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- 저자 : 심지윤(Ji Yun Sim) (검색결과 2 건)
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모야모야병과 폐 신경절신경종이 동반된 신경섬유종증 1례
심지윤(Ji Yun Sim),임정숙(Jeong Sook Lim),한윤수(Youn-Soo Han),김지윤(Ji-Yoon Kim),김원섭(Won Seop Kim) 대한소아신경학회 2009 대한소아신경학회지 Vol.17 No.2
Neurofibromatosis type I is one of the most common neurocutaneous syndrome which is inherited by autosomal dominant manner, characterized by cafe au-lait spots, axillary freckling, Lisch nodules in iris, multiple neurofibromas and bone involvement with pseudoarthrosis, bowing of the long bone. And Moyamoya disease is a specific disease characterized by progressive idiopathic stenosis and eventual occlusion of the large cerebral arteries at the circle of Willis. In response to the stenosis, an abnormal network of small collateral vessels develops, creating the "puff of smoke". Intracranial lesions associated with Neurofibromatosis type I include optic glioma, sphenoid wing dysplasia, "unidentified bright objects" and cerebrovascular lesions such as Moyamoya syndrome and aneurysm. Moyamoya syndrome is an uncommon association of neurofibromatosis type 1 and lung mass has not frequently been found with neurofibromatosis and moyamoya syndrome. We report a case with Neurofibromatosis type I with Moyamoya syndrome and ganglioneuroma in lung with reviewing literatures. 제 1형 신경섬유종증은 혈관 병변을 동반하여 중한 합병증을 나타낼 수 있다. 이러한 혈관 병변은 어느 부위에서도 발생할 수 있으나 폐쇄성 뇌혈관 질환이 동반된 증례는 흔하지 않으며, 뇌자기혈관조영술상 전형적인 모야모야병의 양상을 보이는 경우는 드문 것으로 알려져 있다. 신경섬유종증 및 모야모야병으로 진단받은 사례는 국내에 드물게 보고되었고 더욱더 폐 신경절신경종이 동시에 발견된 예는 없었다. 모야모야병은 염색체 17번 염색체 장완 25.2번에서 신경섬유종증은 염색체 17번 염색체 장완 11.2번에서 유전학적이상이 나타난다. 따라서 모야모야병과 신경섬유종증의 관련성은 그들의 유전자가 17번 염색체에 이웃하므로 인한 것으로 판단할수 있다. 저자들은 출생시 부터 전신에 분포하는 밀크 커피색 반점 및 액와부에 작은 주근깨를 보이며 지능저하와 폐 종괴를 동반한 환아에서 시행한 뇌 자기 공명 영상(MRI)과 뇌 자기혈관 조영술(MRA)상 모야모야병, 폐 조직 검사 상 얼기형 신경섬유종을 배경으로 하는 신경절신경종이 동반된 신경섬유종증 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
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자폐증 환아와 주의력 결핍 과잉운동장애 환아의 신경학적 이상
심지윤(Ji Yun Sim),손정우(Jung-Woo Son),김용대(Yong-Dae Kim),김원섭(Won Seop Kim) 대한소아신경학회 2010 대한소아신경학회지 Vol.18 No.2
Purpose : Autism spectrum disorder (ASD) and attention-deficit hyperactivity disorder (ADHD) are neurodevelopmental disorders. previous report have examined the neurologic problems both in ASD and ADHD. Methods : We studied 49 children with ASD and 51 with ADHD who had visited our hospital's neuropsychiatric clinic from January 1999 to December 2008. We examined age, EEG abnormalities, developmental delays, and seizures in both groups. Results : 1) The mean age of children diagnosed with ASD was 4 years while that for ADHD was 9 years (P =0.000). 2) The percentage of abnormal EEG in children with ASD was 54.5% and that for those with ADHD was 25.4% (P =0.027). 3) The percentage of developmental delays in children with ASD was 89.8% and that for those with ADHD was 39.2% (P =0.000). 4) The mean age of first seizure in children with ASD was 13 years and that for those with ADHD was 6 years (P=0.005). Conclusion : The percentage of abnormal EEG and developmental delay in children with ASD was more than in those with ADHD. The mean age of diagnosis in children with ASD was younger than in those with ADHD and the mean age of first seizure in children with ASD was older than in those with ADHD. The percentage children with ASD who exhibited normalized EEGs upon follow-up was greater than that for children with ADHD.
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