톨로사 헌트 증후군에 대한 임상적 고찰 = Clinical Observations on Tolosa-Hunt Syndrome

국문 초록 (Abstract)

서론: 톨로사 헌트 증후군 환자들을 대상으로 임상양상 및 치료에 대한 반응, 재발률을 알아보고자 하였다.
대상과 방법: 2003년 1월부터 2007년 10월 사이 동통과 외안근마비로 본원에 내원한 24명의 환자 중 2004년 국제두통학회(International
Headache Society, IHS)의 톨로사 헌트 증후군의 진단기준에 부합하는 6예의 톨로사 헌트 증후군 환자들을 분석하였다.
결과: 모든 환자에서 눈 주위의 모호한 통증이 첫 증상으로 나타난 후 외안근 마비가 곧이어 수반되었으며, 제 3뇌신경 마비가 5명(83.3%)
명으로 가장 많았고, 제 6뇌신경 마비가 3명(50%), 제 4뇌신경 마비가 1명(16.7%), 제 5뇌신경의 1지 마비가 1명(16.7%)이었으며, 두개 이상
의 외안근 마비가 복합적으로 나타난 경우는 2명(33.3%)이었다. 모든 환자에서 스테로이드 고용량 치료를 하였고 72시간 이내에 동통의
소실을 보였다. 외안근의 마비의 완전회복은 평균 2.8개월이었으며 평균 29개월간의 추적관찰 중 2명(33.3%)에서 재발을 보였다.
결론: 톨로사 헌트 증후군은 스테로이드 투여에 신속한 반응을 보이고 완전한 회복이 가능하므로 적절한 안과적, 신경과적, 영상학적
검사를 통한 정확한 진단과 치료가 필수적이며 재발이 잦으므로 치료가 되었다 하더라도 적어도 2년 이상의 장기간의 추적관찰기간이
필요하다.

다국어 초록 (Multilingual Abstract)

Purpose: The authors reviewed clinical features, response to treatment and recurrence rate of Tolosa-Hunt syndrome.
Methods: A retrospective chart review was performed on 6 patients, who fulfilled the diagnosis for Tolosa-Hunt syndrome according
to the International Headache Society (IHS) classification of 2004.
Results: Every patient had orbital pain as a first symptom, followed by cranial nerve paresis. The third cranial nerve was most
commonly involved (83.3%), followed by the sixth nerve (50%), the forth nerve (16.7%), and the first branch of the fifth cranial nerve
(16.7%). Two of the patients showed multiple cranial nerve paresis (33.3%, 2 out of 6). All patients received high-dose steroid therapy
for more than 5 days, and all patients had resolution of orbital pain within 72 hours of treatment. Full recovery of cranial nerve paresis
occurred on average in 2.3 months (3 days to 12 months). During the 29 months of follow-up, 2 patients (33.3%) had a recurrence
episode.
Conclusions: Tolosa-Hunt syndrome responds well to steroid therapy, and full recovery is possible with proper treatment. The exact
diagnosis and treatment of Tolosa-Hunt syndrome is important. Because Tolosa-Hunt syndrome often recurs after full recovery, the
authors suggest a minimum follow-up period of 2 years.

참고문헌 (Reference)

• 1 La Mantia L, "Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria" 26 : 772-781, 2006
• 2 Kline LB, "The Tolosa-Hunt syndrome" 71 : 577-582, 2001
• 3 Headache Classification Subcommittee of the International Headache Society, "The International Classification of Headache Disorders: 2nd edition" 24 : 9-160, 2004
• 4 Hannerz J, "Recurrent Tolosa-Hunt syndrome: a report of ten new cases" 19 : 33-35, 1999
• 5 Tolosa E, "Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm" 17 : 300-302, 1954
• 6 Hunt WE, "Painful opthalmoplegia. Its relation to indolent inflammation of the carvernous sinus" 11 : 56-62, 1961
• 7 Gladstone JP, "Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome" 8 : 321-329, 2004
• 8 Smith JL, "Painful ophthalmoplegia. The Tolosa-Hunt syndrome" 61 : 1466-1472, 1966
• 9 Wroe SJ, "Painful intraorbital meningiomas" 54 : 1009-1010, 1991
• 10 Goto Y, "Neurological and radiological studies in painful ophthalmoplegia: Tolosa-Hunt syndrome and orbital pseudotumour" 236 : 448-451, 1989
• 11 Kasner SE, "Neuro-ophthalmologic aspects of aneurysms" 7 : 679-692, 1997
• 12 Holland D, "Metastatic tumors of the orbit: a retrospective study" 22 : 15-24, 2003
• 13 Leijzer CT, "Meningioma presenting as Tolosa-Hunt syndrome" 101 : 19-22, 1999
• 14 Wasmeier C, "Idiopathic inflammatory pseudotumor of the orbit and Tolosa-Hunt syndrome-are they the same disease?" 249 : 1237-1241, 2002
• 15 Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain, "Headache Classification Committee of the International Headache Society" 8 : 1-96, 1988
• 16 Jacobs D, "Diagnosis and management of orbital pseudotumor" 13 : 347-351, 2002
• 17 Keane JR, "Cavernous sinus syndrome. Analysis of 151 cases" 53 : 967-971, 1996
• 18 Attout H, "Cavernous sinus lymphoma mimicking Tolosa-Hunt syndrome" 21 : 795-798, 2000
• 19 Biousse V, "Aneurysms and subarachnoid hemorrhage" 10 : 631-651, 1999