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1 원 저 J Korean Neurol Assoc / Volume 22 / June, 2004 원위부만성염증성탈수초성다발성신경병증 : 원위부근력약화가우세한변형 지방공사강남병원신경과, 성균관대학교의과대학신경과학교실 * 배종석김병준 * Distal Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Variant with Predominant Distal Weakness JongSeok Bae, M.D., ByoungJoon Kim, M.D.* Department of Neurology, Kangnam General Hospital Public Corporation, Seoul; Department of Neurology, Sungkyunkwan University School of Medicine*, Seoul, Korea Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogenous group of acquired peripheral neuropathies. A subset of CIDP involves predominantly distal parts of the limbs, which is similar to axonal polyneuropathy. The clinical course or response to treatment may be different in this group. We investigated the clinical course and electrodiagnostic findings of the distal CIDP. Methods: Twenty five CIDP cases were reviewed retrospectively. Patients with proximal as well as distal involvement were grouped as typical CIDP, and patients with predominantly distal involvement as distal CIDP. We compared the clinical, laboratory and electrophysiological findings of these two groups. Results: Sixteen patients had typical CIDP and nine had distal CIDP. Distal CIDP differed significantly from typical CIDP; later age of onset (p=0.049), less frequent relapses (p=0.041), more rapidly progressive to maximal disability (p =0.01), low disability score at the diagnosis (p=0.02) and after treatment (p=0.01), poor response to immunomodulating therapy (p=0.02), and infrequent conduction blocks or abnormal temporal dispersions (p<0.01). Conclusions: Distal CIDP is a distinctive variant of CIDP different from typical CIDP in clinical and electrophysiological features. Identification of the pathogenesis underlying this new entity may lead to better understanding of the heterogeneous acquired demyelinating neuropathies. J Korean Neurol Assoc 22(3):219~225, 2004 Key Words: Polyradiculoneuropathy, Chronic inflammatory demyelinating, Distal, Variant 서론 Received May 2, 2003 Accepted December 10, 2003 *Address for correspondence ByoungJoon Kim, M.D. Department of Neurology, Sungkyunkwan University School of Medicine, Samsung Medical Center 50 Ilwondong, Gangnamgu, Seoul, 13510, Korea Tel: Fax: bjkim@smc.samsung.co.kr 만성염증성탈수초성다발성신경병증 (chronic inflammatory demyelinating polyradiculoneuropathy; CIDP) 은다양한임상소견과전기생리학적소견, 혈액및뇌척수액검사소견을보이는이질적인질환군이다. 1,2 CIDP 의진단은여러가지임상소견과검사실소견을종합하여이루어지며이러한다양성으로인해 CIDP 의초기진단, 치료의결정과예후를예측하는데어려움이있다. 1,35 CIDP 의특징적인임상적소견중하나는근위약이사지의원위부및근위부모두에서나타난다는것인데특히, Dyck 등 6 은이러한소견이 CIDP 의진단에매우중요한것이라고강조하였고, Barohn 등 1 은원위부, 근위부모두에서균등한근위약이존재하는소위 전형적 22 권 3 호대한신경과학회지 219

2 배종석김병준 인 임상양상을보이는 CIDP 환자군이스테로이드에대한치료반응이높다고하였다. 하지만실제임상에서는균등한임상분포를보이는전형적인 CIDP 외에도불균등한임상분포를보이는다양한 CIDP 를경험하게되며이에대한많은보고들이있다. 이러한 비전형적인 CIDP 에는원위부에국한된근력약화를보이거나,,8 비대칭적인신경침범을보인경우, 9,10 또는하지보다상지에우세한침범이확인된경우 11 등의다양한임상적변형들이포함되어있다. 최근에는 CIDP 의임상적변형들의확인외에도이들의분류가임상적으로유용한지를알아보려는노력이있었다. 대표적으로근위약의분포에따라 CIDP 를분류하면치료의결정과예후를예측하는데유용하다는주장,8 이있는데, 아직이에대하여는논란의여지가있고임상소견과전기생리학및병리학적소견의연관성이확실치않다. American Academy of Neurology (AAN) 의 CIDP 진단기준 4 (Table 1) 은원위부와근위부모두의침범소견이나증상의대칭성을절대적인임상적진단기준으로포함하지않고축삭형말초신경병의특징인원위부침범을주로보이는 비전형적 CIDP 도다른조건이만족된다면 CIDP 진단에포함시키고있다. 따라서임상적으로원위부침범을주로보이는 원위부 CIDP 가임상적경과와치료반응면에서전형적인 CIDP 와차이를보일것인지를분석해볼필요성이대두된다. 본연구에서는 CIDP 의임상및전기생리학적기준 1,36 에합당하면서전형적인근위부위약소견이없는원위부 CIDP 환자의질병경과, 치료반응, 예후를분석하여전형적인 CIDP 와차이가있는지를알아보고이들환자군에서임상소견과전기생리학적소견의연관성을찾 고자하였다. 또한이를통해 CIDP 의임상분포에의한분류가임상적으로유용한지알아보고자하였다. 대상과방법 1. 대상 본연구는삼성서울병원신경과로내원하여 CIDP 로진단되거나추정된 51 명의환자가운데 AAN 의진단기준 4 에합당한 25 명의환자를대상으로하였다. 포함된 CIDP 환자들은임상적으로적어도 2 개월이상의진행성또는재발성의근위부또는원위부근위약소견과 1 지 (limb) 이상의감각이상이상 하지각각또는모두에서존재하고심부건반사의감소또는소실을보였다. 말초신경병증을일으킬수있는약물또는독물에의노출력, 유전성말초신경병증의가족력이나유전성말초신경병증에서나타나는근골격계의변형, 대사성말초신경병증에서나타나는전신성증상및검사실소견, 감각수준 (sensory level) 의존재, 괄약근의이상, 당뇨와비타민부족등을포함한내분비성대사성질환력등이있거나전기생리학적검사상근육병, 운동신경원질환또는신경근접합부질환이의심되는경우, 척추자기공명검사상증상과부합하는압박성신경근병증이의심되는환자는대상에서철저히배제하였다. 2. 임상소견의측정및분류 근력검사를 34 개의근육군에서 modified Medical Research Council (MRC) grading system 을이용하여 Table 1. Electrophysiologic criteria of demyelinating neuropathy (AAN criteria 4 ) Three or four of the following: 1. Reduction in conduction velocity in two or more motor nerves: a. <80% of LLN if amplitude >80% of LLN. b. <0% of LLN if amplitude >80% of LLN. 2. Partial conduction block or abnormal temporal dispersion in one or more motor nerves: either peroneal nerve between ankle and below fibular head, median nerve between wrist and elbow, or ulnar nerve between wrist and below elbow. Criteria for conduction block: <15% change in duration between proximal and distal sites and >20% drop in negative peak area or peaktopeak amplitude between proximal and distal sites. Criteria for abnormal temporal dispersion: >15% change in duration between proximal and distal sites and >20% drop in negative peak area or peaktopeak amplitude between proximal and distal sites. 3. Prolonged distal latencies in two or more nerves: a. >125% of ULN if amplitude >80% of LLN. b. >150% of ULN if amplitude <80% of LLN. 4. Absent F waves or prolonged minimum Fwave latencies (1015 trials) in two or more motor nerves: a. >120% of ULN if amplitude >80% of LLN. b. >150% of ULN if amplitude <80% of LLN. AAN; American Academy of Neurology, LLN; lower limit of normal, ULN; upper limit of normal 220 대한신경과학회지 22 권 3 호

3 원위부만성염증성탈수초성다발성신경병증 : 원위부근력약화가우세한변형 환자가처음병원에방문했을때와이후유의한신경학적변화가있는경우연속하여측정하였다. 원위부와근위부모두에서비슷한위약이있는경우를 typical CIDP (tcidp) 로정의하고, 상 하지원위부에만국한된근위약이있는경우혹은하지근위부에경도의위약 (grade IV 이상 ) 이있으면서상하지원위부에상대적으로심한근위약을보이는경우를 distal CIDP (dcidp) 로분류하였다. 기능적인장애는 modified Rankin disability Scale 12 을이용하여무증상인 0 점에서심한장애로인해전적으로의존적인상태인최대 5 점까지로평가하였다. 질병의임상경과는장애나증상이적어도 1 년이상의기간동안에서서히진행하는진행성과, 재발의사이에부분적또는완전회복이존재하는재발성으로나누었다. 관해는면역치료없이 1 년이상증상이없는경우로하였다. 3. 전기생리학적검사 최대상자극 (supramaximal stimulation) 과표면전극을이용하여표준신경전도검사를하였다. 운동신경은정중신경과척골신경을수관절, 주관절, 액와부위에서자극하고비골신경과경골신경을족근관절, 슬관절부위에서자극하였다. 감각신경은정중, 척골, 비복신경전도를측정하였다. 탈수초성신경병증의전기생리학적기준은 AAN 의기준 4 을이용하였다 (Table 1). 전기생리학적으로탈수초성전도장애의분포는정중운동신경전도검사결과를분석하여구분하였다. 분절성의전도속도저하, 확실한전도차단 (conduction block), 또는비정상적으로증가된시간적분산 (temporal dispersion) 이없으면서종말잠복기만 AAN 진단기준에맞는탈수초범위 (demyelinatiing range) 로연장된경우를 전기생리학적인원위부탈수초형 (electrophysiological distal demyelination type) 으로정의하였다. 반대로종말잠복기의확실한연장없이탈수초범위의분절성전도속도저하나전도차단, 또는비정상적으로증가된시간적분산이존재하는경우를 중간형 (electrophysiological intermediate type), 두가지소견이모두존재하는경우는 미만성탈수초형 (electrophysiological diffuse demyelinating type; distal and proximal demyelinating type) 으로분류하였다. 분류된각각의전기생리학적탈수초분포형과임상적분포형의일치성을알아보았다. 탈수초의분포를구분하는또다른방법으로감각신경전도의분석중에서 비정상정중신경정상비복신경소견 (abnormal mediannormal sural response) 의존재를알아보았다. 이는정중신경전도가비복신경전도에비해기술적으로더원위부에서검사되기때문에탈수초현상이원위부에만국한되어발생하는경우보일수있는소견으로길랑 바레증후군과 CIDP 에서탈수초병 변이원위부신경종말에국한되어있을때나타난다고보고된바있다. 2,13 본연구에서는 tcidp, dcidp 군간의 비정상정중신경정상비복신경소견 의양성빈도를비교하였다. 4. 검사실검사 포함된모든환자에서일반혈액검사, 간기능검사, 일반화학검사, 적혈구침강속도, 비타민 B12, B 형간염항원항체검사, 뇌척수액백혈구세포수, 단백수치를포함한뇌척수액검사를하였다. 항핵항체, 류마토이드인자, 한랭글로불린을포함한기타특수검사들은환자의병력과검사소견에따라검사의필요성에대한담당주치의의판단에의해추가되었다. 16 명의환자에서는 M 단백의확인을위한혈청단백전기영동검사및면역고정전기영동검사를하였다. 일부환자에서는 anti MAG 항체나 antigm1 항체검사를하였다. 5. 치료및치료반응의평가 환자들은 Predinisone, intravenous immunoglobulin (IVIg), azathioprine 치료를받았으며 predinisone 이나 IVIg 가첫단계 (firstline) 치료로선택되었다. Prednisone 으로치료를시작할때용량은 1~1.5 mg/kg 였고환자가기복없는임상적호전을나타내는경우단계적감량이이루어졌고약 2~3 개월의투여에도현저한호전소견이보이지않는경우감량하면서다른치료의선택이고려되었다. 치료경과중의평균용량은약 40 mg 이었다. IVIg 는총 2 g/kg 을 2 일혹은 5 일간투여하였고증상의재발이나악화시에재투여를고려하였다. azathioprine 은다른치료의반응이불량하거나장애의정도가위중한경우추가하였다. Modified Rankin disability scale 이 1 점이상감소하거나어떠한근육에서도 MRC 1 단계이상의근력향상소견이보이면객관적치료반응을보이는것으로간주하였고이상감각등의감각증상이나일반적인전신상태의주관적인호전여부는문진을통해평가하였다. 6 개월이상의기간동안치료없이이러한객관적호전이보이는경우는이를자발적인호전으로평가하였다. 6. 통계학적분석 tcidp 와 dcidp 환자군의임상소견, 검사실소견, 전기생리학적소견을비교하였다. 결과는평균 ± 표준편차로나타내었으며, 명목변수는 chisquare test, 연속변수는 MannWhitney U test 를이용하여분석하였다. p 값이 0.05 이하인경우를통계학적으로유의한것으로해석하였다. 22 권 3 호대한신경과학회지 221

4 배종석김병준 결과 1. 임상소견의특징 25 명의환자중 16 명이 tcidp, 9 명이 dcidp 로분류되었다. dcdip 환자들은 tcidp 환자들에비해높은연령에서증상이시작되었으며, 최대장애에도달하는기간도 tcidp 보다길었다. tcidp 군에서는재발형 (relapsing remitting) 의질병경과가, dcidp 군에서는진행형 (chronic progressive) 의질병경과가유의하게많았다. 그외의다른임상적소견으로질병시작전의상기도염병력이나동통의존재여부, 세섬유 (small fiber) 증상의우세여부에대한항목들을비교해보았으나두군사이의유의한차이는보이지않았다 (Table 2). 2. 전기생리학적소견과탈수초소견의분포 탈수초성전도장애를나타내는항목중에서 tcidp 군에서는전기생리학적으로명확한전도차단과비정상적으로증가된시간적분산소견이전체 16 명중 14 명에서확인된반면, dcidp 군에서는전체 9 명중에서단 1 명만이그런소견을보여유의한차이를보였다. 전기생리학적인탈수초분포의분류, 즉원위부탈수초형, 중간형, 미만성탈수초형으로나누어이를임상적원위부증상인 dcidp 군과미만성증상인 tcidp 군을대응시켜임상적 전기생리학적일치정도를분석하였으 나모든아형에서유의한일치성을보이지않았다. 비정상정중신경정상비복신경소견 의발생빈도도두군사이에유의한차이가없었다 (Table 3). 3. 혈액및뇌척수액검사소견의비교 1 명의환자가 IgG 형 M 단백양성을보였고 anti MAG 항체는 tcidp 환자 1 명에서양성을보였으며 AntiGM1 항체가양성인경우는없었다. 각군의모든환자에서뇌척수액검사를하여뇌척수액단백수치를측정하였다. 평균단백수치는 tcidp 군에서는 121.8±1.5 g/dl, dcidp 군에서는 118.6±44.0 g/dl 로두군사이의유의한차이는없었다. 4. 면역조절치료에대한반응과장애척도의비교 15 명의 tcidp 와 명의 dcidp 환자에서한가지이상의면역조절치료를하였다. 치료후의객관적인호전을보인환자는 tcidp 군에서 dcidp 군보다유의하게많았지만주관적인호전까지포함하여분석하였을때에는두군사이에차이가없었다. Modified Rankin disability score 로측정한장애정도의비교에서는처음진단당시와악화되었을당시모두에서 dcidp 가 t CIDP 군보다유의하게장애정도가낮은소견을보였다 (Table 4). Table 2. Clinical features in patients with tcidp and dcidp tcidp (n=16) dcidp (n=9) p value Age at onset, y 41.1± ± Malefemale ratio 6/10 6/3 Duration of symptoms at diagnosis, mo 14.0± Duration to achiving maximal disability, mo 8.4± ± Followup, mo 3.9± ±18.2 Course No. of patients (%) Relapsing remitting Chronic progressive Pattern No. of patients (%) Sensorimotor Predominantly sensory Pure sensory Special findings No. of patients Presence of URI history Presence of pain Predominantly small fiber symptoms Cranial nerve involvement 11 (69) 5 (31) 14 (88) 2 (12) 0 (0) (22) (8) (8) 1 (11) 1 (11) tcidp; typical chronic inflammatory demyelinating polyradiculoneuropathy, dcidp; distal chronic inflammatory demyelinating polyradiculoneuropathy, ; not significant 대한신경과학회지 22 권 3 호

5 원위부만성염증성탈수초성다발성신경병증 : 원위부근력약화가우세한변형 Table 3. Electrophysiologic findings in patients with tcidp and dcidp Abnormal parameters No. of patients Prolonged terminal latency Slow conduction velocity Conduction block / temporal dispersion Electrophysiologic patterns No. of patients(%) Distal Intermediate Diffuse Distal CMAP duration, ms Median Ulnar Peroneal Posterior tibial tcidp (n=16) dcidp (n=9) p value (19) 6 (38) (43) 9.4± ± ± ± (11) 3 (33) 5 (56) 9.13± ± ± ±13.60 Abnormal mediannoraml sural response 4 1 CMAP; compound muscle action potential, tcidp; typical chronic inflammatory demyelinating polyradiculoneuropathy, dcidp; distal chronic inflammatory demyelinating polyradiculoneuropathy <0.01 Table 4. Treatment response and disability score in patients with tcidp and dcidp Treatment No. of patients Total receiving any treatment Prednisone Intravenous immuno globulin azathioprine Improvement No. of patients (%) subjective improvement Objective motor response Experience of spontaneous recovery Experience of complete remission Disability score Initial At worst After treatment tcidp (n=16) dcidp (n=9) p value (94) 14 (88) 2 (13) 5 (31) 2.3± ± ± (56) 3 (33) 0 (0) 0 (0) 1.4±0.5 1.±0.5 1.±0. tcidp; typical chronic inflammatory demyelinating polyradiculoneuropathy, dcidp; distal chronic inflammatory demyelinating polyradiculoneuropathy, ; not significant 고찰 본연구에서원위부 CIDP 는변형 CIDP 의하나로전형적인 CIDP 에비해특히, 임상적소견, 신경학적장애가심한정도와치료반응성면에서유의하게다름을확인할수있었다. 원위부 CIDP 는전형적인 CIDP 에비해상대적으로고령에서증상이시작되어최대장애를나타내기까지보다긴기간이소요되었다. 기복이심한재발성의경과보다는만성진행성의임상경과를보였으 며경한신경학적장애를보이면서이는경과중에큰변동없이유지되는양상이었다. 하지만면역조절치료에대해서는전형적인 CIDP 에비해불량한반응을보임이확인되었다. 이처럼단순한임상적인분류가질병의진행경과및치료에대한반응을예측하는데도움이될수있다면실제임상에서 CIDP 가의심되는환자에대한접근에매우유용하며 CIDP 라는이질적인질환군을이해하는데도움이될것이다. 근래들어 CIDP 의다양한임상양상만큼이나다양한 22 권 3 호대한신경과학회지 223

6 배종석김병준 CIDP 의임상분류방법들이제시되었다. 이중대표적으로 Katz 등 8 은후천성탈수초성신경병증의범주에서절대적으로원위부에만국한된운동신경또는순수감각신경증상만을보이는신경병증을 CIDP 와는별개의질환으로구분하여원위부후천성대칭성신경병증 (distal acquired demyelinating symmetric neuropathy; DADS) 으로명명하였다. 이들은 M 단백의존재여부에따라서 DADS 를단클론성단백과연관된후천성대칭성신경병증 (DADS associated with a monoclonal protein) 과특발성후천성대칭성신경병증 (idiopathic DADS not associated with a monoclonal protein) 으로분류하고이중 Ig M kappa M 단백과연관된 DADS 는고령에서발병하여느리게진행하며전기생리학적으로는원위부탈수초성전도장애를대부분에서보이며면역조절치료에나쁜반응을보인다고하였다. M 단백음성인특발성 DADS 는순수감각증상만나타나는경우가흔하여감각성 CIDP (sensory CIDP) 14,15 의한형태일수있다고하였다. 그러나특발성 DADS 는본연구의 1 예에서보듯이임상적으로는순수감각증상만을보이지만전기생리학적으로는운동신경을침범하는소견을보이기도하며검사소견이다양하여아직까지구분되는별개의질환으로서인정되지못하고있다. 임상적표현형과이상단백 (paraprotein) 의유무를연관하여분류하고자하는노력은병태생리적인측면에서의 M 단백의역할에무게를두고있지만 Mygland 등 의보고에의하면 M 단백의존재가 DADS 의임상양상및치료반응에아무런영향을주지않았고전체 CIDP 군과 DADS 군을비교할때 M 단백양성비율은약 30% 전후로비슷하였다. 따라서과연 M 단백이병태생리적혹은임상적인면에서얼마나중요한역할을할지의심하게한다. 특발성 DADS 환자를오랫동안추적관찰을해도계속해서원위부에국한된증상을보이면서전형적인 CIDP 와는다른경과를보이는경우가적지않아서특발성 DADS 를단순히전형적인 CIDP 의초기단계라고보기도어렵다. 특발성 DADS 와본연구의 dcidp 가유사한점이많지만정의상구분되는명확한점을지적하면, 첫째, dcidp 는이상단백과같은검사실소견이아닌임상적증상분포에따른분류이고둘째, dcidp 는 DADS 와달리근위부, 원위부모두에서근위약소견이있을수있지만하지근위부근력약화가심하지않으면서 (grade IV 이상 ) 원위부는근위부에비해심한근위약차이 (grade 2 단계이상 ) 를보이는경우를모두포함하였다는것이다. 따라서저자들은 dcidp 를별개의질환이라기보다는 CIDP 에속하는여러가지변형중하나로생각하였고다만이러한변형을구분하는것이임상적으로어떤유용성이있는지를분석하였다. 원위부의선택적인침범이전기생리학적으로특징적인소견을보이는지확인해보고자하였으나본연구에 서사용한방법으로는 dcidp 환자의선택적원위부이상을나타내는소견을발견하지못하였다. 이것은첫째, 말초신경의원위부탈수초성전도장애가곧임상적원위부근위약을반영하지않을가능성과둘째, dcidp 로정의된환자에서보이는근위부와원위부근력차이가전기생리학적으로는명확히구분하기가어렵다는가정을할수있다. 따라서 dcidp 는전기생리학적으로는명확히구별되기어렵고임상소견및치료반응과같은임상적인면에서전형적인 tcidp 와구별이가능한 CIDP 의임상적변형으로생각된다. 물론, dcidp 라는전기생리학적뒷받침이없는임상적변형의개념에는논란의여지가충분히있을수있다. 하지만현재까지받아들여지고있는 CIDP 의진단기준들은임상소견보다는주로전기생리학적소견에중점을둔면이있고전기생리학적기준자체가특이도는높은반면민감도는상대적으로낮은점이있어진단및치료에대한임상적접근보다는연구를위한기준에더합당하다는비판이있다. 1,3,16 따라서이러한 CIDP 진단기준의불완전성은객관적인전기생리학적소견못지않게 CIDP 의매우다양한임상상의분류를실제임상에폭넓게반영함으로써이의유용성을판단해보는시도가필요할것으로생각되며본연구는그중의한시도로생각된다. 본연구에서원위부 CIDP 를단순히근위약의분포만으로정의하였는데근위부와원위부의구분이나근력분포양상을아직검증되지않은임의적인정의에따라분류하였기때문에엄격한진단기준을마련하기어렵다는제한점이있다. 이는향후원위부 CIDP 의임상적, 병인론적연구에의해해결될수있을것이다. 본연구는조절된 (controlled) 연구가아닌후향적병록검토를통한연구라는점에서포함된대부분의환자들이치료면에서나임상적추적과정상비교하는데오류가있을수있다. 마지막으로, 환자수가적었기때문에통계학적인분석에제한이있으며일반적인적용에도미흡한점이있다. 이러한한계점들은앞으로보다많은환자에대한전향적이고조절된연구를통하여확인되어야할것이다. REFERENCES 1. Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol 1989;46: Kuwabara S, Ogawara K, Misawa S, Mori M, Hattori T. Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 2002;2: Bromberg MB. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy. Muscle Nerve 1991;14: 대한신경과학회지 22 권 3 호

7 원위부만성염증성탈수초성다발성신경병증 : 원위부근력약화가우세한변형 4. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology 1991;41: Sander HW, Latov N. Research criteria for defining patients with CIDP. Neurology 2003;60:S8S Dyck PJ, Prineas J, Pollard JD. Chronic inflammatory polyradiculoneuroapthy. In: Dyck PJ, Thomas PK, Griffin JW, Low PAP, Podulso JF, editors. Peripheral neuropathy. 3rd ed.philadelphia:wb Saunders, 1993; Mygland A, Monstad P. Chronic acquired demyelinating symmetric polyneuropathy classified by pattern of weakness. Arch Neurol 2003;60: Katz JS, Saperstein DS, Gronseth G, Amato AA, Barohn RJ. Distal acquired demyelinating symmetric neuropathy. Neurology 2000;54: Thomas PK, Claus D, Jaspert A, Workman JM, King RH, Larner AJ, et al. Focal upper limb demyelinating neuropathy. Brain 1996;119: Midroni G, Dyck PJ. Chronic inflammatory demyelinating polyradiculoneuropathy: unusual clinical features and therapeutic responses. Neurology 1996;46: Gorson KC, Ropper AH, Weinberg DH. Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 1999;22: van Swieten JC, Koudstaal PJ, Visser MC, Schouten HJ, van Gijn J. Interobserver agreement for the assessment of handicap in stroke patients. Stroke 1988;19: Bromberg MB, Albers JW. Patterns of sensory nerve conduction abnormalities in demyelinating and axonal peripheral nerve disorders. Muscle Nerve 1993;16: Berger AR, Herskovitz S, Kaplan J. Late motor involvement in cases presenting as chronic sensory demyelinating polyneuropathy. Muscle Nerve 1995;18: Oh SJ, Joy JL, Kuruoglu R. Chronic sensory demyelinating neuropathy : chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry 1992;55: Thaisetthawatkul P, Logigian EL, Herrmann DN. Dispersion of the distal compound muscle action potential as a diagnostic criterion for chronic inflammatory demyelinating polyneuropathy. Neurology 2002;59: 권 3 호대한신경과학회지 225

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