항GQ1b 항체 양성인 급성 눈근육마비의 임상양상
Clinical Features of Acute Opthalmoplegia Associated with Anti-GQ1b Antibody
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
목적: 항GQ1b 항체 양성인 급성 눈근육마비환자의 임상양상에 대해서 알아보고자 하였다. 대상과 방법: 2011년 1월부터 2018년 7월까지 급성 복시를 주소로 국내 상급 종합병원에 내원한 환자 중 항GQ1b 항체 양성인 눈근육마비 환자 33명의 의무기록을 후향적으로 조사하였다. 환자들의 임상양상, 항GQ1b 항체검사 결과 및 치료 결과를 비교 분석하였다. 결과: 33명 중 8명(24%)에서 전형적인 눈근육마비, 운동실조, 건반사소실의 세 증상을 모두 보였다. 운동실조 없이 눈근육마비와 건반사소실 및 인두-목-위팔 위약을 보인 경우가 1명(3%) 있었으며, 운동실조나 건반사소실 없이 눈근육마비와 인두-목-위팔 위약을 보인 경우가 2명(6%)이 있었다. 다른 신경학적 증상 없이 급성 눈근육마비만 보인 환자가 22명(67%)이 있었다. 33명의 환자 중 16명(48.5%)에서 대증 치료만 하며 경과 관찰하였고, 17명(51.5%)에서 초기 면역억제치료를 시행하였다. 24명(72.7%)에서 증상 호전이 있었고 9명(27.3%)에서 사시 및 복시 증상이 지속되었다. 발병 후 완전 호전까지 걸린 기간은 평균 2.52 ± 2.11개월이었다. 결론: 항GQ1b 항체 양성 급성 눈근육마비의 임상양상은 탈수초성 신경병증의 다양한 증상이 중첩된 형태로 나타날 수 있으며, 이를 인지하는 것이 급성 눈근육마비 환자의 감별에 도움을 줄 수 있다.
Abstract
Purpose: To investigate the clinical features of acute ophthalmoplegia associated with anti-GQ1b antibody in the Republic of Korea. Methods: From January 2011 to July 2018, we retrospectively reviewed the medical records of all patients who tested positive for anti-GQ1b antibody, and who had been concurrently diagnosed with acute ophthalmoplegia at a tertiary hospital in the Republic of Korea. Ophthalmic and neurological features were carefully reviewed. Laboratory results including ganglioside antibody panels and treatment outcomes were analyzed. Results: Thirty-three patients were enrolled in the study and 8 (24%) of them showed pure Miller-Fisher syndrome with all three cardinal symptoms of ophthalmoplegia, ataxia, and areflexia. One patient (3%) showed ophthalmoplegia, areflexia, and pharyngeal- cervical-brachial weakness without ataxia and 2 patients (6%) showed only ophthalmoplegia and pharyngeal-cervical- brachial weakness without ataxia and areflexia. Twenty-two patients (67%) showed only ophthalmoplegia without any other neurological symptom. Early immunosuppressant treatment was used to treat 17 patients, and observation only was conducted as the initial treatment for 16 patients (48.5%). Twenty-four patients (72.7%) showed improvement and 9 patients showed persistent strabismus and diplopia. The average duration until recovery was 2.52 ± 2.11 months. Conclusions: The symptoms of acute ophthalmoplegia associated with anti-GQ1b antibody can manifest in various forms that overlap with clinical features of demyelinating neuropathy, so it is important to recognize such characteristics when treating acute ophthamoplegia.
참고문헌 (27)
- Fisher M, "An unusual variant of acute idiopathic polyneuritis(syndrome of ophthalmoplegia, ataxia and areflexia)", N Engl J Med, 255, pp.57~65 (1956)
- Sekiguchi Y, "How often and when Fisher syndrome is overlapped by Guillain-Barré syndrome or Bickerstaff brainstem encephalitis?", Eur J Neurol, 23, pp.1058~1063 (2016)
- Nishimoto Y, "Usefulness of anti-GQ1b IgG antibody testing in Fisher syndrome compared with cerebrospinal fluid examination", J Neuroimmunol, 148, pp.200~205 (2004)
- Odaka M, "Anti-GQ1b IgG antibody syndrome : clinical and immunological range", J Neurol Neurosurg Psychiatry, 70, pp.50~55 (2001)
- Friedman DI, "Headache associated with miller fisher syndrome", Headache, 47, pp.1347~1348 (2007)
- Kim JK, "Does delayed facial involvement implicate a pattern of "descending reversible paralysis" in Fisher syndrome?", Clin Neurol Neurosurg, 135, pp.1~5 (2015)
- Mori M, "Clinical features and prognosis of Miller Fisher syndrome", Neurology, 56, pp.1104~1106 (2001)
- Ohe Y, "Fisher syndrome with taste impairment", Intern Med, 51, pp.2977~2979 (2012)
- Tan CY, "Delayed facial palsy in Miller Fisher syndrome", J Neurol Sci, 358, pp.409~412 (2015)
- Yagi Y, "Taste impairment in Miller Fisher syndrome", Neurol Sci, 36, pp.809~810 (2015)
- Koga M, "Pain in Miller Fisher syndrome", J Neurol, 247, pp.720~721 (2000)
- Yuki N, "Acute ophthalmoparesis(without ataxia)associated with anti-GQ1b IgG antibody : clinical features", Ophthalmology, 108, pp.196~200 (2001)
- Ropper AH, "Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness", Arch Neurol, 51, pp.671~675 (1994)
- Van der Meché FG, "Diagnostic and classification criteria for the Guillain-Barré syndrome", Eur Neurol, 45, pp.133~139 (2001)
- Uncini A, "Nodo-paranodopathy : beyond the demyelinating and axonal classification in anti-ganglioside antibody-mediated neuropathies", Clin Neurophysiol, 124, pp.1928~1934 (2013)
- Koga M, "GQ1b-seronegative Fisher syndrome : clinical features and new serological markers", J Neurol, 259, pp.1366~1374 (2012)
- Koga M, "Antecedent infections in Fisher syndrome : a common pathogenesis of molecular mimicry", Neurology, 64, pp.1605~1611 (2005)
- Jacobs BC, "The spectrum of antecedent infections in Guillain-Barré syndrome : a case-control study", Neurology, 51, pp.1110~1115 (1998)
- Ropper AH, "Unusual clinical variants and signs in Guillain-Barrésyndrome", Arch Neurol, 43, pp.1150~1152 (1986)
- Willison HJ, "Mechanisms of action of anti-GM1 and anti-GQ1b ganglioside antibodies in Guillain-Barrésyndrome", J Infect Dis, 176(Suppl 2), pp.S144~149 (1997)
- Chapman J, "Antibodies to ganglioside GM1 in patients with Alzheimer's disease", Neurosci Lett, 86, pp.235~240 (1988)
- Chiba A, "Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome : clinical and immunohistochemical studies", Neurology, 43, pp.1911~1917 (1993)
- Yuki N, "Guillain-Barré syndrome", N Engl J Med, 366, pp.2294~2304 (2012)
- Umapathi T, "Non-demyelinating, reversible conduction failure in Fisher syndrome and related disorders", J Neurol Neurosurg Psychiatry, 83, pp.941~948 (2012)
- Jung JH, "Atypical clinical manifestations of Miller Fisher syndrome", Neurol Sci, 40, pp.67~73 (2019)
- Nishijima H, "Taste impairment in Guillain-Barré syndrome: more frequent than thought?", J Peripher Nerv Syst, 16, pp.270~271 (2011)
- 정재우, "밀러-피셔 증후군의 임상양상과 치료경과", 대한안과학회지, 58(2), pp.197~202 (2017), 대한안과학회