CASE REPORT J Neurocrit Care 2015;8(1):25-29 ISSN 정맥내스테로이드투여후빠른회복을보인 Marchiafava-Bignami disease 국립중앙의료원신경과 정진우ㆍ황인건ㆍ박혜성ㆍ고임석ㆍ김현경 Rapid Recove

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1 CASE REPORT ISSN 정맥내스테로이드투여후빠른회복을보인 Marchiafava-Bignami disease 국립중앙의료원신경과 정진우ㆍ황인건ㆍ박혜성ㆍ고임석ㆍ김현경 Rapid Recovery from Marchiafava-Bignami Disease after Intravenous Corticosteroid Administration Jin Woo Jung, MD, In gun Hwang, MD, Hye Sung Park, MD, Im-Seok Koh, MD, and Hyun Kyung Kim, MD Department of Neurology, National Medical Center, Seoul, Korea Background: Marchiafava-Bignami disease (MBD) is a rare disorder characterized by acute demyelination of the corpus callosum in people with chronic alcoholism. Early thiamine treatment can reduce neurological complications of MBD. However, the outcome of MBD is usually unfavorable. Here we report a case of MBD responded to intravenous corticosteroid administration. Case Report: A 46-year-old woman with chronic alcoholism presented to our emergency department with two days of dysarthria, dysphagia, extremity weakness, and decreased consciousness. Diffusion-weighted image and fluid attenuated inversion recovery revealed high signal intensities of the left frontal cortex and the entire corpus callosum. After receiving high dose intravenous thiamine and multivitamin, she did not improve. At the third day of admission, intravenous steroid was started. Her mental status and weakness were rapidly improved. At 2 weeks later, she could walk independently. Follow-up MRI revealed only a small lesion in the splenium of corpus callosum. Conclusion: The MBD in our case is associated with thiamine deficiency with main pathology of edema and demyelination of corpus callosum. Corticosteroid stabilized blood brain barrier and reduced inflammation and edema. Therefore, corticosteroid therapy might have beneficial effects as an alternative therapy for MBD. Key Words: Marchiafava-Bignami disease; Corticosteroid; Extracallosal involvement 서 론 Marchiafava-bignami disease (MBD) 는만성알코올중독이 나영양실조와관련되어발생하는드물고치명적인질환으 로, 병리학적으로뇌량 (corpus callosum) 의괴사 (necrosis) 및탈 수초 (demyelination) 가특징이다. 1 임상적으로는경련, 구음장 애, 사지마비, 보행장애등의신체증상및의식저하, 환각, 망 상, 반구간해리증상 (interhemispheric disconnection symptoms) Received: January 23, 2015 / Revised: March 11, 2015 Accepted: March 11, 2015 Address for correspondence: Hyun Kyung Kim, MD Department of Neurology, National Medical Center, 245 Eulji-ro, Jung-gu, Seoul , Korea Tel: , Fax: twinspica@medimail.co.kr 등의정신증상을보인다. 1,2 확진은사망후병리학적인소견을통해가능하지만, 흔히뇌자기공명영상 (magnetic resonance imaging, MRI) 의특징적인소견을통해진단된다. 보존적치료외에 MBD의정립된치료는없으나일반적으로티아민투여가좋은예후와관련이있으며 3 스테로이드에반응을보인증례보고들도있다. 3-8 국내에서도티아민투여에반응을보인여러보고들이있으며 9-11 스테로이드치료를시도한증례도있으나임상호전을보이지는못했다. 12 저자들은 MBD 환자에서정맥내스테로이드투여를통해좋은경과를보였던예를경험하여문헌고찰과함께보고한다. 증례 46 세여자가내원 2-3 일전부터아급성으로발생하여진행 Copyright 2015 The Korean Neurocritical Care Society 25

하는혼동 (confusion), 구음장애, 사지위약을주소로응급실에내원하였다. 환자는약 6년전부터하루에소주 2-3병정도를매일마시는만성알코올중독자 (alcoholic) 였으며, 평소식사를제대로하지않았다. 수차례알코올전문병원에서입원치료를받았으며다른특이병력은없었다.

2 하는혼동 (confusion), 구음장애, 사지위약을주소로응급실에내원하였다. 환자는약 6년전부터하루에소주 2-3병정도를매일마시는만성알코올중독자 (alcoholic) 였으며, 평소식사를제대로하지않았다. 수차례알코올전문병원에서입원치료를받았으며다른특이병력은없었다. 내원당시혈압은 155/90 mmhg, 맥박수는 103회 / 분, 호흡수 18회 / 분, 체온은 36.6 였다. 매우마른체형이었으며신경학적진찰에서의식은명료하였으나스스로말하기 (spontaneous speech) 가매우감소하였고 1단계명령 (one stage command) 에도따르지못했다. 외안근 (extraocular muscle) 운동의제한이나안진은관찰되지않았다. 구음장애, 연하곤란을보였으며, 우측상지의근력은 Medical Research Council (MRC) grade 2, 좌측상지는 grade 4, 양하지는모두 grade 3으로측정되었다. 심부건반사는전반적으로감소하였으며, 근위약을고려하였을때소뇌실조 (cerebellar ataxia) 는없었고수막자극징후 (meningeal irritation sign) 도없었다. 혈액검사상 aspartate aminotransferase (AST) 는 43 IU/L, gamma glutamyl transferase (γ-gt) 는 74 IU/L로약간상승하 A B C D E F Figure 1. Initial brain imaging of the patient. Computerized tomography (A, B) reveals low attenuation on genu and splenium of the corpus callosum (arrows). Diffusion-weighted image (C, D) demonstrates high signal intensities on the corpus callosum and left frontal cortex (arrow head). On fluid attenuated inversion recovery (FLAIR) image (E, F), signal changes in the corresponding areas are shown. 26

3 Corticosteroid and Marchiafava-Bignami Disease Jung JW, et al. 였고혈당은 105 mg/dl이었다. 혈액요소질소, 크레아티닌수치, 암모니아는정상이었다. 소변검사상케톤체가 1+ 로관찰되었으며그외특이소견은없었다. 뇌전산화단층활영 (computerized tomography, CT) 에서뇌량의저감쇠 (low attenuation) 를보였으며 (Fig. 1A, 1B) MRI 의확산강조영상 (diffusion weighted image) 에서뇌량의무릎 (genu), 몸통 (body), 뇌량팽대 (splenium) 모두에서고신호강도 (hyperintensity) 를보였다 (Fig. 1C). 또한좌측대뇌피질에서도고신호강도가관찰되었다 (Fig. 1D). 액체감쇠역전회복 (fluid attenuated inversion recovery, FLAIR) 에서는주로뇌량의같은부위에서고신호강도를보였다 (Fig. 1E, 1F). 만성음주의과거력과의식변화, 구음장애, 혼동등의중추신경계증상및뇌량의병변등을종합하여볼때 MBD에합당하다고진단하여티아민및복합비타민의정맥내투여를시작하였다. 이틀후환자의의식은간단한명령시행이가능할정도로다소호전을보였으나구음장애, 연하곤란, 사지위약감은변화가없었다. 이에덱사메타손 (dexathetasone) 을정주로하루 10 mg 추가하였다. 투약바로다음날부터위약감, 구음장애가호전되기시작했으며스스로말하기도증가하였다. 약일주일후에는비위관 (Levin tube) 을제거하였고보조기를이 용하여보행이가능해졌으며 2주후에는보조기없이스스로보행하였다. 내원 16일에시행한신경심리검사에서간이정신상태검사 (MMSE) 는 26점으로장소지남력에서 1점, 주의집중및계산에서 1점, 기억회상 1점, 오각형에서 1점이감점되었다. 시각적기억및전두엽수행기능 (frontal executive function) 장애를보였으나언어기억은정상범위였으며실어증및뇌량절단증후군 (callosal disconnection syndrome) 은보이지않았다. 덱사메타손은총 2주간투약되었으며내원 3주후시행한뇌 MRI에서는뇌량팽대의일부를제외한대부분영역에서이전에보이던고신호강도가사라졌다 (Fig. 2). 이후스스로일상생활및집안일이가능한상태로외래에서경과관찰중이다. 고찰 MBD는 1903년병리학자인 Victor 와 Ropper 13 가 3명의알코올중독환자에게서뇌량의변화를기술하면서처음으로알려지게되었다. 주로중년이나노년의성인남자에게많으며만성알코올중독이나영양결핍과관련있다. 3,13 현미경상에서병변은주로뇌량의중간층 (middle lamina) 에국한되며수초 A B C D Figure 2. Follow-up imaging three weeks after treatment. Diffusionweighted image (A, B) and fluid attenuated inversion recovery image (C, D) show only a small lesion in the splenium of the corpus callosum. The other callosal lesions and left premotor cortical lesions are nearly disappeared. 27

4 (myelin) 의손실과풍부한대식세포 (macrophage), 그리고별아교세포 (astrcyte) 의증식을관찰할수있다. 13 병리학적으로뇌량의탈수초와괴사를보이는데, 왜 MBD에서뇌량의손상이발생하는지에대한기전은명확하지않다. 3 MBD는뇌 CT에서뇌량의저밀도 (hypodence) 소견을보이며, 뇌 MRI는급성기에서확산강조영상과 T2 강조영상에서고신호강도 (hyperintence) 와부기 (swelling) 를관찰할수있으며, 만성기에서는 T1 강조영상에서동일부위의저신호강도 (hypointence) 및낭성변화, 위축등이관찰된다. 2 확산강조영상은뇌량의세포독성부종 (cytotoxic edema) 을가장먼저반영하기때문에 MBD 진단의초기지표가된다. 3 MBD는뇌량이외의부위를침범하기도하는데뇌량과인접한백질부위를침범하거나 13 대뇌피질부위를침범하기도하며, 1 대체적으로속섬유막 (internal capsule), 대뇌부챗살 (corona radiate), 소뇌등은보존된다. 13 병리학적으로피질판상경화증 (cortical laminar sclerosis) 이라고하여전두엽과측두엽피질세번째층에있는신경세포 (neuron) 들이사라지고섬유신경교증 (fibrous gliosis) 로대체된다. 13 어떤이유로대뇌피질이알코올중독이나비타민결핍등에취약한지는명확하지않지만, 동물실험에서티아민결핍이전두엽피질의글루탐산염 (glutamate) 의흡수를감소시키는것이관찰되었다. 1 광범위한대뇌피질병변은나쁜예후를야기한다는보고가있으며, 1 뇌량외 (extracallosal) 병변은나쁜예후의지표가된다고보고되었지만아직까지논쟁의여지가있다. 2 MBD의치료는보존적치료외에티아민투여가가장많이행해지고있다. 3 티아민부족은수초 (myelin sheath) 를감소시키고, 젖산염 (lactate) 의침착을유발하며, 자유라디칼 (free radical) 을생산하여신경흥분독성 (neuroexcitotoxicity) 을일으켜신경세포의괴사를초래한다. 티아민투여로이러한과정을막는것이 MBD에서티아민치료의근거이다. 14 티아민은증상발생 2주안에투여하는것이효과적이며티아민치료가늦어지면예후가나빠진다고알려져있다. 3 티아민의용량은베르니케뇌병증의치료용량과동일한용량이추천되며, 치료기간은회복을보일때까지지속하는것이추천된다. 3 티아민결핍외에탈수초및염증반응이 MBD의주요발병기전임을고려할때스테로이드가효과적일수있다. 2000년에 Kikkawa 등 6 이처음으로 MBD에서정맥내스테로이드투여로호전된사례를보고한이후여러증례가보고되었다. 3-5,7,8 스테로이드는뇌부종 (brain edema) 을감소시키고, 탈수초를억제한다. 3 림프구생성을억제하고 T세포의세포자멸사 (apoptosis) 및아라키돈산 (arachidonic acid) 과여러염증물질을억제하여염증반응을감소시킨다. 3,4 또한혈관투과성 (vasogenic permeability) 증가를줄여혈액뇌장벽 (blood-brain barrier) 을 안정시키는작용을한다. 3 일부에서는티아민에는반응이없 고스테로이드투여후뚜렷한임상호전을보인보고들도있 었다 본증례의경우티아민의초기효과는뚜렷하지않았으나 정맥내스테로이드투여이후임상적호전을보였다. 또한내 원당시심한신경학적증상과함께뇌량외병변을동반하여 예후가나쁠것으로예상되는상황에서도치료후경도의인 지저하외에신경학적합병증을최소화할수있었다. 그러나 현재까지연구에서티아민의효과가나타나는시점은불분명 하며상기환자는치료기간대부분에서티아민과스테로이드 가동시에투여되었으므로스테로이드단독효과보다는두가 지모두가임상경과에긍정적인영향을준것으로추정된다. 스테로이드용량과기간은기존증례들에의하면메틸프레 드니솔론 (methylprednisolone) 500 mg 에서 1 g 을 5 일간정맥내 로투약하는충격요법 (pulse therapy) 을주로사용하였으며 4,5,7 3 주간투약하기도하였다. 12 본증례에서는환자가저체중이 었기때문에중간용량의스테로이드를사용하였고임상경과 에따라약 2 주정도투여하였다. 현재 MBD 의스테로이드치 료에대한정립된용법이없으므로향후이에대한연구가필 요하다. 현재까지의연구를종합해볼때티아민에비해스테로이 드의긍정적인효과가일관되지는않다. 3 다만증례들에서스 테로이드투여가뚜렷한부작용을초래하지않았으며 3 MBD 가다양한신경학적합병증을초래하는질환임을고려할때 티아민투여후임상적호전이불분명한경우정맥내스테로 이드치료를시도하는것이환자예후에도움이될것으로생 각된다. REFERENCES 1. Namekawa M, Nakamura Y, Nakano I. Cortical involvement in Marchiafava-Bignami disease can be a predictor of a poor prognosis: a case report and review of the literature. Intern Med 2013;52: Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava-Bignami disease. J Neurol 2004;251: Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and management of Marchiafava-Bignami disease: a review of CT/MRI confirmed cases. J Neurol Neurosurg Psychiatry 2014;85: Gerlach A, Oehm E, Wattchow J, Ziyeh S, Glocker FX, Els T. Use of high-dose cortisone in a patient with Marchiafava- Bignami disease. J Neurol 2003;250: Tao H, Kitagawa N, Kako Y, Yamanaka H, Ito K, Denda K, et al. A case of anorexia nervosa with Marchiafava-Bignami disease that responded to high-dose intravenous corticosteroid 28

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Lumbar spine

Lumbar spine CT 32 111 DOI : 10.3831/KPI.2010.13.2.111 Lumbar Spine CT 32 Received : 10. 05. 23 Revised : 10. 06. 04 Accepted : 10. 06. 11 Key Words: Disc herniation, CT scan, Clinical analysis The Clinical