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124 Sang-Ku Park, et al. Hemifacial Spasm Caused by Brain Tumor CASE REPORT Korean J Clin Lab Sci. 2013, 45(3):124-129 pissn 1738-3544 eissn 2288-1662 Hemifacial Spasm Caused by Brain Tumor Sang-Ku Park 1, Soon-Chul Hyun 1, Sung-Hyuk Lim 1, Chan-Woo Park 1, Jin-Woo Park 1, Dong-Jun Kim 1, Ki-Eob Kim 2, and Gi-Bong Kim 1 1 Department of Neurology Laboratory, Samsung Medical Center, Seoul 135-710, Korea 2 Daejeon Health Sciences College, Daejeon 300-711, Korea Separating of the facial nerve caused by compression near the blood vessels that cause the blood vessels and surgery when the hemifacial spasm, facial spasms, will disappear. These impacts have occurred very rarely and seen in this paper as facial spasms due to a brain tumor. The size of a brain tumor grows, which will put pressure on the surrounding facial spasm. Treated hemifacial spasm symptoms disappear through the removal of a brain tumor that occurs because saw. Keywords: Hemifacial spasm, Brain tumor Corresponding author: Sang-Ku Park Department of Neurology Laboratory, Samsung Medical Center, 50 Ilwon-dong, Gangnam-gu, Seoul 135-710, Korea. Tel: 82-2-3410-2737 E-mail: sk39.park@samsung.com This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Copyright 2013 The Korean Society of Clinical Laboratory Science. All rights reserved. Received: July 26, 2013 Revised: August 26, 2013 Accepted: August 29, 2013 서론얼굴의한쪽면에본인의의지와는상관없이경련이생기는상태를반측성안면경련 (Hemifacial Spasm, HFS) 이라고하며, 안면신경이분포하는얼굴근육에간헐적이고돌발적으로수축이일어나는운동기능항진증상을말한다. HFS는 7번뇌신경안면신경이뇌혈관에의해압박을받아발생하는것으로신경가닥들간에합선현상을발생시켜안면떨림현상이나타나는것으로알려져있다 (Gardner, 1962; Park 등, 2011). 정상에서는우리가생각하는대로전기의흐름을조절하여움직이고싶은안면근육만을움직일수있다. 반측성안면경련의경우뇌혈관에의한안면신경압박이점차심해지면안면신경내의신경가닥들이점차손상을받게되어신경가닥들사이에서합선현상이나타나며이로인해서자기의사와무관하게한쪽얼굴에경련이발생하게된다. 하나의근육이움직이면동시에여러근육을작동시키는동시수축성이중요한특징으로나타난다. 이러한동시수축성은안면신경의일부신경섬유에서전달되는활동전위가병변부위에서다른신경섬유로측방향전파가일어나면서발생하게된다. 안면근육에근전도검사를하면특징적인측면전파반응 (Lateral Spread Response, LSR) 을기록할수있고, 수술중에지속적인검사를하여서측면전파반응이원인혈관과안면신경의감압에의해소실되는지를관찰함으로써수술이잘되었는지의효과를수술중 바로확인할수있다. 수술중청신경이나안면신경의손상등을방지하기위하여수술중집중감시검사 (Intra-operative Neurophysiological Monitoring, INM) 를이용하여청력손상이나안면마비의발생가능성을최소화하며, 수술이잘되었는지의수술효과를수술중즉시확인할수있다 (Hatem 등, 2001; Kong, 2007). 수술중집중감시검사는청신경손상을예방하기위해뇌간청각유발전위 (Brainstem Auditory Evoked Potentials; BAEP) 검사를하고, 안면마비의예방을위해안면신경근전도 (Facial Nerve Electromyography; FNEMG) 검사를하고, 수술이잘되었는지의효과를확인하기위해측면전파반응 (Lateral Spread Response, LSR) 검사를하여평가할수있다 (Kakizawa, 1990; Lee, 2009). 수술장에서사용하는신경계추적감시 (INM) 검사장비는 Xltek Protektor32 (Natus Medical Incorporated DBA Excel-Tech Ltd, XLTEK, Canada) 를사용하였고, 마취과에서사용하는마취장비는흡입마취장비로전신마취기 (anesthesia machine), 환기기 (ventilator), 제거체계 (scavenging system) 로구성되어있는 GE/Datex-Ohmeda Aestiva/5 (Hoja de Ventas en Español) 장치를사용하였다 (Park 등, 2012) (Fig. 1). 이러한반측성안면경련현상의발생원인이뇌혈관의직접적인영향이아니라뇌신경주위의종양에의해발생하는경우도있다. 뇌종양의크기가점점커지면서주변에있는안면신경을압박하게되어발생하는것으로매우보기드문현상으로본논문에서두가

Korean J Clin Lab Sci. Vol. 45, No. 3, Sep. 2013 125 Fig. 1. GE/Datex-ohmeda aestiva/5. Fig. 3. Pre operation BAEP. Fig. 2. Pre operation MRI. 지증례를다루어보려한다. 증례 1. 증례 1 반측성안면경련환자의일반적인수술적요법으로는미세혈관감압술 (Microvascular decompression, MVD) 이라는수술을시행하는데전신마취하에귀뒤쪽의유양돌기뒷부분에약 2 3 cm 정도의머리를여는개두술 (Craniotomy) 을통하여뇌혈관에의해압박되고있는신경근기수부를확인한후뇌혈관을분리해내고 Teflon-felt 라고하는수술재료를신경과뇌혈관사이에끼워넣어줌으로써압박을주던동맥혈관을신경으로부터분리하는수술을한다 (Kim 등, 2001). 50세남자환자로서 3개월전부터오른쪽얼굴에반측성안면경련증상이발생하였고, 간헐적으로어지러움증상을호소하였다. 수술전검사로서영상의학 (Magnetic Resonance Images, MRI) 검사에서는오른쪽소뇌에혈관모세포종양 (Hemangioblastoma) 이관찰되었고 (Fig. 2), 뇌간청각유발전위 (Brainstem Auditory Fig. 4. FNEMG and BAEP in operation. Evoked Potentials; BAEP) 검사는양쪽모두정상소견결과로보아뇌종양으로인하여청신경은영향을안받고 (Fig. 3), 안면신경만이압박을받고있음을알수있었다. 일반적으로뇌종양이커지면서안면신경에영향을미치고있다고판단되는환자의경우는종양 (Tumor) 에대한검사를진행하고, 종양수술을먼저시행하고반측성안면경련증상이남아있을경우, 안면신경과동맥혈관을분리시키는수술인미세혈관감압술을시행하도록계획을한다. 전신마취하에환자의머리쪽소뇌부분이잘보이도록엎드려서뒤통수가위로향하게하는 prone position을취하고, 양쪽귀에는 Tubal insert phone 을삽입하여뇌간청각유발전위 (Brainstem Auditory Evoked Potentials; BAEP) 검사를시행하여청신경손상여부를측정하였고 (Fig. 4), 안면신경근전도 (Facial Nerve Electromyography; FNEMG) 검사를시행하여안면마비의예방을위해검사하였고 (Fig. 4), 소뇌부분의종양을모두제거한후에더깊은뇌심부로접근하여서반측성안면경련이발생하는안면신

126 Sang-Ku Park, et al. Hemifacial Spasm Caused by Brain Tumor Fig. 5. Direct facial nerve stimulation in operation. 경을찾기위해서직접신경자극검사 (Direct Nerve Stimulation, DNS) 를시행하여서안면신경을찾았으며 (Fig. 5), 안면신경을찾은이후에안면신경주변의상태를살펴보는과정에서간헐적으로안면신경근전도파형 (Facial Nerve EMG) 이관찰되었다 (Fig. 6). 반측성안면경련증상이뇌종양으로인한것인지아니면안면신경주변의뇌동맥혈관에의한것인지를판별하기위해측면전파반응 (Lateral Spread Response, LSR) 검사를시행하여수술하는동안지속적으로관찰을하였다. 뇌종양을제거하기전에잘관찰되던측면전파반응이뇌종양을모두제거하고안면신경주변의상태를살피는과정에소실되었다 (Fig. 7). 이로서환자의반측성안면경련증상은뇌종양으로인한안면신경의압박으로발생한것으로판단되었고더이상의수술을진행하지않고수술을종료하였다. 본사례의환자는 2012년8월1일수술을받았고, 2012년10월18일외래방문시에반측성안면경련증상이모두사라진상태였다. 2013년1월17 일재진시에뇌종양수술에의한후유증으로말이조금어눌한증세가일시적으로있었으나 2013년 10월18일외래방문시에는모든증상이개선되었다. 2. 증례 2 Fig. 6. Wave of facial nerve electromyography. 54세남자환자로 1년전오른쪽얼굴이떨리는반측성안면경련증세가발생하여한방치료와약복용을하였으나증상의호전이전혀없었다. 수술전검사로영상의학 (Magnetic Resonance Images, MRI) 검사에서유피낭종 (epidermoid cyst) 이오른쪽소뇌각교 (cerebello pontine angle, CPA) 부위에서관찰되었다 (Fig. 8). 소뇌각교 (cerebello pontine angle, CPA) 라는곳은소뇌 (cerebella) 와뇌교 (pons) 의사이를말하며이곳을중심으로위와아래로뇌신경 (Intracranial Nerve) 들의분지가이루어지는곳이다. 그러므로뇌종양을제거하면서뇌신경이손상받지않도록하 Fig. 7. Wave change of Lateral Spread Response. a Before tumor remove. b After tumor remove.

Korean J Clin Lab Sci. Vol. 45, No. 3, Sep. 2013 127 Fig. 8. Pre and Post operation MRI. a Before tumor remove. b After tumor remove. Fig. 10. FNEMG and BAEP in operation. Fig. 9. Tubal insert phone for BAEP. 는것이반측성안면경련증상을해결하는것보다더중요하다고할수있다. 전신마취하에환자의머리쪽유양돌기 (mastoid tip) 부분이잘보이도록옆으로누워서오른쪽귀가위로향하게하는 park bench position 을취하고, 양쪽귀에는 Tubal insert phone을삽입하여뇌간청각유발전위 (Brainstem Auditory Evoked Potentials; BAEP) 검사를시행하여청신경손상여부를측정하였고 (Fig. 9), 안면신경근전도 (Facial Nerve Electromyography; FNEMG) 검사를시행하여안면마비의예방을위해검사하였다 (Fig. 10). 뇌경막 (dura) 을열어서뇌척수액 (CSF) 을배양 (drain) 시켜뇌종양과뇌신경들의간격을벌어지게하여수술이용이하도록하는과정에서경막을열기전에잘관찰되던측면전파반응 (Lateral Spread Response, LSR) 이소실되었다 (Fig. 11). 이러한현상은뇌압이감소하고뇌척수액의배액으로인하여안면신경에압력을가하던동맥혈관의영향이약해짐으로써발생하는현상이다. 소뇌교각부위의종양을제 거하는과정에서안면신경과청신경을관찰할수있었고삼차신경은뇌종양에의하여멀리밀려져있는상태였다. 반측성안면경련증상을발생시키는원인혈관을찾으려고안면신경에접근하는과정에서청신경에일시적인손상이발생하여뇌간청각유발전위 (Brainstem Auditory Evoked Potentials; BAEP) 파형이감소되었다가회복되었다 (Fig. 12). 뇌종양에의하여오른쪽전하뇌동맥 (Rt. anterior inferior cerebral artery, AICA) 이안면신경을압박하는것이관찰되어서종양을제거하면서뇌동맥과안면신경사이에 teflon felt를넣어주어미세혈관감압술 (mircovascular decompression, MVD) 도동시에시행하여주었다. 본사례의환자는 2010년 4월 23 일수술을받았고, 2010년6월14일외래방문시수술로인한후유증으로약간의쉰목소리와약간의이명증상이있었다. 2012년7월 25일외래방문시에는약간의이명은남아있지만반측성안면경련증상과쉰목소리증상은완전히개선되었다.

128 Sang-Ku Park, et al. Hemifacial Spasm Caused by Brain Tumor Fig. 11. Wave change of Lateral Spread Response. a Before dura open. b After dura open. Fig. 12. Wave change of BAEP. a Before dura open. b Tumor removing. c Tumor removing. d After tumor remove. 고찰반측성안면경련증상환자를대상으로수술을하는경우에는수술이잘되었는지의여부를수술중바로판단할수있도록측면전파반응 (Lateral Spread Response, LSR) 검사를시행한다. 하지만반측성안면경련증상을수반한뇌종양환자의수술에서측면전파반응검사를시행한경우는보고된사례가없다. 즉, 뇌종양에비중을더두어서뇌종양수술에적용할수있는검사만을시행하여서수술을하고회복실에서환자의상태를살펴보아서뇌종양을모두제거하였더라도반측성안면경련증상이남아있다면다음에다시 수술을하는방식으로진행을한다. 이러한경우환자가경제적, 육체적으로많은부담을해야만한다. 수술중집중감시검사 (Intra-operative Neurophysiological Monitoring, INM) 는수술장에서수술을하면서환자의기능적인평가를할수있는유일한검사이다. 신경외과, 정형외과, 흉부외과, 이비인후과, 성형외과등에서신경계통의손상이우려되는수술에모두적용이가능하며, 수도권위주의병원에서만가능하던것이이제는전국으로확산되어가고있다. 환자를중심으로해당과집도의와신경과의사그리고신경생리기사가어떻게도움을주는것이가장좋을지회의를하여서최적의검사를수술에적용하는것

Korean J Clin Lab Sci. Vol. 45, No. 3, Sep. 2013 129 이바람직하겠다. 뇌종양이있는환자에서반측성안면경련증상이뇌종양때문인지, 뇌종양과별개로안면신경주변의동맥혈관에의한것인지는정확하게파악하기어렵다. 그러므로수술중에이러한면을모두고려하여검사를시행하는것이바람직하다고사료된다. 또한반측성안면경련증상으로미세혈관감압술 (mircovascular decompression, MVD) 을하기전에반드시영상의학 (Magnetic Resonance Images, MRI) 검사를시행하여서증상의발생원인이뇌종양으로인한것인지의감별을미리하는것도매우중요하다. 참고문헌 Gardner WJ. Concernibg the mechanism of trigeminal neuralgia and hemifacial spasm. J Neurosurg. 1962, 19:947-958. Hatem J, Sindou M, Vial C. Intraoperative monitoring of facial EMG responses during microvascular decompression for hemifacial spasm. Prognostic value for long-term outcome: a study in a 33 patients series. Br J Neurosurg. 2001, 15:496-499. Kakizawa T, Shimizu T, Fukushima T. Monitoring of auditory brainstem response (ABR) during microvascular decompression (MVD): result in 400 case. Noto Shinkei. 1990, 42:991-998. Kim EY, Park HS, Kim JJ, Lee SC, Park HC. A more basal approach in microvascular decompression for hemifacial spasm the paracondylar fossa approach. Acta Neurochir (Wien). 2001, 143:141-144. Kong DS, Park K, Hhin BG, Lee JA, Eum DO. Prognostic value of the lateral spread response for intraoperative electromyography monitoring of the facial muscular true during microvascular decompression for hemifacial spasm. J Neurosurg. 2007, 106:384-387. Lee SH, Song DG, Lee JH, Kang DG. Result of auditory brainstem response monitoring of microvascular decompression: a prospective study of 22 patients with hemifacial spasm. Larngoscope. 2009, 119:1887-1892. Park SK, Lim SH, Park CW, Park JW, Chang SH, Park Kh, et al. Diagnostic significance of brainstem evoked potentials in microvascular decompression of patients with hemifacial spasm or trigeminal neuralgia. Korean J Clin Lab Sci. 2011, 43:19-25. Park SK, Lim SH, Park CW, Park JW, Kim DJ, Kim GB, et al. Intra-operative neurological monitoring and anesthesia. Korean J Clin Lab Sci. 2012, 44:184-198.