Intracranial Hypertension after COVID-19 Infection

Headache is a frequent complaint in coronavirus disease-19 (COVID-19) patients. Nevertheless, no detailed information on the pathophysiology of headache in COVID-19 infection is currently limited. We encountered a patient developing headache, diplopia, and intracranial hypertension after COVID-19 infection. The patient no more complained of headache and diplopia after the intracranial pressure was normalized. Our case suggests that intracranial hypertension is a mechanism of headache in COVID-19 infection. We report herein a case of transient intracranial hypertension after COCVID-19 infection.

The First Reported Case of Infant Botulism in Korea: Treatable Infantile Neuromuscular Disease

Botulism is a rare neuromuscular disorder caused by neurotoxins produced by Clostridium botulinum. The diagnosis of infant botulism may be obscured or delayed, as its presentation is similar to that of infantile neuromuscular disorders. We report the first Korean case of infant botulism in an acute progressive floppy infant with poor sucking and a weak cry. No abnormalities were found in all blood, cerebrospinal fluid, genetic test, nerve conduction study, and imaging studies. A stool-toxin test was finally performed under suspicion of infant botulism, and the result was positive. The patient was immediately treated with heptavalent botulism antitoxin. Follow-up after 3 months showed normal development with a complete resolution of all symptoms. Therefore, clinical suspicion of infant botulism, which is a treatable infantile neuromuscular disease, is essential for early diagnosis and prompt treatment in the differential diagnosis of a floppy infant.

The First Reported Case of Infant Botulism in Korea: Treatable Infantile Neuromuscular Disease

Botulism is a rare neuromuscular disorder caused by neurotoxins produced by Clostridium botulinum. The diagnosis of infant botulism may be obscured or delayed, as its presentation is similar to that of infantile neuromuscular disorders. We report the first Korean case of infant botulism in an acute progressive floppy infant with poor sucking and a weak cry. No abnormalities were found in all blood, cerebrospinal fluid, genetic test, nerve conduction study, and imaging studies. A stool-toxin test was finally performed under suspicion of infant botulism, and the result was positive. The patient was immediately treated with heptavalent botulism antitoxin. Follow-up after 3 months showed normal development with a complete resolution of all symptoms. Therefore, clinical suspicion of infant botulism, which is a treatable infantile neuromuscular disease, is essential for early diagnosis and prompt treatment in the differential diagnosis of a floppy infant.

The Reliability and Validity of the Korean Version of the Autism-Spectrum Quotient

OBJECTIVE: This study was conducted to investigate the reliability and validity of the Korean version of Autism-Spectrum Quotient (AQ). METHODS: 20 participants with high-functioning autism (HFA) and 99 normal participants were recruited. All participants were completed the AQ and Empathy Quotient (EQ), and parents of the HFA group completed the parent-report AQ. For testing the reliability, we examined Cronbach’s alpha, performed item analysis, and compared self versus parent report score of HFA participants. For testing the validity, we compared the difference of the score of AQ among HFA and control group using independent t-tests, and performed correlation analysis between AQ and EQ. The receiver operation characteristic curve analysis was performed to determine a cut-off. RESULTS: The Korean version of the AQ exhibited adequate internal consistency, and in most items, the HFA group scored higher in comparison to the control group. It was demonstrated that AQ has good discriminant validity through the confirmation of the significant difference in the AQ score between two groups. The concurrent validity was established through the significant correlation between AQ and EQ in the HFA group. The best estimate cut-off score of AQ for screening was 23. CONCLUSION: The Korean version of the AQ was determined as a reliable and valid instrument to assess HFA in Korean population.

Protective Role of Parenting Attitude on the Behavioral and Neurocognitive Development of the Children from Economically Disadvantaged Families

OBJECTIVE: Association between home environment and the behavioral and neurocognitive development of children from a community childcare center for low-income families was examined (aged 6 to 12 years, n=155). METHODS: The parents performed a questionnaire on home environment (K-HOME-Q) to assess home environment including parenting attitude and the Child Behavior Checklist (K-CBCL). The children performed the Wechsler Intelligence (IQ) Scale, Stroop interference test (Stroop), word fluency test (WF), and design fluency test (DF) to assess their neurocognitive development. RESULTS: ‘Nurturing of Development’ and ‘Variety of Language Interaction’ scores from the K-HOME-Q, were inversely associated with total behavior problems, externalization, rule-breaking, and aggressive behavior subscales of K-CBCL, and ‘Emotional atmosphere’ and ‘Tolerance toward the child’ scores showed inverse associations with the total behavior problems, rule-breaking, aggressive behavior, and withdrawn/depressed subscales. Despite economic hardship, the mean scores of the neurocognitive tests were comparable to the average level of Korean children’s normative sample. However, ‘Nurturing of Development’ and ‘Tolerance toward the Child’ score of K-HOME-Q were associated with better executive function (IQ, WF, DF). CONCLUSION: These results suggest that parental stimulation of development and tolerant parenting attitude may offer protection against the negative effects of suboptimal economic environment on children’s behavior and neurocognitive development.

Radiation Dose Reduction without Compromise to Image Quality by Alterations of Filtration and Focal Spot Size in Cerebral Angiography

OBJECTIVE: Different angiographic protocols may influence the radiation dose and image quality. In this study, we aimed to investigate the effects of filtration and focal spot size on radiation dose and image quality for diagnostic cerebral angiography using an in-vitro model and in-vivo patient groups. MATERIALS AND METHODS: Radiation dose and image quality were analyzed by varying the filtration and focal spot size on digital subtraction angiography exposure protocols (1, inherent filtration + large focus; 2, inherent + small; 3, copper + large; 4, copper + small). For the in-vitro analysis, a phantom was used for comparison of radiation dose. For the in-vivo analysis, bilateral paired injections, and patient cohort groups were compared for radiation dose and image quality. Image quality analysis was performed in terms of contrast, sharpness, noise, and overall quality. RESULTS: In the in-vitro analysis, the mean air kerma (AK) and dose area product (DAP)/frame were significantly lower with added copper filtration (protocols 3 and 4). In the in-vivo bilateral paired injections, AK and DAP/frame were significantly lower with filtration, without significant difference in image quality. The patient cohort groups with added filtration (protocols 3 and 4) showed significant reduction of total AK and DAP/patient without compromise to the image quality. Variations in focal spot size showed no significant differences in radiation dose and image quality. CONCLUSION: Addition of filtration for angiographic exposure studies can result in significant total radiation dose reduction without loss of image quality. Focal spot size does not influence radiation dose and image quality. The routine angiographic protocol should be judiciously investigated and implemented.

Prediction of Neurodevelopmental Outcome in Hypoxic Ischemic Encephalopathy at 12 Months: Correlation of Brain MRI and EEG / 대한주산의학회잡지

PURPOSE: The aims of this study were to estimate the neurodevelopmental outcome of hypoxic-ischemic encephalopathy (HIE) at 12 months, and determine the usefulness of brain magnetic resonance imaging (MRI) and electroencephalography (EEG) to predict neurodevelopmental outcome in term infants with HIE at 12 months. METHODS: This study was conducted retrospectively on term infants with HIE from January 2009 to June 2013. Based on neurodevelopmental outcome at 12 months, infants were categorized into 2 groups. Brain MRI and EEG findings were stratified into 4 categories as normal, mild, moderate and severe groups. RESULTS: Total 42 term infants were enrolled. Fifty seven point one percent (24/42) of total infants had favorable neurodevelopmental outcome at 12 months (favorable outcome, n=24). Thirty eight point one percent (16/42) of total infants had significant neurodevelopmental deficit at 12 months of age, and 4.8% (2/42) had mortality within 12 months (poor outcome, n=18). In brain MRI and EEG findings, there were significant correlations with neurodevelopmental outcome. Brain MRI showed sensitivity of 88.9%, specificity of 70.8%, positive predictive value of 69.6% and negative predictive value of 89.5%, while EEG showed sensitivity of 70.6%, specificity of 82.6%, positive predictive value of 75%, and negative predictive value of 79.2%. In the multivariate analysis, moderate-to-severe findings in brain MRI were the strongest risk factor (odds-ratio, 11.24; 95% confidence interval, 1.36-92.89; P=0.025). CONCLUSION: Forty two point nine percent of total infants had poor neurodevelopmental outcome at 12 months. Brain MRI and EEG findings were correlated with neurodevelopmental outcome of term infants with HIE at 12 months.

Predictive Factors of Postneonatal Epilepsy: The Significance of Follow-up EEG Changes / 대한소아신경학회지

PURPOSE: To describe the prevalence and severity of postneonatal epilepsy after neonatal seizures in term neonates as well as to evaluate the predictive factors of postneonatal epilepsy. METHODS: Retrospective analysis of 50 children who experienced neonatal seizures. In children with at least 12 months of follow-up data, the univariate and multivariate logistic regression analysis was applied in order to determine the predictive factors of postneonatal epilepsy. Electroencephalography (EEG), neuroimaging studies, and other clinical variables were systematically analyzed. Infants with abnormal EEG recordings in the initial studies underwent a follow-up EEG examination at 1 or 3 months later. RESULTS: Twelve of 50 neonates with neonatal seizures (24%) developed postneonatal epilepsy. Seventy-five percent (9 of 12) of the children with postneonatal epilepsy were eventually seizure-free without AED (antiepileptic drug), and 25% (3 of 12) had seizures at the last follow-up (modified angel classification 2 or 3, mean follow-up period; 52 months). On the univariate logistic regression analysis, abnormal EEG, Magnetic resonance imaging (MRI) findings, combined with encephalopathy and the number of AEDs were correlated with postneonatal epilepsy (P<0.05). On the multivariate analysis, the persistent abnormality on the follow-up EEG was correlated with postneonatal epilepsy (adjusted odds ratio=20.78; P=0.016). CONCLUSION: The number of intractable cases was relatively low, indicating good prognosis in postneonatal epilepsy. Abnormal EEG, MRI findings, combined with encephalopathy, and the number of AEDs were very good predictors of postneonatal epilepsy. The persistent abnormality in the follow-up EEG was more frequently seen in postneonatal epilepsy patients.

Risk Factors for Neurologic Complications of Hand, Foot and Mouth Disease in the Republic of Korea, 2009

In 2009, the first outbreak of hand, foot and mouth disease (HFMD) or herpangina (HP) caused by enterovirus 71 occurred in the Republic of Korea. This study inquired into risk factors associated with complications of HFMD or HP. A retrospective medical records review was conducted on HFMD or HP patients for whom etiologic viruses had been verified in 2009. One hundred sixty-eight patients were examined for this investigation. Eighty patients were without complications while 88 were accompanied by complications, and 2 had expired. Enterovirus 71 subgenotype C4a was the most prevalent in number with 67 cases (54.9%). In the univariate analysis, the disease patterns of HFMD rather than HP, fever longer than 4 days, peak body temperature over 39degrees C, vomiting, headache, neurologic signs, serum glucose over 100 mg/dL, and having an enterovirus 71 as a causative virus were significant risk factors of the complications. After multiple logistic analysis, headache (Odds ratio [OR], 10.75; P < 0.001) and neurologic signs (OR, 42.76; P < 0.001) were found to be the most significant factors. Early detection and proper management of patients with aforementioned risk factors would be necessary in order to attain a better clinical outcome.

Metastatic Hepatocellular Carcinoma to the Distal Phalanx: a Case Report and Review of Literature / 한국호스피스완화의료학회지

Metastatic tumors to the hand are rare and often overlooked by clinicians. Here, we report a rare case of phalangeal metastasis of right 4th finger in a patient with hepatocellular carcinoma. Treatment was given with right 4th finger disarticulation. This report suggests that physicians must take consideration into the possibility of metastatic tumors in patients with bone lesions on hands and a history of malignancy.

A case of Hashimoto's encephalopathy presenting with seizures and psychosis / 소아과

Hashimoto's encephalopathy (HE) is a rare, poorly understood, autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased anti-thyroid antibody levels. Here, we report a case of a 14-year-old girl with HE and briefly review the literature. The patient presented with acute mental changes and seizures, but no evidence of infectious encephalitis. In the acute stage, the seizures did not respond to conventional antiepileptic drugs, including valproic acid, phenytoin, and topiramate. The clinical course was complicated by the development of acute psychosis, including bipolar mood, insomnia, agitation, and hallucinations. The diagnosis of HE was supported by positive results for antithyroperoxidase and antithyroglobulin antibodies. Treatment with methylprednisolone was effective; her psychosis improved and the number of seizures decreased. HE is a serious but curable, condition, which might be underdiagnosed if not suspected. Anti-thyroid antibodies must be measured for the diagnosis. HE should be considered in patients with diverse neuropsychiatric manifestations.

Multimodal neuroimaging in presurgical evaluation of childhood epilepsy / 소아과

In pre-surgical evaluation of pediatric epilepsy, the combined use of multiple imaging modalities for precise localization of the epileptogenic focus is a worthwhile endeavor. Advanced neuroimaging by high field Magnetic resonance imaging (MRI), diffusion tensor images, and MR spectroscopy have the potential to identify subtle lesions. 18F-FDG positron emission tomography and single photon emission tomography provide visualization of metabolic alterations of the brain in the ictal and interictal states. These techniques may have localizing value for patients which exhibit normal MRI scans. Functional MRI is helpful for non-invasively identifying areas of eloquent cortex. These advances are improving our ability to noninvasively detect epileptogenic foci which have gone undetected in the past and whose accurate localization is crucial for a favorable outcome following surgical resection.

Neurological Manifestations and Experience with IV Immunoglobulin in Children with Enterovirus 71 Infections / 대한소아신경학회지

PURPOSE: The objective of the study was to report the neurological manifestations and response to high-dose immunoglobulin (IVIG) therapy in children with enterovirus 71 (EV 71) infections. METHODS: Eleven patients who had the primary clinical marker for enteroviral infection-herpangina or hand-foot-mouth-disease (HFMD) followed by acute neurological manifestations were admitted to Ajou University Hospital from June to September, 2009. All patients were positive for enterovirus (EV) as indicated by reverse transcription polymerase chain reaction (RT-PCR). Among them, EV 71 was confirmed by semi-nested PCR in five patients. All patients received IVIG soon after admission. RESULTS: The mean age of the patients who were EV 71 positive was 2.5 years (range, 4 months to 5.3 years). Five neurological complications associated with EV 71 infection were identified: meningoencephalitis (3 patients); acute cerebellar ataxia (1 pationt); and complex febrile seizure in another patient. Three patients (60%) had HFMD, and two(40%) had herpangina. None of the patients had neurological sequelae at follow-up. CONCLUSION: EV 71 infection should be suspected in young children with epidemic HFMD or herpangina complicated by a variety of neurological manifestations. We have reported the response to high dose IVIG therapy in children with EV 71 infection.

Clinical and Laboratorial Characteristics of Korean Children with Mitochondrial Respiratory Chain Defect / 대한소아신경학회지

PURPOSE:The study was carried out to characterized the clinical and the laboratorial features of children with mitochondrial respiratoy chain disorders in Korea. METHODS:We retrospectively analyzed the clinical and the loboratorial data of 28 children with significantly low activities in respiratory chain complexes of muscle using spectrophotometry. RESULTS:The mean age was 6.67+/-4.44 years and the ratio males to female was 1.15:1. Eighteen patients (64.3%) showed defects in Complex I, 8 (28.6%) in Complex VI, 1 (3.6%) in Complex II, and 1 in Complex I and IV. Eight cases (28.6%) were diagnosed with Leigh disease, one with MELAS, Kearns-Sayre syndrome, and Alpers disease retrospectively, but the predominant clinical presentations were a nonspecific encephalopathy (17/28, 60.7%). Epilepsy was seen in 21 (75.0%) patients, while developmental delay in 27 (96.4%) patients. Fifteen out of 28 children (53.6%), clinical symptoms mostly appeared below age of 1 year. The brain MRI showed diffuse cortical atrophy in 18 (64.3%) patients and basal ganglia signal changes in 12 (42.9%) patients. CONCLUSION:The defects in mitochondrial respiratory chain complexes should be considered in any children with an unexplained neurological condition including even epilepsy.

A case of a child with non-parasitic chyluria / 소아과

Chyluria is the passage of milky urine due to the leakage of lymph into the urinary tract. Chyluria occurs predominantly in adults and is rare in children. We present an unusual case in which a child with proteinuria, hematuria and milky urine was subsequently diagnosed with non-parasitic chyluria. Retrograde cystogram confirmed a lymphatico-calyceal communication. This case showed spontaneous remission. The etiology of this case was not exactly known; however, the prognosis of non-parasitic chyluria (or idiopathic chyluria) is usually very good and the treatment is mostly conservative.

Steroid and enalapril therapy: possible cause of toxic epidermal necrolysis / 소아과

Toxic epidermal necrolysis (TEN) is a rare, acute and life-threatening cutaneous drug reaction. TEN is characterized by the sudden onset of extensive necrosis in the epidermis and frequent mucous membrane involvement. The pathogenesis has not yet been elucidated. In addition, no particular treatment for TEN has been established. We report a case of TEN in a 14-year-old-boy, which might have been caused by steroids with enalapril treatment for membranous nephropathy. He recovered after intravenous immunoglobulin therapy.

Bronchiolitis Obliterans with Finger Clubbing after Measles Infection / 소아알레르기및호흡기학회지

A 5-year-old Korean boy was admitted with dyspnea, intermittent cyanosis and finger clubbing. He was diagnosed to be suffering measles pneumonia 3 years ago. Plain radiograph showed collapse of the right lung, mediastinal shifting and infiltration on both lower lung fields. High resolution computerized tomography revealed a central bronchiectasis with mosaic hyperinflation. All of the clinical information indicated that the severity of illness score was 4 of bronchiolitis obliterans (BO). The bronchoalveolar lavage cellular profile showed predominant neutrophilia. BO with finger clubbing has been rarely reported previously in childhood. We report a case of BO with finger clubbing in children with regard to clinical presentation of measles complicated pneumonia.

Bronchoalveolar Cellularity and IL-8 Levels in Patients with Post-measles Wheezing

PURPOSE: This study was conducted to investigate the pulmonary cellular profiles and IL-8 levels in patients with post-measles wheezing. METHODS: Twelve previously healthy infants with a minimum of three episodes of wheezing after measles pneumonia(Measles wheezing, median age, 1.3 years) were recruited by a retrospective examination of hospital records. They underwent bronchoalveolar lavage(BAL) with flexible bronchoscopy, and high-resolution computed tomography(HRCT) with a mean six(1-15) months interval. Comparisons were made with seven normal controls(Control, median age: 7.4 years). BAL cell counts and differentials were determined. IL-8 levels also were measured by ELISA. RESULTS: The BAL cellular profiles were characterized by a significantly increased percentage of neutrophils in the Measles wheezing group(median 16.0%) compared to the control group(median 3.8%)(P<0.01). IL-8 levels were markedly increased in the Measles wheezing group(mean+/-SD, 512.7+/-324.0 pg/mL) compared to the control group(41.7+/-67.7 pg/mL)(P<0.01). Furthermore, IL-8 levels correlated significantly(r=0.816, P=0.001) with neutrophil percentages in BAL fluids in the Measles wheezing group. Abnormal HRCT findings were mosaic perfusion, bronchiectasis, bronchial wall thickening, and decreased vascularity. CONCLUSION: These results suggest that pulmonary neutrophils and IL-8 may play an important role in the pathogenesis of the post-measles wheezing.

Urinary Excretion of Various Urinary Proteins in Children with Vesicoureteral Reflux

PURPOSE: This study aimed to examine the excretion of various urinary proteins in children with a history of urinary tract infection(UTI), with or without vesicoureteral reflux(VUR) or reflux nephropathy, and to identify means of predicting the severity of VUR or the presence of reflux nephropathy as indicated by these markers, and to know how these markers are changed after resolution of VUR. METHODS: We studied 30 children with previous UTI, without VUR and renal scarring(group I), 12 children with VUR, without evidence of renal scarring(group II), and 34 children with VUR and renal scarring(group III). 24-hour or 12-hour urine beta2 microglobulin(beta2 MG), microalbumin and N- acetyl-beta-D-glucosaminidase(NAG) were measured in each child. Urinary protein excretions were analyzed according to the degree of VUR(mild VUR : a grade reflux I-III, severe VUR : a grade reflux IV-V). Cases of bilateral VUR were graded by the higher grade of reflux detected. A total of 46 children with primary VUR were followed. Among these patients, VUR was completely resolved in 16 children. Voiding cystourethrography(VCUG) and DMSA scan were performed every year. Values for urinary markers were estimated every year. RESULTS: 24 or 12 hour urine microalbumin and NAG excretions were significantly increased in group III compared to group I(microalbumin : 27.7+/-26.0 mg/gCr vs 15.0+/-10.7 mg/gCr, P<0.05, NAG : 15.2+/-18.7 U/gCr vs 3.4+/-2.2 U/gCr, P<0.05). Urinary beta2 MG excretions were not significantly different between groups. Urinary NAG excretions were elevated in the group of children with severe VUR compared to mild VUR(26.8+/-27.1 U/gCr vs 7.6+/-3.8 U/gCr, P<0.05). After resolution of VUR, urinary microalbumin and NAG excretions were decreased(P<0.05). CONCLUSION: Urinary microalbumin and NAG may be useful clinical indicators to predict the presence of reflux nephropathy and the resolution of VUR. Especially, urinary NAG excretions may be used as a possible method to predict the severity of VUR.

The Cases of Surgical Treatment of Intractable Childhood Epilepsy with Tuberous Sclerosis / 대한소아신경학회지

Tuberous sclerosis(TS) is a disorder of aberrant neuronal differentiation and proliferation manifesting as multiple central nervous system(CNS) hamartomas. Seizures in patients with TS are often intractable to anticonvulsant therapy. Although TS shows various lesions including multiple tubers, many patients with well localized epileptic focus to some tubers may have a benefit from epileptic surgery. We experienced three cases who had clinical improvement after epileptic surgery in intractable epilepsy complicated by TS. We report three cases and the brief review of literatures.