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Song-Hwa Chae, MD, Ji-Yoon Kim, MD a, Dae-Seong Kim, MD, Jin-Hong Shin, MD Department of Neurology, Pusan National University Yangsan Hospital, Yangsan, Korea Department of Neurology, MH Yeonse

Selective postganglionic autonomic denervation could be the differential diagnostic point for other diseases of the autonomic nervous system.

n evaluation of sweating and the pupillary response together with generalized areflexia confirmed the diagnosis of Ross syndrome.

1 증례 분절무한증과동공부등을주소로내원한로스증후군증례 : 손가락주름유발검사의적용 채송화김지윤 a 김대성신진홍 양산부산대학교병원신경과, MH 연세병원신경과 a Ross Syndrome with Segmental nhidrosis and nisocoria: pplication of Finger Winkle Test Song-Hwa Chae, MD, Ji-Yoon Kim, MD a, Dae-Seong Kim, MD, Jin-Hong Shin, MD Department of Neurology, Pusan National University Yangsan Hospital, Yangsan, Korea Department of Neurology, MH Yeonse Hostpial a, Changwon, Korea Ross syndrome is characterized by a triad of segmental anhidrosis, tonic pupil, and generalized areflexia. Selective postganglionic autonomic denervation could be the differential diagnostic point for other diseases of the autonomic nervous system. Here we report a patient with regional anhidrosis in his left hand and sole, and anisocoria. n evaluation of sweating and the pupillary response together with generalized areflexia confirmed the diagnosis of Ross syndrome. The finger wrinkle test is a simple and useful tool for revealing segmental sympathetic denervation. J Korean Neurol ssoc 34(1):57-61, 2016 Key Words: Ross syndrome, utonomic nervous system disease, Finger wrinkle test 로스증후군 (Ross syndrome) 은 1958년 Ross에의해처음기술되었으며, 긴장동공, 분절무한증과건반사저하를세징후로하는드문증후군이다. 1 주요증상은시야흐림이나빛번짐, 피부건조감, 보상다한증, 더위못견딤증등으로별다른증상없이지내는경우도있다. 2 동공이상없이분절무한증만있는경우광대증후군 (harlequin syndrome) 으로부르며, 호너증후군이동반되기도한다. 우연히발견되어수년에걸쳐진행하거나유지되는경우가많은데, 심한다한증이나실신이동반될경우치료적개입이필요하다. 3 저자들은동공부등과분절무한증으로내원하여, 진찰소견및임상검사로세징후를확인하고로스증후군으로진단된증례를보고하면서, 손가락주름유발검사의유용성을기술하고자한다. D C E Received July 27, 2015 Revised October 26, 2015 ccepted October 26, 2015 ddress for correspondence: Jin-Hong Shin, MD Department of Neurology, Pusan National University Yangsan Hospital, 20 Geumoro, Mulgeum-eup, Yangsan 50612, Korea Tel: Fax: shinzh@gmail.com Figure 1. Tonic pupils responsive to low concentration pilocarpine. () nisocoria. (, right; C, left) Pupils are of irregular shapes, and unresponsive to light. (D, right; E, left) Pupils contricted on administration of 0.125% pilocarpin solution. J Korean Neurol ssoc Volume 34 No. 1,

채송화김지윤김대성신진홍 증례 43세남자가좌측손발의땀분비저하로왔다. 5년전문고리를잡고돌릴때나바닥을걸을때좌측손바닥과발바닥이건조하며미끄러워손잡이를돌리지못하거나마루에서넘어질뻔한일이종종생겼으며, 당시좌측손에땀이나지않는것을발견하였다. 3년전우연히거울을보던중좌우동공의크기가 다른것을발견했고별다른평가없이지내던중좌측손의건조감이악화되어병원에방문하였다.

Starch test reveals anhidrosis on the left palm (), while it shows color change by sweating on the right palm ().

2 채송화김지윤김대성신진홍 증례 43세남자가좌측손발의땀분비저하로왔다. 5년전문고리를잡고돌릴때나바닥을걸을때좌측손바닥과발바닥이건조하며미끄러워손잡이를돌리지못하거나마루에서넘어질뻔한일이종종생겼으며, 당시좌측손에땀이나지않는것을발견하였다. 3년전우연히거울을보던중좌우동공의크기가 다른것을발견했고별다른평가없이지내던중좌측손의건조감이악화되어병원에방문하였다. 어린시절부터빈뇨로 2-3 시간마다화장실을드나들었으며배변장애나기립시어지러움, 두근거림은동반되지않았다. 빛번짐이나시야흐림증상도없었다. 복용중인약도없었으며, 유사증상의가족력이나유해물질노출의과거력도없었다. 신체진찰에서좌측손이건조하고매끄러웠으며상대적으로 C Figure 2. Segmental anhidrosis. Starch test reveals anhidrosis on the left palm (), while it shows color change by sweating on the right palm (). (C) QSRT by iontophoresis of acetylcholine shows no sweating on the left forearm (L-F), while normal response is obtained on the right forearm (R-F). Hung-up response pattern is noted on the left lower leg (L-LL), which produced sweat volume less than half of that on the right lower leg (R-LL) (abscissa, minutes of iontophoresis; ordinate, sweat rate in nl/min). 58 대한신경과학회지제 34 권제 1 호, 2016

() Skin temperature was higher on the left palm when measured by digital infrared thermal imaging. 우측손에는땀이흥건하였다. 동공의모양이불규칙하였으며, 우측동공은 5 mm로좌측에비해컸다 (Fig. 1-, ).

3 분절무한증과동공부등을주소로내원한로스증후군증례 : 손가락주름유발검사의적용 Figure 3. Evaluation of distal sympathetic denervation. () Finger wrinkle test by warm water immersion produces skin wrinkle on the right fingers, while left fingers remain unwrinkled. () Skin temperature was higher on the left palm when measured by digital infrared thermal imaging. 우측손에는땀이흥건하였다. 동공의모양이불규칙하였으며, 우측동공은 5 mm로좌측에비해컸다 (Fig. 1-, ). 빛반사검사에서우측동공은고정된채좌측동공이미약하게반응하였고, 조절반사에서는좌측동공만수축하였다. 세극등검사에서홍체조임근의움직임이저하되어있었으며, 0.125% pilocarpin 용액을점안했을때양안의동공수축을확인함으로써양안의긴장동공을증명하였다 (Fig. 1-, C). 뇌신경검사및운동-감각기능검사는정상이었으나, 우측무릎의심부건반사는저하, 다른모든심부건반사는소실되어있었다. 분절무한증에대한객관적인평가를위해전분검사를시행하였는데양하지와오른쪽손바닥에서전분색의변화를관찰할수있었으나왼쪽손에서는전분색의변화가없었다 (Fig. 2-, ). 정량땀분비축삭반사검사 (quantitative sudomotor axon reflex test; QSRT) 에서오른팔의땀분비는정상범위였으나, 왼팔의땀분비는소실되어있었다. 왼쪽정강이는오른쪽에비해절반수준의땀분비가측정되었으나양측다정상범위에들었다 (Fig. 2-C). 말단부자율신경기능의평가를위해손가락주름검사 (finger wrinkle test) 를시행하였다. 양손을따뜻한물에 5분간담근뒤관찰하였을때오른쪽손가락말단부에현저한피부주름이생겼으나, 왼손손가락에서는주름이거의관찰되지않았다 (Fig. 3-). 체열측정검사에서는땀분비가저하된왼손이높은온도를보였다 (Fig. 3-). 심혈관계자율신경조절장애평가를위한발살바수기검사, 심박변이도검사, 기립혈압검사결과는정상범위였으며, 말초신경병동반유무확인위해시행한운동- 감각신경전도검사에서 H반사소실이외에특이소견은관찰되지않았다. 빈뇨에대한평가위하여배뇨전요의를최대한느낄때측정한방광용적은 ml로정상에비해방광용량이적으나, 배뇨후잔뇨량은 25 ml로정상범위에속했다. 뇌와경부자기공명영상검사에서 C5/6 추간판돌출외에는이상소견이없었다. 자가면역질환, 염증성질환, 내분비장애와관련한혈액검사에서항핵항체양성소견이외에는특이소견이없었다. 고찰 로스증후군은자율신경계의부분적이상을보이는질환으로, 진단을위해서는전형적세징후의확인과각증상의다른원인에대한배제가필요하다. 한편질환의양성경과로인해적극적인평가를받지않은미진단증례가많이있을것으로추정되기도한다. 국내에서는안과학회지에호너증후군과동반된증례가보고된바있다. 4 부분자율신경이상의범주에속하는질환으로한쪽얼굴을포함한신체상부의홍조와과발한을특징으로하는광대증후군, 긴장동공과건반사저하를특징으로하는 Holmes-die증후군이있다. 각증후군의증상또는징후가겹치는경우가종종보고되면서, 이러한질환들은땀분비신경섬유, 혈관운동신경섬유그리고동공조절섬유등수초화되지않은자율신경말단의선택적손상으로인한자율신경계부분부전을일으키는스펙트럼질환으로파악되고있다. 3 발병원인은잘알려져있지않으나거대세포바이러스감염이후로스증후군의형태로발생한자율신경계부전사례와 5 로스 J Korean Neurol ssoc Volume 34 No. 1,

4 채송화김지윤김대성신진홍 증후군이두일란성쌍둥이모두에이환된사례로 6 미루어감염후자가면역또는유전적인기전이관여할것으로추정된다. 동공조임근에작용하는신경절이후부교감신경섬유와섬모체신경절의변성으로긴장동공이유발되고건방추와알파운동신경사이의무수초연접효능감소로건반사의가소실또는저하가초래되며, 국소피부땀샘유출과피부혈관수축반응과같은피부교감신경활성도소실혹은저하로분절무한증이발생한다는보고가있다. 교감신경절세포의손상또는신경절후섬유의결손으로무한증이유발되며등뿌리신경집또는척수사이신경세포의소실에의해건반사가소실되었을것으로추정하나손상기전은알려진바가없다. 말초자율신경계와등뿌리신경절모두신경능선세포기원으로이러한공통점에근거하여특정자극에의한조직손상취약성이제기되기도한다. 다른자율신경조절장애와달리로스증후군에서는체위변화에따른혈압변화나심박수에관여하는근육교감신경활성도는정상으로피부교감신경에선택적인자율신경부전을초래한다고알려져있으나, 부전의정도에따라다양하고전신적으로영향을미쳐기립저혈압, 두통, 발살바수기시심박수저하, 설사, 배뇨 / 배변장애를호소할수있다는보고도있다. 3 본증례의경우동반된배뇨장애가자율신경조절장애의일환으로추정되나, 방광용적의감소만이확인되어연관성은불확실하다. 분절무한증의비교적흔한원인으로는호너증후군을들수있으며, 그밖에쇼그렌증후군, 전신홍반루푸스, 피부경화증등의자가면역질환또는뇌줄기하부또는경부상방의병변에서동반되기도한다. 드문원인으로전자율신경기능이상의제한된형태로발현하는감염후무한증증후군, 특발성순수땀샘운동부전등을고려할필요가있다. 본증례에서는이러한질환들과달리체간의뚜렷한비진행분절무한증이있어임상적으로감별이가능하였다. 저자들이사용한 QSRT와전분검사는발한장애의평가에있어표준으로자리잡은검사이다. QSRT 는정량분석이가능하나, 신경절후섬유이후만을평가할수있으며, 고가의장비와유발약물을이용한검사과정이필요하다. 또한발한장애환자들이가장불편감을호소하는말단부의평가에적합하지못하다. 전분검사는장비가필요하지는않으나역시번거로운과정을거쳐야하며, 정량적이지못할뿐아니라기저발한양에따라위음성결과가나올수있다. 체열측정검사는말단부혈관운동성을반영하는데반사교감신경이상증의평가에제한적으로권장되고있다. 본증례에서는말단부평가가어려운 QSRT검사의보조적인수단으로적용하였다. 손가락과발가락주름은땀분비와마찬가지로교감신경에의해조절된다. 교감신경말단의무수신경섬유는진피와피하에 위치한말초혈관및진피와연결되어있는사구체혈관을수축시켜피부주름을형성하는데, 교감신경의말단가지가손상된환자에서는이러한과정이제대로이루어지지않아주름이관찰되지않는다. 손가락주름유발검사는따뜻한물에양손을 5분간담그는것만으로결과를확인할수있어, 말초신경손상의응급평가를위해사용되기도한다. 7 이방법은정량적이지는않으나, 말단부를평가할수있는장점이있고, 본증례와같이편측성자율신경장애가있는경우는간편하게적용할수있다. 한편손발을섭씨 40도의소금물에 30분간담그거나국소마취제의공융혼합물을사용한손가락주름유발방법으로소섬유신경병을반정량적으로평가하는방법이시도되고있는데, 8 피부생검을통한표피내신경섬유밀도치와비교하여소섬유신경병진단에거의비슷한민감도를보였으며, 교감피부반응보다뛰어났다. 9 또한교감신경차단술환자에서의손가락주름유발검사연구에의하면교감피부반응과의일치율이전분검사에비해월등히뛰어났다. 10 로스증후군은자율신경계의선택적부전으로요약할수있다. 자율신경계이상을보이는의심증례에서는로스증후군의세징후에대한적극적인평가가권장된다. 이중분절무한증의확인을위해서는 QSRT나전분검사보다간단하고비침습적으로시행할수있는손가락주름유발검사가유용할것이다. cknowledgements 본연구는 2014년도양산부산대학교병원임상연구비지원으로이루어졌음. REFERENCES 1. Ross T. Progressive selective sudomotor denervation; a case with coexisting die s syndrome. Neurology 1958;8: Nolano M, Provitera V, Perretti, Stancanelli, Saltalamacchia M, Donadio V, et al. Ross syndrome: a rare or a misknown disorder of thermoregulation? skin innervation study on 12 subjects. rain 2006;129: allestero-díez M, García-Río I, Daudén E, Corrales-rroyo M, García- Díez. Ross syndrome, an entity included within the spectrum of partial disautonomic syndromes. J Eur cad Dermatol Venereol 2005;19: Choi MJ, Choi DG, Kim SY. case of Ross syndrome with Horner s syndrome. J Korean Ophthalmol Soc 1998;39: Nagane Y, Utsugisawa K. Ross syndrome associated with cytomegalovirus infection. Muscle Nerve 2008;38: Nolano M, Provitera V, Donadio V, Stancanelli, Saltalamacchia, Caporaso G, et al. Ross syndrome: a lesson from a monozygotic twin pair. Neurology 2013;80: 대한신경과학회지제 34 권제 1 호, 2016

5 분절무한증과동공부등을주소로내원한로스증후군증례 : 손가락주름유발검사의적용 7. Tindall, Dawood R, Povlsen. Case of the month: the skin wrinkle test: a simple nerve injury test for paediatric and uncooperative patients. Emerg Med J 2006;23: Wilder-Smith EP. Stimulated skin wrinkling as an indicator of limb sympathetic function. Clin Neurophysiol 2015;126: Teoh HL, Chow, Wilder-Smith EP. Skin wrinkling of diagnosing small fibre neuropathy: comparison with epidermal nerve density and sympathetic skin response. J Neurol Neurosurg Psychiatry 2008;79; Vasudevan TM, van RiJ M, Nukada H, Taylor PK. Skin wrinkling for the assessment of sympathetic function in the limbs. ust N Z J Surg 2000;70; J Korean Neurol ssoc Volume 34 No. 1,

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Jksvs019(8-15).hwp Grade I Grade II Grade III 12 대한혈관외과학회지 : 제 20 권 제 1 호 2004 Control Group A Group B Fig. 4. Microscopic findings of vein wall in control, group A and group B on the day of 7 after venous occlusion. The