시신경을 침범한 밀러피셔증후군
A Case of Miller Fischer Syndrome With Optic Nerve Involvement
1
조회
0
다운로드
6
인용
0
참고문헌
키워드
초록
목적: 시신경을 침범한 밀러피셔증후군 1예를 경험하였기에 이를 보고하고자 한다. 증례 요약: 74세 여자환자가 한 달 전 발생한 양안 시력저하를 주소로 내원하였다. 환자는 한 달 전 갑자기 구음장애, 좌측의 근력저하가 발생하였고 수일 후에 구음장애와 연하곤란이 심해지면서 외전장애와 안검하수가 발생하였다. 항GQ1b항체에 대한 혈청학적 검사가 양성이어서 밀러피셔증후군으로 확진하였으며, 5일간 타과에서 면역글로불린치료를 시행하였다. 발병 한달 후에도 복시, 시력저하, 보행장애가 계속되어 안과에 내원하였다. 양안 최대교정시력은 0.5 이었고 양안 눈꺼풀처침과 내측편위가 있었으며, 안구운동장애가 관찰되었다. 안저검사에서 양안 경도의 시신경유두이측창백이 보였다. 양안에 색각이상이 있었고, 시유발전위검사에서 진폭감소, 잠복 기지연이 있었다. 발병 두 달 후부터 안과적 증상이 호전되었고, 네 달 후엔 교정시력이 0.8로 호전되고, 눈운동 장애와 눈꺼풀처짐이 완전히 회복되었으며, 시유발전위검사의 이상소견도 거의 회복되었다. 결론: 밀러피셔증후군이 시신경을 침범하는 것은 대단히 드문 경우로 발병 4개월 후에 회복된 증례 1예를 경험하였기에 이를 보고하고자 한다.
Abstract
Purpose: To report an extremely rare case of optic nerve involvement in Miller-Fisher syndrome. Case Summary: A 74-year-old woman presented to our clinic with decreased visual acuity in both eyes. Such symptoms started 1 month prior to her visit, after sudden onset of left-side motor power weakness and dysphasia. Following the initial symptoms, our patient consecutively experienced worsening dysphagia, ptosis, and lateral gaze limitation. We confirmed the diagnosis as Miller Fischer syndrome with a positive anti-GQ1b antibody serology. She received immunoglobulin treatment for 5 days in other departement. She visited our clinic due to continuing diplopia, decrease of visual acuity and gait disturbance. On initial examination, the corrected visual acuity was 0.5 in both eyes. Ptosis of both eyelids, esodeviation, limitation in ocular movement was noted. The fundoscopic examinations revealed relatively pale optic disc. There was decrease in color vision in both eyes. The visual evoked potential test showed low amplitude and delayed latency in P100 wave in both eyes. Two months after her initial visit to our department her symptoms started to improve, and after 4 months all the initial problems resolved completely and her corrected visual acuity checked out to be 0.8 in both eyes and VEP abnormality was recovered. Conclusions: It is extremely uncommon for Miller Fisher syndrome to involve the optic nerve. We present such a case of a 74-year-old woman whose symptoms improved 4 months after its first attack with treatment.
참고문헌 (19)
- Fischer M, "An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia)", N Engl J Med, 255, pp.57~65 (1956)
- Chiba A, "Ganglioside composition of the human cranial nerves, with special refrence to pathophysiology of Miller Fischer syndorme", Brain Res, 745, pp.32~36 (1997)
- Berlit P, "Miller Fischer syndrome. Review of the literature", J Clin Neurophthalmol, 12, pp.57~63 (1992)
- LI H, "Miller Fisher syndrome: toward a more comprehensive understanding", Chin Med J, 104, pp.235~239 (2001)
- Toshniwal P, "Demyelinating optic neuropathy with Miller Fischer syndrome: the case for overlap syndromes with central and peripheral demyelination", J Neurol, 234, pp.353~358 (1987)
- Colding-Jorgensen E, "Visual impairment in anti-GQ1b positive Miller Fischer syndrome", Acta Neurol Scand, 103, pp.259~260 (2001)
- Chan J, "Optic neuritis in anti-GQ1b positive recurrent Miller Fischer syndrome", Br J ophthalmol, 87, pp.1185~1186 (2003)
- Lo YL, "Clinical and immunological spectrum of the Miller Fischer syndrome", Muscle Nerve, 36, pp.615~627 (2007)
- Roberts M, "Serum factor in Miller Fischer varient of Guillain-Barre syndrome and neurotransmitter release", Lancet, 343, pp.454~455 (1994)
- Buchwald B, "Pre- and postsynaptic blockade of neuromuscular transmission by Miller Fischer syndrome IgG at mouse motor nerve terminals", Eur J Neurosci, 10, pp.281~290 (1998)
- Odaka M, "nti-GQ1b IgG antibody syndrome: clinical and immunological range", J Neurol Neurosurg Psychiatry, 70, pp.50~55 (2001)
- Goodall JA, "Effect of corticosteroids on course of Guillain-Barre syndrome", Lancet, 1, pp.524~526 (1974)
- Zifko U, "High dose immunoglobulin therapy is effective in the Miller Fischer syndrome", J Neurol, 241, pp.178~179 (1994)
- Yeh JH, "Miller Fischer syndrome with central involvement: successful treatment with plasmapheresis", Ther Apher, 3, pp.69~71 (1993)
- Son MK, "Miller Fischer syndrome", J Korean Ophthalmol Soc, 38, pp.1470~1479 (1997)
- 안재환, "동공근을 침범한 항 GQ1b 항체 양성의 비전형적 밀러피셔증후군 1예", 대한안과학회지, 50(4), pp.645~648 (2009), 대한안과학회
- Yuki N, "An immunologic abnormality common to Bickerstaff’s brain stem encephalitis and Fischer’s syndrome", J Neurol Sci, 118, pp.83~87 (1993)
- Robbins MS, "Optic neuritis and palatal dysarthria as presenting features of post-infectious GQ1b antibody syndrome", Clin Neurol Neurosurg, 111, pp.465~466 (2009)
- Mater H, "Central nervous system pathology in patients with the Guillain-Barrë syndrome", Brain, 120, pp.451~464 (1997)