• 재활복지
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• 제20권4호
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• pp.79-106
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• 2016

본 연구는 중증뇌병변장애인 자녀를 둔 어머니들에게 장애교육 현상이 어떻게 경험되고 있으며 그 경험의 의미와 본질을 분석하는 것을 목적으로 한다. 연구를 위해 7명의 중증뇌병변장애인 자녀를 둔 어머니들을 연구참여자로 선정하여 심층면담을 실시하였고, 구술 원자료를 Rennie(2000; 2006; 2007)의 해석학적 질적연구방법론을 적용하여 분석하였다. 분석 결과 53개의 의미단위와 16개의 하위범주 및 7개의 해석학적 범주를 구성하였다. 해석학적 범주는 '매일매일 통곡하는 마음', '자원의 사회적 동원', '앞만 보고 전진', '계란으로 부순 바위', '먼 곳 바라보기', '더불어 사는 교육', '장애교육의 주체화'이었다. 연구참여자들에게 장애자녀 교육은 체념단계, 추스림 단계, 비전세우기 단계와 도전단계, 작은 성취 단계를 통하여 최종적으로는 공동체에 희망걸기 단계로 나아가는 좌절과 희망세우기의 동시경험으로 나타났다. 연구참여자들이 보이는 반복적인 공통의 행위패턴을 분석한 결과 추수형, 현실전략형 및 불굴의 도전형의 세 가지 유형을 보이고 있었다. 이들의 교육경험의 핵심범주는 '중증장애 절망을 교육을 통해 희망으로 바꾸어가는 연단의 과정'이었다고 결론지었다. 이와 같은 연구결과를 바탕으로 중증뇌병변장애인 자녀를 둔 어머니들의 교육경험의 실태와 사회구조적 문제에 대한 논의를 하였고, 어머니들의 장애자녀 교육지원을 위한 구체적 제언을 하였다.

• Clinical and Experimental Pediatrics
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• 제58권9호
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• pp.354-357
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• 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.

• 한국치위생학회지
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• 제9권3호
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• pp.357-368
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• 2009

The purpose of this study was to examine the state of dental treatment among disabled patients by the type of disability. After the medical records of 531 disabled patients who received treatment at the pediatric dentistry in K university hospital, the following findings were given: 1. As for age distribution by year, the rate of patients aged 10 or down rose to 42.5 from 5.1 percent, and the 16-20 age group increased from 16.7 to 24.8 percent. But the rates of patients aged between 11 and 15 and aged 21 and up were on the rise(p<.05). 2. Concerning the type of disability by year, there was an increase in the number of patients with brain lesions, mental retardation, developmental disorder and Down's syndrome(p<.05). As to the number of dental caries by the type of disability, the patients with heart diseases had the most dental caries that numbered 8.49, followed by Down's syndrome, metal retardation, brain lesions, the other disabilities and developmental disorder. 3. In relation to dental treatment experiences by the type of disability, the patients with developmental disorder(57.5%) received the most dental treatment, followed by mental retardation, the other disabilities, brain lesions, Down's syndrome and heart diseases(p<.05). 4. Regarding general anesthesia experience by the type of disability, the patients with mental retardation(31.6%) were put under general anesthesia the most, followed by developmental disorder, brain lesions, the other disabilities, heart diseases and Down's syndrome(p<.05). In conclusion, nationwide efforts to nurture separate dental personnels responsible for the disabled, to expand relevant facilities and to improve the health care insurance are required to promote the oral health of disabled children.

• 대한통합의학회지
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• 제9권3호
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• pp.9-18
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• 2021

Purpose : In this study, we applied a computerized cognitive rehabilitation program (CoTras-C) for children with cerebral palsy. Research was conducted to investigate the impact of upper limb function, sensory function, and activities of daily living. Methods : The study period lasted 10 weeks from October 2019 to December 2019. The study subjects were 12 subjects according to the selection criteria, and a computerized cognitive rehabilitation program (CoTras-C) was conducted twice a week for 30 minutes before and after the application of basic occupational therapy. Results : As a result of the computerized cognitive rehabilitation program, scores of upper limb function (QUEST), sensory function (SSP-2), and daily life activity (WeeFIM) were significantly improved (p>.05). From the result of examining the motor area, improvement in fine-motor function and protective extension through touch pad or controller operation was found. It also showed improvement in activities of daily living including motor and activities of daily living including social cognition. In the sensory function evaluation, it was not significant in movement sensitivity. Significant differences were shown in the items excluding olfactory/taste sensitivity. Conclusion : The application of the computerized cognitive rehabilitation program (CoTras-C) showed significant results in upper limb function, sensory function, and daily life activities of children with brain lesions. Based on these results, future studies need to generalize the study by expanding the age or population of children with brain lesions, or by expanding the diversity of diseases and environments.

• Clinical and Experimental Pediatrics
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• 제59권6호
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• pp.280-284
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• 2016

Cerebral cavernous malformation (CCM) is a vascular malformation characterized by abnormally enlarged capillary cavities without any intervening neural tissue. We report 2 cases of familial CCMs diagnosed with the CCM1 mutation by using a genetic assay. A 5-year-old boy presented with headache, vomiting, and seizure-like movements. Brain magnetic resonance imaging (MRI) revealed multiple CCM lesions in the cerebral hemispheres. Subsequent mutation analysis of his father and other family members revealed c.940_943 del (p.Val314 Asn315delinsThrfsX3) mutations of the CCM1 gene. A 10-month-old boy who presented with seizure-like movements was reported to have had no perinatal event. His aunt was diagnosed with cerebral angioma. Brain and spine MRI revealed multiple angiomas in the cerebral hemisphere and thoracic spinal cord. Mutation analysis of his father was normal, although that of the patient and his mother revealed c.535C>T (p.Arg179X) mutations of the CCM1 gene. Based on these studies, we suggest that when a child with a familial history of CCMs exhibits neurological symptoms, the physician should suspect familial CCMs and consider brain imaging or a genetic assay.

• Clinical and Experimental Pediatrics
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• 제64권3호
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• pp.103-110
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• 2021

Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders.

• Pediatric Infection and Vaccine
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• 제30권2호
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• pp.91-96
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• 2023

Enterovirus (EV) 71은 무균성 뇌수막염 환자로부터 처음 분리된 이후로 주로 수족구병으로 나타났으며 소아에서 전형적인 피부 발진, 구강 병변 및 발열을 동반한다. 피부 발진은 수포성으로 손과 발에 영향을 미치고 수포가 없는 반구진 양상일 수도 있으며 특히 어린 소아 및 유아에서는 팔꿈치, 무릎 그리고 엉덩이를 침범할 수도 있다. EV 71 감염으로 인한 신경학적 합병증은 매우 드물지만 주로 뇌간 뇌염으로 나타난다. 대근육 운동 발달 지연 이외에 건강했던 30개월 된 남아가 발열, 혼수 증상으로 내원하였고 입원 후 무호흡 및 호흡 정지 증상이 동반되어 뇌척수액 검사 및 뇌 MRI, 뇌파검사를 하였다. 검사 결과 뇌간 뇌염 및 미만성 대뇌 기능 장애가 확인되었고 비인두도말 및 대변 검체에서 EV 71이 검출되었으며 유전자 검사에서 myoclonic epilepsy with ragged red fiber syndrome이 확인되었다. 저자들은 기저미토콘드리아 질환으로 인해 인후 및 피부 발진의 증상 없이 발열 하루 만에 빠르게 진행되는 EV 71 감염으로 인한 뇌간 뇌염 1예를 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제55권1호
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• pp.40-42
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• 2014

A correlation between radiation therapy and cavernoma has been suspected since 1994. Since then, only a few cases of radio-induced cavernomas have been reported in the literature (85 patients). Most of them were children, and the most frequent original tumour had been medulloblastoma. The authors report a case of two cystic cavernous angiomas after radiation therapy for atypical meningioma in adult woman. This is the first case of cavernous angioma after radiotherapy for low grade meningioma. A 39-year-old, Latin american woman was operated on for a frontal atypical meningioma with intradiploic component and adjuvant radiotherapy was delivered (6000 cGy local brain irradiation, fractionated over 6 weeks). Follow-up MR imaging showed no recurrences of the tumour and no other lesions. Ten years later, at the age of 49, she consulted for progressive drug-resistant headache. MR imaging revealed two new well defined areas of different signal intensity at the surface of each frontal pole. Both lesions were surgically removed; the histopathological diagnosis was cavernous angioma. This is the first case of cavernous angioma after radiation therapy for atypical meningioma : it confirms the development of these lesions after standard radiation therapy also in patients previously affected by non-malignant tumours.

• Toxicological Research
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• 제21권2호
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• pp.135-140
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• 2005

Although the pathogenesis of cerebral cavernous malformation (CCM) is unknown, a familial predisposition has been recognized, with up to $55\%$ of patients having an affected relatives. Genetic linkage studies have recently mapped a gene causing CCM to a segment of the long arm of chromosome 7 (7q). We report herein a genetic linkage analysis conducted on a Korean three generation family with CCM. It's first report in Korean family. A Korean family in which one member had undergone surgery for ubtracerebrak hematoma (ICH) and confirmed the CCM, was evaluated. They were examined clinically (n=18) and by magnetic resonance (MR) imaging (n=10). Polymorphic markers (D7S1813, D7S1789) spanning the CCM1 locus on 7q were genotyped by the polymerase chain reaction and analysis of linkage was performed in this family (n=17). Six had multiple lesions on brain MR image, one of them being symptomatic, and five were asymptomatic. Seven remaining members were asymptomatic and refused MR image study. One had died of ICH from presumed CCM. Analysis of the pedigree was consistent with an autosomal dominant pattern of inheritance. All affected patients were linked to CCM1. Linkage to CCM1 can account for inheritance of CCM in this family. They had some striking features with a low clinical penetrance and the presence of multiple lesions. These findings have implications for genetic testing of this disorder and represent an important step toward identification of the gene responsible for the pathogenesis of this disease.

• Clinical and Experimental Pediatrics
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• 제57권6호
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• pp.264-270
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• 2014

Purpose: Acute necrotizing encephalopathy (ANE) is a fulminant disease of the brain characterized by bilateral thalamic lesions, and is prevalent among children in East Asia. The prognosis of ANE is usually poor with a high mortality rate and neurological sequelae. This study aimed to delineate the clinical characteristics and prognostic factors of ANE. Methods: We retrospectively analyzed clinical data of 399 pediatric patients with encephalitis who were admitted to Samsung Medical Center from December 1998 to March 2011. We enrolled ten patients (11 cases) with ANE and analyzed their demographic, clinical, and neuroimaging data. The location and extent of the brain regions were checked based on fluid-attenuated inversion recovery, T1-, and T2-weighted imaging findings; the presence of contrast enhancement, restricted diffusion, and hemorrhage. Results: Ten patients were identified, including one patient with two episodes. The median age of onset was 1.5 years (0.4-8.4 years). The mortality rate was 40%, and only 30% of patients survived without neurological sequelae. The definite involvement of the brainstem on brain magnetic resonance imaging was significantly correlated with mortality (P =0.04). Conclusion: Broad and extensive brainstem involvement suggested the fulminant course of ANE. Early diagnosis of ANE before brainstem involvement, through careful identification of symptoms of brain dysfunction, may be the best way to achieve better neurological outcomes.