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1 증례접수번호 :09-029(2 차 -0710) 전북대학교의학전문대학원신경과학교실 김도형오선영신병수정슬기서만욱 Secondary Intracranial Hypertension Due to Unusual Causes Do-Hyung Kim, MD, Sun-Young Oh, MD, Byoung-Soo Shin, MD, Seul-Ki Jeong, MD, Man-Wook Seo, MD Department of Neurology, Chonbuk National University Medical School, Jeonju, Korea Secondary intracranial hypertension (IH) is a syndrome with various etiologies, including a brain tumor, head trauma, hypoparathyroidism, hydrocephalus, meningitis, drug use, or venous obstruction. It is reasonably straightforward to diagnose secondary IH induced by these diseases. However, diagnosing secondary IH induced by unpredictable extracranial lesion is difficult, and is based largely on subtle neurological symptoms or signs and usually can be achieved by an extracranial evaluation. Here we describe four cases of secondary IH with unusual causes. J Korean Neurol Assoc 31(1):45-49, 2013 Key Words: Headache, Secondary intracranial hypertension, Spinal cord tumor 특발두개내압상승 (idiopathic intracranial hypertension) 은뇌에기질적인병변없이두개내압이상승하는질환으로, 1 두통, 어지럼, 이명, 구역, 시력감소, 복시같은증상이생기며신경계질환에서시신경유두부종이보인다. 이와는달리뇌압을상승시킬수있는중추신경계의기질적병변때문에생기는두개내압상승을이차두개내압상승 (secondary intracranial hypertension) 이라한다. 뇌는두개골로둘러싸여한정된공간안에위치하므로두개내압의변화에민감한데, 신생물처럼공간을차지하는병변이나뇌척수액의흐름을막는구조이상은두개내압을상승시켜뇌에비가역손상을주기도한다. 두개내압이상승하여영구적인시력저하같은이차적인신경계이상이올수있으므로조기에진단하여적극적으로치료하는것이중요하다. 그러므로두개내압상승이의심되면두개내압항진을초래할수있는이차병변에대한세심한진찰이필수적이다. 대개이차두개내압상승은뇌영상검사로진단이어렵지않으나, 최근저자들은특발두개내압상승으로초기에진단하였지만두 Received July 16, 2012 Revised October 4, 2012 Accepted October 4, 2012 *Sun-Young Oh, MD Department of Neurology, Chonbuk National University Medical School, 20 Geonji-ro, Deokjin-gu, Jeonju , Korea Tel: Fax: ohsun@jbnu.ac.kr 개외종양에의한이차두개내압상승환자들을경험하여정리해보았다. 증례증례 1 19세여자가내원 3주전부터지속되던두통이내원 2일전부터더심해져응급실로내원하였다. 2일전두통이심해지며구역, 구토가동반되었으며양쪽눈에통증이있었다. 체위변화에따라증상이변하여앉으면두통이심해졌다. 두통은이마양측을찌르는듯한양상이었으나시력변화나안구충혈, 눈물, 콧물, 발열같은증상은동반되지않았으며최근감염이나자가면역질환을의심할만한증상이나약물복용에대한과거력은없었다. 신장 163 cm, 체중 47 kg로신체비만지수 (BMI, Body Mass Index) 17.7로저체중이었으며그외신체검사와신경계진찰은정상이었다. 전체혈구계산, 요검사, 일반화학검사, 혈청검사와심전도검사는정상이었다. 뇌척수액검사에서염증은없었고두개내압이 260 mmh 2O로증가되었으며안저촬영상시신경유두부종이확인되어특발두개내압상승으로생각하였다. 입원해서촬영한단순흉부방사선사진에서우측폐문종양발견되었고흉부컴퓨터단층촬영 (Fig. A) 에서보이는병변을경화 J Korean Neurol Assoc Volume 31 No. 1,

김도형 A E I 오선영 신병수 정슬기 서만욱 B C F G D HH J Figure.

G and H image were belonged to the case 3. I and J image were belonged to the case 4.

The CT scan obtained at admission day (A).

intra-alveolar hemorrhage (right fragment), and granulation tissue with hemosiderin-laden

HRCT scan obtained 1 week after intracranial pressure control, shows evidence of the mass

Note decrease in size of the mass (arrow) (C).

Sagittal T1- and T2-weighted image of the cervical spine shows intradural-extraspinal cord

(G, H) A 43-year-old man had headache, nausea, vomiting, blurred vision, lower back pain, and

Sagittal T1-weighted image of the cervical spine shows multiple vertebral bone metastases (G).

2 김도형 A E I 오선영 신병수 정슬기 서만욱 B C F G D HH J Figure. A, B, C, and D images were belonged to the case 1. E and F image were belonged to the case 2. G and H image were belonged to the case 3. I and J image were belonged to the case 4. (A-D) A 19-year-old woman had a right lung hilar mass on Chest CT image. The CT scan obtained at admission day (A). Biopsy of the right lung hilar mass shows reactive bronchial epithelial cells and intra-alveolar hemorrhage (right fragment), and granulation tissue with hemosiderin-laden macrophages (left fragment) on lung needle biopsy (H&E; original magnification, x200) (B). HRCT scan obtained 1 week after intracranial pressure control, shows evidence of the mass reduction within the region compared to the baseline CT. Note decrease in size of the mass (arrow) (C). Diffusion weighted image of the brain shows high signal intensity at splenium of corpus callosum (arrow) (D). (E, F) A 41-year-old man had headache, blurred vision, and both leg weakness. Sagittal T1- and T2-weighted image of the cervical spine shows intradural-extraspinal cord tumor at the T2-3 level (E, F). There is compression of the thoracic spinal cord. (G, H) A 43-year-old man had headache, nausea, vomiting, blurred vision, lower back pain, and voiding difficulties. Sagittal T1-weighted image of the cervical spine shows multiple vertebral bone metastases (G). PET-CT image of the torso shows multiple metastases to both lungs and multiple abdominal organs (H). (I, J) A 27-year-old woman had headache, horizontal diplopia, dizziness, nausea, vomiting, and neck stiffness. Coronal contrast-enhanced T1 weighted image of the brain shows enlarged 3rd ventricle and normal pituitary, though, downward displacement of the some suprasellar cistern into sellar (I). Midline sagittal contrast-enhanced T1-weighted image of the brain shows widen 3rd ventricle (J). 46 대한신경과학회지 제31권 제1호, 2013

3 혈관종 (sclerosing hemangioma), 선종 (adenoma), 또는결핵종 (tuberculoma) 인지감별하기위해침흡인생검 (needle aspiration biopsy) 을하였다. 생검에서과립조직상의출혈 (Fig. B) 이보여자궁내막증 (endometriosis) 을의심할수있었으며일주일후고해상도흉부컴퓨터단층촬영 (High Resolution CT, HRCT) 추적검사에서병변크기는현저히감소되었다 (Fig. C). 입원해서촬영한뇌컴퓨터단층촬영은정상이었으나뇌자기공명영상추적검사에서발견된뇌량병변은허혈병변과뇌의자궁내막증의감별이어려운상태였으며조직검사는시행하지못하였다 (Fig. D). 두통은많이호전되었으며안저이상도정상으로회복되었고우측폐문종양의치료를위하여산부인과와호흡기내과로전과하였다. 증례 2 41세남자가 2개월전부터나타난두통과시야흐림으로응급실에왔다. 이마우측에국한된박동성두통이 2주전부터더심해져하루 3차례나타났으며 2시간정도지속되었다. 두통이심해지며구역과구토가동반되고, 체위변화에영향을받지않았으며뒷목뻣뻣함도호소하지않았다. 시야흐림과함께좌안시야에검은것이떠다니는것같다고하였다. 시신경유두부종이있었으며신체검사와신경계진찰은정상이었다. 전체혈구계산, 요검사, 일반화학검사, 혈청검사, 심전도검사와단순흉부방사선검사도정상이었으며흉부컴퓨터단층촬영도정상이었다. 뇌척수액검사에서감염은없었으며두개내압이 330 mmh 2O로증가되었고단백질증가가있었다. 신체비만지수는 23.94로과체중이었으며 3개월전외부병원에서기립저혈압을진단받고치료하였으며, 두개내압을상승시킬만한약을복용한적도없었다. 특발두개내압상승으로진단하고만니톨치료를시작하였다. 2일째부터두통과시야흐림은호전되었으나양하지에힘이빠져보행이힘든증상이수차례발생하여척추자기공명영상을촬영하였고흉추 (T2, T3) 의경막과척수사이에종양이발견되었다 (Fig. E, F). 추가로실시한뇌척수액검사에서세포수는정상이었다. 입원 8일째두통은거의호전되었으며시력과시신경유두부종도호전되었으나척수종양에의한두개내압상승으로판단하고종양제거수술을위해전원하였다. 증례 3 20갑년의흡연력외다른과거력이없는건강한 43세남자가한달전부터시작되고누워있으면호전되고앉거나서면 심해지는욱신거리는두통이이마와후두부에발생하였다. 약두달전부터시작된허리통증으로물리치료와경막외차단술을받은후부터두통이발생하여두개내압저하때문으로생각하고대증치료를하였으나호전이없고시력저하가생겨촬영한안저촬영에서양안시신경유두부종이있었다. 구역, 구토와배뇨장애도나타났으며양측시야가흐려졌고입원후두통은체위의영향을받지않고지속되는양상으로변하였다. 신체비만지수는 22.7로정상이었으며신경계진찰에서양안시력저하와우안의상대구심동공결손 (relative afferent papillary defect) 이관찰되었다. 전체혈구계산, 요검사, 일반화학검사, 혈청검사, 심전도검사와단순흉부방사선사진결과모두정상이었다. 뇌자기공명영상도정상이었고뇌척수액검사에서염증없이두개내압 500 mmh 2O로측정되어특발두개내압상승으로진단후만니톨과스테로이드를투여하였다. 두통은호전되지않고시력저하와시신경유두부종이지속되고요통과배뇨장애의원인을찾기위하여척추자기공명영상을촬영하였다. 영상검사에서다발성골전이가발견되었고 (Fig. G), 뇌에병변은없었으나양전자방출컴퓨터단층촬영 (PET-CT) 에서양쪽폐와복부여러장기로의전이된종양을확인하였다 (Fig. H). 뇌척수액세포검사에서악성세포는보이지않았으나다수의비정형세포 (atypical cell) 를확인하였다. 두통과시력저하가지속되어뇌실복강션트 (ventriculoperitoneal shunt) 수술을하였으며종양에대한검사와치료는원치않아퇴원하였다. 증례 4 이전에건강했던 27세여자가내원 3개월전부터서서히나타난두통과양안복시때문에병원에왔다. 내원 3개월전부터간간히이마와양측측두부통증이시작되었으며, 약 2개월전부터는복시가발생하였고안과에서유두부종이있음을확인후신경과로의뢰되었다. 어지럼, 구역과구토증상이동반되었으나이명은없었고체위변화와관련없이뒷목뻣뻣함을호소하였다. 상하주시에서는정상이었으나좌우주시에서복시가발생하였으며안구운동검사에서양측외전신경마비가있었다. 신체비만지수는 38.2로고도비만이었으며전체혈구계산, 요검사, 일반화학검사, 혈청검사, 심전도검사와단순흉부방사선사진은정상이었다. 뇌척수액검사에서염증은없었으나개방압이 400 mmh 2O로매우증가되었고뇌자기공명영상에서안장위수조 (suprasellar cistern) 일부가안장 (sellar) 안으로밀려들어와있으나뇌하수체는정상이었고 (Fig. I), 대뇌수도관협착과제3뇌실의심한확장을확인할수있었다 (Fig. J). 만니톨, 아세타졸아미드, 스테로이드치료를시작하였으나시 J Korean Neurol Assoc Volume 31 No. 1,

4 김도형오선영신병수정슬기서만욱 력저하와두통, 시신경유두부종이호전되지않아뇌실복강션트를하였고두통은호전되었으며시신경유두부종은경미한변화만을보인채로외래에서경과관찰중이다. 고찰 두개내압의조절과유지는크게용적완충 (volume buffering), 자동조절 (autoregulation), 쿠싱반응의세가지기전에의해정상적으로이루어진다. 용적완충은수정된 Monro-Kellie가설로두개강내세가지구조물인뇌, 뇌척수액, 혈액중어느한가지의용적이증가하면두개내압력을일정하게유지하기위하여다른구조물의용적을줄이는변화가일어나는것을말한다. 두개내압의조절에관여하는이러한세가지기전중어느하나라도손상되면두개내압은높아지거나낮아지게된다. 2 이차두개내압상승은이와같은기전으로설명되는뇌종양, 뇌농양, 동정맥기형, 뇌부종과같은뇌의공간점유병소뿐만아니라, 심한두부외상, 부갑상샘저하증, 수두증, 뇌수막염, 뇌정맥폐쇄, 테트라사이클린, 리튬, 비타민A나성장호르몬등의사용과같은두개내압을상승시킬수있는여러원인이나상황에의해유발된다. 두개내압상승과관련된두통이특별한원인없이발생하는특발성인지, 뇌내원인질환에동반되는이차성인지를구분하는것이치료방향결정에매우중요하다. 두개내압상승을시사하는증상이있는경우특발성또는이차성을감별하기위해영상검사를하지만, 본증례처럼특발두개내압상승으로생각하였지만이후두개외종양과같은병인에의한이차두개내압상승으로밝혀진경우도많아원인에대한신속한감별과적극적인치료가중요하다. 증례 1 (Table) 은뇌자기공명영상에서뇌량병변이발견되어, 흉부컴퓨터단층촬영과조직검사에서임상적으로진단한자궁내막증 (endometriosis) 의뇌전이가능성을시사하였다. 자궁내막증진단을위해서는조직검사에서자궁내막고유의분비샘이있어야하나침흡인검사로정확한조직을채취하지못하면생리초기조직에서다량의출혈만발견되는경우가많다. 또한생리주기에따라짧은기간내단순흉부방사선사진에서급격한종괴의크기감소가보이고본증례처럼젊은나이라면자궁내막증을먼저고려할수있다. 3 자궁내막증의뇌전이는매우드물며뇌내자궁내막증에의한이차두개내압상승의발생기전역시확실히밝혀지지않았지만, 두개내압증가와함께뇌자기공명영상에서이상이발견되어자궁내막증에의한이차두개내압상승을생각해볼수있다. 증례 2 (Table) 는흉추의경막과척수사이종양에의해두개내압상승과이로인한시신경유두부종이나타난것으로생각하며, 증례 3 (Table) 도다발성뼈전이와함께척수강내종양이척수주위를압박하여두개내압이상승되었을것으로추정한다. 두개내병변과달리척수종양에의한두개내압상승이나시신경유두부종은흔하지않으며다음과같은기전으로생긴다. 첫째, 척수관내종양이척수정맥얼기를직접누르고이로인해척수주위에서뇌척수액흡수가저하되어두개내압상승과시신경유두부종같은증상이초래될수있다. 4-6 이러한증상은척수주변의뇌실막세포종 (ependymoma) 에서가장잘나타나며슈반세포종 (Schwannoma), 수막종 (meningioma), 신경섬유종 (neurofibroma), 교종 (glioma) 에서잘나타난다. 6 둘째, 척수내종양에의해뇌척수액에포함된단백양이증가되고, 변성된단백이지주막융모 (arachnoid villi) 표면에결합하여뇌척수액이지주막융모로흡수되는것을방해하여두개내압이상승되는경우이다. 특히척수종양환자에서뇌척수액내섬유소원 (fibrinogen) 의증가가있으면증가된섬유소원이섬유소 (fibrin) 로전환되어지주막하공간의혈관표면을덮어뇌척수액이정맥내로흡 Table. Summary on clinical features of our patients with secondary intracranial hypertension Pt./Age/ Sex Symptom 1/19/F HA, N/V, Both eyeball pain 2/41/M 3/43/M 4/27/F HA, blurred vision, N/V, both leg weakness HA, lower back pain, N/V, voiding difficuly, blurred vision, RAPD (Rt) HA, Horizontal diplopia, dizziness, N/V, neck stiffness CSF pressure (mmh 2O) Papilledema BMI Possible cause of IICP Treatment Prognosis Endometriosis at remote Mannitol and steroid IV Improved sites (lung & brain) Anti PLT agent Extraspinal intradural tumor Mannitol and steroid IV Extraspinal intradural Mannitol and steroid IV, tumor and multiple tumor V P shunt metastasis (except brain) Cerebral aqueduct obstruction with hydrocephalus Mannitol and steroid IV, Acetazolamide, V L shunt Partially improved, Refer to OS Improved Improved HA; headache, N/V; nausea/vomiting, IICP; increased intracranial hypertension, RAPD; relatively afferent papillary defect, Rt.; right, V-P shunt; ventriculo-peritoneal shunt, BMI; body mass index. 48 대한신경과학회지제 31 권제 1 호, 2013

5 수되는것을막아두개내압상승을더악화시키는것으로알려져있다. 7 그외에도지주막염 (arachnoiditis), 뇌척수액에대한척수탄력예비력 (spinal elastic reservoir) 의손상이척수종양에의한두개내압상승에관여할것으로생각한다. 또척수병증과관련된증상없이두개내압만을상승시키고시신경유두부종을초래하는증례도있다. 5,6 따라서교통수두증 (communicating hydrocephalus) 이있거나두개내압이상승된환자가뇌영상검사에서병소가보이지않지만뇌척수액검사에서비정상적인단백의증가가있으면척수종양에의한두개내압상승을의심해야한다. 5,6 이차두개내압상승의진단에도움이되는검사는뇌컴퓨터단층촬영나뇌자기공명영상또는자기공명혈관조영술 (MRA) 이며, 헤르페스뇌염이나뒷머리뼈우묵, 뇌하수체, 연수막의종양에의한이차두개내압상승이의심되는경우조영증강뇌자기공명영상이도움이되고, 정맥동혈전으로인한두개내압상승이의심되면뇌자기공명정맥조영술 (magnetic resonance venography) 이필요하다. 8 두개내압이상승되면두통, 시력저하, 이명, 외전신경손상으로인한복시가발생할수있으며, 두통은뇌척수액누출검사에서호전될수있다. 9 두개내압상승을치료하기위해서는원인을먼저찾아야한다. 뇌척수액순환과흡수장애를초래하는두개내종양이나척수관내종양에대한적절한치료가필요하며감염이나뇌경색, 뇌출혈과같은원인이두개내압의상승을유발한다면이에대한치료를해야한다. 그외에도필요에따라과호흡, 예방적저체온, 마취제, 진정제도사용할수있다. 따라서저자들은두개외원인에의한이차두개내압상승의드문증례를소개하였고, 이에임상의는이차두개내압상승을염두에두고원인질환을찾으려는세심한노력을기울여야하겠다. REFERENCES 1. Skau M, Brennum J, Gjerris F, Jensen R. What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia 2006;26: Singhi SC, Tiwari L. Management of intracranial hypertension. Indian J Pediatr 2009;76: Woodward PJ, Sohaey R, Mezzetti TP Jr. Endometriosis: radiologicpathologic correlation. Radiographics 2001;21: Kobayashi M, Hara K, Nakatsukasa M, Murase I, Toya S. Primary spinal leptomeningeal gliomatosis presenting visual disturbance as the initial symptom: case report. Acta Neurochir (Wien) 1996;138: Kounin GK, Romansky KV, Traykov LD, Shotekov PM, Stoilova DZ. Primary spinal melanoma with bilateral papilledema. Clin Neurol Neurosurg 2005;107: Porter A, Lyons MK, Wingerchuk DM, Bosch EP. Spinal cord astrocytoma presenting as idiopathic intracranial hypertension. Clin Neurol Neurosurg 2006;108: Ko MW, Osborne B, Jung S, Jacobs DA, Marcotte P, Galetta SL. Papilledema as a manifestation of a spinal subdural abscess. J Neurol Sci 2007;260: Dodick DW. Clinical clues and clinical rules: primary Vs secondary headache. Avd Stud Med 2003;3:S550-S Macsween KF, Bicanic T, Brouwer AE, Marsh H, Macallan DC, Harrison TS. Lumbar drainage for control of raised cerebrospinal fluid pressure in cryptococcal meningitis: case report and review. J Infect 2005;51:e221-e224. J Korean Neurol Assoc Volume 31 No. 1,

Trd022.hwp

Trd022.hwp 김신태, 이선녕, 이석정, 정필문, 박홍준, 신명상, 김종환, 이부길, 김상하, 리원연, 신계철, 용석중 Shin-Tae Kim, M.D., Shun Nyung Lee, M.D., Seok Jeong Lee, M.D., Pil Moon Jung, M.D., Hong Jun Park, M.D., Myung Sang Shin, M.D., Chong Whan Kim,