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A Case of Desanctis-Cacchione Syndrome

Kim JS, Suh ES

  • KMID: 2177064
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):383-387.
Xeroderma pigmentosum is autosomal recessive, degenerative disease generated by abnormal repair of DNA damaged by ultraviolet radiation and environmental mutagens. DeSanctis-Cacchione syndrome is the most severe form of xeroderma pigmentosum...
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A Case of Minicore Myopathy

Shim KJ, Kim SK, Yoon HS

  • KMID: 2177063
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):378-382.
Minicore myopathy, an uncommon condition, is one of congenital myopathies. It is characterized by multifocal areas of degeneration in muscle fibers. The minicores consist of numerous small areas of decreased...
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Two Cases of Lowe Syndrome

Choi BS, Lee YJ, Park SE, Nam SO

  • KMID: 2177062
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):374-377.
Lowe syndrome is, also known as oculocerebrorenal syndrome, a rare X-linked disorder characterized by congenital cataract, hypotonia, developmental delay, cogntive impairment, renal tubular dysfunction, and growth retardation. Recently the defevtive...
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Two Cases of Familial Hypokalemic Periodic Paralysis Caused by Salty Foods

Kim HJ, Lee SY, Kim MY, Kim NY, Lee SJ, Kim WD, Jo SM, Lee DS, Kim DK, Choi SM

  • KMID: 2177061
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):369-373.
Hypokalemic periodic paralysis is an autosomal dominant disorder characterized by episodic weakness of skeletal muscle associated with hypokalemia. It may be presented as familial or sporadic. Familial hypokalemic periodic paralysis...
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A Case of Ramsay Hunt Syndrome Associated with Aseptic Meningitis

Kim JS, Oh CK, Han DK, Yi JS, Woo YJ

  • KMID: 2177060
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):362-368.
Ramsay Hunt syndrome(RHS or herpes zoster oticus) is caused by reactivation of latent varicella-zoster virus(VZV) in the geniculate ganglion of the seventh cranial nerve. Unilateral facial paralysis is accompanied by...
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A Case of Cerebral Vasculitis in Henoch-Schonlein Purpura

Lee SH, Lee SY, Kim SM, Jeong DC, Kang JH, Lee JH, Chung SY

  • KMID: 2177059
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):356-361.
Henoch-Schonlein purpura(HSP), also known as anaphylactoid purpura, is a vasculitis of small vessels. Skin, gastrointestinal tract, joints, and kidney are usual location of the acute small vascular damage. Neurologic complications...
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Intracranial Hypertension without Hydrocephalus in an Infant with a Spinal Lipoma

Lee JH, Kim HM

  • KMID: 2177058
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):351-355.
A 6-month-old female infant presented initially with a bulging of anterior fontanelle. A CT scanning of the brain was normal. On lumbar puncture, intrathecal pressure was increased to 60 cmH2O...
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A Case of Homocystinuria Complicated with Systemic Deep Vein Thrombosis and Venous Infarction of Thalamus

Kang JH, Koo NH, Kim SH, Cho JH, Kim SY

  • KMID: 2177057
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):344-350.
Homocystinuria is an autosomal recessive inherited disorder of methionine metabolism. The most common cause of homocystinuria is cystathione-beta-synthase deficiency, which has the characteristic clinical features such as ectopia lentis, Marfanoid...
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A Case of Childhood Ataxia with Diffuse Central Nervous System Hypomyelination(CACH) Syndrome

Kim KT, Kim HS, Nam SO, Kim HJ

  • KMID: 2177056
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):338-343.
Childhood ataxia with diffuse central nervous system hypomyelination(CACH) syndrome is a recently described leukodystrophy of unknown etiology. The patients show normal development until the age from 1.5 to 5 years,...
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Cerebral Salt Wasting Syndrome Associated with Meningitis in a Child

Kim SH, Jang HO, Kim DW, Jo HS, Moon JS, Jang GY, Nam SY, Lee CG

  • KMID: 2177055
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):333-337.
Hyponatremia is commonly seen in those patients with central nervous system injury associated with infection or trauma. And decreasing intracranial pressure through restriction of maintenance fluid and salt is practiced...
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A Case of EBV-associated Hemophagocytic Lymphohistiocytosis with Seizure and Loss of Consciousness

Jeong HC, Kim DS, Kim WS, Cha SH, Bae IH, Shin KS, Son BR, Park HJ

  • KMID: 2177054
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):325-332.
Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Persistent fever, hepatosplenomegaly and pancytopenia are observed in the most cases with the characteristic change of serum triglyceride, fibrinogen, ferritin...
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The Cases of Surgical Treatment of Intractable Childhood Epilepsy with Tuberous Sclerosis

Jung DE, Park JC, Chung HJ, Kang HC, Kim HD, Hwang YS

  • KMID: 2177053
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):314-324.
Tuberous sclerosis(TS) is a disorder of aberrant neuronal differentiation and proliferation manifesting as multiple central nervous system(CNS) hamartomas. Seizures in patients with TS are often intractable to anticonvulsant therapy. Although...
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Frequency of Neurologic Disorders in the Neonatal Intensive Care Unit and Associated Factors

Kim DW, Suh ES

  • KMID: 2177052
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):305-313.
PURPOSE: Recent neonatal care unit survival rates have improved. These increasing survival rates have resulted in increased neurologic disorders. So we investigated the sorts and frequency of neurologic disorders at...
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Extensor Toe Signs Elicited by Various Methods in Cerebral Palsy Children

Cheon SH, Kim JC, Lee KS

  • KMID: 2177051
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):298-304.
PURPOSE: Extensor toe signs are neurologic abnormal finding in upper motor neuron impairment, such as spastic diplegia in cerebral palsy. Though classic Babinski sign is widely used, many other methods...
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Clinical Features of Arachnoid Cyst in Childhood

Lee JU, Lee DH, Park SB, Kim JS, Lee HJ

  • KMID: 2177050
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):290-297.
PURPOSE: Intracranial arachnoid cysts are benign neurodevelopmental anomalies that are often diagnosed in childhood incidently. They are clinically asymptomatic or could be related to headache, seizure, devlopmental delay, hydrocephalus and...
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Subacute Sclerosing Panencephalitis: Clinical Experience of 6 Cases

Lee TY, Kim SD, Lim BC, Hwang H, Kang YJ, Chae JH, Kim KJ, Hwang YS, Kim IO

  • KMID: 2177049
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):281-289.
PURPOSE: Subacute sclerosing panencephalitis(SSPE) is a severe and usually fatal neurodegenerative disorder of childhood and adolescence. The etiology is related to previous measles infection especially during the first 2 years...
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A Clinical Study of Aseptic Meningitis in Busan and Kyoungsangnamdo from April to July, 2002

Lee KH, Son YH, Jung YH, Kim CH, Hwang TG

  • KMID: 2177048
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):273-280.
PURPOSE: There was an outbreak of aseptic meningitis in Busan and Kyoungsangnamdo area from April to July, 2002. In this study, we reported the clinical manifestations, laboratory data and causative...
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Ischemic Stroke in Children: Analysis of Risk Factors

Choi YJ, Kang JH, Kim SH

  • KMID: 2177047
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):262-272.
PURPOSE: This paper evaluate and classified the risk factors that could possibly cause ischemic stroke in children, and investigate whether TOAST classification could be applied to the ischemic stroke in...
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The Efficacy of Add-on Therapy of Clobazam in Epileptic Children

Choi BS, Nam SO

  • KMID: 2177046
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):255-261.
PURPOSE: Clobazam is a 1,5-benzodiazepine which has a unique structure different from other benzodiazepines and shows different spectrum in treatment of epilepsy. We evaluated the efficacy of clobazam as an...
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Clinical Features of Nocturnal Seizure in Childhood

Lee JS, Kim J, Yoo HJ

  • KMID: 2177045
  • J Korean Child Neurol Soc.
  • 2002 Nov;10(2):248-254.
PURPOSE: Nocturnal seizure is a common seizure disorder in childhood. But there are a few study about nocturnal seizure. To be a great of help, We investigated clinical characteristics, response...
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