• 四川大學(xué)華西醫(yī)院神經(jīng)內(nèi)科（成都，610041）;

【摘要】　目的　 分析合并免疫指標(biāo)異常的視神經(jīng)脊髓炎臨床特點(diǎn)。　方法　 回顧性分析2009年5月-2010年11月收治的62例視神經(jīng)脊髓炎患者中24例合并免疫指標(biāo)異?；颊叩呐R床資料。24例均為女性，發(fā)病年齡14~53歲。對(duì)其臨床表現(xiàn)、視覺(jué)誘發(fā)電位、影像學(xué)檢查結(jié)果、免疫檢查結(jié)果進(jìn)行分析。　結(jié)果　 所有患者均有脊髓和視神經(jīng)同時(shí)或先后受累的表現(xiàn)。24例視覺(jué)誘發(fā)電位檢查23例異常。脊髓MRI顯示病變集中于頸段、上胸段脊髓。頸段和胸段脊髓同時(shí)受累17例，單純頸段脊髓受損6例，單純胸段脊髓受損1例。所有患者抗核抗體滴度均≥1∶100，合并抗SSA抗體陽(yáng)性14例（55.5％），同時(shí)合并抗SSB抗體陽(yáng)性11例（45.8％），合并抗Rib抗體陽(yáng)性1例，合并抗SCL-70抗體陽(yáng)性1例，合并抗dsDNA抗體1例。　結(jié)論　 視神經(jīng)脊髓炎合并免疫指標(biāo)異常的患者以女性較為多見(jiàn)，易復(fù)發(fā)，青壯年患者發(fā)病率最高。脊髓MRI示病變集中于頸段、上胸段脊髓，表現(xiàn)為長(zhǎng)節(jié)段脊髓損害。視神經(jīng)脊髓炎患者合并結(jié)締組織病的病例較多。
【Abstract】　Objective　 To analyze the clinical features of neuromyelitis optica (NMO) combined with abnormal immune parameters.　Methods　 We retrospectively reviewed the clinical data of 24 patients with NMO and abnormal immune parameters among the 62 NMO patients who were admitted into our department between May 2009 and November 2010. All patients were female, aged from 14 to 53 years. We analyzed their clinical manifestations, visual evoked potentials, imaging results, and immunological examinations.　Results　 All patients had simultaneous or successive spinal cord and optic nerve involvement. Twenty-three patients had abnormal visual evoked potential. MRI showed that the lesions focused on the cervical and upper thoracic spinal cord. Both cervical and thoracic spinal cord were involved in 17 cases; there were 6 cases of simple cervical spinal cord injury and 1 case of simple thoracic spinal cord damage. Antinuclear antibody titer of all the patients was ≥1∶100. Combined positive anti-SSA antibody occurred in 14 patients (55.5%); Concomitant positive anti-SSB antibodies occurred in 11 patients (45.8%); Combined positive anti-Rib antibodies occurred in 1 patient; Combined positive anti-SCL-70 antibody occurred in 1 patient; and combined positive anti-dsDNA antibodies occurred in 1 patient.　Conclusions　 NMO combined with abnormal immune parameters mainly occurs in female patients, especially in young people. Recurrence rate is high. MRI shows that the lesions focus mainly on the cervical and upper thoracic spinal cord, manifesting the characteristic of long segment damage. And NMO is frequently combined with connective tissue disease.

引用本文： 韓文杰,何曉非,王露,周紅雨. 合并免疫指標(biāo)異常的視神經(jīng)脊髓炎臨床特點(diǎn). 華西醫(yī)學(xué), 2011, 26(11): 1639-1641. doi: 復(fù)制

1. 　Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica[J]. Lancet Neurol, 2007, 6(9): 805-815.
2. 　Wingerchuk DM, Hogancamp WF, O’Brien PC, et al. The clinical course of neuromyelitis optica (Devic’s syndrome)[J]. Neurology, 1999, 53(5): 1107-1114.
3. 　Wingerchuk DM, Lennon VA, Pittock SJ, et al. Revised diagnostic criteria for neuromyelitis optica[J]. Neurology, 2006, 66(10): 1485-1489.
4. 　Wingerchuk DM. Neuromyelitis optica: effect of gender[J]. J Neurol Sci, 2009, 286(1-2): 18-23.
5. 　Ghezzi A, Bergamaschi R, Martinelli V, et al. Clinical characteristics, course and prognosis of relapsing Devic′s neuromyelitis optica[J]. J Neurol, 2004, 251(1): 47-52.
6. 　Nasir S, Kerr DA, Birnbaum J. Nineteen episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus[J]. Arch Neurol, 2009, 66(9): 1160-1163.
7. 　Pellkofer HL, Hohlfeld R, Kuempfel T. Thirty-one episodes of myelitis and optic neuritis in a woman with neuromyelitis optica and systemic lupus erythematosus[J]. Arch Neurol, 2010, 67(6): 779-780.
8. 　江天麗, 鄭金甌. 35例視神經(jīng)脊髓炎的臨床分析[J]. 廣西醫(yī)學(xué), 2007, 11(29): 1787-1788.
9. 　郁冰冰, 劉建勇, 鄧勇, 等. MRI在視神經(jīng)脊髓炎診斷及隨訪觀察中的應(yīng)用價(jià)值[J]. 醫(yī)學(xué)影像學(xué)雜志, 2006, 16(1): 28-29.
10. 　Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis[J]. Lancet, 2004, 364(9451): 2106-2112.
11. 　Poser CM, Brinar VV. Disseminated encephalomyelitis and multiple sclerosis: two different diseases-a critical review[J]. Acta Neurol Scand, 2007, 116(4): 201-206.
12. 　何曉非, 郭端, 周紅雨, 等. 視神經(jīng)脊髓炎臨床特征分析[J]. 四川大學(xué)學(xué)報(bào)(醫(yī)學(xué)版), 2010, 41(5): 919-921.
13. 　Javed A, Balabanov R, Arnason BG, et al. Minor salivary gland inflammation in Devic′s disease and longitudinally extensive myelitis[J]. Mult Scler, 2008, 14(6): 809-814.
  1. 1. 　Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica[J]. Lancet Neurol, 2007, 6(9): 805-815.
  2. 2. 　Wingerchuk DM, Hogancamp WF, O’Brien PC, et al. The clinical course of neuromyelitis optica (Devic’s syndrome)[J]. Neurology, 1999, 53(5): 1107-1114.
  3. 3. 　Wingerchuk DM, Lennon VA, Pittock SJ, et al. Revised diagnostic criteria for neuromyelitis optica[J]. Neurology, 2006, 66(10): 1485-1489.
  4. 4. 　Wingerchuk DM. Neuromyelitis optica: effect of gender[J]. J Neurol Sci, 2009, 286(1-2): 18-23.
  5. 5. 　Ghezzi A, Bergamaschi R, Martinelli V, et al. Clinical characteristics, course and prognosis of relapsing Devic′s neuromyelitis optica[J]. J Neurol, 2004, 251(1): 47-52.
  6. 6. 　Nasir S, Kerr DA, Birnbaum J. Nineteen episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus[J]. Arch Neurol, 2009, 66(9): 1160-1163.
  7. 7. 　Pellkofer HL, Hohlfeld R, Kuempfel T. Thirty-one episodes of myelitis and optic neuritis in a woman with neuromyelitis optica and systemic lupus erythematosus[J]. Arch Neurol, 2010, 67(6): 779-780.
  8. 8. 　江天麗, 鄭金甌. 35例視神經(jīng)脊髓炎的臨床分析[J]. 廣西醫(yī)學(xué), 2007, 11(29): 1787-1788.
  9. 9. 　郁冰冰, 劉建勇, 鄧勇, 等. MRI在視神經(jīng)脊髓炎診斷及隨訪觀察中的應(yīng)用價(jià)值[J]. 醫(yī)學(xué)影像學(xué)雜志, 2006, 16(1): 28-29.
  10. 10. 　Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis[J]. Lancet, 2004, 364(9451): 2106-2112.
  11. 11. 　Poser CM, Brinar VV. Disseminated encephalomyelitis and multiple sclerosis: two different diseases-a critical review[J]. Acta Neurol Scand, 2007, 116(4): 201-206.
  12. 12. 　何曉非, 郭端, 周紅雨, 等. 視神經(jīng)脊髓炎臨床特征分析[J]. 四川大學(xué)學(xué)報(bào)(醫(yī)學(xué)版), 2010, 41(5): 919-921.
  13. 13. 　Javed A, Balabanov R, Arnason BG, et al. Minor salivary gland inflammation in Devic′s disease and longitudinally extensive myelitis[J]. Mult Scler, 2008, 14(6): 809-814.