• Title/Summary/Keyword: 신경발달장애

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Application of Occupation-Based Practice for Neurodevelopmental Children's Subway Use (신경발달 장애 아동의 지하철 이용하기 작업에 대한 작업기반 중재의 적용)

  • Ra, Dae-Yeop;Kong, Myung-Ja;Chang, Ki-Yeon
    • Korean Journal of Occupational Therapy
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    • v.26 no.4
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    • pp.27-41
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    • 2018
  • Objective : This study implemented occupation-based practice as a group program for children with neurodevelopmental disabilities, and investigated the quality of occupational performance, performance and satisfaction, and efficacy levels of the program. Methods : ADOC-S(Aid for Decision making in Occupation Choice-School) and COPM(Canadian Occupational Performance Measure) were used to establish common meaningful occupational goals for 10 children with neurodevelopmental disabilities. After occupational analysis of the 'subway use', the Performance Quality Rating Scale(PQRS) were used to evaluate the quality of occupational performance. Occupation-based interventions were implemented by participants individually performing meaningful occupations in the most natural and real-life environments as possible. The trial consisted of 1 session of pre-intervention assessments, 8 sessions of intervention, where a single session of 100 minutes is provided once a week. Post-intervention evaluation was conducted over one session. Results : The quality of occupational performance by PQRS was 38.10 higher than before intervention. The occupation-based intervention as a group program used the maintain and modify(compensation, adaptation) approaches and acquisition model. In the early stages of intervention (1 to 4 sessions), the occupational performance was greatly improved. The performance level of COPM increased by 5.80 and the satisfaction level was increased by 7.00. In addition, high scores were found in program satisfaction through parental interview. Conclusion : The results of implementing occupation-based interventions to children with neurodevelopmental disabilities showed increase in quality of occupational performance, performance, and satisfaction along with positive effects of satisfaction levels for the program.

Early Diagnosis of KBG Syndrome Using Diagnostic Exome Sequencing (Diagnostic exome sequencing을 통한 KBG 증후군의 조기 진단)

  • Hong, Jun Ho;Kim, Se Hee;Lee, Seung Tae;Choi, Jong Rak;Kang, Hoon Chul;Lee, Joon Soo;Kim, Heung Dong
    • Journal of the Korean Child Neurology Society
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    • v.26 no.4
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    • pp.272-275
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    • 2018
  • KBG syndrome is a rare neurodevelopmental disorder characterized by intellectual disability, skeletal anomalies, short stature, craniofacial dysmorphism, and macrodontia. ANKRD11 gene mutation and 16q24.3 microdeletion have been reported to cause KBG syndrome. Here, we report two patients with ANKRD11 mutations who initially presented with neurologic symptoms such as developmental delay and seizures. Patient 1 was a 23-month-old boy who presented with a global developmental delay. Language delay was the most dominant feature. He had hypertelorism, hearing impairment, and behavior problems characterized as hyperactivity. A c.1903_1907delAAACA (p.Lys635GInfsTer26) mutation in ANKRD11 was identified with diagnostic exome sequencing. Patient 2 was a 14-month-old boy with developmental delay and seizure. He also had atrial septum defect, and ventricular septal defect. Generalized tonic seizures began at the age of 8 months. Electroencephalography showed generalized sharp and slow wave pattern. Seizures did not respond to antiepileptic drugs. A loss of function mutation c.5350_5351delTC (p.ser1784HisfsTer12) in ANKRD11 was identified with diagnostic exome sequencing. In both cases, characteristic features of KBG syndrome such as short stature or macrodontia, were absent, and they visited the hospital due to neurological symptoms. These findings suggest that more patients with mild phenotypes of KBG syndrome are being recognized with advances in diagnostic exome sequencing genetic technologies.

TIC DISORDER AND OBSESSIVE COMPULSIVE DISORDER IN CHILDHOOD (틱 장애 및 소아기 발병 강박 장애)

  • Hong, Hyun-Ju;Song, Dong-Ho
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.16 no.2
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    • pp.183-191
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    • 2005
  • Tic disorder including Tourette's disorder is a neurodevelopmental disorder that appears in childhood and characterized by the presence of motor and vocal tics. Childhood-onset obsessive-compulsive disorder (OCD) is suggested to be a phenomenologically and etiologically distinct subtype of OCD, bearing a close genetic relationship to tic-disorders. Tourette's disorder and OCD are comorbid in $40-75\%$ of patients initially diagnosed with either disorder. Basal ganglia and cortico-striato-thalamic circuits are implicated in the pathophysiology of both disorders and these disorders have similar clinical features. Over the past decades, the progress in research on Tourette's disorder and OCD has been extraordinary. This review describes some of important insights from these work, involving these areas : 1) clinical implication 2) genetics and epidemiology 3) brain imaging study 4) neuroche-mistry 5) pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS).

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AUTISTIC DISORDER - AN OVERVIEW OF THE NATURE AND THE CHANGING CONCEPTS IN COMMEMORATION OF KANNER'S ORIGINAL PUBLICATION - (자폐장애 - 자폐장애의 본질과 개념변천에 관한 고찰 -)

  • Hong, Kang-E.M.
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.4 no.1
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    • pp.3-26
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    • 1993
  • Leo Kanner (1943)의 자폐증에 관한 획기적 논문발표 50주년을 맞아, 자폐증의 개념변천, 분류, 의학, 원인설 및 자폐증의 본질을 문헌고찰을 통해 살펴 보았다. 초기에 자폐증을 정신병의 아형으로 보다가 1980년 (DMS-III)을 기점으로 전반적 발달장애로의 개념 변천이 일어났다 원인설도, 초기의 심리${\cdot}$환경설은 지지 받지 못하고 1960년대에는 신경${\cdot}$생물학적 이상이 자폐병리의 기저를 이룸이 분명해 졌고 1970년대에는 지각과 운동, 감각과 인지 통합의 결함, 심각한 언어, 인지의 장해가 일차적인 결함으로 생각 되었다 최근 1980년 후반기 부터 상징적${\cdot}$표상적 인지의 결함, 타인의 감정과 생각의 이해 결함, 사회적${\cdot}$정감적 표현의 결함등 사회${\cdot}$정서발달의 이상이 자폐의 근본적 결함이라는 비교 관찰 연구가 많이 보고되어, 자폐증의 근본적이고 일차적인 결함이 정감적 접촉의 선천적 장애라는 Kanner의 놀라운 임상적 통찰을 증명해 주고 있다. 저자는 이상의 광범위한 문헌 고찰을 통해 자폐장애를 일차성 애착장애로 개념화하고 앞으로 치료, 교육의 방향도 일차적으로 사회${\cdot}$정서발달에 촛점을 두어야 하며, 특히 어머니와의 애착증진 치료가 필요함을 제안하고 있다.

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A study on the realization of community care for the developmentally disabled: Focusing on Japanese community practice (발달장애인 커뮤니티 케어 실현방안 연구: 일본의 지역사회 실천을 중심으로)

  • Shin, Kyung-An;Lee, Eun-A;Kim, Do-Hoon
    • Journal of Industrial Convergence
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    • v.18 no.5
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    • pp.88-96
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    • 2020
  • The rate of admission to facilities for people with developmental disabilities in Korea is very high for other types of disabilities. Therefore, 'community care', which supports life in the community for people with developmental disabilities, is very important compared to other types of disabilities. However, in Korea, families with disabilities are the problem of care and are appealing for the burden of support. This study analyzed practical cases through welfare institutional visits and interviews on support for community independence in Japan. As a result of the study, the transition of people with developmental disabilities to communities in Japan was centered on group home. The private sector is providing support for people with severe disabilities in group home to live in communities, and Sapporo City Hall is conducting private connections to solve the problem of caring for the developmentally disabled elderly parents. Accordingly, as Korean policy recommendations, it is proposed to expand group home, switch functions of living facilities for the disabled, cope with the problem of caring for the disabled by elderly parents, and provide preventive services through surveys on the actual condition of adults living alone. As practical suggestions, it is necessary to develop and distribute educational textbooks such as pictures to improve daily life skills for self-reliance, expand sufficient manpower and facilities in vocational training for self-reliance, and operate shelters for adults with developmental disabilities and their guardians.

CHILDHOOD TRAUMA:PSYCHIATRIC OVERVIEW (아동기 외상의 정신과적 개관)

  • Han, Sung-Hee
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.13 no.1
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    • pp.3-14
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    • 2002
  • Childhood psychic trauma appears to be a crucial factor in the development of serious disorders both in childhood and in adulthood. Traumatized children show strong tendency to revisualize or re-feel a traumatic events. Play and behavioral reenactments are frequent manifestations of both the single blow and the long-standing traumas in childhood. Those children who suffer the results of single, intense terror appear to exhibit detailed memory, retrospective reworkings and misperceptions. In long-standing or repetitive trauma, children would show psychic numbing, self-hypnosis, dissociation and rage. Child's brain is undergoing critical and sensitive periods of differentiation. During this time, developing central nervous system is exquisitely sensitive to stress. Stressor-activated neurotransmitters and hormones can play major roles in neurogenesis, migration, synaptogenesis, and neurochemical differentiation. Internal opiate system operates in some trauma and causes the victim to fail to respond, to avoid, to shut off feelings. Evidence is also accumulating in traumatology that dysfuntion of locus coeruleus and ventral tegmental neucleus system leads to catecholamine receptors hypersensitivity. This change result in hypervigilance, increased startle, affective lability, and increased autonomic nervous system hyperreactivity. Another site of action of trauma on the brain is hypothalamic-pituitary-adrenal axis. Individuals with PTSD do not have enough cortisol to halt the alarm reaction. When children are exposed to long-standing extreme events, massive attempts to protect the psyche and to preserve the self are put into gear. These developmental traumas mobilize various kinds of defense mechanisms. Massive denial, dissociation, self anesthesia, identification with aggressor and aggression turned against the self often lead to profound character changes in the youngsters.

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Differential Diagnostic Characteristics of Movement Disorders in Children With Lesch-Nyhan Syndrome (LNS): A Case Report (Lesch-Nyhan 증후군 아동의 운동장애에 대한 감별진단 특성)

  • You, Sung H.;Bunker, Linda K.
    • Physical Therapy Korea
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    • v.9 no.4
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    • pp.13-35
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    • 2002
  • Lesch-Nyhan 증후근(LNS)은 hypoxanthine guanine phosphoribosyle transferase(HGPRT) 효소를 암호화 하는 X 염색체가 불완전해서 일어나는 유전적인 추제외로계(또는 기저핵)의 드문 병변이다. 출생시 LNS 유아는 정상적인 운동발달이 관찰되어진다. LNS에게서 현저하게 진단적인 특징으로 보여지는 운동심리적 행동인 self-mutilating 행위는 4살 이후에나 나타난다. LNS 아이들은 오히려 초기에 Rett's 증후근, 뇌성마비, 자폐, 다운증후근과 유사한 운동행위를 보인다. 그래서 LNS 아이들은 앞에 기술한 신경학적 장애로 오진을 받을 수가 있다. 오진으로 인해 초기에 적절한 치료를 받지 못한다면 LNS는 결과적으로 합병증(신장부전)과 self-mutilating 행위로 인하여 치명적일 수가 있다. 그러므로, 이 연구의 목적은 LNS 평가 동안 더 나은 진단을 하도록 하기 위하여 LNS와 관련된 기능부전에 대한 지식을 임상가들에게 제공하고자 함이었다. 연구 대상자는 10살인 2명의 쌍둥이 남아이었으며 실험은 뻗기 과제 수행(reaching task)시 움직임 특성을 보기 위하여 운동형상학적과 비디오 분석을 사용하였다. 기술통계로 분석 결과 움직임 시간과 단위가 증가됨을 보였고 사지의 분절적 움직임이 협응되지 않음을 보였다. ballistic과 jerky 움직임 양상은 dysmetric과 비긴장성 운동 행위에서 우세하였다. LNS은 추체로계 운동 장애 (과근긴장도나 저긴장도) 와 추체외로계의 운동 장애(dystonia와 choreoathetosis)의 혼합된 형태를 보였다. 결론으로 이 연구는 운동발달 장애를 가진 아이들을 치료하고자 할 때 임상가들한테 LNS 아이들의 움직임 장애의 다른 진단적 특징을 알아야 한다는 것을 제시하고자 한다.

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Changes of neurodevelopmental outcomes and risk factors of very low birth weight infants below 1,500 g, in the last 10 years (최근 10년간 1,500 g이하 극소 저출생 체중아의 신경학적 위험 요인 및 예후 변화에 관하여)

  • Lee, Se Kyu;Lee, Ji Hyun;Lee, Sang Geel
    • Clinical and Experimental Pediatrics
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    • v.49 no.10
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    • pp.1050-1055
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    • 2006
  • Purpose : As a result of advances in neonatal intensive care and perinatal care, neurodevelopmental outcomes of very low birth weight infant(VLBWIS) is expected to lead to improvement. The aim of this study was to report neurodevelopmental outcomes and risk factors of neurologic impairment of very low birth weight infants during the past 10 years. Method : We performed a retrospective study of 447 newborn infants below 1,500 gm admitted to neonatal intensive care unit of Taegu Fatima Hospital between Janury 1996 and December 2004. Infants were subdivided into group 1(Jan. 1996 to Dec. 1998), group 2(Jan. 1999 to Dec. 2001), and group3(Jan. 2002 to Dec. 2004). We analyzed epidemiologic data to study changes of neurodevelopmental outcomes and risk factors of neurologic impairment. Result : The incidence of cerebral palsy and developmental delay decreased significantly in periods 2 and 3(vs period 1; cerebral palsy 10 percent, developmental delay; 18 percent, P<0.05). Periventricular leukomalacia incidence decreased in period 3(vs period 1; 14.5 percent, P<0.05). The overall survival rate of VLBWIS increased significantly in period 2 and 3(vs period 1; 90.0 percent, P<0.05). The risk factors of neurologic impairment are long-term ventilator care(above 1 wk), low Apgar score, low gestational age and low birth weight. Conclusion : In the most recent 10 years, neurologic impairments of VLBWIS significantly decreased, as a result of advances in neonatal intensive care and perinatal care.

Development of interactive self-system based on artificial intelligent speaker for treatment of children with developmental disabilities (발달 장애 아동 치료를 위한 인공지능 스피커 기반 대화형 자가 시스템 개발)

  • Wee, YeJin;Kye, SeulA;Bae, SeoYeon;Choi, SeoungPyo;Lee, OnSeok
    • Proceedings of the Korea Information Processing Society Conference
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    • 2019.10a
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    • pp.1151-1152
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    • 2019
  • 발달 장애는 신체 및 정신이 해당하는 나이에 맞게 발달하지 않은 상태로, 다른 아동에 비해 신경정신과적 질환 발생 확률이 높기 때문에 발달장애 아동의 치료는 매우 중요하다. 그러나 주관적 판단에 의해 이루어지는 기존 작업치료의 경우, 정량적 성과 지표를 확인하기 힘들고 대상자 스스로 지속적으로 진행하기에 한계가 있다. 본 연구에서는 치료 모델을 가상 공간상에 구현하여 공간에 구애받지 않고 치료를 진행할 수 있으며, 수행 결과에 대한 자료를 정확하고 지속적으로 기록하며 확인할 수 있도록 하였다. 또한, AI 스피커를 통해 치료에 대한 피드백을 줌으로써, 대상자 스스로 실시하여 치료자의 개입을 줄여 심리적 부담을 덜어 더욱 정확한 수행이 이루어지도록 하였다.

A Study for Developing Music Therapy Activity Program for Development of Rudimentary Movement Phase of Spastic Cerebral Palsied Infant : Applying the techniques of Neurological Music Therapy (경직형 뇌성마비 유아의 초보운동단계 발달을 위한 음악치료활동 프로그램 개발 - 신경학적 음악치료의 기법을 활용하여)

  • Lee, Yoon Jin
    • Journal of Music and Human Behavior
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    • v.4 no.2
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    • pp.84-105
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    • 2007
  • Cerebral palsy is a collection of motor disorders resulting from damage to the central nervous system that arise in multiple handicaps including cognitive disorders, speech disorders, epilepsy, perception disorders, and emotion disorders. Today spastic cerebral palsy has become more prevalent because intensive care for newborns has resulted in higher survival rates for very small premature babies. Since the children grow the fastest in order for a development during one year after birth, the therapeutic intervention is provided as early as possible to the children with cerebral palsy. After seven year old, there is no effect of intervention. So, the necessity of early intervention to spastic cerebral palsied infants is increasing. The purpose of this study is to develop the music therapy activity program using the techniques of neurological music therapy(NMT), the therapeutic application of music to dysfunctions due to neurologic disease of the human nervous system, for rudimentary movement phase of spastic cerebral palsied infant. This music therapy activity program was developed on the basis of the major developmental tasks of the rudimentary movement phase, the period that children can acquire the most basic movement function at the 0 to 2. Then the developmental characteristics of spastic cerebral palsy were applied to this music therapy activity program. This music therapy activity program was classified to three domains, those are stability, locomotion, and manipulation. This study has been consisted of three steps, those are the development of the activities, the evaluation of the activities by th panels, and the adjustment and complement of the activities. Reviewing literatures and interviews were done for the development of the activities, and the evaluation the activities was done by seven music therapists. In the evaluation steps, the questionnaire was used for estimating the content validity and application efficiency. The adjustment and complement of the activities were evaluated by the panels who were participating in the music therapy for cerebral palsied children in the clinical setting, and the results of the adjustment and complement were confirmed by the panels. The evaluation was presented in a mean value with the comment of the panels. In conclusion, the music therapy activity program for the spastic cerebral palsied infants using the techniques of NMT was developed on the basis of the major developmental tasks of the rudimentary movement phase. The program is comprised of 38 activities, those are 14 activities for developing the stability, 10 activities for developing the locomotion, and 14 activities for developing the manipulation. The programed activities would bring out the answers in the affirmative for the conformance with infants' development phase, the harmony between the objective and the activity, the conformance with the cerebral palsied infants, the properness of the music and the instruments, and the utility in the clinic field. This results mean that this developed music activity program is appropriate to help spastic cerebral palsied infants progress their movement development by stages.

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