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Intravenous Immunoglobulin in the Treatment of Neurological Diseases

Kim SH, Lee YM

Intravenous immunoglobulin (IVIG) is used in treating many cases of autoimmune and inflammatory conditions thanks to its multiple anti-inflammatory and immunomodulatory properties. The clinical use of IVIG has been for...
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Alice in Wonderland Syndrome in a Child with an Electroencephalographic Abnormality: A Case Report

Koo CM, Kang HC

Alice in wonderland syndrome (AWS) is a paroxysmal disorder with distorted body image, including altered perceptions of size, mass, or shape. In fact, altered body image is the classic symptom...
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A Case Report of Precocious Puberty in Children Associated with Hypothalamic Hamartoma in Neurofibromatosis Type 1

Lee SA, Kim JH, Kim SJ

Neurofibromatosis type 1 (NF1) is a common neurocutaneous syndrome that presents with multiple café-au-lait spots, skinfold freckling, dermatofibromas, neurofibromas, and Lisch nodules. Mutations of the NF1 gene, encoding the protein...
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Outbreak of Influenza B related to Benign Acute Childhood Myositis

Hur YJ

PURPOSE: Benign acute childhood myositis (BACM) is a rare syndrome caused by a viral infection and is characterized by gait disturbance with calf pain in children. I investigated whether BACM...
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A Case of Severe Encephalitis with Mycoplasma pneumoniae Infection in a 4-Year-Old Boy

Lee HE, Lee SJ

Mycoplasma pneumoniae (MP) infection can result in extrapulmonary and respiratory manifestations. The direct invasion by MP and the indirect invasion by immune-mediated response have been suggested as the pathogenesis of...
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Varicella Zoster Meningitis in an Immunocompetent Child

Kim YJ, Choi YB, Kim DH, Kwon YS

Neurologic complications associated with varicella zoster virus (VZV) are rare in children. A 13-year-old boy was hospitalized due to headache, fever, and vomiting. Aseptic viral meningitis was strongly suspicious based...
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A Case of Dopa-responsive Dystonia with a Mutation in the GCH1 Gene Misdiagnosed as Cerebral Palsy for 2 Years

Yi CH, Yang HJ, Kim HJ, Lee KY

Dopa-responsive dystonia (DRD) is characterized by lower limb-onset, diurnally fluctuating dystonia and dramatic and sustained response to levodopa treatment. Segawa disease, an autosomal dominant deficiency of guanosine triphosphate cyclohydrolase 1...
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Late Infantile-Onset Globoid Cell Leukodystrophy: Treatment using Hematopoietic Stem Cell Transplantation

Lee SH, Ko A, Lyu CJ, Lee JS, Lee JS

Globoid cell leukodystrophy is a rare autosomal recessive disorder of the brain white-matter caused by galactosylceramidase deficiency; the disorder is classified into four types based on the age of onset....
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Concurrence of Diabetic Ketoacidosis and Acute Ischemic Stroke due to Moyamoya Disease

Lee H, Lee J, Kim S, Oh MS, Han KH, Kang KS, Kim S

Although the etiology of moyamoya disease (MMD) remains unknown, autoimmunity is one of the proposed pathogeneses. Unlike other autoimmune disorders that are associated with cerebral arteritis, concurrence of MMD and...
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A Case of Effective Treatment of a Patient with PCDH 19-Related Epilepsy using Corticosteroid

Lee DM, Chung HJ

An uncommon female-limited intractable epilepsy, protocadherin (PCDH) 19-related epilepsy, is characterized by mutations in the PCDH 19 gene, located on chromosome X. Clinical symptoms include early onset, fever sensitivity, focal...
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Clinical Features and Practices of Bell Palsy of Children and Adolescent in a Single Center

Kim YH, Lee KH

PURPOSE: Bell palsy is an idiopathic peripheral facial nerve paralysis of sudden onset. Though the incidence in children is relatively lower than adult population, it is not uncommon. Because of...
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Two Cases of Hirayama Disease in a Pediatric Clinic

Ahn H, Yum MS, Kim HJ, Yeh HR, Ko TS

We report two pediatric cases with Hirayama disease—a 16-year-old boy with a left wrist drop and a 14-year-old-boy with weakness and muscle atrophy of right hand. Motor nerve conduction study...
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Acute Childhood Encephalitis and Encephalopathy following Infectious Symptoms: a Single Center Study

Choi GE, Hong SH, Kim YO, Woo YJ

PURPOSE: Acute encephalitis and encephalopathy are preceded by respiratory or enteric infection, whose pathogens can be detected more easily with advanced tools. However, studies for pathogens in Korea remain scarce....
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A Case of Acute Hemorrhagic Encephalomyelitis associated with Mycoplasma Pneumoniae Infection

Choi Y, Lee J, Suh ES

Acute hemorrhagic encephalomyelitis (AHEM) is a severe form of encephalitis characterized by fulminant clinical course and presence of hemorrhagic necrosis of the white matter. Mycoplasma pneumoniae (M. pneumoniae) have severe...
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Leigh Syndrome: Subgroup Aanalysis according to Mitochondrial DNA Mutation

Jee NL, Her SM, Kim SH, Lee MJ, Lee CH, Lee YM

PURPOSE: Leigh syndrome (LS) is a rare, progressive neurodegenerative disorder with characteristic abnormalities in the central nervous system. Such patients present with heterogeneous clinical symptoms and genetic abnormalities; thus, prognosis...
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HLA DRB1*04:03 and Drug Reaction with Eosinophilia and Systemic Symptoms Induced by Oxcarbazepine: A Case Report

Yeo HR, Yoo HW, Yoon L, Kim HY, Lee Y, Nam SO, Kim YM

Drug reaction with eosinophilia and systemic symptoms(DRESS), which occurs 2–8 weeks after taking a medication is a rare and potentially life-threatening drug-induced hypersensitivity reaction, which includes skin eruption, hematologic abnormalities,...
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