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1 대한안과학회지제 47 권제 7 호 2006 J Korean Ophthalmol Soc 47(7): , 2006 임산부에서시력저하로발견된림프구성뇌하수체염 1예 김상우권정도 박도훈 왈레스기념침례병원안과 목적 : 시력저하를주소로내원한임산부에서발견된림프구성뇌하수체염 1 례를보고하고자한다. 대상과방법 : 임신 36주인 31세여자가 3주간의두통과서서히진행되는시력저하및시야장애를주소로내원하였다. 최대교정시력은우안 0.02, 좌안안전수지 20 cm 이었으며, 양안에상대구심성동공운동장애는없었고, 안압은우안 14 mmhg, 좌안 15 mmhg 이었다. 두부 MRI 소견상뇌하수체에종괴가관찰되었다. 결과 : 비기능성뇌하수체거대샘종이의심되어접형동을통한수술(transsphenoidal approach, TSA) 을시행하였고, 술중조직검사상림프구성뇌하수체염으로진단되어부분절제술과함께뇌하수체기능저하에대한내과적인치료를병행하였다. 술후 5주째최대교정시력은양안 1.0 으로회복되었고, 시야검사도정상소견을보였으며, 술후 7개월째까지재발의소견은보이지않았다. 결론 : 임산부에서뇌하수체종괴가있을때, 뇌하수체샘종및배아종, 림프종, 결핵, 랑게르한스조직구등의감별진단외에본증례와같이발생빈도는드물지만, 림프구성뇌하수체염도주의깊게감별진단에반드시포함시켜야할것이다. < 한안지 47(7): , 2006> 뇌병변은시력및시야변화등의안과적증상과연관되는경우가흔한데, 1 특히터어키안및안배상병변 (suprasellar lesion) 은그위치상시신경의교차부위에있으면서시신경의압박으로인한특징적인시신경교차증후군을야기할수있다. 2 이러한시신경교차증후군에서는주로두통이나양이측반맹, 편측또는양측색각및시력저하, 상대구심성동공운동장애등의안과적증상을호소하여병원을찾게되고, 환자의전신증상및혈액학적검사, 신경학적검사, 뇌전산화단층촬영또는뇌자기공명영상등의신경영상을통해병변의크기, 성격, 치료방향을결정하게된다. 그러나, 이러한검사들을통한병변에대한진단은간혹오류를나타낼때가있는데, 이것은치료방침의결정에따라예후가크게달라지는뇌병변의성격상큰문제가아닐수없다. 본원에서는 3주간서서히진행하는두통및시력저하, 시야장애를주소로안과로내원해내분비학적, 뇌자기공명영상소견상비기능성뇌하수체거대샘종이의 < 접수일 : 2006년 1월 18 일, 심사통과일 : 2006년 5월 25 일> 통신저자 : 박도훈부산시금정구남산동 왈레스기념침례병원안과 Tel: , Fax: oph@wmbh.co.kr 심되던임산부에서술후조직검사상임상적으로매우드문질환인림프구성뇌하수체염으로진단된 1예를경험하였기에문헌고찰과함께보고하고자한다. 증 임신 36주인 31세여자가내원 3주전부터간간히발생하는두통과함께서서히진행되는시력저하를주소로본원안과를방문하였다. 환자는 11년전갑상선암으로갑상선전절제술을받고현재까지갑상선약을복용중이었으며, 임신이후철분제제를복용하고있는것외에는특이사항은없었다. 음주나흡연의과거력은없었으며, 이번이첫임신이었고, 가족력상특이사항은없었다. 환자는전두부와측두부쪽으로방사되는중등도의두통이내원 3 주전부터시작되었으며, 구토증은동반되지않았고, 하루중수시간지속되는양상이었다고한다. 또, 환자는내원 1주전부터보는것이갑자기흐려지기시작하였다고하였다. 내원시환자의최대교정시력은우안 0.02, 좌안안전수지 20 cm 이었고, 동공은양안 3 mm 로상대구심성동공운동장애는없었다. Humphery 자동시야계에의한시야검사상우안은상외측의일부분을제외하고는완전맹소견이었으며좌안은완전맹소견을보였다 (Fig. 1). 례 1183

2 김상우외 : 림프구성뇌하수체염 Figure 1. Findings from automated perimetry (Humphrey 30-2 threshold program) show nearly total defect sparing only supratemporal area in the right eye and total defect in the left eye on gray scales. 안압은우안 14 mmhg, 좌안 15 mmhg 이었으며, 산동검사를통한후극부검사상시신경유두부위의충혈이나부종은보이지않았으며, 황반부도특이소견은없었다. 광간섭단층촬영 (OCT) 상에서도시신경부위와황반부의특이소견은보이지않았다. 뇌병변이의심되어신경영상검사를실시하였는데, 두개골 X-선소견에서터어키안의확장소견과 Sella MRI 소견에서뇌하수체샘및뇌하수체줄기의비후가관찰되었고, 이비후된종괴는크기가 cm (6.8 cc) 으로시신경교차부위를압박하고있었으며, T1 가중영상에서강한초기조영증강소견을보였다(Fig. 2). 뇌하수체호르몬검사에서는 ACTH 68.8 pg/ml, TSH 0.17 uiu/ml, FSH 0.0 miu/ml, LH 0.0 miu/ml, prolactin ng/ml, GH 0.11 ng/ml, T ng/ml, free T ng/ml, cortisol ug/dl로각각측정되었다. 임상적으로드물게젖분비호르몬분비샘종 (prolacti noma) 에서역설적으로 prolactin 수치의감소가있을수있으나, 3 정상적인임신 3기의 prolactin 수치에비해비정상적인수준으로떨어진점으로보아비기능성뇌하수체거대샘종과뇌하수체염이의심되었고, 이들질환에대한감별이필요하였다. Figure 2. A simple skull X-ray shows enlarged sella turcica (A). T1-weighted MR images demonstrate an isosignal intense, homogenous, intrasellar mass with suprasellar extension (B, C) and strong homogenous enhancement of the lesion after the administration of gadolinium-dtpa (D). 1184

대한안과학회지제 47 권제 7 호 2006 년 누두신경뇌하수체염 (infundibuloneurohypophy sitis) 인경우 MRI 소견상 T1 가중영상에서의특징적인후엽의고강도신호소실로뇌하수체샘종과감별이가능하지만, 4,5 본증례의경우에는 T1 가중영상에서강한초기조영증강소견만을보여 MRI상으로는두질환의감별이어려웠으며,

Permanent section pathology of the pituitary mass demonstrates infilteration of the pituitary tissue with lymphocytes. (H&E, 400) 복용중이어서이차적 TSH 분비감소를배제할수없었으므로비기능성뇌하수체거대샘종으로추정진단하였다.

3 대한안과학회지제 47 권제 7 호 2006 년 누두신경뇌하수체염 (infundibuloneurohypophy sitis) 인경우 MRI 소견상 T1 가중영상에서의특징적인후엽의고강도신호소실로뇌하수체샘종과감별이가능하지만, 4,5 본증례의경우에는 T1 가중영상에서강한초기조영증강소견만을보여 MRI상으로는두질환의감별이어려웠으며, 단지내분비학적으로뇌하수체염에의한뇌하수체기능저하증에서는 ACTH 또는 TSH 분비의감소가다른뇌하수체호르몬분비감소에선행되는것에반해비기능성뇌하수체거대샘종인경우생식샘자극호르몬또는 GH 분비의감소가 ACTH나 TSH 분비의감소에선행하는것으로알려져있고, 4 본증례의경우기존의갑상선약을 Figure 3. Permanent section pathology of the pituitary mass demonstrates infilteration of the pituitary tissue with lymphocytes. (H&E, 400) 복용중이어서이차적 TSH 분비감소를배제할수없었으므로비기능성뇌하수체거대샘종으로추정진단하였다. 시야장애와시력소실이임박하여제왕절개술과함께경접형동접근법으로수술을시행하였는데, 종괴가단단하고박리가용이하지않았다. 술중시행한생검조직의병리검사상림프구의침윤및섬유화소견이관찰되었으며, 뇌하수체선종은관찰되지않았고, 건락괴사나육아종괴같은결핵의소견은없었고, 뇌하수체에서원발성으로발생하는 X-조직구증및배아종(germinoma) 을의심할만한세포도관찰되지않았다(Fig. 3). 이와같은조직학적소견으로림프구성뇌하수체염이확진되었고, 종괴에대한부분절제만을시행하였다(Fig. 4). 술후스테로이드투여와함께뇌하수체기능부전에대한내과적치료를병행하였다. 환자는특별한신경학적증상이나합병증없이회복되었으며, 수술후 7일째시행한추적호르몬검사상혈청 TSH, LH, FSH의감소외에는특이소견은없었다. 환자의시력은술후 6일째양안 0.3 이었으며, 술후 5주째최대교정시력은양안 1.0으로호전되었다. 시야검사에서는술후 6일째부터정상소견을나타내었다 (Fig. 5). 6 여성호르몬, 갑상선호르몬및스테로이드대체요법을시행하며술후 7개월까지재발의소견없이통원가료중여성호르몬대체요법에의한합병증으로생각되는자궁내막증이발생하여부인과적으로경과관찰중이다. 고 찰 림프구성뇌하수체염은자가면역에의한염증성질환으로알려져있으며, 7,8 매우드물고병변의위치에따라선뇌하수체염과누두신경뇌하수체염으로분류되어진다. 4,5,9,10 Figure 4. Saggital (A) section (T2-weighted image) on postoperative 1 week shows fat packed into the sella turcia and optic chiasm decompressed. The increased signal intensity of the sellar contents and sphenoid sinus in gadolinium-dtpa enhanced saggital (B) and coronal (C) sections (T1-weighted image) is due to a combination of subacute hemorrhage and fat. 1185

4 김상우외 : 림프구성뇌하수체염 Figure 5. Findings from automated perimetry (Humphrey 30-2 threshold program) after surgery revealed marked resolution of previous defects. 선뇌하수체염은 1962 년 Goudie and Pinkerton 11 에의해 22세여자환자에서첫증례가보고된질환으로, 뇌하수체기능저하와관련이있는것으로알려져있다. 이질환은주로여자에서임신말기나출산후에발생한다고하며뇌하수체종괴와함께두통및구토, 시력저하및시야장애와뇌하수체기능부전증등이나타나며주로뇌하수체의전엽에림프구성침윤, 뇌하수체선세포의파괴와섬유화가특징이다 모체사망률은약 50% 에달하는것으로알려져있고, 조직병리학적으로는주로뇌하수체전엽에림프구성침윤및뇌하수체선의파괴소견이특징적인것으로알려져있다. 10,15-18 가장흔한임상증상은두통이며, 시야장애의경우약 32% 정도에서관찰된다. 19 그외뇌하수체기능저하에따른여러증상이나타날수있는데, 오심, 식욕부진, 구토, 체중감소, 어지러움, 출산후무월경등이있을수있다. 10 방사선학적영상에서종괴의터키안상부확장소견은약 64% 에서관찰된다고하며, 자기공명영상에서보이는특징적인소견으로는시상하부로설상의확장과뇌하수체줄기의비후등이있으며, 뚜렷한조영증강, 중심성괴사에의한윤상의조영증강, 병소부의삼각형의조형증강, 안격막의조영증강등을보일수있 다 뇌하수체염의내분비학적소견으로는범뇌하수체기능저하증이흔하고, 특히 ACTH와 TSH 저하의빈도가높은데, 이는자가면역질환과연관성이높은것으로알려져있으며, 누두신경뇌하수체염의경우요붕증도발생하는것으로보고되고있다. 9,19 또한임신에관계없이고프로락틴혈증이뇌하수체염환자에서드물지않게보고되고있는데이의원인으로추정되는가설로첫째, 뇌하수체종괴가뇌하수체줄기를압박함으로써 prolactin 억제제인 dopamine 이뇌하수체전엽으로전달되는것이감소되기때문이고, 둘째, 염증반응으로인해 prolactin 분비억제에관여하는 dopamine 수용체에직접적인변화가발생하기때문이며, 셋째, 염증반응으로인한전반적인조직파괴로야기된 prolactin 의직접적인혈관내누출을들수있다. 10 본증례에서의 prolactin 의비정상적인감소는 FSH, LH와함께염증에의해이미뇌하수체전엽조직이전반적으로파괴된것에기인한것으로사료되었다. 이질환의원인은아직정확히알려진바는없으나, Levine 23 이백서를이용한실험적연구에서동종의뇌하수체조직을주입하여림프구성뇌하수체염을유발함으로써자가면역이중요한병인이라고주장하였다. 또 1186

5 대한안과학회지제 47 권제 7 호 2006 년 한, 갑상선염, 부신염, 난소염, 고환염등과같은다른자가면역질환과연관되어발생하는경우가있고, 24 드물지만뇌하수체에대한자가항체를포함한여러자가항체들이발견되는것으로보아자가면역에의한염증성질환으로생각된다. 7,8 이러한림프구성뇌하수체염과의감별진단으로는뇌하수체샘종, 육아종, 조직구증, 매독, 결핵, 과립성뇌하수체염등이있다. 3,25 진단은주로환자의증상과자가면역성질환의유무, 신경영상학적소견및내분비학적소견에의하며생검에의해확진된다. 임상적으로환자의증상및신경영상검사로는감별이어려운경우가많고자가면역성질환의병발은매우드문것으로알려져있다. 일반적으로, 림프구성뇌하수체염에대한치료는내과적으로스테로이드가사용될수있으며감별진단의수단으로도이용되고있고, 3,26-28 내분비학적으로뇌하수체기능부전에의한전신적합병증이나종괴로인한시력소실및시야장애가심할경우에는수술적부분절제와함께내과적치료를병행하는것으로보고되고있다. 10,14,29 최근에스테로이드단독에의한치료성적이보고되고있는데, 염증의억제로종괴의감소와질병의진행억제를유도하는것으로나타났으며, 뇌하수체저하증으로부터의회복을나타낸경우도있었다. 26,27 그러나, 염증에의한조직파괴가진행되어섬유화가심한경우에는스테로이드치료에반응을잘안하는것으로나타났다. 14,28 또한, 스테로이드치료후감량시질병의재발이흔한것으로나타났으며, 결과적으로질병의경과를길게한다는문제점이대두되었다 그래서, 몇몇저자들은림프구성뇌하수체염의경우진단및뇌하수체기능의보존을위하여조기에수술을시행하고수술후병소가남고증상이악화되는경우에는스테로이드의투여로치료를한다고보고하고있다. 3,30 수술후뇌하수체전엽의기능부전이회복된예도보고된바가있으나, 대부분은염증성변화에의한뇌하수체의파괴로인하여수술후에도기능부전은지속되는것으로알려져있다. 31 저자들은임신말기여성에서뇌하수체종괴와함께뇌하수체기능부전이있을경우뇌하수체샘종및배아종, 림프종, 결핵, 랑게르한스세포조직구등의감별진단외에, 본증례와같이발생빈도는드물지만림프구성뇌하수체염도주의깊게감별진단에반드시포함되어야할것으로사료되어보고하였다. 참고문헌 1) Min IS, Jung HS, Kim MW. A clinical case report of right occipital lobe infarction. J Korean Ophthalmol Soc 1982;23: ) Mejico LJ, Miller NR, Dong LM. Clinical features associated with lesions other than pituitary adenoma in patients with an optic chiasmal syndrome. Am J Ophthalmol 2004;137: ) Kerrison JB, Lee AG, Weinstein JM. Acute loss of vision during pregnancy due to a suprasellar mass. Surv Ophthalmol 1997;41: ) Hashimoto K, Takao T, Makino S. Lymphocytic adenohypophysitis and lymphocytic infundibuloneurohypophysitis. Endocr J 1997;44: ) Son YJ, Wang KC, Choe GY, et al. Lymphocytic infundibuloneurohypophysitis: case report and review of the literature. J Korean Neurosurg Soc 2000;29: ) Kerrison JB, Lynn MJ, Baer CA, et al. Stages of improvement in visual fields after pituitary tumor resection. Am J Ophthalmol 2000;130: ) Ozawa Y, Shishiba Y. Recovery from lymphocytic hypophysitis associated with painless thyroiditis: clinical implications of circulating antipituitary antibodies. Acta Endocrinol 1993;128: ) Nishiyama S, Takano T, Hidaka Y, et al. A case of postpartum hypopituitarism associated with empty sella: possible relation to postpartum autoimmune hypophysitis. Endocr J 1993;40: ) Kojima H, Nojima T, Nagashima K, et al. Diabetes insipidus caused by lymphocytic infundibuloneurohypophysitis. Arch Pathol Lab Med 1989;113: ) Thodou E, Asa SL, Kontogeorgos G, et al. Clinical case seminar: lymphocytic hypophysitis: clinicopathological fingings. J Clin Endocrinol Metab 1995;80: ) Goudie RB, Pinkerton PH. Anterior hypophysitis and Hashimoto's disease in a young women. J Pathol Bacteriol 1962;83: ) Patel MC, Guneratne N, Haq N. Peripartum hypopituitarism and lymphocytic hypophysitis. QJM 1995;88: ) Abe T, Matsumoto K, Sanno N. Lymphocytic hypophysitis: case report. Neurosurgery 1995;36: ) Nishioka H, Ito H, Miki T, et al. A case of lymphocytic hypophysitis with massive fibrosis and the role of surgical intervention. Surg Neurol 1994;42: ) Sautner D, Saeger W, Ludecke DK, et al. Hypophysitis in surgical and autoptical specimens. Acta Neuropathol 1995;90: ) Jenkins PJ, Chew SL, Lowe DG, et al. Lymphocytic hypophysitis: unusual features of a rare disorder. Clin Endocrinol 1995;42: ) Magner JA, West RL. Lymphocytic hypophysitis. West J Med 1994;160: ) Naik RG, Ammini A, Shah P. Lymphocytic hypophysitis. J Neurosurg 1994;80: ) Cho CS, Park BJ, Kim YJ, et al. Lymphocytic hypophysitis: case report. J Korean Neurosurg Soc 2003;34: ) Powrie JK, Powell M, Ayers AB, et al. Lymphocytic adenohypophysitis: Magnetic resonance imaging features of two new cases and a review of the literature. Clin Endocrinol 1995;42:

6 김상우외 : 림프구성뇌하수체염 21) Ahmadi J, Meyers GS, Segall HD, et al. Lymphocytic adenohypophysitis: contrast-enhanced MR imaging in five cases. Radiology 1995;195: ) Levine SN, Benzel EC, Fowler MR, et al. Lymphocytic adenohypophysitis: clinical, radiological, and magnetic resonance imaging characterization. Neurosurgery 1988;22: ) Levine S. Allergic adenohypophysitis: new experimental disease of the pituitary gland. Science 1967;158: ) Lim DJ, Chung YG, Lee HK, et al. Lymphocytic hypophysitis: a case report. J Korean Neurosurg Soc 1998;27: ) Yu DK, Choi MY. Clinical assessment of the patients diagnosed with intracranial lesion at an ophthalmology clinic. J Korean Ophthalmol Soc 2004;45: ) Feigenbaum SL, Martin MC, Wilson CB, et al. Lymphocytic adenohypophysitis: a pituitary mass lesion occurring in pregnancy. Proposal for medical treatment. Am J Obstet Gynecol 1991;164: ) Beressi N, Cohen R, Beressi JP, et al. Pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroid alone: first case report. Neurosurgery 1994;35: ) Reusch JE, Kleinschmidt-DeMasters BK, Lillehei KO, et al. Preoperative diagnosis of lymphocytic hypophysitis (adenohypophysitis) unresponsive to short course of dexamethasone: case report. Neurosurgery 1992;30: ) Skandarajah A, Ng WH, Gonzales M, et al. Lymphocytic hypophysitis mimicking pituitary macroadenoma. J Clin Neurosci 2002;9: ) Honegger J, Fahlbusch R, Bornemann A, et al. Lymphocytic and granulomatous hypophysitis: experience with nine cases. Neurosurgery 1997;40: ) Bitton RN, Salvin M, Decker RE, et al. The course of lymphocytic hypophysitis. Surg Neurol 1991;36:40-3. =ABSTRACT= A Case of Lymphocytic Hypophysitis during Pregnancy Sang Woo Kim, M.D., Jung Do Kwon, M.D., Do Hoon Park, M.D. Department of Ophthalmology Wallace Memorial Baptist Hospital, Pusan, Korea Purpose: To report a rare case of lymphocytic hypophysitis in a 31-year-old woman who presented with gradually progressive bilateral visual loss during the third trimester of pregnancy. Methods: Ophthalmologic examination revealed best corrected visual acuity of 0.02 OD and counting fingers at 20 cm OS. Pupil examination revealed no relative afferent pupillary defect in either eye and intraocular pressure was normal in both eyes. A visual field test revealed nearly total visual defect sparing superotemporal area OD and total defect OS. An MRI of the head was performed. Results: At the time of transsphenoidal surgery following the patient's delivery, a frozen biopsy of the lesion revealed diffuse lymphocytic infilteration and fibrosis of the pituitary gland consistent with the diagnosis of lymphocytic hypophysitis. Postoperatively the patient was treated for hypopituitarism. At 5 weeks postoperative, her best corrected visual acuity was 1.0 OU, and visual field defects resolved in both eyes. On follow-up by telephone for postoperative 7 months, the patient remained visually asymptomatic. Conclusions: The clinical presentation of lymphocytic hypophysitis may mimic pituitary adenoma, lymphoma, germinoma, and histiocytosis. The diagnosis should be suspected in any pregnant or postpartum patient with an intrasellar or suprasellar mass. J Korean Ophthalmol Soc 47(7): , 2006 Key Words: Lymphocytic hypophysitis, Pituitary adenoma, Pregnancy Address reprint requests to Do Hoon Park, M.D. Department of Ophthalmology Wallace Memorial Baptist Hospital # Namsan-dong, Kumjung-gu, Pusan , Korea Tel: , Fax: , oph@wmbh.co.kr 1188

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<31382EB1E8BFEBC0CF2E687770> 대한내과학회지 : 제 69 권제 5 호 2005 라트케열낭종과동반된림프구성뇌하수체염 1예 을지의과대학교내과학교실, 신경외과학교실 2 2 김용일 전재석 김영지 박상원 변영혜 이종용 강희인 =Abstract= A case of lymphocytic hypophysitis associated with Rathke's cleft cyst Yong Il Kim, M.D.,