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Case Report
Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia
Yun-Jin Lee, Kee-Yang Chung, Hoon-Chul Kang, Heung Dong Kim, Joon Soo Lee
Clin Exp Pediatr. 2015;58(9):354-357.   Published online September 21, 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the...

Original Article
Applying the bacterial meningitis score in children with cerebrospinal fluid pleocytosis: a single center's experience
Jungpyo Lee, Hyeeun Kwon, Joon Soo Lee, Heung Dong Kim, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(7):251-255.   Published online July 22, 2015
Purpose

The widespread introduction of bacterial conjugate vaccines has decreased the risk of cerebrospinal fluid (CSF) pleocytosis due to bacterial meningitis (BM) in children. However, most patients with CSF pleocytosis are hospitalized and treated with parenteral antibiotics for several days. The bacterial meningitis score (BMS) is a validated multivariate model derived from a pediatric population in the postconjugate vaccine era and...

Case Report
Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses
Soo Jin Chang, Ji Hyun Lee, Shin Hye Kim, Joon Soo Lee, Heung Dong Kim, Joon Won Kang, Young Mock Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(5):194-198.   Published online May 22, 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at...

Original Article
Screening for depression and anxiety disorder in children with headache
Sang Mi Lee, Jung-Rim Yoon, Yoon Young Yi, Soyong Eom, Joon Soo Lee, Heung Dong Kim, Keun-Ah Cheon, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(2):64-68.   Published online February 28, 2015
Purpose

The purpose of this study was to investigate the importance of initial screening tests for depression and anxiety disorders in children with headache. In addition, this study evaluated whether the Children's Depression Inventory (CDI) and Revised Children's Manifest Anxiety Scale (RCMAS) are suitable for screening symptoms of depression and anxiety.

Methods

A retrospective chart review was conducted of 720 children aged 7-17...

Case Report
Wernicke's encephalopathy in a child with high dose thiamine therapy
So Won Park, Yoon Young Yi, Jung Woo Han, Heung Dong Kim, Joon Soo Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2014;57(11):496-499.   Published online November 30, 2014

Wernicke's encephalopathy is an acute neurological disorder characterized by mental confusion, oculomotor dysfunction, and ataxia. It has been reported in individuals with alcohol dependence, hyperemesis gravidarum, and prolonged parenteral nutrition without vitamin supplementation. Here we present the case of a 13-year-old male patient with neuroblastoma and a history of poor oral intake and nausea for 3 months. After admission, he...

Original Article
Myocardial atrophy in children with mitochondrial disease and Duchenne muscular dystrophy
Tae Ho Lee, Lucy Youngmin Eun, Jae Young Choi, Hye Eun Kwon, Young-Mock Lee, Heung Dong Kim, Seong-Woong Kang
Clin Exp Pediatr. 2014;57(5):232-239.   Published online May 31, 2014
Purpose

Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution and functional changes of the myocardium in patients with MD or DMD.

Methods

We retrospectively evaluated the echocardiographic data of 90 children with...

Case Report
Congenital muscular dystrophy type 1A with residual merosin expression
Hyo Jeong Kim, Young-Chul Choi, Hyung Jun Park, Young-Mock Lee, Heung Dong Kim, Joon Soo Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2014;57(3):149-152.   Published online March 31, 2014

Congenital muscular dystrophy type 1A (MDC1A) is an autosomal recessive disorder characterized by hypotonia, elevated serum creatine kinase level, delayed motor milestones, white matter changes observed by brain magnetic resonance imaging, and normal intelligence. A mutation in the laminin α2 (LAMA2) gene, located at 6q22-23, is a genetic cause of MDC1A. Patients have merosin (laminin α2)-deficient skeletal muscles. However, the...

Review Article
Lower fat and better quality diet therapy for children with pharmacoresistant epilepsy
Jung-Rim Yoon, Heung Dong Kim, Hoon-Chul Kang
Clin Exp Pediatr. 2013;56(8):327-331.   Published online August 27, 2013

The ketogenic diet (KD) is an established, effective, nonpharmacologic treatment for children with pharmacoresistant epilepsy. Although the KD is the most well-established dietary therapy for epilepsy, it is too restrictive and is associated with serious complications; therefore, alternative lower-fat diets, including a modified Atkins diet and low-glycemic index diet, have been developed. Recent ongoing clinical evidence suggests that other dietary...

Original Article
Epilepsy in Korean patients with Angelman syndrome
Sung-Hee Park, Jung-Rim Yoon, Heung Dong Kim, Joon Soo Lee, Young-Mock Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2012;55(5):171-176.   Published online May 21, 2012
Purpose

The aim of this study was to investigate the natural history of epilepsy and response to anti-epileptic drug treatment in patients with Angelman syndrome (AS) in Korea.

Methods

We retrospectively reviewed the clinical records of 14 patients diagnosed with epilepsy out of a total of 17 patients with a genetic diagnosis of AS. These patients were seen at the Department of Pediatric...

Risk factors of ocular involvement in children with mitochondrial respiratory chain complex defect
Jung Hyun Chae, Jung Hun Lee, Kyo Ryung Kim, Suk Ho Byeon, Young Mock Lee, Hoon Chul Kang, Joon Soo Lee, Heung Dong Kim
Clin Exp Pediatr. 2010;53(12):994-999.   Published online December 31, 2010
Purpose

Mitochondrial dysfunction can present with various symptoms depending on the organ it has affected. This research tried to analyze the ophthalmologic symptoms and ophthalmologic examination (OE) results in patients with mitochondrial disease (MD).

Methods

Seventy-four patients diagnosed with mitochondrial respiratory chain complex defect with biochemical enzyme assay were included in the study. They were divided into 2 groups based on the OE...

Electrocardiography as an early cardiac screening test in children with mitochondrial disease
Ran Baik, Jung Hyun Chae, Young Mock Lee, Hoon Chul Kang, Joon Soo Lee, Heung Dong Kim
Clin Exp Pediatr. 2010;53(5):644-647.   Published online May 31, 2010
Purpose

To evaluate myocardial conductivity to understand cardiac involvement in patients with mitochondrial disease.

Methods

We performed retrospective study on fifty-seven nonspecific mitochondrial encephalopathy patients with no clinical cardiac manifestations. The patients were diagnosed with mitochondrial respiratory chain complex defects through biochemical enzyme assays of muscle tissue. We performed standard 12-lead electrocardiography (ECG) on all patients.

Results

ECG abnormalities were observed in 30 patients (52.6%)....

Ocular manifestations in Leigh syndrome
Kyo Ryung Kim, Suk Ho Byeon, Young Mock Lee, Hoon Chul Kang, Joon Soo Lee, Heung Dong Kim
Clin Exp Pediatr. 2010;53(2):163-166.   Published online February 15, 2010
Purpose : Leigh syndrome is a typical type of mitochondrial disease. This study was conducted to analyze the types of ophthalmologic symptoms and results of funduscopy conducted in the ophthalmologic examination of patients with Leigh syndrome. Methods : Funduscopy was conducted on 24 subjects, who were chosen among those diagnosed as having mitochondrial respiratory chain complex defect and who were clinically...
Case Report
A case of hippocampal sclerosis diagnosed as cortical dysplasia due to preoperative brain MRI finding
Jun Seok Lee, Kyo Ryung Kim, Jeong Tae Kim, Min Jung Choi, Young Mock Lee, Heung Dong Kim, Joon Soo Lee, Dong Seok Kim, Tae Seong Kim
Clin Exp Pediatr. 2010;53(1):106-110.   Published online January 15, 2010
Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white...
Original Article
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)...
Clinical manifestations and neuroimaging findings of schizencephaly in children
Jae Rang Lee, Seung Kim, Young Mock Lee, Joon Soo Lee, Heung Dong Kim
Clin Exp Pediatr. 2009;52(4):458-463.   Published online April 15, 2009
Purpose : Schizencephaly is a uncommon congenital brain anomaly characterized by congenital clefts spanning the cerebral hemispheres from pial surface to lateral ventricles and lined by gray matter. In this study, we investigated the clinical manifestation and radiologic findings of pediatric schizencephaly. Methods : The data of 13 patients who were diagnosed with schizencephaly in Severance Childrens Hospital and Yongdong...
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