• Journal of Korean Neurosurgical Society
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• 제53권3호
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• pp.187-189
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• 2013

We report an unusual case of cerebral aneurysmal subarachnoid hemorrage (SAH) with Fabry's disease. A 42-year-old woman presented with aneurysmal SAH originated from a saccular aneurysm of the right posterior communicating artery. The patient was treated by an endovascular coil embolization of aneurysm. Postoperatively the patient recovered favorably without any neurological deficit. During her admission, the patient had a sign of proteinuria in urine analysis. The pathologic findings of kidney needle biopsy implied nephrosialidosis (mucolipidosis of lysosomal stroage disease), which is consistent with a Fabry's disease. It is uncommon that Fabry's disease is presented with aneurysmal SAH, especially in middle-aged patients, but could be a clinical concern. Further investigations are needed to reveal risk factors, vascular anatomy, and causative mechanisms of Fabry's disease with aneurysmal SAH.

• Journal of Korean Neurosurgical Society
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• 제40권5호
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• pp.391-393
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• 2006

Dermoid cysts of the spinal canal are rare benign congenital tumors, accounting for $1{\sim}2%$ of all intraspinal tumors. We report a case of lumbar extramedullary cyst, combined with congenital sacral meningocele. The clinical features, characteristics on MRI, pathologic findings, and surgical treatment of such a rare extramedullary benign tumor is discussed with the relevant literature.

• Annals of Clinical Neurophysiology
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• 제25권2호
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• pp.106-109
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• 2023

Inclusion body myositis (IBM) is a late-onset myopathy that manifests as distinct muscle weakness in the quadriceps, finger flexors, and ankle dorsiflexors. T-cell large granular lymphocyte (T-LGL) leukemia is a late-onset clonal disorder of CD8+ cytotoxic T-cells that is often accompanied by autoimmune diseases. To date, the association between IBM and T-LGL leukemia has been infrequently reported. Here, we report a case of a patient with T-LGL leukemia who developed IBM, along with in-depth laboratory, electrophysiological, and pathologic findings.

• Investigative Magnetic Resonance Imaging
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• 제20권2호
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• pp.114-119
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• 2016

We report a case of vegetation in a 4-year-old female with infective endocarditis, diagnosed by late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) imaging. The patient had a history of primary closure for ventricular septal defect and presented with mild febrile sensation. No remarkable clinical symptoms or laboratory findings were noted; however, transthoracic echocardiography demonstrated a 14 mm highly mobile homogeneous mass in the right ventricle. On LGE CMR imaging, the mass showed marginal rim enhancement, which suggested the diagnosis of vegetation rather than thrombus. The extracellular volume fraction (${\geq}42%$) of the lesion was higher than that of normal myocardium. Based on the patient's clinical history of congenital heart disease and pathologic confirmation of the lesion, a diagnosis of infective endocarditis with vegetation was made.

• Investigative Magnetic Resonance Imaging
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• 제12권2호
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• pp.188-196
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• 2008

목적: 이 연구의 목적은 소아 수막종의 임상적, 영상의학적, 그리고 병리학적 특징을 기술함에 있다. 대상 및 방법: 병리학적으로 진단된 수막종을 가진 16예의 소아환자를 대상으로 임상기록과 자기공명영상을 후향적으로 분석하였다. 평균연령은 14세 (3-18세)였다. 자기공명영상은 병변의 크기, 신호강도, 경계부의 특징, 내부 구조, 조영증강, 그리고 경막과 뇌실질의 변화를 검토하였고 다른 영상 방법 및 병리소견을 함께 분석하였다. 결과: 종양의 평균 크기는 5.24 cm (1.3-18.1 cm)였으며 12예는 천막상부에 4예는 천막하부에 위치하였다. T2 강조 영상에서 높은 신호강도를 보이는 종양이 9예 있었고 4예에서 동신호강도를, 3예에서 낮은 신호강도를 보였다. T1 강조 영상에서는 낮은 신호강도 11예, 동신호강도 4예, 그리고 높은 신호강도 1예가 있었다. 모든 종양의 경계가 좋았고 조영증강을 보였다. 5예에서 종양은 균질한 양상을 보였으며 나머지에는 낭종이나 괴사에 의해 비균질한 양상을 보였다. 경막 부착 소견이 11예에서, 뇌실질 부종이 10예에서 동반되었다. 컴퓨터 단층촬영 소견은 6예에서 밝은 음영을 보였으며 5예에서 석회화를 동반하였다. 혈관 조영술 상 3예는 내경동맥에서, 4예는 외경동맥으로부터 혈액 공급이 이루어졌다. 병리검사결과 종양의 아형은 이행성 (4예), 수막세포성 (4예), 척삭성 (2예), 섬유성 (2예), 투명세포성 (1예), 유리질화성 (1예), 횡문근양 유두모양 (1예), 그리고 비정형(1예) 수막종으로 진단되었다. 결론: 소아의 수막종은 주로 10대에 발생하며 다양한 병리학적 아형을 보일뿐만 아니라 비정형적인 영상 소견으로 인해 축내 종양으로 오인될 수 있다.

• Clinical and Experimental Pediatrics
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• 제46권11호
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• pp.1107-1111
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• 2003

목 적 : 진행된 신경모세포종 환아의 약물치료 후 수술한 예에서 조직변화를 관찰하여 예후와의 상관관계를 알아보고자 이 연구를 시행하였다. 방 법 : 1991년 1월부터 2000년 6월까지 계명대학교 동산병원 소아과에서 진단된 신경모세포종 환아 중 항암치료 후 종양절제를 시행한 6례를 대상으로 하였다. 진단 당시 침생검소견과 항암치료 후 조직소견에서 신경모세포의 분화정도와 핵 모양 및 간질조직의 기질화 양상과 석회와 유무 등을 비교하여 변형된 Shimada 분류에 따라 분류하였다 결 과 : 항암치료 전과 후에 조직소견의 변화를 관찰하였으며 변형된 Shimada 분류에 따라 2가지 예후군으로 분류하였으며, 좋은 예후군 3례 중 2명은 생존해 있으며, 1명은 추적되지 않고 사망한 것으로 추정하였으며, 나쁜 예후군의 3례는 각각 패혈증과 retinoic acid 복용 중 발생한 출혈성 췌장염으로 사망하였다. 결 론 : 항암치료 전과 후에 조직소견의 변화를 관찰하였으며 Shimada 분류에 따라 비교하고, 치료 후 종양세포의 성숙을 관찰하였으며, 좋은 예후군에서 생존수가 많은 것을 확인할 수 있었다.

• Tuberculosis and Respiratory Diseases
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• 제82권4호
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• pp.277-284
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• 2019

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.

• 대한골관절종양학회지
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• 제2권1호
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• pp.60-64
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• 1996

Giant cell tumor of tendon sheath is a slow-growing, unilateral and solitary lesion that is most commonly seen in the digits of the hand and knee, but occasionally occurs in the hips, ankles, toes and wrists. The lesion is asymptomatic or mildly painful, especially if it is diffuse and located in a major joint. The concepts concerning about the pathogenesis of these lesion have undergone constant revision, which include neoplastic process, inflammation and lipid metabolism. Authors analysized 20 patients with giant cell tumor of tendon sheath about the etiology, clinical findings, pathologic findings and treatment results. The results were as follows: 1. 13 cases were females (65%) and 7 cases were males (35%), and the range of age was from 9 years old to 60 years old. 2. The hand was most frequently involved site in which 14 cases (70%) were included, and the foot was involved in 5 cases (25%). 3. Solitary lesions were 15 cases and multiple lesions were 5 cases. 4. Radiographically, in 3 cases bony erosion was seen. 5. All cases were treated by surgical excision and presented no recurrence. In conclusion, the giant cell tumor of tendon sheath, which has been considered to be benign tumorous conditions appeared to be necessary for complete surgical excision to prevent recurrence.

• 대한골관절종양학회지
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• 제4권2호
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• pp.88-93
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• 1998

Neurilemoma usually discovered incidentally, is a benign nerve-sheath tumor which has been described as a painless mass. In most cases, the size of the mass was smaller than 5cm. However, it was reported that there were masses, sometimes associated with local tenderness and pain, whose size was over 6cm. Then, we have found there is a relationship between mass size and neurologic symptoms, as well as with, postoperative complications. It is important to diagnose early and to treat it. Twenty neurilemoma patients, who were treated at Keimyung University Dongsan Medical Center were analyzed using their clinical symptoms, pathologic findings, radiologic findings and complications. There was no sexual difference in tumor incidence. The anatomical locations of tumors were as follows. ; upper extremities in 15 cases(69%), axilla in 1 case(4%) and lower extremities in 6 cases(27%). Symptoms were palpable mass in 22 cases, local tenderness in 8 cases(36%), radiating pain in 6 cases(27%) and paresthesia in 6 cases(27%), Median nerve was involved most frequently(33%). There were 2 patients(10%) with multiple symptoms. Tumor enucleation was done in all cases. The size of tumors in longest axis was smaller than 2cm in 2 cases, between 2 and 4cm in 11 cases and more than 5cm in 9 cases. There was no case of malignant transformation or recurrence. In conclusion, incidence of clinical symptom and postoperative complications are increased with the size of the tumor especially over the 5cm. Preoperative MRI finding was the most accurate method of diagnosis and most helpful in determining surgical resection margin.

• Endocrinology and Metabolism
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• 제32권3호
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• pp.353-359
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• 2017

Background: Cushing syndrome is characterized by glucose intolerance, cardiovascular disease, and an enhanced systemic inflammatory response caused by chronic exposure to excess cortisol. Eosinopenia is frequently observed in patients with adrenal Cushing syndrome, but the relationship between the eosinophil count in peripheral blood and indicators of glucose level in patients with adrenal Cushing syndrome has not been determined. Methods: A retrospective study was undertaken of the clinical and laboratory findings of 40 patients diagnosed with adrenal Cushing syndrome at Chungnam National University Hospital from January 2006 to December 2016. Clinical characteristics, complete blood cell counts with white blood cell differential, measures of their endocrine function, description of imaging studies, and pathologic findings were obtained from their medical records. Results: Eosinophil composition and count were restored by surgical treatment of all of the patients with adrenal Cushing disease. The eosinophil count was inversely correlated with serum and urine cortisol, glycated hemoglobin, and inflammatory markers in the patients with adrenal Cushing syndrome. Conclusion: Smaller eosinophil populations in patients with adrenal Cushing syndrome tend to be correlated with higher levels of blood sugar and glycated hemoglobin. This study suggests that peripheral blood eosinophil composition or count may be associated with serum glucose levels in patients with adrenal Cushing syndrome.