-
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 the patients with primary immunodeficiencies, but lately it is expanding its usage to the realms of treating patients with neurological conditions. Both the efficacy and safety of IVIG treatment in chronic inflammatory demyelinating polyradiculoneuropathy and Guillain?Barr? syndrome have been studied successfully. However, the use of IVIG treatment in other neurological conditions still remains investigational despite several successful reports. Considerable numbers of mechanisms have been suggested in order to explain the effects of IVIG, but the exact mechanisms are not understood yet. This review covers the new developments in clinical fields and the possible ways in which IVIG could help in the future.
-
PURPOSE: We analyze the brain magnetic resonance imaging (MRI) findings of children with epilepsy and concomitant attention deficit hyperactivity disorder (ADHD) to investigate the correlation between brain MRI and ADHD, and to determine whether abnormal MRI finding can be a risk factor for the development of ADHD. METHODS: A total of 55 patients (36 male, 19 female) were diagnosed as ADHD in children with epilepsy at the pediatric neurology department of Seoul St. Mary hospital from March, 2009 to December, 2013. The records of these patients were retrospectively reviewed. RESULTS: 29 patients (52.7%) had normal MRI findings, and 26 patients (47.3%) had abnormal MRI findings. The inattention type of ADHD (96.5%) was the largest type in a group of normal brain MRI findings, but the combined type (53.8%) and the inattention type (46.2%) occupied the majority in a group of abnormal MRI findings. The score of symptom in inattention was 7.44/9 in a group of normal MRI findings, while 8.2/9 in a group of abnormal MRI findings. And the score of symptom in hyperactivity was 2.93/9 in a group of normal MRI findings, while it was 4.8/9 in a group of abnormal MRI findings (P
-
Spontaneous intracranial hypotension in childhood is rare, and a few cases have been reported as a cause of headache in children. A 9-year-old boy was admitted to our hospital with a 3-day history of new-onset headache that worsened upon standing or walking, and aggravating low back pain. No medical history of injury, connective tissue disorder or migraine was detected. A neurological examination revealed neck stiffness. His initial blood tests suggested acute kidney injury by increased blood urea nitrogen (BUN) and creatinine. Brain computed tomography (CT) and cerebral spinal fluid (CSF) analysis were normal: however, opening pressure was low (
-
Menkes disease (also known as kinky hair disease) is an X-linked recessive neurodegenerative disorder caused by diverse mutations in a copper-transport gene, ATP7A. Affected patients are characterized by kinky hair, hypotonia, and generalized myoclonic seizures. Here, we report a case of Menkes disease in which the patient presented with progressive hypotonia and intractable seizures. A 4-month-old male infant visited our pediatric clinic for focal seizures with blinking eyes. He was generally hypotonic and suffered from malnutrition. The focal seizures became more frequent, and the patient became intractable to anti-seizure medications. An electroencephalogram (EEG) indicated diffuse cerebral dysfunction with focal seizure, and a brain magnetic resonance imaging (MRI) showed tortuous and ectatic intracranial arteries, as well as several ischemic lesions. A genetic analysis was performed, and a c.2473_2474del (p.Leu825fsX1) of the ATP7A gene was detected.
-
Posterior reversible encephalopathy syndrome (PRES, or posterior leukoencephalopahty syndrome) is a neurological condition caused by reversible cortical/subcortical vasogenic brain edema secondary to hypertension, cytotoxic drugs, immunosuppressants, autoimmune diseases, renal disease, eclampsia or pre-eclampsia. It is characterized by acute neurological symptoms such as headache, seizures, visual disturbances, and impaired levels of consciousness. Brain imaging usually reveals bilateral, cortical/subcortical vasogenic edema. Completely unilateral PRES constituted only 2.6% of the cases in a previous study. Here we report the case of a pediatric patient with completely unilateral PRES. A 13-year-old boy was admitted with acute gastroenteritis. On the fourth day of hospitalization, he started to complain of headache and vomiting. He then developed generalized tonic-clonic seizure 3 times. His blood pressure was 180/121 mmHg during the first seizure, 188/112 mmHg during the second seizure and 152/92 mmHg during the third seizure. T2-weighted imaging with fluid attenuation by inversion recovery (T2 FLAIR) demonstrated high-signal intensity in the cortical gyri of the left frontal, parietal, and occipital lobes. Follow-up magnetic resonance imaging (MRI) was performed 2 weeks after the seizure onset, which indicated a significant improvement in the patient's condition. Abdominal pelvic computed tomography (CT) and renal CT angiography showed abnormal narrowing of the left renal artery. In summary, we present a case report of unilateral PRES secondary to renovascular hypertension due to left renal arterial obstruction.
-
Mycoplasma pneumoniae is a respiratory pathogen responsible for various upper and lower respiratory tract diseases. This also induces extra-pulmonary manifestations, with encephalitis being the most frequent and critical manifestation in pediatric populations. This report describes an 8-year-old boy who presented with repetitive motions in both hands, agitation, muttering and increased appetite after seizure. He had started treatment with antibiotics for M. pneumoniae infection 14 days prior to this event. Electroencephalography (EEG) showed slow and disorganized background rhythms and polymorphic slowing on both frontal areas, whereas brain magnetic resonance imaging was normal. He was diagnosed with M. pneumoniae-related encephalitis and treated with intravenous immunoglobulin (500 mg/kg/day for 4 days). After 3 days, his impulsive behaviors disappeared and, after 4 days, his EEG became normal. Abrupt hyperactive behavior in children may be a sign of M. pneumoniae-related encephalitis.
-
Neuronal ceroid lipofuscinoses (NCLs) are inherited neurodegenerative disorders, which are caused by the accumulation of lipopigment in lysosomes. Variant forms of late infantile NCLs (vLINCLs) characterized by a later onset of seizures and visual impairment (3?8 years) than in the classic form (2?4 years) are caused by mutations of the gene encoding ceroid lipofuscinosis neuronal protein 6 (CLN6). In a girl with progressive myoclonus epilepsy, we found heterozygous variants of CLN6 (NM_017882.2; NP_060352.1): c.296A>G (p.Lys99Arg) and c.307C>T (p.Arg103Trp). They were identified with whole-exome sequencing and verified with Sanger sequencing. At 7 years and 9 months, our patient had developed multiple types of seizures, prominent myoclonus with photosensitivity, regression in motor and language skills, pyramidal and extrapyramidal signs, and brain atrophy in brain images, all of which were progressive and were compatible with vLINCLs. However, this first Korean report shows no visual impairment, which resembles the previously reported Japanese case.
-
Advances in network science and computer engineering have enabled brain connectivity analysis using clinical big data such as brain magnetic resonance imaging (MRI), electroencephalography (EEG), or magnetoencephalography (MEG). Resting-state functional connectivity analysis aims to reveal the characteristics of functional brain network in various diseases and normal brain maturation using resting-state EEG. Simplified sequence of resting-state functional connectivity analysis methods will be reviewed in this article. The outcomes from EEG resting-state connectivity analysis are comprised of connectivity itself of the specific condition and the network topology measure which describe the characteristics of specific connectivity. An increasing number of studies report the differences in the functional connection itself, global network measures including segregation (connectedness), integration (efficiency), and importance of specific nodes (centrality or node degree). Several issues that are relevant in the resting-state connectivity analysis are obtaining good quality EEG for analysis, consideration of particular features of EEG signal, understanding different types of association measures, and statistics for comparison of connectivities. Well-designed and carefully analyzed EEG resting-state connectivity analysis can provide useful information for patient care in pediatric neurology.
-
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. We investigated the clinical characteristics and pathogens in childhood encephalitis and encephalopathy. METHODS: We retrospectively reviewed the records of children with acute encephalitis and encephalopathy admitted to our hospital between March 2013 and February 2017. RESULTS: The 51 included patients were aged 5.8
-
PURPOSE: To investigate the relationship between the smart devices usage-related factors and self-regulation ability development in early childhood. METHODS: Parental questionnaires of 187 children aged 3?6 years were analyzed. The metrics included smart device usage frequency (times/week, scored as uFreq), smart device usage time (hours/day, scored as uTime), parental scale for appropriate smart device usage level (scored as uLevel), the Korean-developmental screening test (K-DST), and the scale for self-regulation ability in young children (scored as SRS, and including four sub-categories: self-appraisal, self-determination, behavior inhibition, and emotionality). The correlations were analyzed by total age group and by each age. RESULTS: In the total age group analysis, uFreq and uTime were negatively correlated with mean SRS (rs =?0.366, ?0.330; P
-
PURPOSE: The purpose of this study was to identify the risk factors for developmental delays in preterm infants. METHODS: We studied 151 preterm infants admitted to the neonatal intensive care unit (NICU) at the Hallym University Kangnam Sacred Heart Hospital from January 2013 to November 2016. After discharge, the infants were evaluated by a pediatric neurologist via the developmental screening test K-ASQ:SE II, which consists of five domains: communication (CC), gross motor (GM), fine motor (FM), problem solving (PS), and social-emotional (SE). The subjects were divided into a normal group and an abnormal group (abnormal results on at least one of the five domains). Several variables were compared between the two groups and risk factors for developmental delays were analyzed. RESULTS: Several factors, such as birth weight (BW), gestational age (GA), Apgar score at 1 and 5 min (AS1, AS5), hospital days (HDs), respiratory distress syndrome (RDS), chronic lung diseases, intraventricular hemorrhage (IVH), early sepsis, retinopathy of prematurity (ROP), and history of management of invasive ventilators, dexamethasone, anti-hypotensive, were significantly different between the normal and abnormal groups. BW was a risk factor for developmental delay according to the binary logistic regression analysis. On individual domain analysis, risk factors were lower GA for domains CC and FM, lower AS1 for GM domain, lower BW for PS domain, and longer HDs for SE domain. CONCLUSION: In preterm infants, regular developmental screening especially follow-up observation, is important for early detection of developmental delay, considering the risk factors, such as GA (
-
PURPOSE: Ischemic stroke is rarely seen in children, but it could cause mortality and result in developmental disabilities such as motor paralysis, cognitive dysfunction, and epilepsy. In this study, the neurological outcomes of ischemic stroke in children were reviewed and the factors associated with the neurological outcomes were to be analyzed. METHODS: Medical records of patients younger than 15 years of age who were newly diagnosed with ischemic stroke between January 2006 and December 2016 in Chonnam National University Hospital were reviewed. RESULTS: This study consisted of 38 patients with ischemic stroke (male/female= 18/20, mean age=6 years 1 month
-
PURPOSE: Despite the fact that enteroviral meningitis is the most common cause of meningitis in children with signs of meningeal irritation and has benign course, most clinicians routinely perform an invasive lumbar puncture (LP) that result in inadequate antibiotic therapy and unnecessary long-term hospitalization. This study was aimed to compare the clinical characteristics of childhood enteroviral meningitis according to LP. METHODS: Children over 2 years of age who can clearly express signs of meningeal irritation in the pediatric department of Eulji university hospital from July 2013 and August 2016 were enrolled. The patients were diagnosed by reverse transcriptase polymerase chain reaction in stool and/or cerebrospinal fluid. We retrospectively reviewed their clinical records. RESULTS: A total of 257 patients were diagnosed with enterovirus meningitis and median age was 6.3 (2.1?7.9) years. One hundred fifteen patients (44.7%) with confirmed enterovirus in the stools underwent supportive care without LP. Mostly, there was no statistically significant difference in age, sex, clinical symptoms, except gastrointestinal involvement (abdominal pain, diarrhea), and serologic findings when compared with patients who underwent LP. But patients who underwent LP had longer hospital stay (4 vs 3 days, P
-
PURPOSE: To identify the significance of the 12-month developmental assessment in high-risk neonates by comparing their 12 month and later childhood development. METHODS: We retrospectively reviewed the records of high risk neonates admitted to neonatal intensive care units of Korea University Ansan Hospital for ten years. Data of 146 patients, who underwent the Bayley test at 12 months of age and retook the same test at 24?36 months, was analyzed. Changes in mental developmental index (MDI) and psychomotor developmental index (PDI) were assessed and
-
PURPOSE: Herpes zoster appears in all ages and its incidence progressively increase. It is more common in elderly people or immunocompromised people and can be accompanied by serious complications. This study was performed to investigate the clinical manifestation of herpes zoster according to immune status in children. METHODS: This study was retrospectively included 307 children under 18 years who were diagnosed and treated with herpes zoster at Inha University Hospital from 1997 to 2017 based on medical records. These patients were divided into two groups according to their immunity and their clinical features were compared. RESULTS: The mean age of the total 307 patients was 10.2 years, 151 (49.2%) in males. Eighty-seven patients were hospitalized and 220 patients were treated in an outpatient clinic. Most patients received antiviral treatment. The most common dermatomal distribution of the skin lesion was the thoracic region, followed by trigeminal, lumbar and sacral, cervical region. Twenty-one patients were immunocompromised and fifteen of them were hematologic disorders. Admission rate, history of chickenpox and mean duration of treatment were significantly higher in immunocompromised group (P
-
Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous syndrome caused by mutations in the neurofibromin gene. NF-1 patients have a high risk of tumors, and optic glioma is the most commonly observed central nervous system tumor in these patients. However, glioblastoma is extremely rare in pediatric NF-1 patients. Here we report the discovery of a novel heterozygous c.6766_6767insAA (p.Ser2256Lysfs*4), pathogenic mutation in the neurofibromin gene in a 17-year-old boy with NF-1-associated glioblastoma.
-
Viral encephalitis can lead to serious neurological sequelae and death among younger children. It is also known that the mortality rate in encephalitis with cerebral edema or transtentorial brain herniation is higher. A 4-year-old boy visited our emergency department exhibiting mental change. The patient had a high fever for four and a whole-body rash for three days prior to his visit. He had displayed irritable symptoms and been vomiting for six hours before his visit, accompanied by seizure. After 13 hours of admission, the patient's right pupil became fixed and fully dilated, and the left pupil also became fixed and fully dilated within 30 minutes. Brain computed tomography (CT) was performed immediately, and severe brain swelling with transtentorial brain herniation was found. The mannitol dose was increased and dexamethasone was also added. Hyperventilation was performed through intubation to reach PaCO
-
Seizure is a temporary symptom or sign which is caused by an abnormal electrical stimulation of brain. Depending on whether a seizure has preceding factors or not, it can be further categorized into provoked and unprovoked seizure. In provoked seizure, it is important to find a cause for treatment. In this study, we would like to report a case of a 6 year-old male child with seizure caused by organophosphate poisoning. The patient's chief complaint was his decreased mental status accompanying seizure. Initially, status epilepticus was suspected but the response to anticonvulsants was not good, and resulted in prolonged respiratory failure. After 3 hours, the patient showed signs of cholinergic crisis. In response, atropine was administered and the condition improved. If respiratory failure or mental confusion persists even after cessation of seizure in status epilepticus, repetitive physical and neurological examinations should be carried out to find preceding factors. Even though the recent incidence of organophosphate poisoning has decreased, we would like to emphasize from our study that it should be considered as a preceding factor for seizure.
-
Purpose: Floppy infants or congenital hypotonia indicates decreased muscle tone in infants secondary to abnormalities of the central or the peripheral nervous system, or both. Previous literature classified its causes as those attributable to a central vs. peripheral origin; however, recent studies have introduced a newer classification describing a combined origin. We invenstigated floppy infants by applying the new etiological classification and reviewed the most common etiologies based on the age of presentation. We additionally reviewed the clinical characteristics, diagnoses, and the developmental outcomes in these infants.
Methods: We retrospectively reviewed the electronic medical charts and recruited 116 infants diagnosed with floppy infant syndrome between January 2005 and December 2016 at Severance Children
-
Purpose: West syndrome is a severe form of age-specific epilepsy that typically affects infants younger than 2 years of age with mitochondrial disease. We aimed to examine age-specific characteristics of the syndrome in these patients.
Methods: We retrospectively analyzed 54 patients with West syndrome diagnosed with mitochondrial disease between March 2006 and March 2016. We compared treatment strategies and diagnostic and clinical variables between patients with early-onset (
-
Purpose: Several studies have shown that magnesium plays an important role in modulating N-methyl-D-aspartate (NMDA)-related seizures by blocking NMDA ion channel receptors. Clinicians usually measure total serum magnesium levels instead of biologically active ionized magnesium levels. We compared the serum ionized magnesium (iMg
2+) level between epileptic children with and without a history of fever-triggered seizure (FTS).
Methods: All epileptic children who visited the outpatient clinic or pediatric emergency department at Korea University Guro Hospital between January 2015 and July 2017 were included. Only epileptic children aged 1-8 years who were newly diagnosed within 2 years were included.
Results: There were 12 children with FTS and 16 without FTS. Median serum iMg
2+ level was 0.93 (0.85-1.14, quartile) mEq/L. Serum iMg
2+ level was significantly lower in epileptic children with FTS (0.86 mEq/L) compared to those without FTS (1.10 mEq/L) (
P =0.005). No difference was noted in clinical variables between the two groups. Lower serum iMg
2+ level significantly increased the risk of having FTS in epileptic children based on multivariable logistic regression analysis (odds ratio [OR]=0.028). Conclusion: Serum iMg
2+ level was significantly lower in epileptic children with FTS than in those without FTS. Measurement of biologically active serum iMg
2+ level could be considered in epileptic children with recurrent FTS. A large-scale prospective study is warranted.
-
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 is related to the incidences of seasonal influenza and of specific influenza subtypes in Korea. METHODS: Patients diagnosed with BACM between March, 2010 and December, 2014 were investigated retrospectively. The occurrence of BACM was compared with the incidence of influenza for each influenza season, as obtained from the Korea Centers for Disease Control and Prevention. RESULTS: All patients had previous infection preceding calf muscle pain or gait disturbance. Laboratory findings showed elevated levels of creatine kinase (median, 1243 U/L) and leukopenia (
-
Purpose: A relationship between Febrile seizure (FS) and iron deficiency anemia (IDA) has been found in several studies. However, few studies have focused on the role of IDA in complex febrile seizures (CFS) and simple febrile seizures (SFS) and there is no report on whether IDA is a risk factor for recurrence. The aim of this study was to investigate the role of IDA in SFS and CFS and to examine the effect of IDA on recurrence.
Methods: Patients (n=166) who had been diagnosed with FS were enrolled in our study. Subjects were divided into the following groups for analysis: the SFS and CFS groups, recurrence and non-recurrence groups. The onset age was compared in each group of patients and laboratory test results based on IDA were compared.
Results: Between the SFS and the CFS groups, there was no significant difference in laboratory test results based on IDA. There was a significant difference in onset age between the two groups and the onset age tended to be lower in the CFS group (24.00 vs. 16.49 months) (
P=0.004). Comparing recurrence and non-recurrence groups, the mean corpuscular volume was significantly different (
P=0.043) with the recurrence group having a lower mean corpuscular volume level (78.92 vs. 77.48). The onset age in the recurrence group was lower (26.02 vs. 19.68 months).
Conclusion: This study suggests that onset age could be a risk factor for CFS, and IDA may not contribute to elevating the risk of CFS. However, IDA may play an important role in the recurrence of FS.
-
Purpose: Few reports have described the prognostic factors affecting the occurrence of subsequent unprovoked seizure in patients who present with febrile seizure (FS) after 6 years of age. We investigated the prognostic factors affecting the development of unprovoked seizures after FS among patients from Jeju Island.
Methods: We included patients who developed FS after 6 years of age, who presented to our outpatient clinic between January, 2011 and June, 2017. Clinical data were obtained through chart reviews and phone call interviews. We used logistic regression analysis to analyze the risk factors associated with the occurrence of subsequent unprovoked seizure.
Results: Of the 895 patients who presented to our hospital due to their febrile seizure, 83 developed FS after 6 years of age. Among them, 3 patients were prescribed antiepileptic drugs before the onset of the unprovoked seizure, and 4 patients developed an unprovoked seizure before 6 years of age. Thus, overall, 76 patients were included in the study. 51 patients developed first FS before 6 years of age. In the remaining patients, the first FS developed after 6 years of age. The mean observational period since the last outpatient follow-up visit was 3.2 years (median 3.04 years, range: 1.42-4.71 years). Among them, 21% developed an unprovoked seizure. Logistic regression analysis showed that electroencephalographic (EEG) abnormalities served as an independent risk factor for a subsequent unprovoked seizure.
Conclusion: EEG is the proper diagnostic tool to predict the risk of a subsequent unprovoked seizure in patients with FS after 6 years of age.
-
Purpose: Febrile seizures (FSs) are the most common type of seizure in the first 5 years of life and are frequently associated with viral infections. Influenza infection is associated with a variety of neurological conditions, including FSs. The purpose of this study was to evaluate the clinical implications of influenza infection in FSs.
Methods: In total, 388 children with FS were divided into two groups: FS with influenza infection (n=75) and FSs without influenza infection (n=313). Their medical records, including seizure type, frequency, duration, and familial history of FSs or epilepsy, were retrospectively reviewed and the clinical characteristics of the two groups were compared.
Results: In total, 75 of the 388 children (19.3%) had FSs associated with influenza infection; such children were significantly older than those with FSs without influenza infection (34.9
