Servicio de Neurología, Departamento de Medicina, Hospital Daniel Alcides Carrión, Callao, Perú
[2] Unidad de Post Grado, Facultad de Medicina, Universidad Nacional Mayor de San Marcos, Lima, Perú
[3] Red de Eficacia Clínica y Sanitaria, REDECS, Lima, Perú
[4] U.S. Naval Medical Research Unit 6, Lima, Perú

E-mail: carlos.alexander.alva@gmail.com

Author address:
[1] Red de Eficacia Clínica y Sanitaria
Urbanización Principal 396
Distrito Surquillo
Lima
Perú

Citation: Alva Díaz C, Andamayo Villalba L, Mori N, Ventura Chilón JJ, Romero R. Neuromyelitis optica spectrum disorders as initial presentation of Sjögren's syndrome: A case report. Medwave 2016 Feb;16(1):e6388 doi: 10.5867/medwave.2016.01.6388

Acceptance date: 24/1/2016

Publication date: 29/2/2016

Origin: not requested

Type of review: reviewed by three external peer reviewers, double-blind

PubMed record

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1. Chiquete E, Navarro-Bonnet J, Ayala-Armas R, Gutiérrez-Gutiérrez N, Solórzano-Meléndez A, Rodríguez-Tapia D, Gómez-Rincón M, Ruiz-Sandoval JL.[Neuromyelitis optica: a clinical update]. Rev Neurol. 2010 Sep 1;51(5):289-94. | PubMed |
2. Mauch E, Völk C, Kratzsch G, Krapf H, Kornhuber HH, Laufen H, et al. Neurological and neuropsychiatric dysfunction in primary Sjögren's syndrome. Acta Neurol Scand. 1994 Jan;89(1):31-5. | PubMed |
3. Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren's syndrome. Nat Rev Rheumatol. 2013 Sep;9(9):544-56. | CrossRef | PubMed |
4. Fox PC. Autoimmune diseases and Sjogren's syndrome: an autoimmune exocrinopathy. Ann N Y Acad Sci. 2007 Mar;1098:15-21. | PubMed |
5. Hammi AR, Al-Hashimi IH, Nunn ME, Zipp M. Assessment of SS-A and SS-B in parotid saliva of patients with Sjögren's syndrome. J Oral Pathol Med. 2005 Apr;34(4):198-203. | PubMed |
6. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. 2004 Jun 28;164(12):1275-84. | PubMed |
7. Tucci M, Quatraro C, Silvestris F. Sjögren's syndrome: an autoimmune disorder with otolaryngological involvement. Acta Otorhinolaryngol Ital. 2005 Jun;25(3):139-44. | PubMed |
8. Segerberg-Konttinen M, Konttinen YT, Bergroth V. Focus score in the diagnosis of Sjögren's syndrome. Scand J Rheumatol Suppl. 1986;61:47-51. | PubMed |
9. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8. | PubMed |
10. Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, et al. American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken). 2012 Apr;64(4):475-87. | PubMed |
11. Kurien B, SR, Sjogren's síndrome 2015 [on line], | Link |
12. Chourkani N, El Moutawakil B, Sibai M, Bourezgui M, Rafai MA, Slassi I.[Primary Sjögren's syndrome and neuromyelitis optica]. Rev Med Interne. 2010 Sep;31(9):e13-5. | CrossRef | PubMed |
13. Tristano AG. A case of Sjögren's syndrome with acute transverse myelitis and polyneuropathy in a patient free of sicca symptoms. Clin Rheumatol. 2006 Feb;25(1):113-4. | PubMed |
14. Manabe Y, Sasaki C, Warita H, Hayashi T, Shiro Y, Sakai K, et al. Sjögren's syndrome with acute transverse myelopathy as the initial manifestation. J Neurol Sci. 2000 Jun 15;176(2):158-61. | PubMed |
15. de Seze J, Stojkovic T, Hachulla E, Breteau G, Michon-Pasturel U, Mounier-Vehier F, et al. [Myelopathy - Sjogren's syndrome association: analysis of clinical and radiological findings and clinical course]. Rev Neurol (Paris). 2001 Jul;157(6-7):669-78. | PubMed |
16. Wingerchuk DM, Hogancamp WF, O'Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology. 1999 Sep 22;53(5):1107-14. | PubMed |
17. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007 Sep;6 9):805-15. | PubMed |
18. Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2):177-89. | CrossRef | PubMed |
19. Wingerchuk DM, Weinshenker BG. The emerging relationship between neuromyelitis optica and systemic rheumatologic autoimmune disease. Mult Scler. 2012 Jan;18(1):5-10. | CrossRef | PubMed |
20. Pittock SJ, Lennon VA, de Seze J, Vermersch P, Homburger HA, Wingerchuk DM, et al. Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol. 2008 Jan;65(1):78-83. | CrossRef | PubMed |
21. Carvalho DC, Tironi TS, Freitas DS, Kleinpaul R, Talim NC, Lana-Peixoto MA. Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu. Arq Neuropsiquiatr. 2014 Aug;72(8):619-24. | PubMed |
22. de Seze J, Stojkovic T, Ferriby D, Gauvrit JY, Montagne C, Mounier-Vehier F, et al. Devic's neuromyelitis optica: clinical, laboratory, MRI and outcome profile. J Neurol Sci. 2002 May 15;197(1-2):57-61. | PubMed |
23. Alhomoud IA, Bohlega SA, Alkawi MZ, Alsemari AM, Omer SM, Alsenani FM. Primary Sjogren's syndrome with central nervous system involvement. Saudi Med J. 2009 Aug;30(8):1067-72. | PubMed |
24. Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004 Sep;83(5):280-91. | PubMed |
25. Escudero D, Latorre P, Codina M, Coll-Cantí J, Coll J. Central nervous system disease in Sjögren's syndrome. Ann Med Interne (Paris). 1995;146(4):239-42. | PubMed |
26. Javed A, Balabanov R, Arnason BG, Kelly TJ, Sweiss NJ, Pytel P, et al. Minor salivary gland inflammation in Devic's disease and longitudinally extensive myelitis. Mult Scler. 2008 Jul;14(6):809-14. | CrossRef | PubMed |
27. Kahlenberg JM. Neuromyelitis optica spectrum disorder as an initial presentation of primary Sjögren's syndrome. Semin Arthritis Rheum. 2011 Feb;40(4):343-8. | CrossRef | PubMed |
28. Tan P, Yu WY, Umapathi T, Lim SA. Severe optic neuritis in a patient with combined neuromyelitis optica spectrum disease and primary Sjögren's syndrome: a case report. J Med Case Rep. 2012 Nov 24;6:401. | CrossRef | PubMed |
29. Beck RW, Cleary PA, Anderson MM Jr, Keltner JL, Shults WT, Kaufman DI, et al. A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med. 1992 Feb 27;326(9):581-8. | PubMed |
30. Sherman E, Han MH. Acute and Chronic Management of Neuromyelitis Optica Spectrum Disorder. Curr Treat Options Neurol. 2015 Nov;17(11):48. | CrossRef | PubMed |

Chiquete E, Navarro-Bonnet J, Ayala-Armas R, Gutiérrez-Gutiérrez N, Solórzano-Meléndez A, Rodríguez-Tapia D, Gómez-Rincón M, Ruiz-Sandoval JL.[Neuromyelitis optica: a clinical update]. Rev Neurol. 2010 Sep 1;51(5):289-94. | PubMed |

Mauch E, Völk C, Kratzsch G, Krapf H, Kornhuber HH, Laufen H, et al. Neurological and neuropsychiatric dysfunction in primary Sjögren's syndrome. Acta Neurol Scand. 1994 Jan;89(1):31-5. | PubMed |

Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren's syndrome. Nat Rev Rheumatol. 2013 Sep;9(9):544-56. | CrossRef | PubMed |

Fox PC. Autoimmune diseases and Sjogren's syndrome: an autoimmune exocrinopathy. Ann N Y Acad Sci. 2007 Mar;1098:15-21. | PubMed |

Hammi AR, Al-Hashimi IH, Nunn ME, Zipp M. Assessment of SS-A and SS-B in parotid saliva of patients with Sjögren's syndrome. J Oral Pathol Med. 2005 Apr;34(4):198-203. | PubMed |

Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. 2004 Jun 28;164(12):1275-84. | PubMed |

Tucci M, Quatraro C, Silvestris F. Sjögren's syndrome: an autoimmune disorder with otolaryngological involvement. Acta Otorhinolaryngol Ital. 2005 Jun;25(3):139-44. | PubMed |

Segerberg-Konttinen M, Konttinen YT, Bergroth V. Focus score in the diagnosis of Sjögren's syndrome. Scand J Rheumatol Suppl. 1986;61:47-51. | PubMed |

Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8. | PubMed |

Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, et al. American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken). 2012 Apr;64(4):475-87. | PubMed |

Kurien B, SR, Sjogren's síndrome 2015 [on line], | Link |

Chourkani N, El Moutawakil B, Sibai M, Bourezgui M, Rafai MA, Slassi I.[Primary Sjögren's syndrome and neuromyelitis optica]. Rev Med Interne. 2010 Sep;31(9):e13-5. | CrossRef | PubMed |

Tristano AG. A case of Sjögren's syndrome with acute transverse myelitis and polyneuropathy in a patient free of sicca symptoms. Clin Rheumatol. 2006 Feb;25(1):113-4. | PubMed |

Manabe Y, Sasaki C, Warita H, Hayashi T, Shiro Y, Sakai K, et al. Sjögren's syndrome with acute transverse myelopathy as the initial manifestation. J Neurol Sci. 2000 Jun 15;176(2):158-61. | PubMed |

de Seze J, Stojkovic T, Hachulla E, Breteau G, Michon-Pasturel U, Mounier-Vehier F, et al. [Myelopathy - Sjogren's syndrome association: analysis of clinical and radiological findings and clinical course]. Rev Neurol (Paris). 2001 Jul;157(6-7):669-78. | PubMed |

Wingerchuk DM, Hogancamp WF, O'Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology. 1999 Sep 22;53(5):1107-14. | PubMed |

Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007 Sep;6 9):805-15. | PubMed |

Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2):177-89. | CrossRef | PubMed |

Wingerchuk DM, Weinshenker BG. The emerging relationship between neuromyelitis optica and systemic rheumatologic autoimmune disease. Mult Scler. 2012 Jan;18(1):5-10. | CrossRef | PubMed |

Pittock SJ, Lennon VA, de Seze J, Vermersch P, Homburger HA, Wingerchuk DM, et al. Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol. 2008 Jan;65(1):78-83. | CrossRef | PubMed |

Carvalho DC, Tironi TS, Freitas DS, Kleinpaul R, Talim NC, Lana-Peixoto MA. Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu. Arq Neuropsiquiatr. 2014 Aug;72(8):619-24. | PubMed |

de Seze J, Stojkovic T, Ferriby D, Gauvrit JY, Montagne C, Mounier-Vehier F, et al. Devic's neuromyelitis optica: clinical, laboratory, MRI and outcome profile. J Neurol Sci. 2002 May 15;197(1-2):57-61. | PubMed |

Alhomoud IA, Bohlega SA, Alkawi MZ, Alsemari AM, Omer SM, Alsenani FM. Primary Sjogren's syndrome with central nervous system involvement. Saudi Med J. 2009 Aug;30(8):1067-72. | PubMed |

Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004 Sep;83(5):280-91. | PubMed |

Escudero D, Latorre P, Codina M, Coll-Cantí J, Coll J. Central nervous system disease in Sjögren's syndrome. Ann Med Interne (Paris). 1995;146(4):239-42. | PubMed |

Javed A, Balabanov R, Arnason BG, Kelly TJ, Sweiss NJ, Pytel P, et al. Minor salivary gland inflammation in Devic's disease and longitudinally extensive myelitis. Mult Scler. 2008 Jul;14(6):809-14. | CrossRef | PubMed |

Kahlenberg JM. Neuromyelitis optica spectrum disorder as an initial presentation of primary Sjögren's syndrome. Semin Arthritis Rheum. 2011 Feb;40(4):343-8. | CrossRef | PubMed |

Tan P, Yu WY, Umapathi T, Lim SA. Severe optic neuritis in a patient with combined neuromyelitis optica spectrum disease and primary Sjögren's syndrome: a case report. J Med Case Rep. 2012 Nov 24;6:401. | CrossRef | PubMed |

Beck RW, Cleary PA, Anderson MM Jr, Keltner JL, Shults WT, Kaufman DI, et al. A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group. N Engl J Med. 1992 Feb 27;326(9):581-8. | PubMed |

Sherman E, Han MH. Acute and Chronic Management of Neuromyelitis Optica Spectrum Disorder. Curr Treat Options Neurol. 2015 Nov;17(11):48. | CrossRef | PubMed |