Older Patients May Have More Frequent Seizures among Children Diagnosed as Benign Convulsions with Mild Gastroenteritis.

Purpose: Benign convulsions with mild gastroenteritis (CwG) is a condition that does not usually require treatment. However, when the patient experiences multiple seizures or prolonged seizures, anticonvulsant treatment may be required. We investigated this study to identify the factors that may influence on the number or duration of seizures at CwG.

Human Herpesvirus-6 may be Neurologically Injurious in Some Immunocompetent Children.

Purpose: Human herpesvirus 6 (HHV-6) can infect the central nervous system in immunocompromised individuals. Less is known, however, about HHV-6 infection in immunocompetent patients. This study evaluated the neurologic features and prognosis of HHV-6 infection in immunocompetent patients.

Evaluation of hematologic profile may be needed for patients treated with oxcarbazepine.

Purpose: The major side effects of treatment with oxcarbazepine (OXC) are skin rash and hyponatremia. Hematologic side effects are reported rarely. The aim of this study was to investigate the rate and types of the hematologic side effects of OXC.

A long-term subacute sclerosing panencephalitis survivor treated with intraventricular interferon-alpha for 13 years.

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and fatal central nervous system disorder resulting from persistent measles virus infection. Long-term data are scarce, with a maximum follow-up period of 10 years. Interferon-alpha (IFN-α) is a protein that exerts its antiviral activity via enhancement of cellular immune response and is reported to be an effective drug for the treatment of SSPE.

Factors associated with electroencephalographic and clinical remission of benign childhood epilepsy with centrotemporal spikes.

Purpose: Benign childhood epilepsy with centrotemporal spikes (BECTS) is strongly related to age, both to age at the time of seizure onset and to age at remission. However, the age of remission varies. The present study analyzed factors associated with remission of BECTS.

A 6-Month-Old Girl with Incontinentia Pigmenti Presenting as Status Epilepticus.

Incontinentia pigmenti (IP) is an uncommon neurocutaneous syndrome. Its initial diagnosis is based primarily on characteristic papulovesicular skin lesions and early-onset neonatal seizures. In contrast to typical early neurologic manifestations, we encountered a normally developed 6-month-old female patient with hyperpigmented whorls on her body.

Short-Term Outcome of Intravenous Methylprednisolone Pulse Therapy in Patients With Infantile Spasms.

Background: Many studies advocate hormonal treatments including high-dose oral prednisolone as an effective treatment for epileptic spasms. However, little is known about the effects of intravenous methylprednisolone pulse therapy on infantile spasms. We investigated the short-term response to intravenous methylprednisolone pulse therapy for the treatment of infantile spasms.

Paroxysmal kinesigenic dyskinesia in a patient with a mutation and centrotemporal spike discharges on electroencephalogram: case report of a 10-year-old girl.

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.

Prognostic factors of neurological outcomes in late-preterm and term infants with perinatal asphyxia.

Purpose: This study aimed to identify prognostic factors of neurological outcomes, including developmental delay, cerebral palsy and epilepsy in late-preterm and term infants with perinatal asphyxia.

Methods: All late-preterm and term infants with perinatal asphyxia or hypoxic-ischemic insults who admitted the neonatal intensive care unit of Inje University Sanggye Paik Hospital between 2006 and 2014 and were followed up for at least 2 years were included in this retrospective study. Abnormal neurological outcomes were defined as cerebral palsy, developmental delay and epilepsy.

An interictal EEG can predict the outcome of vagus nerve stimulation therapy for children with intractable epilepsy.

Purpose: This study aimed to evaluate the long-term efficacy of vagus nerve stimulation (VNS) in children and adolescents with intractable epilepsy and identify predictive factors for responsiveness to VNS.

Methods: Medical records of pediatric patients who underwent VNS implantation at two Korean tertiary centers were reviewed. At 0.

The relationship between primary headache and constipation in children and adolescents.

Purpose: Many patients presenting with headache also complain of constipation; the relationship between these two symptoms has not been explored in detail. The aim of this study was to investigate the association between primary headache and constipation.

Methods: This retrospective study included all children who attended the Inje University Sanggye Paik Hospital complaining of headache, and who had been followed up for at least 100 days.

Maternal immune activation promotes hippocampal kindling epileptogenesis in mice.

Objective: Maternal immune activation (MIA) triggered by infections has been identified as a cause of autism in offspring. Considering the involvement of perturbations in innate immunity in epilepsy, we examined whether MIA represents a risk factor for epilepsy as well. The role of specific MIA components interleukin (IL)-6 and IL-1β was also addressed.

Human parechovirus-3 infection in children, South Korea.

Background: Human parechoviruses (HPeVs) have recently been recognized as important viral pathogens causing sepsis-like illness and meningitis in children, but the data on these infections in Korea is limited. Klassevirus is emerging as a novel etiologic agent of acute gastroenteritis, but its role in meningitis remains unclear.

Objectives: To understand the epidemiology of HPeVs and klassevirus in sepsis-like illness and meningitis through the detection and typing of the virus in cerebrospinal fluid (CSF) samples.

Magnetic resonance imaging and spectroscopic analysis in 5 cases of Pelizaeus-Merzbacher disease: metabolic abnormalities as diagnostic tools.

Pelizaeus-Merzbacher disease (PMD) is a rare, X-linked recessive disorder characterized by dysmyelination in the central nervous system. PMD results from deletion, mutation, or duplication of the proteolipid protein gene (PLP1) located at Xq22, leading to the failure of axon myelination by oligodendrocytes in the central nervous system. PMD may be suspected when there are clinical manifestations such as nystagmus, developmental delays, and spasticity, and genetic analysis can confirm the diagnosis.

A multicenter trial of oxcarbazepine oral suspension monotherapy in children newly diagnosed with partial seizures: a clinical and cognitive evaluation.

Purpose: We conducted a prospective, multicenter, open label trial to evaluate the effectiveness of oxcarbazepine (OXC) oral suspension as monotherapy for children newly diagnosed with partial seizures.

Methods: This trial included a two- to eight-week titration and stabilization period to achieve effective target doses and a 24-week maintenance phase. The primary outcome measure was the seizure-free rate over six months, while a secondary measure was the change in cognition and behavior from screening to the end of the maintenance phase.

Cognitive function of idiopathic childhood epilepsy.

Most children with epilepsy are of normal intelligence. However, a significant subset will have temporary or permanent cognitive impairment. Factors that affect cognitive function are myriad and include the underlying neuropathology of the epilepsy, seizures, epileptiform discharges, psychosocial problems, age at seizure onset, duration of epilepsy, and side effects associated with antiepileptic drugs.

A case of acute necrotizing encephalopathy associated with parainfluenza virus infection.

Acute necrotizing encephalopathy (ANE) may be suspected when a young child presents with abrupt onset of altered mental status, seizures, or both. Definitive clinical diagnosis is based on magnetic resonance imaging (MRI) results. ANE is associated with influenza virus infections.

Staged total callosotomy for lennox-gastaut syndrome: a case report.

We report a case with Lennox-Gastaut syndrome (LGS) who underwent staged total callosotomy with a favorable outcome. A 6-year-old boy began having myoclonic seizures at the age of 8 months and was diagnosed with LGS when he was 27 months old. Various antiepileptic drugs and a ketogenic diet failed to control his multiple types of seizures.

A case of midgut malrotation presenting as multiple afebrile seizures.

Provoked seizures are a common pediatric problem and can be caused by multiple etiologies. However, there have been few reports of benign infantile seizures associated with gastrointestinal tract abnormality. We report on a 3-month-old male infant who presented with multiple afebrile seizures, EEG abnormalities and a midgut malrotation.

Comparison of short- versus long-term ketogenic diet for intractable infantile spasms.

Purpose: To compare the prognoses between short-term (8 months) and conventional long-term (> 2 years) trials involving patients with refractory infantile spasms who successfully completed the ketogenic diet (KD).

Methods: Of 40 patients who achieved seizure-free outcomes and showed improvement in hypsarrhythmic patterns within 6 months of the KD, with a 3:1 fat to nonfat ratio as an add-on treatment, 16 patients were randomized into the short-term trial group and the diet was tapered throughout two additional months. Twenty-four patients were randomized into a long-term trial group, and 19 patients could successfully discontinue the diet after 2 years.

Neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy.

We characterized the neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy and measured differences in IQ between children with different types of epilepsy. The Korean Education Development Institute-Wechsler Intelligence Scale for Children (KEDI-WISC) was administered to 72 patients (35 males and 37 females), of mean age 8.7±2.

Factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome.

This study examines factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome. The study population comprised 98 patients diagnosed with West syndrome and monitored for at least 3 years. During follow-up, West syndrome evolved to Lennox-Gastaut syndrome in 48 of the 98 patients.

Identification of a de novo Lys304Gln mutation in the glycine receptor alpha-1 subunit gene in a Korean infant with hyperekplexia.

Startle disease or hyperekplexia (STHE; MIM 149400) is a rare disorder that is characterized by marked muscular hypertonia in infancy and an exaggerated startle response to unexpected acoustic or tactile stimuli. Mutations in the gene encoding the alpha-1 subunit of the inhibitory glycine receptor (GLRA1) were reported as causes of STHE. Recently, we encountered a Korean male infant with generalized stiffness that was observed from the first 3 days of life.