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A Case of Cystathioninuria with Delayed Development

Byun SH, Lee HS, Pee DH, Lee KH, Eun BL, Hahn SH

  • KMID: 2329309
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):184-190.
Cystathionine is well-known intermediate in the metabolism of methionine. It is cleaved to cysteine and homoserine by gamma-cystathionase. This enzyme utilize pyridoxal 5'-phosphate as coenzyme. gamma-cystathionase deficiency leads to persistent...
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A Case of Juvenile Dermatomyositis

Yom HW, Park JH, Seo JW, Choi HY, Gu H, Lee SW, Kang YJ

  • KMID: 2329308
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):178-183.
Juvenile dermatomyositis is an uncommon autoimmune disease with classic heliotrope discoloration of eyelids, erythematous skin rash of joints and proximal muscle weakness. Quite different from adults, malignancy is rarely accompanied...
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Two Cases of Sporadic Hemiplegic Migraine

Hwang EK, Park HY, Chae KY

  • KMID: 2329307
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):173-177.
Hemiplegic migraine is characterized by the occurrence of migraine attacks with unilateral weakness. The attack usually starts in childhood, adolescence, or early adulthood. The diagnosis may be delayed if there...
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A Case of Choroid Plexus Papilloma of a Child with Anorexia and Failure to Thrive

Hong SC, Chae SA, Lee DK, Min BK

  • KMID: 2329306
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):168-172.
Choroid plexus papilloma is a very rare disease in children. The manifestations of the disease differ according to the tumor size and the location. Increased CSF production by the tumor,...
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A Case of Exon 7 and 8 Deletion of Survival Motor Neuron Gene in Spinal Muscular Atrophy

Kim YD, Kim JC, Hwang CK, Lee KS

  • KMID: 2329305
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):163-167.
Spinal muscular atrophy(SMA) is a genetic disorder of the motor neurons that cause muscular weakness and muscular atrophy due to anterior horn cell degeneration. Classic spinal muscular atrophy patient is...
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A Case of Ullrich's Disease

Lee BH, Lim JH, Bae SN, Lee KW, Nam SO, Choi KU

  • KMID: 2329304
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):158-162.
Ullrich's disease is a congenital muscular dystrophy clinically characterized by generalized muscle weakness, multiple contractures of the proximal joints, and hyperextensibility of the distal joints. All the patients develop rigidity...
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A Case of Linear and Whorled Nevoid Hypermelanosis with Intracranial Lipoma

Song TW, Yoo BW, Lee YM, Kang DC, Lee JS, Coe CJ

  • KMID: 2329303
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):150-157.
Linear and whorled nevoid hypermelanosis(LWNH) is characterized by macules in streaks and whorls along Blaschko's lines, that are not preceded by inflammation. LWNH can present with various findings such as...
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A Case of Parry-Romberg Syndrome with Intractable Seizure

Heo YJ, Kim SW, Song DW, Chung HJ

  • KMID: 2329302
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):144-149.
Parry-Romberg syndrome, first described in 1825 by Parry and in 1846 by Romberg, is a rare disorder characterized by a progressive hemifacial atrophy of the skin and adipose tissue and...
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MELAS : Clinical Features according to Genetic Characteristics

Hwang H, Chae JH, Kim KJ, Hwang YS

  • KMID: 2329301
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):135-143.
PURPOSE: This study was performed to estimate the frequency of mitochondrial DNA (mtDNA) A3243G point mutation in MELAS(mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) of our population and to identify...
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A Clinical Profile of Peripheral Neuropathy in Korean Children

Lee HS, Park WS, Ko CW, Sohn YK, Kwon SH

  • KMID: 2329300
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):128-134.
PURPOSE: To analyze the clinical features of peripheral neuropathy in Korean children. METHODS: A total of 62 children with acute flaccid paralysis, longstanding weakness of extremities, or abnormal electrophysiological studies, suggestive...
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Childhood Myasthenia Gravis: Clinical Features and Response to Steroid Treatment

Lim BC, Kang YJ, Hwang H, Chae JH, Kim KJ, Hwang YS

  • KMID: 2329299
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):120-127.
PURPOSE: Myasthenia gravis(MG) is relatively rare in childhood and shows some distinct epidemiologic and clinical features according to ages and races. The role of immunosuppressive therapy such as steroid treatment...
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The Relationship between Underlying Diseases and the Patterns of Abnormal MR Image Findings in Childhood White Matter Diseases

Lim KH, Moon HK

  • KMID: 2329298
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):111-119.
PURPOSE: In children disorders affecting brain white matter are not uncommon and MRI has high sensitivity to detect white matter lesions. We performed this study to find out the distribution...
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Clinical Manifestations and Surgical Outcome of Medically Refractory Epilepsy in Childhood

Lee HY, Cheon JM, Chung SH, Lee M, Won SD, Hong SB, Hong SC, Yoon HK

  • KMID: 2329297
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):100-110.
PURPOSE: The cognitive and psychosocial morbidity of medically refractory epilepsy is considerable. Epilepsy surgery can play a important role in these patients. We investigated the clinical manifestations and the surgical...
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Surgical Outcomes in Children with Intractable Temporal Lobe Epilespy:Electroencephalographic and Pathologic Findings

Kim SH, Kim JS, Kim CJ, Woo YJ, Lee MC, Kim HI

  • KMID: 2329296
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):90-99.
PURPOSE: Epilepsy surgery has become increasingly available in children with medically intractable epilepsy including temporal lobe epilepsy(TLE). TLE in children, however, has many different clinical and electrophysiologic characteristics which make...
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Factors Influencing on Perceived Stigma of Parents with Epileptic Children

Kim BY, Lee SJ, Kim WD, Cho SM, Kim DK, Choi SM

  • KMID: 2329295
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):82-89.
PURPOSE: Epilepsy, a typical chronic disease, may cause severe of psychosocial difficulties for all family members, including stigmatization. The present study focused on how children with epilepsy affect their parents'...
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Weight Change associated with Oxcarbazepine Monotherapy of Childhood Epilepsy

Kim YM, Nam SO

  • KMID: 2329294
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):73-81.
PURPOSE: To evaluate the changes of bodyweight associated with oxcarbazepine monotherapy in children with epilepsy. METHODS: We conducted a retrospective review of 71 childhood epileptic patients(40 boys, and 31 girls) attending...
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Change of Body Weight According to Antiepileptic Drugs in Children with Epilepsy:Valproic Acid vs. Topiramate

Kim SJ, Park HS, Hwang JS, Jung YJ, Ko TS

  • KMID: 2329293
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):68-72.
PURPOSE: The use of antiepileptic drugs for the seizure control has been a remarkable breakthrough. However, excessive body weight gain is a common side effect of some antiepileptic drugs. Topiramate...
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Comparative Study for the Diagnostic Usefulness of Brain MRI and CT in Children with Seizure

Shin SA, Kim DK, Park HK, Kim NS, Seol IJ

  • KMID: 2329292
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):61-67.
PURPOSE: This study was performed to know and compare the diagnostic usefulness of Magnetic Resonance Imaging(MRI) and Computed Tomography(CT) for the evaluation of patients with seizure. METHODS: We studied retrospectively 96...
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The Relationship between Iron Deficiency Anemia and Febrile Seizure

Im S, Ah JK, Choi BJ, Lee IG, Whang KT

  • KMID: 2329291
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):55-60.
PURPOSE: A part of seizure disorders, hemosiderin deposits are noted in epileptogenic lesions cytopathologically and iron status may affect the seizure threshold. To investigate this possibility, measures of iron sufficiency...
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Mutations of SCN1A in Familial Febrile Seizures

Kang JM, Roh SM, Kim YH, Chung SY, Lee IG, Whang KT

  • KMID: 2329290
  • J Korean Child Neurol Soc.
  • 2003 May;11(1):47-54.
PURPOSE: Febrile seizures affect 2-5% of all children younger than 6 years old. A small proportion of children with febrile seizures later develop epilepsy. Muations in the voltage-gated sodium channel...
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