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Original Article
Neurology
Long-term prenatal stress increases susceptibility of N-methyl-D-aspartic acid-induced spasms in infant rats
Hyeok Hee Kwon, Taekwan Lee, Jinpyo Hong, Dong Woon Kim, Joon Won Kang
Clin Exp Pediatr. 2018;61(5):150-155.   Published online May 28, 2018
Purpose

Infantile spasms, also known as West syndrome, is an age-specific epileptic seizure. Most patients with this condition also exhibit delayed development. This study aimed to determine the effect of long-term prenatal stress on susceptibility to infantile spasms.

Methods

We subjected pregnant rats to acute or chronic immobilization stress. Resulting offspring received N-methyl-D-aspartic acid (15 mg/kg, intraperitoneally) on postnatal day 15, and their...

Case Report
Neurology
Glucose transport 1 deficiency presenting as infantile spasms with a mutation identified in exon 9 of SLC2A1
Hyun Hee Lee, Yun Jung Hur
Clin Exp Pediatr. 2016;59(Suppl 1):S29-S31.   Published online November 30, 2016

Glucose transport 1 (GLUT-1) deficiency is a rare syndrome caused by mutations in the glucose transporter 1 gene (SLC2A1) and is characterized by early-onset intractable epilepsy, delayed development, and movement disorder. De novo mutations and several hot spots in N34, G91, R126, R153, and R333 of exons 2, 3, 4, and 8 of SLC2A1 are associated with this condition. Seizures,...

Original Article
Long-term outcomes of infantile spasms
Seak Hee Oh, Eun-Hye Lee, Min-Hee Joung, Mi-Sun Yum, Tae-Sung Ko
Clin Exp Pediatr. 2010;53(1):80-84.   Published online January 15, 2010
Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone...
Ictal single-photon emission computed tomography with slow dye injection for determining primary epileptic foci in infantile spasms
Yun Jung Hur, Joon Soo Lee, Hoon Chul Kang, Hye Jung Park, Mi Jin Yun, Heung Dong Kim
Clin Exp Pediatr. 2009;52(7):804-810.   Published online July 15, 2009
Purpose : We investigated whether ictal single-photon emission computed tomography (SPECT) with prolonged injection of technetium-99m (99mTc) ethyl cysteinate dimer during repeated spasms can localize the epileptogenic foci in children with infantile spasms. Methods : Fourteen children with infantile spasms (11 boys, 3 girls; mean age, 2.2¡¾1.3 years) were examined. When a cluster of spasms was detected during video electroencephalography (EEG)...
Case Report
A case of Menkes disease with unusual hepatomegaly
Go Un Jeong, Anna Cho, Hee Hwang, Yong Seung Hwang, Ki Joong Kim, Jong Hee Chae, Jeong Kee Seo
Clin Exp Pediatr. 2008;51(5):538-541.   Published online May 15, 2008
Menkes disease is an X-linked recessive copper transport disorder characterized by neurological deterioration, connective- tissue damage, and abnormal hair growth. It is caused by the mutation of the ATP7A gene. This report describes a four- month-old boy with neurological symptoms typical of Menkes disease plus unusual liver involvement. He developed seizures at three months of age and exhibited hypotonia, cephalhematoma,...
A Case of Krabbe Disease with Infantile Spasm
Ja Kyoung Kim, Dal Hyun Kim, Bo Young Kang, Young Se Kwon, Young Jin Hong, Byong Kwan Son, Hye Ran Yoon
Clin Exp Pediatr. 2003;46(1):95-99.   Published online January 15, 2003
Krabbe disease is a rare autosomal recessive disorder clinically characterized by retardation in motor development, prominent spasticity, seizures, and optic atrophy. Pathologically, there are many globoid cells in the white matter, in addition to the lack of myelin and the presence of severe gliosis. Hence Krabbe disease is known as globoid cell leukodystrophy. Biochemically, the primary enzymatic deficiency in Krabbe...
Original Article
A Clinical Study on Infantile Spasms with Vigabatrin Therapy
Seung Jung Oh, Sung Gun Park, Hyun Ho Kang, Sa Jun Chung
Clin Exp Pediatr. 1999;42(2):233-238.   Published online February 15, 1999
Purpose : Infantile spasms are considered an age-specific and malignant epilepsy. It is not controlled easily by general anticonvulsants. Therefore, this study aimed to assess vigabatrin(VGT) efficacy in infantile spasms. Methods : From January 1992 to December 1995, 35 children with infantile spasms, who were diagnosed at Kyung Hee University Hospital and treated vigabatrin, were analyzed retrospectively. Results : The ratio of...
A Clinical Study on Infantile Spasms with Prednisolone Therapy
Hye Sun Yoon, Young Hoon Choi, Ho Suk Lee, Yong Mook Choi, Sa Jun Chung
Clin Exp Pediatr. 1996;39(4):522-529.   Published online April 15, 1996
Purpose : Infantile spasms are considered malignant epilepsy of infancy. Primary objectives of treatment are complete control of seizure attack and prevention of further brain damage. The aim of this study is to assess prednisolone(PDL) efficacy in infantile spasms. Methods : From June 1985 to July 1994, 20 children with infantile spasms who were diagnosed at Kyung Hee University Hospital and were...
A Clinical Study on Infantile Spasms with ACTH Therapy
Se Hee Hwang, Yong Seung Hwang
Clin Exp Pediatr. 1992;35(4):459-469.   Published online April 15, 1992
Infantile spasms is a disease, causes or treatments of which are not clearly delineated. Pri-mary objectives of treatment are complete control of seizure attack and prevention of further brain damage. Forty-one patients were managed with ACTH, who were diagnosed as infantile spasms and admitted to the Department of Pediatrics, Seoul National University Hospital from July 1985 till June 1990. The results...
11 q Deletion Syndrome.
Y B Im, S B Park, B Y Pyun, J O Park, S J Lee, S Y Moon
Clin Exp Pediatr. 1989;32(2):239-243.   Published online February 28, 1989
llq deletion syndrome is a rare chromosomal anomaly. The authors experienced a case of llq deletion syndrome with congenital glaucoma and infantile spasm in a female child. She showed delayed psychomotor development, flat occiput, hypertelorism, low set malformed ears, flat nasal bridge, short neck, simian line on right palm and throm. bocytopenia. Chromosomal study showed the deletion of the long arm of chromosme 11, karyotypically...
2 Cases of Infantile Spasms(Cryptogenic Type) Treated with ACTH Therapy.
Shin Heh Kang, Chang Jun Coe
Clin Exp Pediatr. 1987;30(8):928-933.   Published online August 31, 1987
We have experienced 2 cases of infantile spasms treated with ACTH. They were diagnosed as cryptogenic type within relatively short duration after the onset. With the administration of ACTH, flexor spasms and hypsarrhythmia disappeared. They did not demonstrate any intellectual impair- ment on OPD follow up study. A brief review of literatures was made.
Clinical Study of Epilepsy in Children.
C J Coe, D K Han, K Y Lee, B S Kim
Clin Exp Pediatr. 1982;25(12):1234-1242.   Published online December 31, 1982
From November 1980 to June 1982, for 19 months, 636 cases of child epileptic patients were clinically investigated at Pediatric department of Yonsei Medical school, and obtained follo- wing results. 1. The epileptic patients was 4.4% of all the pediatric patients, visited or hospitalized during the same period of time. 2. Epilepsy was commoner in male child compare to female representing male to female ratio, 1.3:1. 3. The most common...