안근무력증으로 내원한 환자에서 진단된 그레이브스병 1예

최준호 ( Jun Ho Choi ),노동효 ( Dong Hyo Noh ),박병현 ( Byoung Hyun Park ),조정구 ( Chung Gu Jo ) 대한내과학회 2008 대한내과학회지 Vol.75 No.1

Myasthenia gravis and autoimmune thyroid disorders often overlap. It is known that hyperthyroidism occurs in 2~17.5% of patients with myasthenia gravis. Thyrotoxicosis may influence the clinical course of myasthenia gravis. Overlapping clinical features may cause diagnostic confusion when Graves` disease and myasthenia gravis co-exist. Thus, various tests may be needed to distinguish these two diseases. It is clinically important to screen patients with myasthenia gravis for the co-existence of autoimmune thyroid disorders and vice versa. We cared for a patient with Graves` disease associated with ocular myasthenia gravis who presented with fluctuating double vision and ptosis. Ocular myasthenia gravis was diagnosed by electrophysiologic study and presence of acetylcholine receptor antibody. The patient had a favorable clinical and laboratory response to treatment with an anticholinesterase (pyridostigmine) and an antithyroid drug (propylthiouracil), and he had minimal symptoms at the 9-month follow-up examination. (Korean J Med 75:98-102, 2008)

재발된 중증근무력증에 병발된 그레이브스병 1예

임동현,권수경,강상중,김진구,최영식,박요한,김광수,장희경 고신대학교(의대) 고신대학교 의과대학 학술지 2000 고신대학교 의과대학 학술지 Vol.15 No.1

Myasthenia gravis is considered as a systemic autoimmune disorder of acetylcholine receptor and often presented with other autoimmune diseases such as autoimmune thyroid disease, systemic lupus erythematosus, and Rheumatoid arthritis. Graves' disease occurs in 3 to 6 percent of patients association with myasthenia gravis. However, Graves' disease associated with recurrent myasthenia gravis is rare. Recently we experienced a case of Graves' disease associated with recurrent myasthenia gravis. A 25-year-old woman presenting with palpitation, tremor, ptosis and diplopia visited our hospital. Thirteen years ago, she diagnosed myasthenia gravis and treated for 3 months. Chest CT shows enlarged thymus. We performed thymectomy. Histologic examination showed thymic lymphoid hyperplasia. The thyroid function of patient became euthyroid and the clinical symptom related myasthenia gravis improved after the medication of anticholinesterase and propylthiouracil. We reported this case with a brief review of literature.

재발된 중증근무력증에 병발된 그레이브스병 1예

임동현,권수경,강상중,김진구,최영식,박요한,김광수,장희경 고신대학교 의학부 2000 고신대학교 의학부 학술지 Vol.15 No.1

재발된 중증근무력증에 병발된 그레이브스병 1예

임동현,권수경,강상중,김진구,최영식,박요한,김광수,장희경 고신대학교 의학부 2000 高神大學校 醫學部 論文集 Vol.15 No.1

최근 저자들은 흉선증식증을 가진 재발현 중증근무력증 환자에서 동봔된 그레이브스병 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Myasthenia gravis is considered as a systemic autoimmune disorder of acetylcholine receptor and often resented with other autoimmune disease such as autoimmune thyroid disease, systemic lupus erythematosus, and Rheumatoid arthritis. Graves' disease occurs in 3 to 6 percent of patients association with myasthenia gravis. However, Graves' disease associated with recurrent myasthenia gravis is rare. Recently we experienced a case of Graves' disease associated with recurrent myasthenia gravis. A 25-year-old woman presenting with palpitation, tremor, ptosis, and diplopia visited our hospital. Thirteen years ago, she diagnosed myasthenia gravis and treated for 3 months. Chest CT shows enlarged thymus. We performed thymectomy. Histologic examination showed thymic lymphoid hyperplasia. The thyroid function of patient became euthyroid and the clinical symptom related myasthenia gravis improved after the medication of anticholinesterase and propylthiouracil. We reported this case with a brief review of literature.

한국 소아중증근무력증 환아의 HLA type의 특성에 대한 연구

고창준(Chang Jun Coe),이준수(Joon Soo Lee),차병호(Byung Ho Cha) 대한소아신경학회 1995 대한소아신경학회지 Vol.2 No.2

배 경 : 중증근무력증은 아세틸콜린 수용체에 대한 항체의 생성에 의해 신경근 세포막의 후접합부에서의 아세틸콜린 수용체의 차단, 파괴로 수의근의 피로와 쇠약을 보이는 자가면역질환으로 최근 중증근무력증에서의 유전적 요인에 대한 활발한 연구가 진행되어 Human Leukocyte Antigen(HLA)과 밀접한 상관관계가 있다고 알려졌으나 아직 국내에서 소아 중증근무력증과 HLA type에 대한 연구가 없는 실정으로 연구자들은 본 연구를 통하여 한국 소아중증근무력증의 HLA type의 상관관계 및 특징을 일반인의 빈도와 비교 고찰하고 유전적 요인을 분석 연구하여 향후 소아 중증근무력증의 유전적, 신경면역학적 연구의 기초자료로 삼고자 본 연구를 시행하였다. 대상 및 방법 : 1980년 3월 1994년 12월까지 연세의대 소아신경과에서 중증근무력증으로 진단된 증세발현 시기가 15세 이전인 환아 30명과 대조군으로 친인척 관계가 없는 정상인 50례를 대상으로 하였으며 전례에서 Biotest-Lymphotype ABC-72-Oriental well tray(Biotest AG 63303 Dreieich/Germany)로 HLA -A,B C typing 하였고 Terasaki Second DR(60) Tissue Typing Tray, Lot #6A로 HLA- DR, DQ typing을 시행하여 환자군과 대조군을 발병빈도, 임상정도 및 발병연령에 따라 비교 분석하였다. 통계 분석은 SAS법의 Chi-Square test 및 Fisher exact 2-tail test를 사용하여 통계적 유의성을 판정하였다. 결 과 : 1) 30례의 환자 중 남녀비는 1:1.1로 남녀간에 큰 차이가 없었으며 이들의 평균 증세 발현시기는 4.6±3.6세였으며 각 증세 발현 시기별로 남녀간의 통계학적 차이는 없었다(P>0.05). 2) Osserman의 분류에 따라 분류시 Group Ⅰ이 18례(60%)로 가장 많았고 Ⅱa 8례(27%), Ⅱb 1례(3%), Ⅲ 3례(10%)였으며 Group Ⅳ는 한례도 없었다. 3) HLA Antigen의 발현빈도를 대조군과 환자군을 비교할 때 HLA antigen A30, Bw4, Bw6, Cw4, DR9, DQ9의 발현빈도가 환자군에서 의의있게 높았으며(P<0.05) HLA Cw3, DR4는 발현빈도가 환자군에서 의의 있게 낮았다(P<0.05). 4) 증세 발현시기와 HLA Antigen의 발현빈도와의 상관관계는 6세 이전에 A30, DR9, DQ9 등의 발현빈도가 의의있게 높음을 알 수 있었으며 CW4는 6세 이후에서 의의있게 발현빈도가 높았고 Bw4, Bw6는 2세이하와 6세이후에서 높은 발현빈도를 나타냈었다.(P<0.05). 5) Cw3는 특히 3-5세에, DR4는 6세 이전에서 의의 있게 낮은 발현빈도를 보였다(P<0.05). 6) Ocular type과 Non-ocular type으로 분류시 HLA type과의 상관관계를 보면 Bw4, Bw6,DR9은 Ocular type에서, A30은 Non-oular type에서, DQ9은 type에 관계없이 높은 발현빈도를 보였고 CW3의 경우는 Non-oular type에서 더 낮은 발현빈도를 보였다(P<0.05). Cw4는 Ocular type에서 다소 높은 발현빈도를 보였으며 DR4는 Ocular type에서 낮은 발현빈도를 보였으나 모두 통계적으로 유의하진 않았다(P>0.05). 결 론 : HLA type A30, Bw4, Bw6, Cw4, DR9, DQ9 등은 한국 소아중증근무력증 환자에서 정상인 집단보다 더 높은 발현빈도를 보였고 Cw3, DR4는 의의있게 낮은 발현빈도를 보였으며 특히 Bw4, Bw6, DR9은 6세 이전 안형에서 높은 발현빈도를 보였으나 좀 더 의미있는 통계적 유의성을 갖기 위해서는 보다 광범위한 연구가 필요할 것으로 사료된다. In myasthenia gravis, the nicotinic acetylcholine receptor at the neuromuscular junction is the target of an autoimmune reaction. One of the important features in this disorder is a strong association with HLA antigens. The association of HLA A1, B8 and DR3 with generalized myasthenia gravis in Caucasoids is well established but no particular gene has been implicated and there is still no adequate explanation in functional terms. In this study. we determined HLA antigen frequencies and corresponding relative risks in 30 Koreans with juvenile myasthenia gravis and normal controls. Using a microcytotoxicity assay and commercially prepared typing trays, we found that HLA A30, Bw4, Bw6 and Cw3, DR9, DQ9 frequencies were significantly increased in patients compared with the control group and there was a decrease in Cw3 and DR4 significantly. Further analysis between different subgroup of patients showed A30, DR9 and DQ9 were more significantly increased in the cases with onset below the age of 6. But Cw4 frequency was increased in patient with over the age of 6. HLA Bw4, Bw6 and DR9 frequencies had a significantly increased prevalence in patient with ocular myasthenia gravis. But A30. with generalized myasthenia gravis. There was an excess of Bw4, Bw6 and DR9 in both ocular type patients and cases with onset below the age of 6. The Korean patients with juvenile myasthenia gravis are characterised by earlier age at onset. more ocular forms and less clinically severe illness than in whites and these characteristics indicate a special subgroup that correlates with the strong Bw4, Bw6, DR9 association.

중증 근무력증의 외과적 치료

이광선 대한흉부심장혈관외과학회 1994 Journal of Chest Surgery (J Chest Surg) Vol.27 No.7

Myasthenia gravis is a rare condition, affecting only 1/75000. It is characterized by weakness and fatigue of the voluntary muscles due to impaired neuromuscular transmission at the acetylcholine receptor site. It is probably caused by an autoimmune mechanism leading to reduction of the available nicotinic acetylcholine receptors at the neuromuscular junction. A relationship between the thymus and myasthenia gravis was suggested by Weigert in 1901, and Blalock was the first to report the efficiency of thymectomy in the treatment of myasthnia gravis in 1939. Since that time, thymectomy has become increasingly important in managing patients with myasthenia gravis. Fourteen patients with myasthenia gravis underwent thymectomy between September 1987 and March 1994 and got the following results; 1. The sex distribution was 10 females and 4 males with ages ranging from 15 to 51 years[mean, 34.8 years]. 2. Clinical manifestation of ocular symptoms were seen in all cases, dysphagia in 12[85.7%], extremity weakness in 11 [78.6 %], and dyspnea in 3 [21.4 %]. 3. According to the modified Osserman`s classification, 8 patients were in group IIb, 3 in IIa, 2 in I and 1 in IIc, respectively. 4. Histopathologic examination of the resected thymuses revealed hyperplasia in 8 patients, benign thymoma in 3, and malignant thymoma in 3. 5. There were no perioperative or immediate postoperative deaths but one patient died about two months after the thymectomy due to respiratory failure. 6. During the follow-up period, 11 patients [78.6 %] showed clinical improvement. Among them, 4 cases [28.6 %] showed complete remission, 3 [21.4 %] showed marked improvement, and 4 [28.6 %] showed subjective improvement. 7. Among the 8 patients with thymic hyperplasia, clinical improvement was noticed in 7 patients 5 %]. Among the 6 patients with thymoma, 4 patients [66.7 %] showed improvement.

중증근무력증의 증상으로 발현된 대변실금

이상수 대한신경과학회 2022 대한신경과학회지 Vol.40 No.4

I report three cases of fecal incontinence during the clinical course of myasthenia gravis. Fecal incontinence developed after withdrawal from the pyridostigmine and improved after readministration of the pyridostigmine. Myasthenia gravis could affect external anal sphincter and puborectalis function. Myasthenia gravis is presumably one of the causes of fecal incontinence. We must check for fecal incontinence in the patients with myasthenia gravis.

광범위 전신성 경화증 환자에서 D-penicillamine 치료 중에 발생한 중증 근무력증

박도현 ( Do Hyun Park ),이은영 ( Eun Young Lee ),안만수 ( Man Su Ahn ),김인 ( In Kim ),정선미 ( Son Mi Chung ),신정현 ( Jung Hyun Shin ),이창근 ( Chang Keun Lee ),조유숙 ( You Sook Cho ),유빈 ( Bin Yoo ),김광국 ( Kwang Kuk Kim ) 대한류마티스학회 2002 대한류마티스학회지 Vol.9 No.2

D-penicillamine has been used to reduce skin thickening and prevent the development of significant organ involvement in the treatment of scleroderma. This drug has a number of serious adverse reactions including glomerulonephritis with nephrotic syndrome, aplastic anemia, thrombocytopenia, and myasthenia gravis. A 44-year-old woman was admitted for weakness of the extremity muscle during repeated use. Eight months before admission, she visited dermatology department of our hospital. She was diagnosed as having scleroderma. D-penicillamine was started for the treatment of skin lesions. Based on the fluctuation of proximal muscle weakness, high titer of acetylcholine receptor antibody and definite decremental response of Jolly test, she was diagnosed as myasthenia gravis. D-penicillamine was discontinued because of the suspicion of D-penicillamine induced myasthenia gravis. Muscle weakness improved after D-penicillamine was withdrawn. The development of reversible myasthenia gravis may be regarded as a part of general predisposition for autoimmune disease related to the D-penicillamine therapy.

중증근무력증 환자에서의 사시수술

천보영,김승현 대한안과학회 2009 대한안과학회지 Vol.50 No.12

Purpose: To report the results of strabismus surgery in five patients diagnosed with myasthenia gravis with strabismus. Case summary: The authors retrospectively analyzed the effect and timing of surgery for patients who developed strabismus after being diagnosed for myasthenia gravis. Cases 1 and 2 were female myasthenia gravis patients, eight and 45 years of age, who underwent surgery after symptoms developed for correction of exotropia at 33 months and ten years after diagnosis, respectively. Case 3 was a 33-year-old male hyperthyroidism patient who had an exotropia operation six years after his strabismus diagnosis. Cases 4 and 5 were a 22–year-old female and a 50-year-old male patient, who underwent surgery for correction of left hypotropia at 14 months and ten months after diagnosis, respectively. Eventually, a total of three cases of horizontal deviation and two cases of vertical deviation had successful outcomes resulting within ten prism diopters. The patients in cases 4 and 5, both of whom had vertical deviations, experienced a short wait time from the date of symptom presentation until they were able to receive surgery. In particular, case 5 developed left hypertropia two months prior to surgery and the strabismus angle increased until six months prior to surgery. However, the hypertropia stabilized afterwards and the patient finally obtained orthophoria after a left inferior rectus advancement operation. Conclusions: In patients diagnosed with myasthenia gravis and a large angle strabismus, a longer follow-up period from diagnosis to operation is necessary for a successful postoperative outcome and stable strabismus angle. 목적: 사시가 동반된 5명의 중증근무력증 환자에서 사시수술을 시행 후 결과에 대해 보고하고자 한다. 증례요약: 중증근무력증으로 진단 후 사시가 발생한 5명에 대해 수술을 시행한 후 후향적으로 분석하였다. 증례 1, 2는 각각 8세 여아, 45세 여자로 사시 발생 33개월, 10년 후에 외사시로 수술을, 증례 3은 갑상선 항진증과 동반된 33세 남자로 사시 발생 6년 후에 외사시로 수술을 시행하였다. 증례 4는 22세 여자로 복시 발생 후 14개월째 하사시로 수술을 시행 받았고, 증례 5는 50세 남자로 복시 발생 10개월 후에 하사시로 수술을 시행하였다. 증례 1, 2, 3은 수평사시, 4, 5는 수직사시로 모든 증례에서 10PD 이내의 수술결과를 보였다. 증례 4와 5는 사시 발생 후부터 수술일까지 시일이 짧았으며 특히 증례 5의 경우 술 후 2개월째 좌안에 상사시가 나타나 6개월까지 진행한 후 안정되는 경과를 보여 좌안 하직근 전진술을 시행한 후 정위를 보였다. 결론: 큰 각의 사시가 동반된 안근무력증 환자에서 술 전 관찰기간을 충분히 하여, 사시각이 안정된 경우에는 사시수술을 시행하여 술 후 안정된 사시각과 만족스러운 결과를 얻을 수 있었다.

중증근무력증에 의한 개의 거대식도증 3례

이희천,전준혁,조규완,강병택,정동인 한국임상수의학회 2012 한국임상수의학회지 Vol.29 No.1

Three dogs (An 8 years-old intact female Poodle, a 7 years-old intact male Schunauzer, and an 8 yearsold Golden Retriever) were presented due to acute vomiting, dyspnea, and generalized weakness. Megaesophagus was confirmed through radiographic examination in all 3 dogs. Relative oesophageal diameter (ROD) was measured and results of ROD measurements showed the possibility of megaesophagus secondary to myasthenia gravis in three dogs. Thus we performed anticholinesterase test as screening test for myasthenia gravis. In all three dogs, esophageal diameter was reduced after neostigmine methylsulfate administration. For definite diagnosis of acquired myasthenia gravis, serum acetylcholine receptor antibody titer was measured, but definite diagnosis was confirmed only in one case. However,based on history, radiographic findings, anticholinesterase test, ROD measurement, other two cases were still suspected as megaesophagus secondary to myasthenia gravis. Treatment with pyridostigmine bromide was initiated in all dogs,and improvement of esophageal diameter was shown in all dogs. One dog was successfully managed for 15 months after initial treatment and, is still alive, but other two dogs were died shortly after initial treatment, because of severe aspiration pneumonia.