• Title/Summary/Keyword: 소아발달장애

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PHARMACOLOGICAL TREATMENT IN PERVASIVE DEVELOPMENTAL DISORDERS (전반적발달장애의 약물치료)

  • Choi, Jin-Sook
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.4 no.1
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    • pp.27-38
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    • 1993
  • Pervasive developmental disorder is one of the most severe clinical disorder in child psychiatry and is associated with deviancies in multiple areas of development. Medication does not cure pervasive developmental disorder and its effectiveness is generally nonspecific. But psychopharmacological treatment can be important for some children with pervasive developmental disorder and can make many young autistics more amenable to behavior modification and education. Haloperidol, the most widely studied antipsychotics, was statistically and clinically superior to placebo, and furthermore, was known to facilitate the positive functioning such as, discrimination learning and imitative communication, without side effects. However, administration of haloperidol is associated with drug related dyskinesia, and it warrants the introduction and use of the other novel drugs. Several biochemical studies suggest that subgroups of children with pervasive developmental disorder show hyperserotonemia and increased endogenous opioid level as compared with controls. Psychopharmacological trials were conducted according to these findings(ex : fenfluramine, naltrexone), with mixed results till now. These and another drugs that have been used in children with pervasive developmental disorder and their effectiveness are reviewed.

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A CASE OF THE CHILDHOOD DISINTEGRATIVE DISORDER (소아기 붕괴성 장애 1례)

  • Kang, Byung-Goo;Cho, Soo-Churl;Hong, Kang-E
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.7 no.1
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    • pp.110-119
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    • 1996
  • Heller recognized the children exhibiting developmental regression after normal period of development and proposed the term 'dementia' infanilis for the condition and reported it 1908. But the foolowing studies were insufficient, the diagnostic concept was not definded, and various names such as dementia infantilis, Heller's syndrome, disintegrative psychosis and childhood disintegrative disorder have been used for that condition. Recently the term childhood disintegrative disorder fir that condition was proposed as independent disease entity by DSM-IV and ICD-10, and the interset in that condition is increasing now. But because of insufficient previous studies on that condition, the definite concept, the characteristics, the relationship with autism or other similar conditions, the treatment and prognosis of that condition are not clear by this time, therefore we have not seen the official report on the condition in our country. These authors experienced a case considered as childhood disintegrative disorder and report it with the review of literatures.

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Clinical Significance of Auditory Brainstem Response(ABR) in Speech/Language Disorders (언어발달장애에 있어서 청성뇌간반응의 임상적 의의)

  • Oh, Ki Won;Park, Woo Saeng;Kwon, Soon Hak;Kim, Jin Kyung;Lee, Jun Hwa
    • Clinical and Experimental Pediatrics
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    • v.45 no.10
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    • pp.1199-1203
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    • 2002
  • Purpose : The study aimed to evaluate the efficacy of auditory brainstem response(ABR) as a screening tool in children with speech and language disorders. Methods : Between January 1, 1999 and December 31, 2001, 139 patients with chief complaints of speech and language delay were recruited from the pediatric neurology clinic, Kyungpook National University Hospital, Daegu, Korea. They had ABR on entry and the clinical data were then analyzed. Results : Fifteen out of 139 cases(10.8%) showed abnormal findings; seven had pervasive developmental disorders, four had developmental language disorders, and four were noted to have other conditions. Among them, seven cases were noted to have conductive hearing loss and eight had sensoryneuronal hearing loss. We also evaluated the normal values in children at the ages of 18 months to seven years. The mean latency of wave I and V were $1.40{\pm}0.13$ and $5.57{\pm}0.26$ respectively. Interpeak latency of I-V was $4.18{\pm}0.24$. Conclusion : Based on these findings, ABR has proved to be a highly sensitive and specific index of hearing impairment. It should be used as a screening tool in children with speech and language disorders.

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.

GENETIC CONTROL MECHANISM AND MOLECULAR BASIS OF NEURODEVELOPMENT (유전인자 조절기전과 신경발달의 분자 생물학적 특성)

  • Joung, Yoo-Sook
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.16 no.1
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    • pp.5-14
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    • 2005
  • There has been an enormous progress in understanding of how genes contribute to both normal and abnormal development. Also many laboratory works are exploring the intricacies of how to develop in the human central nervous system. Understanding the mechanisms of cortical development gives essential insight into the pathogenesis of many genetic and acqured developmental psychiatric disorders, including autism, schizophrenia, and teaming disorder. Genes have been implicated in an ever-increasing number of disorders. Advance in genetics have begun to clarify the molecular basis of not only single-gene disorders, but also more complex phenotypes.

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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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