Characteristics in Pediatric Patients with Coronavirus Disease 2019 in Korea

Background@#Based on the reports of low prevalence and severity of pediatric severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections, the Korean government has released new SARS-CoV-2 infection response and treatment guidelines for children under the age of 12 years. The government has further directed school reopening under strict preventive measures. However, there is still considerable concern on the impact of school reopening on community transmission of Coronavirus disease 2019 (COVID-19). In the present study, we aimed to evaluate the appropriateness of these directives and the severity of SARS-CoV-2 infections in children as compared to adults using sufficient national sample data. @*Methods@#In the present study, we evaluated the severity of SARS-CoV-2 infection in pediatric patients as compared to adults by analyzing the length of hospital stays (LOS), medical expenses, and hospital and intensive care unit (ICU) admission rates. A multivariate linear regression analysis was carried out to examine the effects of COVID-19 patients that the characteristics on the LOS and medical expenses, and multivariate logistic regression analysis were performed to identify COVID-19 characteristics that affect hospital and ICU admission rates and to prove the low SARS-CoV-2 infection severity in pediatric patients. @*Results@#The hospitalization period for children aged 0–9 was 37% shorter and that of patients aged 10–19 years was 31% shorter than those of older age groups (P < 0.001). The analysis of the medical expenses by age showed that on average, medical expenses for children were approximately 4,900 USD lower for children than for patients over 80 years of age. The linear regression analysis also showed that patients who were 0–9 years old spent 87% and those aged 10–19 118% less on medical expenses than those aged 70 and over, even after the correction of other variables (P < 0.001). The probability of hospitalization was the lowest at 10–19 years old (odds ratio [OR], 0.05; 95% confidence interval [CI], 0.03–0.09), and their ICU admission rate was also the lowest at 0.14 (OR, 0.14; 95% CI, 0.08–0.24). On the other hand, the likelihood of hospitalization and ICU admission was the highest in children aged 0–9 years, and among patients under the age of 50 years in general. @*Conclusion@#This study demonstrated the low severity of SARS-CoV-2 infection in younger patients (0–19 years) by analyzing the LOS, medical expenses, hospital, and intensive care unit admission rates as outcome variables. As the possibility to develop severe infection of coronavirus at the age of 10–19 was the lowest, a mitigation policy is also required for middle and high school students. In addition, children with underlying diseases need to be protected from high-risk infection environments.

Risk Factors of Outcomes of COVID-19 Patients in Korea: Focus on Early Symptoms

Background@#Coronavirus disease 2019 (COVID-19) has spread around the globe, and it is important to determine the risk factors of death in the general population. Our study aimed to determine the risk factors of death and severe illness requiring supplemental oxygen therapy based on the demographic and clinical characteristics of COVID-19 patients in Korea. @*Methods@#In this study, we used data provided by the Korea Disease Control and Prevention Agency (KDCA) and analyzed a total of 5,068 patients with COVID-19, excluding 19 pregnant women and 544 individuals with missing data. We performed logistic regression analysis to determine the impact of early symptoms on survival and severe disease. Logistic regression models included sex, age, number of comorbidities, symptoms on admission, blood pressure, heart rate, and body temperature as explanatory variables, and death and oxygen therapy as outcome variables. @*Results@#Logistic regression analyses revealed that the male sex, older age (≥ 60 years), higher number of comorbidities, presence of symptoms on admission, heart rate ≥ 120 bpm, and body temperature ≥ 37.5°C presented with higher risk of in-hospital death and oxygen therapy requirement. Conversely, rhinorrhea and headache were associated with a low risk of death and oxygen therapy requirement. The findings showed that cough, sputum, and fever were the most common symptoms on admission, while 25.3% of patients with COVID-19 were asymptomatic. @*Conclusion@#COVID-19 patients with high-risk early symptoms on admission, such as dyspnea and altered mental status, and those without low-risk symptoms of rhinorrhea and headache should be included in priority treatment groups.

Development of the Parental Questionnaire for Cerebral Visual Impairment in Children Younger than 72 Months

Background@#and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. @*Methods@#The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. @*Results@#The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. @*Conclusions@#The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.

Characteristics in Pediatric Patients with Coronavirus Disease 2019 in Korea

Background@#Based on the reports of low prevalence and severity of pediatric severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections, the Korean government has released new SARS-CoV-2 infection response and treatment guidelines for children under the age of 12 years. The government has further directed school reopening under strict preventive measures. However, there is still considerable concern on the impact of school reopening on community transmission of Coronavirus disease 2019 (COVID-19). In the present study, we aimed to evaluate the appropriateness of these directives and the severity of SARS-CoV-2 infections in children as compared to adults using sufficient national sample data. @*Methods@#In the present study, we evaluated the severity of SARS-CoV-2 infection in pediatric patients as compared to adults by analyzing the length of hospital stays (LOS), medical expenses, and hospital and intensive care unit (ICU) admission rates. A multivariate linear regression analysis was carried out to examine the effects of COVID-19 patients that the characteristics on the LOS and medical expenses, and multivariate logistic regression analysis were performed to identify COVID-19 characteristics that affect hospital and ICU admission rates and to prove the low SARS-CoV-2 infection severity in pediatric patients. @*Results@#The hospitalization period for children aged 0–9 was 37% shorter and that of patients aged 10–19 years was 31% shorter than those of older age groups (P < 0.001). The analysis of the medical expenses by age showed that on average, medical expenses for children were approximately 4,900 USD lower for children than for patients over 80 years of age. The linear regression analysis also showed that patients who were 0–9 years old spent 87% and those aged 10–19 118% less on medical expenses than those aged 70 and over, even after the correction of other variables (P < 0.001). The probability of hospitalization was the lowest at 10–19 years old (odds ratio [OR], 0.05; 95% confidence interval [CI], 0.03–0.09), and their ICU admission rate was also the lowest at 0.14 (OR, 0.14; 95% CI, 0.08–0.24). On the other hand, the likelihood of hospitalization and ICU admission was the highest in children aged 0–9 years, and among patients under the age of 50 years in general. @*Conclusion@#This study demonstrated the low severity of SARS-CoV-2 infection in younger patients (0–19 years) by analyzing the LOS, medical expenses, hospital, and intensive care unit admission rates as outcome variables. As the possibility to develop severe infection of coronavirus at the age of 10–19 was the lowest, a mitigation policy is also required for middle and high school students. In addition, children with underlying diseases need to be protected from high-risk infection environments.

Risk Factors of Outcomes of COVID-19 Patients in Korea: Focus on Early Symptoms

Background@#Coronavirus disease 2019 (COVID-19) has spread around the globe, and it is important to determine the risk factors of death in the general population. Our study aimed to determine the risk factors of death and severe illness requiring supplemental oxygen therapy based on the demographic and clinical characteristics of COVID-19 patients in Korea. @*Methods@#In this study, we used data provided by the Korea Disease Control and Prevention Agency (KDCA) and analyzed a total of 5,068 patients with COVID-19, excluding 19 pregnant women and 544 individuals with missing data. We performed logistic regression analysis to determine the impact of early symptoms on survival and severe disease. Logistic regression models included sex, age, number of comorbidities, symptoms on admission, blood pressure, heart rate, and body temperature as explanatory variables, and death and oxygen therapy as outcome variables. @*Results@#Logistic regression analyses revealed that the male sex, older age (≥ 60 years), higher number of comorbidities, presence of symptoms on admission, heart rate ≥ 120 bpm, and body temperature ≥ 37.5°C presented with higher risk of in-hospital death and oxygen therapy requirement. Conversely, rhinorrhea and headache were associated with a low risk of death and oxygen therapy requirement. The findings showed that cough, sputum, and fever were the most common symptoms on admission, while 25.3% of patients with COVID-19 were asymptomatic. @*Conclusion@#COVID-19 patients with high-risk early symptoms on admission, such as dyspnea and altered mental status, and those without low-risk symptoms of rhinorrhea and headache should be included in priority treatment groups.

Development of the Parental Questionnaire for Cerebral Visual Impairment in Children Younger than 72 Months

Background@#and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. @*Methods@#The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. @*Results@#The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. @*Conclusions@#The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.

De Novo Pure Trisomy 20p: Report of a Novel Case of a Marker Chromosome and Literature Review

No abstract available.

A Novel VPS33B Variant Identified by Exome Sequencing in a Patient with Arthrogryposis-Renal Dysfunction-Cholestasis Syndrome / 대한소아소화기영양학회지

Arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome is a rare autosomal recessive multisystemic disease that is associated with the liver, kidney, skin, and central nervous and musculoskeletal systems. ARC occurs as a result of mutations in the VPS33B (Vacuolar protein sorting 33 homolog B) or VIPAR (VPS33B interacting protein, apical-basolateral polarity regulator) genes. A female infant presented with neonatal cholestasis with a severe clinical outcome. She was diagnosed with ARC syndrome using targeted exome sequencing (TES). Exome sequencing revealed compound heterozygous mutations, c.707A>T and c.239+5G>A, in VPS33B, where c.707A>T was a novel variant; the resultant functional protein defects were predicted via in silico analysis. c.239+5G>A, a pathogenic mutation that affects splicing, is found in less than 0.1% of the general population. Invasive techniques, such as liver biopsies, did not contribute to a differential diagnosis of ARC syndrome; thus, early TES together with clinical presentations constituted an apparently accurate diagnostic procedure.

Association of Low Serum Ionized Magnesium Level with Fever-Triggered Seizures in Epileptic Children / 대한소아신경학회지

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 (iMg2+) 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 iMg2+ level was 0.93 (0.85–1.14, quartile) mEq/L. Serum iMg2+ 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 iMg2+ level significantly increased the risk of having FTS in epileptic children based on multivariable logistic regression analysis (odds ratio [OR]=0.028). CONCLUSION: Serum iMg2+ level was significantly lower in epileptic children with FTS than in those without FTS. Measurement of biologically active serum iMg2+ level could be considered in epileptic children with recurrent FTS. A large-scale prospective study is warranted.

Impact of Weather on Prevalence of Febrile Seizures in Children / 대한소아신경학회지

PURPOSE: Febrile seizure (FS) is the most common type of seizure in children between 6 months to 5 years of age. A family history of febrile seizures can increase the risk a child will have a FS. Yet, prevalence of FS regarding external environment has not been clearly proved. This study attempts to determine the association between prevalence of FS and weather. METHODS: This study included medical records from the Korea National Health Insurance Review and Assessment Service. Data were collected from 29,240 children, born after 2004, diagnosed with FS who were admitted to one of the hospitals in Seoul, Korea, between January 2009 and December 2013. During the corresponding time period, data from the Korea Meteorological Administration on daily monitoring of four meteorological factors (sea-level pressure, amount of precipitation, humidity and temperature) were collected. The relationships of FS prevalence and each meteorological factor will be designed using Poisson generalized additive model (GAM). Also, the contributory effect of viral infections on FS prevalence and weather will be discussed. RESULTS: The amount of precipitation was divided into two groups for comparison: one with less than 5 mm and the other with equal to or more than 5 mm. As a result of Poisson GAM, higher prevalence of FS showed a correlation with smaller amount of precipitation. Smoothing function was used to classify the relationships between three variables (sea-level pressure, humidity, and temperature) and prevalence of FS. FS prevalence was correlated with lower sea-level pressure and lower humidity. FS prevalence was high in two temperature ranges (-7 to -1℃ and 18–21℃). CONCLUSION: Low sea-level pressure, small amount of precipitation, and low relative air humidity may increase FS prevalence risk.

Prognosis in Late-Onset Febrile Seizure

PURPOSE: There is a paucity of evidence about prognosis after a first febrile seizure in older children. We investigated the prognosis and potential risk factors associated with subsequent unprovoked seizures in children who had experienced a first febrile seizure over 6 years of age, which we termed as late-onset febrile seizure. METHODS: We included all patients six years or older who presented to the emergency department with a febrile seizure between 2009 and 2015. Clinical data was collected by chart review and parents were contacted for information on seizure progress. We used the Cox proportional-hazards model and Kaplan-Meier analysis for evaluating the risk factors for subsequent unprovoked seizures. RESULTS: Of 247 patients, we excluded 168 children who had a history of epilepsy, unprovoked, or febrile seizure and who were followed-up for period less than six months. Overall, 79 patients were classified as having had a first late-onset febrile seizure. During follow-up of 34.9±25.7(mean±SD) months, unprovoked seizure recurred in 7 of 79 patients (9%). The cumulative probability of seizure recurrence was 4% at 6 months, 6% at 1 year and 9% at 2 years. Clinical variables predictive of subsequent unprovoked seizures were not proved. CONCLUSION: This is the first multicenter study focusing on prognosis after a late-onset febrile seizure in children six years or older. The percentage of subsequent unprovoked seizure in patients with late-onset febrile seizure was 9% at 2 years of follow-up. Prospective follow-up study with longer duration is warranted.

Neuroprotective Effect of Lacosamide on Hypoxic-Ischemic Brain Injury in Neonatal Rats

BACKGROUND AND PURPOSE: Lacosamide (LCM) is an antiepileptic drug that enhances the slow inactivation of sodium channels and modulates collapsin response mediator protein-2. LCM was recently demonstrated to exert a neuroprotective effect in a murine model of traumatic brain injury and status epilepticus. Assuming the same underlying excitotoxicity-related brain injury mechanism, we hypothesized that LCM would have a neuroprotective effect in hypoxic-ischemic brain injury. METHODS: We divided rats into three groups at each testing session: pre- or postfed with LCM, fed with normal saline, and sham. A hypoxic-ischemic brain injury was induced by subjecting 7-day-old rats to right carotid artery coagulation followed by 2.5 h of exposure to 8% oxygen. The animals were killed on postnatal day 12 to evaluate the severity of brain damage. Open field testing was also performed between week 2 and week 6, and the Morris water maze test was performed in week 7 after hypoxia-ischemia. RESULTS: The incidence of liquefactive cerebral infarction was lower in rats prefed with LCM at 100 mg/kg/dose, with the mortality rate being higher at higher doses (200 and 300 mg/kg/dose). The infarct areas were smaller in LCM-prefed rats in several brain regions including the hemisphere, hippocampus, cortex, and striatum. Spatial learning and memory function were better in LCM-prefed rats (p<0.05). No effect was observed in postfed rats. CONCLUSIONS: This study suggests that LCM pretreatment exerts a neuroprotective effect on hypoxia-ischemia in neonatal rats. The obtained results suggest that LCM pretreatment could be used as an effective neuroprotective method for neonates under hypoxic-ischemic conditions including heart surgery.

Childhood Idiopathic Occipital Lobe Epilepsy: Clinical Characteristics and Prognostic Factors

PURPOSE: Among syndromes of idiopathic occipital lobe epilepsy there are Panayiotopoulos syndrome (PS) and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G). However, the classification of epilepsy syndrome is difficult to diagnose at the first seizure because of varying characteristics. We analyzed the different clinical characteristics and prognostic factors of idiopathic occipital lobe epilepsy. METHODS: Forty-three patients who had at least two unprovoked seizures, no abnormalities on magnetic resonance imaging, no known etiology of seizure, and mainly occipital spikes on electroencephalography were retrospectively enrolled at the Korea University Medical Center. A good prognosis was defined as being seizure-free and taking two or less drugs for 1 year. RESULTS: Among all patients, the proportion of PS was 44.2%, ICOE-G was 7.0% and unclassified group was 48.8%. The age at seizure onset was 4.5±2.6 years (mean±SD) in the PS group, and 8.3±2.1 years in the ICOE-G group. The follow-up duration was 10.8±6.0 years. The percentage that the initial diagnosis had not been changed was 66.7% in the PS group and 100% in the ICOE-G group. Among other related symptoms only emesis (P<0.001) and visual symptoms (P=0.007) had varying characteristics between the PS and ICOE-G groups. Patients with PS had a better prognosis than those with ICOE-G or unclassified group (odds ratio [OR]=58.8). Patients with 1 more autonomic symptoms had a worse prognosis (OR=3.8). CONCLUSION: This study showed that only symptoms of emesis and visual symptoms can differentiate between PS and ICOE-G. More autonomic symptoms were associated with worse prognosis in childhood occipital lobe epilepsy.

Characterization and Predictors of Headache-Related Disability among School Children: A Population-Based Study

PURPOSE: Headache is a frequent neurological symptom in school aged individuals and recurrent headache has significant disabling effects among children and adolescents that manifest as school absenteeism, decreased extracurricular activities, and poor academic performance, as shown in previous studies. In Korea, there has not yet been a population-based study of headache-related disability in children and adolescents. We sought to estimate headache-related disability and investigate relevant predictors of disability due to headaches among schoolchildren in South Korea. METHODS: This was a cross-sectional school-based study. We surveyed 5,039 (boys 2,405, girls 2,634) students aged 6-18 years. Among 1,465 students with headache, six hundred sixty-six schoolchildren (225 boys, 441 girls) completed all questionnaires. The questionnaires collected demographic data, in addition to headache specific questions consistent with International Classification of Headache Disorder criteria, 2nd edition. Disability was evaluated using the 6-question Pediatric Migraine Disability Assessment (PedMIDAS). RESULTS: Six hundred sixty six school children and adolescents (225 boys, 441 girls) among 1,465 students with headaches completed all questionnaires. The percentage of headache sufferers with grade I disability was 88.6%. The mean (±standard deviation) PedMIDAS score was 5.11±11.17. There was a trend towards more severe disability in the older age groups, particularly among the 16 to18 year-olds. Students with migraine had the highest PedMIDAS scores (6.69±10.66) whereas students reporting other types of headache had the lowest scores (3.81±7.52). The predictors of headache-related disability were intensity (P=0.028), frequent headache (P=0.003), and longer duration of symptoms prior to presentation (P=0.008). CONCLUSION: A trend towards a more severe disability was observed in the older age group. Schoolchildren with migraine had the most headache-related disability. The predictors for headache-related disability were intensity, frequent headache, and longer duration of symptoms prior to presentation.

Characterization and Predictors of Headache-Related Disability among School Children: A Population-Based Study

PURPOSE: Headache is a frequent neurological symptom in school aged individuals and recurrent headache has significant disabling effects among children and adolescents that manifest as school absenteeism, decreased extracurricular activities, and poor academic performance, as shown in previous studies. In Korea, there has not yet been a population-based study of headache-related disability in children and adolescents. We sought to estimate headache-related disability and investigate relevant predictors of disability due to headaches among schoolchildren in South Korea. METHODS: This was a cross-sectional school-based study. We surveyed 5,039 (boys 2,405, girls 2,634) students aged 6-18 years. Among 1,465 students with headache, six hundred sixty-six schoolchildren (225 boys, 441 girls) completed all questionnaires. The questionnaires collected demographic data, in addition to headache specific questions consistent with International Classification of Headache Disorder criteria, 2nd edition. Disability was evaluated using the 6-question Pediatric Migraine Disability Assessment (PedMIDAS). RESULTS: Six hundred sixty six school children and adolescents (225 boys, 441 girls) among 1,465 students with headaches completed all questionnaires. The percentage of headache sufferers with grade I disability was 88.6%. The mean (±standard deviation) PedMIDAS score was 5.11±11.17. There was a trend towards more severe disability in the older age groups, particularly among the 16 to18 year-olds. Students with migraine had the highest PedMIDAS scores (6.69±10.66) whereas students reporting other types of headache had the lowest scores (3.81±7.52). The predictors of headache-related disability were intensity (P=0.028), frequent headache (P=0.003), and longer duration of symptoms prior to presentation (P=0.008). CONCLUSION: A trend towards a more severe disability was observed in the older age group. Schoolchildren with migraine had the most headache-related disability. The predictors for headache-related disability were intensity, frequent headache, and longer duration of symptoms prior to presentation.

Utility of Follow-up Diffusion Tensor Imaging in Acute Hemorrhagic Leukoencephalitis: a Case Report

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.

Anti-N-methyl-D-aspartate Receptor (NMDAR) Encephalitis: Neuronal Burden of a Comorbid Ovarian Teratoma

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient's clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas.

Utility of Follow-up Diffusion Tensor Imaging in Acute Hemorrhagic Leukoencephalitis: a Case Report

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.

Anti-N-methyl-D-aspartate Receptor (NMDAR) Encephalitis: Neuronal Burden of a Comorbid Ovarian Teratoma

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient's clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas.

Norovirus Associated Cerebellitis in a Previous Healthy 2-year-old Girl

Norovirus causes acute gastroenteritis, occasional afebrile seizures, and rarely encephalitis. We describe a child with cerebellitis due to norovirus that has not been reported previously. A previously healthy 2-year-old girl with a recent history of acute gastroenteritis, who presented with acute cerebellar ataxia. She exhibited marked truncal ataxia, was barely able to walk, and was prone to sitting or lying down. Multiplex PCR using stool samples revealed norovirus (genogroup II) and magnetic resonance imaging showed increased T2 signal in the hemi-cerebellum. Norovirus may be a potential pathogenic cause of acute cerebellitis in children.