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Case Report
Neurology
A pediatric case of idiopathic Harlequin syndrome
Ju Young Kim, Moon Souk Lee, Seung Yeon Kim, Hyun Jung Kim, Soo Jin Lee, Chur Woo You, Jon Soo Kim, Ju Hyung Kang
Clin Exp Pediatr. 2016;59(Suppl 1):S125-S128.   Published online November 30, 2016

Harlequin syndrome, which is a rare disorder caused by dysfunction of the autonomic system, manifests as asymmetric facial flushing and sweating in response to heat, exercise, or emotional factors. The syndrome may be primary (idiopathic) with a benign course, or can occur secondary to structural abnormalities or iatrogenic factors. The precise mechanism underlying idiopathic harlequin syndrome remains unclear. Here, we...

Ictal sinus pause and myoclonic seizure in a child
Hye Ryun Kim, Gun-Ha Kim, So-Hee Eun, Baik-Lin Eun, Jung Hye Byeon
Clin Exp Pediatr. 2016;59(Suppl 1):S129-S132.   Published online November 30, 2016

Ictal tachycardia and bradycardia are common arrhythmias; however, ictal sinus pause and asystole are rare. Ictal arrhythmia is mostly reported in adults with temporal lobe epilepsy. Recently, ictal arrhythmia was recognized as a major warning sign of sudden unexpected death in epilepsy. We present an interesting case of a child with ictal sinus pause and asystole. A 27-month-old girl was...

A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures
Jee-Yeon Han, Mi-Sun Yum, Eun-Hee Kim, Seokho Hong, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(Suppl 1):S139-S144.   Published online November 30, 2016

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable...

A young child of anti-NMDA receptor encephalitis presenting with epilepsia partialis continua: the first pediatric case in Korea
Eun-Hee Kim, Yeo Jin Kim, Tae-Sung Ko, Mi-Sun Yum, Jun Hwa Lee
Clin Exp Pediatr. 2016;59(Suppl 1):S133-S138.   Published online November 30, 2016

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis...

1p36 deletion syndrome confirmed by fluorescence in situ hybridization and array-comparative genomic hybridization analysis
Dong Soo Kang, Eunsim Shin, Jeesuk Yu
Clin Exp Pediatr. 2016;59(Suppl 1):S14-S18.   Published online November 30, 2016

Pediatric epilepsy can be caused by various conditions, including specific syndromes. 1p36 deletion syndrome is reported in 1 in 5,000–10,000 newborns, and its characteristic clinical features include developmental delay, mental retardation, hypotonia, congenital heart defects, seizure, and facial dysmorphism. However, detection of the terminal deletion in chromosome 1p by conventional G-banded karyotyping is difficult. Here we present a case of...

Type I Chiari malformation presenting orthostatic syncope who treated with decompressive surgery
Hyun-Seung Shin, Jeong A Kim, Dong-Seok Kim, Joon Soo Lee
Clin Exp Pediatr. 2016;59(Suppl 1):S149-S151.   Published online November 30, 2016

Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical...

Megalencephaly-capillary malformation-polymicrogyria syndrome: the first case report in Korea
Yeon-Chul Choi, Mi-Sun Yum, Min-Jee Kim, Yun-Jung Lee, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(Suppl 1):S152-S156.   Published online November 30, 2016

Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP), previously known as macrocephaly-cutis marmorata telangiectatica congenita and macrocephaly-capillary malformation syndrome, is a rare multiple-malformation syndrome that is characterized by progressive megalencephaly, capillary malformations of the midline face and body, or distal limb anomalies such as syndactyly. Herein, we report a female infant case that satisfies the recently proposed criteria of MCAP and describe the distinctive...

Paroxysmal kinesigenic dyskinesia in a patient with a PRRT2 mutation and centrotemporal spike discharges on electroencephalogram: case report of a 10-year-old girl
Sun Young Seo, Su Jeong You
Clin Exp Pediatr. 2016;59(Suppl 1):S157-S160.   Published online November 30, 2016

Coexistence of paroxysmal kinesigenic dyskinesia (PKD) with benign infantile convulsion (BIC) and centrotemporal spikes (CTS) is very rare. A 10-year-old girl presented with a 3-year history of frequent attacks of staggering while laughing and of suddenly collapsing while walking. Interictal electroencephalogram (EEG) revealed bilateral CTS, but no changes in EEG were observed during movement. The patient's medical history showed afebrile...

Concurrency of Guillain-Barre syndrome and acute transverse myelitis: a case report and review of literature
Orkun Tolunay, Tamer Çelik, Ümit Çelik, Mustafa Kömür, Zeynep Tanyeli, Abdurrahman Sönmezler
Clin Exp Pediatr. 2016;59(Suppl 1):S161-S164.   Published online November 30, 2016

Guillain-Barré syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength...

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,...

A neonate with Joubert syndrome presenting with symptoms of Horner syndrome
Narae Lee, Sang-Ook Nam, Young Mi Kim, Yun-Jin Lee
Clin Exp Pediatr. 2016;59(Suppl 1):S32-S36.   Published online November 30, 2016

Joubert syndrome (JS) is characterized by the “molar tooth sign” (MTS) with cerebellar vermis agenesis, episodic hyperpnea, abnormal eye movements, and hypotonia. Ocular and oculomotor abnormalities have been observed; however, Horner syndrome (HS) has not been documented in children with JS. We present the case of a 2-month-old boy having ocular abnormalities with bilateral nystagmus, left-dominant bilateral ptosis, and unilateral...

Review Article
Neurology
Enterovirus 71 infection and neurological complications
Kyung Yeon Lee
Clin Exp Pediatr. 2016;59(10):395-401.   Published online October 17, 2016

Since the outbreak of the enterovirus 71 (EV71) infection in Malaysia in 1997, large epidemics of EV71 have occurred in the Asia-Pacific region. Many children and infants have died from serious neurological complications during these epidemics, and EV71 infection has become a serious public health problem in these areas. EV71 infection causes hand, foot and mouth disease (HFMD) in children,...

Erratum
Neurology
Erratum: Developmental profiles of preschool children with delayed language development
Ji Eun Jeong, Hyung Jik Lee, Jin Kyung Kim
Clin Exp Pediatr. 2016;59(9):386-386.   Published online September 21, 2016
Case Report
Neurology
Painful legs and moving toes syndrome in a 16-year-old girl
Seung Soo Kim, Yong Seung Hwang, Young Chang Kim
Clin Exp Pediatr. 2016;59(9):381-383.   Published online September 21, 2016

Painful legs and moving toes (PLMT) syndrome is characterized by spontaneous movements of the digits and pain in one or both lower extremities. Of the reported cases, a majority of the patients was female, and the mean age of onset was 58 years. Only one pediatric case has been reported so far. Herein, we report the first adolescent case of...

Original Article
Neurology
Venous angioma may be associated with epilepsy in children
Bo Ryung Kim, Yun Jin Lee, Sang Ook Nam, Kyung Hee Park
Clin Exp Pediatr. 2016;59(8):341-345.   Published online August 24, 2016
Purpose

Venous angioma (VA) is the most common congenital abnormality of the intracranial vasculature. This study aimed to investigate the relationship between VA and epilepsy and to identify the characteristics of children with VA and epilepsy.

Methods

The records of all patients aged less than 18 years who underwent brain magnetic resonance imaging (MRI) at Pusan National University Hospital were retrospectively reviewed. Patients...

Review Article
Neurology
The use of complementary and alternative medicine in children with common neurologic diseases
Gyu-Min Yeon, Sang Ook Nam
Clin Exp Pediatr. 2016;59(8):313-318.   Published online August 24, 2016

Complementary and alternative medicine (CAM) is a phrase used to describe additional health care methods such as mind/body practices and natural products not regarded as treatments by conventional medicine. The use of CAM in children with common neurologic diseases is more frequent than its use in healthy children (24%–78% vs. 12%). However, less than half of patients report such use...

White matter injury following rotavirus infection in neonates: new aspects to a forgotten entity, 'fifth day fits'?
Jung Sook Yeom, Chan-Hoo Park
Clin Exp Pediatr. 2016;59(7):285-291.   Published online July 31, 2016

That rotavirus infection can cause neurological symptoms in young children has been well established. However, it is surprising why rotavirus infection has been overlooked as a cause of neonatal seizures for many years, despite significant research interest in neonatal rotavirus infection. Neonates are the age group most vulnerable to seizures, which are typically attributed to a wide range of causes....

Case Report
Neurology
Two cases of familial cerebral cavernous malformation caused by mutations in the CCM1 gene
Im-Yong Yang, Mi-Sun Yum, Eun-Hee Kim, Hae-Won Choi, Han-Wook Yoo, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(6):280-284.   Published online June 30, 2016

Cerebral cavernous malformation (CCM) is a vascular malformation characterized by abnormally enlarged capillary cavities without any intervening neural tissue. We report 2 cases of familial CCMs diagnosed with the CCM1 mutation by using a genetic assay. A 5-year-old boy presented with headache, vomiting, and seizure-like movements. Brain magnetic resonance imaging (MRI) revealed multiple CCM lesions in the cerebral hemispheres. Subsequent...

Original Article
Neurology
Clinical importance of F-waves as a prognostic factor in Guillain-Barré syndrome in children
Eung-Bin Lee, Yun Young Lee, Jae Min Lee, Su Min Son, Su-Kyeong Hwang, Soonhak Kwon, Sae Yoon Kim
Clin Exp Pediatr. 2016;59(6):271-275.   Published online June 30, 2016
Purpose

A limited number of studies have examined the link between F-wave abnormalities and clinical presentation in pediatric Guillain-Barré syndrome (GBS). Therefore, this study examined the importance of F-wave abnormalities as a prognostic factor in pediatric GBS patients.

Methods

The records and electrodiagnostic studies (EDS) of 70 GBS patients were retrospectively evaluated, and divided into 2 groups according to the results of EDS....

Clinical features and prognostic factors in drowning children: a regional experience
Kyung Lae Son, Su Kyeong Hwang, Hee Joung Choi
Clin Exp Pediatr. 2016;59(5):212-217.   Published online May 31, 2016
Purpose

This study aimed to evaluate the clinical features of children who have survived a water submersion incident, and to identify risk factors for prognosis.

Methods

We retrospectively reviewed the medical records of patients who experienced submersion between January 2005 and December 2014. The patients were classified into 2 groups, according to complications, and prognostic factors were evaluated.

Results

During the study period, 29 children...

Review Article
Neurology
Cognitive impairment in childhood onset epilepsy: up-to-date information about its causes
Eun-Hee Kim, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(4):155-164.   Published online April 30, 2016

Cognitive impairment associated with childhood-onset epilepsy is an important consequence in the developing brain owing to its negative effects on neurodevelopmental and social outcomes. While the cause of cognitive impairment in epilepsy appears to be multifactorial, epilepsy-related factors such as type of epilepsy and underlying etiology, age at onset, frequency of seizures, duration of epilepsy, and its treatment are considered...

Letter to the Editor
Neurology
Blood glucose level after febrile convulsion
Mohamed Amin Ghobadifar, Naser Honar, Parisa Jooya, Fatemeh Hassani
Clin Exp Pediatr. 2016;59(3):153-154.   Published online March 31, 2016
Case Report
Neurology
Complete occlusion of the right middle cerebral artery associated with Mycoplasma pneumoniae pneumonia
Ben Kang, Dong Hyun Kim, Young Jin Hong, Byong Kwan Son, Myung Kwan Lim, Yon Ho Choe, Young Se Kwon
Clin Exp Pediatr. 2016;59(3):149-152.   Published online March 31, 2016

We report a case of a 5-year-old girl who developed left hemiparesis and left facial palsy, 6 days after the initiation of fever and respiratory symptoms due to pneumonia. Chest radiography, conducted upon admission, showed pneumonic infiltration and pleural effusion in the left lung field. Brain magnetic resonance imaging showed acute ischemic infarction in the right middle cerebral artery territory....

A new mosaic der(18)t(1;18)(q32.1;q21.3) with developmental delay and facial dysmorphism
Young-Jin Choi, Eunsim Shin, Tae Sik Jo, Jin-Hwa Moon, Se-Min Lee, Joo-Hwa Kim, Jae-Won Oh, Chang-Ryul Kim, In Joon Seol
Clin Exp Pediatr. 2016;59(2):91-95.   Published online February 29, 2016

We report the case of a 22-month-old boy with a new mosaic partial unbalanced translocation of 1q and 18q. The patient was referred to our Pediatric Department for developmental delay. He showed mild facial dysmorphism, physical growth retardation, a hearing disability, and had a history of patent ductus arteriosus. White matter abnormality on brain magnetic resonance images was also noted....

Original Article
Neurology
Epilepsy in children with a history of febrile seizures
Sang Hyun Lee, Jung Hye Byeon, Gun Ha Kim, Baik-Lin Eun, So-Hee Eun
Clin Exp Pediatr. 2016;59(2):74-79.   Published online February 29, 2016
Purpose

Febrile seizure, the most common type of pediatric convulsive disorder, is a benign seizure syndrome distinct from epilepsy. However, as epilepsy is also common during childhood, we aimed to identify the prognostic factors that can predict epilepsy in children with febrile seizures

Methods

The study comprised 249 children at the Korea University Ansan Hospital who presented with febrile seizures. The relationship between...

Intravenous levetiracetam versus phenobarbital in children with status epilepticus or acute repetitive seizures
Yun-Jeong Lee, Mi-Sun Yum, Eun-Hee Kim, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(1):35-39.   Published online January 22, 2016
Purpose

This study compared the efficacy and tolerability of intravenous (i.v.) phenobarbital (PHB) and i.v. levetiracetam (LEV) in children with status epilepticus (SE) or acute repetitive seizure (ARS).

Methods

The medical records of children (age range, 1 month to 15 years) treated with i.v. PHB or LEV for SE or ARS at our single tertiary center were retrospectively reviewed. Seizure termination was defined...

Factors associated with mumps meningitis and the possible impact of vaccination
Kyuyol Rhie, Heung-Keun Park, Young-Soo Kim, Jung Sook Yeom, Ji Sook Park, Ji-Hyun Seo, Eun Sil Park, Jae-Young Lim, Chan-Hoo Park, Hyang-Ok Woo, Hee-Shang Youn
Clin Exp Pediatr. 2016;59(1):24-29.   Published online January 22, 2016
Purpose

Mumps meningitis is a common complication of mumps infection; however, information on mumps meningitis in the postvaccine era is limited. The purpose of the present study was to determine factors associated with mumps meningitis and to discuss the effect of vaccination on this disease.

Methods

We retrospectively reviewed patients younger than 19 years with mumps, diagnosed at a university hospital in Korea...

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