증 례 online ML Comm 대한간질학회지 2009;13(1):22-26 중심이랑부위에서발생한난치성간질의수술적치료의 1 례 오세미 1,2 서대원 1,3 주은연 1,3 홍승철 2,3 홍승봉 1,3 성균관대학교의과대학삼성서울병원뇌신경센터, 3 신경과학교실, 1 신경외과학교실 2 Surgical Treatment of Intractable Epilepsy Originating from Central Region: A Case Report Semi Oh, M.D. 1,3, Dae-Won Seo, M.D., Ph.D. 1,3, Eun Yeon Joo, M.D., Ph.D. 1,3, Seung-Chyul Hong, M.D., Ph.D. 2,3 and Seung Bong Hong, M.D., Ph.D. 1,3 Departments of Neurology, 1 Neurosurgery 2 and Neuroscience Center, 3 Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Localization of epileptogenic zone in central epilepsy is difficult because of extent and rapidity of ictal spread. Epilepsy surgery in central region is challenging because of the risk of sensorimotor deficits related to excision of eloquent areas. We present a case of 27- year-old woman with a successful surgical treatment for intractable epilepsy from the central region. (J Korean Epilep Soc 2009;13(1): 22-26) KEY WORDS: Epilepsy Central epilepsy Epilepsy surgery Perirolandic epilepsy. 서 론 비관자엽간질 (extratemporal lobe epilepsy) 은간질환자중 20~55% 에달하는것으로알려져있으며, 1-3 항간질약의치료에발작이조절되지않을경우간질수술을생각해볼수있다. 그러나아직비관자엽간질수술은간질조절효과가안쪽관자엽간질 (mesial temporal lobe epilepsy) 만못하고수술후유증역시높아간질수술을신중하게결정해야한다. 특히, 일차운동-감각중추를포함하는중심구부위간질은수술후운동 -감각마비등의심각한신경결손을초래할수있기때문에보다정확한병소의국소화와철저한수술전평가를필요로한다. 1 중심구와인접한뇌기능부위의병변에대해서는이전부터뇌기능평가후외과적절제수술을시행해왔다. 최근발달된간질수술전검사후시행한중심구주변의수 Received 19 April 2009 Accepted 27 July 2009 Corresponding author: Dae-Won Seo, M.D., Ph.D., Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul 135-710, Korea E-mail: daewon@skku.edu 술을받은 52명환자에서 27명 (51.9%) 에서후유증이없었고, 16명 (31%) 에서간질발작이없었다. 3 그러나국내에서는새겉질간질 (neocortical epilepsy) 의겉질절제술 (neocortical resection) 에대해서구체적인증례로제시되지는않았다. 최근에는신경영상 (neuroimaging) 과침습적뇌파 (invasive EEG) 의발달에힘입어간질병소를정확히확인하게되었고, 경막하전극설치후수술장외에서정확히뇌기능을평가하여보다안전한간질수술이가능하게되었다. 4,5 저자들은중심구병변에서발생한난치성간질환자에서최근의신경영상방법인 subtracted ictal SPECT coregistered to MRI (SISCOM) 및삼차원적영상합치술을이용하여간질발생부위를정확히국소화한후에, 두개강내경막하전극 (subdural electrode) 을이용한기능적지도화를시행하여운동기능을확인하고성공적으로간질수술을시행하여이에대하여보고하고자한다. 증례 간질발작을갖는 27세여자가수술적치료를위하여 2005년 10월본원으로전원되었다. 발작은 12년전부터 22 대한간질학회지 2009;13(1):22-26
오세미 서대원 주은연등 목안에무엇인가걸리고조이는느낌이들은후입이좌측으로당겨지면서고개가좌측으로편향되다가전신적발작으로진행하였다. 단순부분발작은 carbamazepine CR, topiramate, clobazam 및 levetiracetam 등복수의항간질약치료에도평균이틀에한번발생하고, 이차적전신발작역시연간 4회정도발생하였다. 신경계진찰은정상이었고, 임상신경심리검사에서주의집중력, 언어적및시각적기억력에서보통수준보다다소저하되었고, 경미한우측이마엽 (frontal lobe) 실행능력저하가관찰되었다. 혈액검사에서 carbamazepine 은 12.7 μg/ml 로측정되었다. 외래에서시행한일반뇌파는정상이었고뇌 MRI 에서우측이마엽의겉질에경계가분명한조양증강되지않는병변이관찰되었다 (Figure 1A and D). 수술전검사로시행한 4일간의비디오-뇌파집중감시검사에서발작간 (interictal) 간질파 (epileptic discharges) 는관찰되지않았고간질발작은 3회기록되었다. 발작은병력과같이목주위 ( 후두 ) 가저린전조로시작하여말을하고싶어도혀를움직일수없어말을할수없다가좌측안면강직 성발작이일어나며, 1회에서전신적강직간대성발작으로진행하기도하였다. 발작중뇌파 (ictal EEG) 에서는뚜렷한이상을관찰할수없었다. 발작중시행한 SPECT 를이용한 SISCOM 에서우측하-이마엽종양부위의바로후상방에서혈류증가가관찰되었다 (Figure 1B and C). 병변과과혈류부위의관계는 3차원영상합치술로중심이랑 (central sulcus) 전후에각각위치하는것을확인할수있었다 (Figure 1E and F). 기능적 MR 검사상언어중추는좌측에위치하였다. 경막하전극을뇌영상의병변과과혈류부위를중심으로두개의 4 8 격자전극 (grid electrode) 을삽입후 7 일간비디오-뇌파검사를시행하였다 (Figure 2A and B). 전극의정확한위치는수술중촬영한사진과 MRI-CT 영상합치술을이용한 3차원입체영상을검토하여확인할수있었다 (Figure 2C). 발작중간질파는 K3, H10 부위에서평균시간당 1회관찰되었다. 이전과같은간질발작은 16회기록되었는데전극 K4 와 K12 에서국소적으로시작되어 K3-6 과 K11-14 로퍼지면서주변전극으로파급되는발작중간질파를얻을수있었다 (Figure 2D). 경 A B C D E F Figure 1. Preoperative imaging findings of the patient. A and D: T2-weighted coronal and axial MR images show a well-demarcated lesion in the inferior part of right precentral gyrus (red arrow). B and C: Subtraction of ictal SPECT coregistered to MR images reveal ictal hyperperfusion in cortical and subcortical areas just around the lesion. E and F Three-D rendering images demonstrate the lesion (green area) in the precentral gyrus and the hyperperfused area (red area) just posterior to the central sulcus (yellow line). 대한간질학회지 2009;13(1):22-26 23
Central Epilepsy A B C D E Figure 2. Results of invasive study of the patient. A and B: Intraoperative photographic pictures show the exposed right pericentral area and the location of the subdural electrode arrays. C: The relation of all the electrodes to the central sulcus and the lesion is indicated on 3-D rendering MRI. D: Ictal electrocorticographic recording demonstrates the focal ictal onset over the lower bank of the precentral area [K4, K12 electrodes; D(1)], spreading to the surrounding regions [K3-6, K11-14; D(2), D(3)]. E: The results of direct electrical cortical stimulation show the electrodes with positive sensory or motor functions (especially face sensation on K4, larynx motor on K12). 막하전극에서시행한뇌자극검사에서인두 (larynx), 혀, 손가락, 손목, 등운동영역과두부및상지의체성감각영역과이마엽안구영역 (frontal eye field) 을확인할수있었다 (Figure 2E). 간질발생부위인전극 K4, K12 를포함한종양절제술을시행하였으며 (Figure 3A-C), 병리학적으로배아형성장애신경상피종양 (dysembryoplastic neuroepithelial tumor, DNT) 이확인되었다 (Figure 3D). 수술후 1년이지난현재 carbamazepine CR 300 mg bid와 topiramate 100 mg bid 복용하며전조와발작이모두없는 Engel class I의결과를보이고있다. 6 간혹피곤할때수초간혀좌측부위에둔한느낌이드는것이외에기능적이상은없었다. 고찰 약물치료로호전되지않는난치성간질환자는수술 적치료를고려하게되는데연령, 병변의유무, 위치및원인에따라수술방법이다를수있다. 또한수술결과도다를수있다. 1 일반적으로병변이있는난치성간질에서중심이랑주변에병변이위치할경우수술후운동및감각기능결손에대한우려로수술을쉽게결정하기어렵다. 본증례는이러한중심이랑주변병변에의한간질환자에서병변위치확인및뇌기능평가에의해효과적으로간질수술을시행할수있다는예가될수있다. 본증례에서 SISCOM 에서우측하-이마엽종양부위후상부의혈류증가가관찰되었고, 병변과과혈류의위치를삼차원합치영상에서확인할수있었다. SISCOM에서관찰된과혈류부위는발작중뇌파의발작시작부위 (ictal onset zone; K4, K12) 보다발작중뇌파가성장된 (builtup) 부위 (K3-6, K11-14) 와일치하였다. 일반적으로새겉질간질 (neocortical epilepsy) 에서 SISCOM의특이도 (specificity) 는약 60% 로알려져있다. 7 중심간질은운동영역과간질발생부위와의관계를정확히파악하는것 24 대한간질학회지 2009;13(1):22-26
오세미 서대원 주은연등 A B Figure 3. Post-operative features. A and C: Post-operative follow-up MRI show complete removal of the lesion in the right precentral gyrus. B: Intraoperative view shows the removed region. D: Histopathologic finding demonstrates oligodendroglia-like cells and floating neurons in myxoid matrix, indicating dysembryoplastic neuroepithelial tumor (HE, 200). C D 이필요한데 SISCOM 은이러한면에서한계가있을수있으며본증례는발작시작부위보다발작성장부위와일치하는것을보여주었다. 이전연구에의하면 DNT 는발작유발부위가병변내보다는병변주변에위치하는경우가더많고, 여러개의유발병변을갖는경우가많은것으로보고되었다. 8 본증례에서는발작파가시작되는지점이병변내에위치하였다. 이처럼 DNT 에서수술전침습적발작중뇌파기록을이용해서정확히간질발생부위를국소화하는것이중심간질수술에서필요하다. 본증례의경우침습적경막하전극삽입후시행한뇌자극검사에서발작시뇌파가시작되었던위치에해당하는 K4는안면감각그리고 K12 는후두발화운동부위였다. 중심병변수술에서중심앞이랑 (precentral gyrus) 안의병변위치에따라수술결과가다를수있는데, 수술결과가좋은부위는 precentral gyrus의하부 (lower bank) 와안면기능과관련된부위였다. 이러한부위의국소절제후좋은결과를보인 Engel 분류 class I 및 II는 60~75% 인데비해, 9,10 이에비하여상부 (upper bank) 와상지및하지기능과관련된부위절제술후결과는 class I 및 II가 46% 로상대적으로나빴다. 3 본증례에서병변이중심앞이랑부위하부에위치한안면감각과인두운동이관여한곳으로수술후간질발작은물론주요운 동감각기능결손도없었다. 중심구간질은일차감각및일차운동영역이분포하는기능적으로중요한부위로이부위에간질병변이위치할경우, 수술전검사로정확한위치의파악및경막하전극에서기록된뇌파및기능적지도를통해간질병소와기능부위를확인하여정교한간질수술을시행할수있다. 본증례는특히병변이중심구하부에위치할경우좋은결과를예상할수있다라는례가될수있다. REFERENCES Cascino GD. Surgical treatment for extratemporal epilepsy. Curr Treat Options Neurol 2004;6:257-62. Kutsy RL. Focal extratemporal epilepsy: Clinical features, EEG patterns, and surgical approach. J Neurol Sci 1999;166:1-15. Pondal-Sordo M, Diosy D, Tellez-Zenteno JF, Girvin JP, Wiebe S. Epilepsy surgery involving the sensory-motor cortex. Brain 2006; 129:3307-14. Cohen-Gadol AA, Britton JW, Collignon FP, Bates LM, Cascino GD, Meyer FB. Nonlesional central lobule seizures: Use of awake cortical mapping and subdural grid monitoring for resection of seizure focus. J Neurosurg 2003;98:1255-62. Lee CC, Ward HA, Sharbrough FW, et al. Assessment of functional MR imaging in neurosurgical planning. Am J Neuroradiol 1999; 20:1511-9. Engel J, Jr., Burchfiel J, Ebersole J, et al. Long-term monitoring for epilepsy. Report of an IFCN committee. Electroencephalogr Clin Neurophysiol 1993;87:437-58. Lee SK, Lee SY, Yun CH, Lee HY, Lee JS, Lee DS. Ictal SPECT in neocortical epilepsies: Clinical usefulness and factors affecting the 대한간질학회지 2009;13(1):22-26 25
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