Listeria meningitis with rapid progression of hydrocephalus: a case of a 16-month-old girl with febrile status epilepticus as the first symptom

Listeria monocytogenes is a rare cause of bacterial meningitis, particularly beyond the neonatal period. This article describes a 16-month-old girl who presented with a new-onset febrile status epilepticus, and subsequently developed altered mentality and lethargy. L. monocytogenes was detected on a point-of-care, multiplex polymerase chain reaction using the cerebrospinal fluid. On day 3, she developed a rapidly progressive hydrocephalus. Her consciousness improved after placement of an external ventricular drain. After 3-week antibiotic therapy, she was successfully discharged without residual complications.

Treatment Outcomes of Vagus Nerve Stimulation in Lennox-Gastaut Syndrome / 대한소아신경학회지

PURPOSE@#Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies and frequently patients with this syndrome respond poorly to antiepileptic drugs. The aim of this study was to evaluate the treatment outcomes of vagus nerve stimulation (VNS) in LGS patients with drug-resistant epilepsy.@*METHODS@#We analyzed the clinical files, collected over 5 years, of children with LGS who received VNS treatment.@*RESULTS@#Seven children were included in this study (four males, three females; mean age of VNS insertion 12.4±3.5 years). All the patients had generalized tonic seizures and there were various other seizure types including myoclonic seizures, atonic seizures, and atypical absence seizures. Although two patients had normal imaging, five patients had abnormalities on imaging, including pachygyria, cortical dysplasia, kernicterus, and a chromosomal anomaly. Comparing the baseline seizure frequency to the frequency after the VNS surgery, the seizure frequency at the last follow-up showed a decrease of 57.2% (0% to 100%) on average (P=0.028) and one patient achieved seizure free status. Only two children were given additional antiepileptic drugs with the aim of managing their seizures. There was no mortality or complications related to the VNS therapy except one case requiring intensive care unit admission due to pneumonia. Comparing the results before and after VNS surgery, the VNS therapy also had a tendency to have a positive effect on quality of life (P=0.066).@*CONCLUSION@#In LGS patients with drug resistant epilepsy who are not candidates for a corpus callosotomy or resective surgery, VNS could be an effective, low-risk adjunct therapy for decreasing seizure frequency.

Applications and Outcomes of Epilepsy Surgery in Pediatric Population / 대한소아신경학회지

Epilepsy is one of the world's most common neurologic disorders and epilepsy surgery is a major treatment option for intractable epilepsy in pediatric patients. Understanding the sequential steps of epilepsy surgery is important for the selection of appropriate candidate for surgery. Surgical methods in epilepsy surgery should be decided according to the results of the presurgical evaluation and they can be categorized into resective surgery and disconnective surgery. The implantation of neuromodulation is also a potential surgical approach. Here, we reviewed the current state of presurgical assessment for intractable epilepsy in pediatric patients, novel surgical procedures, and associated outcome data.

Tuberous Sclerosis Complex with Crohn's Disease / 대한소아신경학회지

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutation of one of two genes, TSC1 (encoding hamartin, 9q34) and TSC2 (encoding tuberin, 16p13). It invades the central nervous system and various parts of the body, causing various symptoms. Crohn's disease (CD) is a chronic immune-mediated disease that has not been clearly elucidated. It is thought to be caused by an excessive immune response of the body to bacteria that normally exist in the digestive tract with genetic factors. No cases have been reported in which both of the above-mentioned diseases occurred simultaneously. We report a case of CD in a patient with TSC. A 12-year-old boy was brought to our hospital because of abdominal pain. Skin lesions were observed in the TSC. Fundus examination revealed a hamartoma in the right retina. Brain magnetic resonance imaging revealed a subendothelial giant cell astrocytoma (SEGA). On the basis of these findings, he was diagnosed as having TSC. Blood test results showed increased levels of inflammatory markers. On abdominal ultrasonography, his colon walls were observed to be thickened with increased vascularity of the proximal ascending colon, ileocecal valve, and terminal ileum. Colonoscopy revealed discontinuous ulcerations and inflammations of the ileum, IC valve, and cecum, similar to those found in CD. Everolimus was administered orally for the SEGA but was discontinued frequently owing to the exacerbation of CD. The possibility of CD should be kept in mind in patients with TSC considering to undergo treatment for SEGA.

Effect of Media Exposure to Language Development / 대한소아신경학회지

PURPOSE: This study was performed in order to evaluate the association of media exposure with language developmental delay. METHODS: The sample consisted of 40 patients with language developmental delay who visited the pediatric clinic of Dongtan Sacred Heart Hospital from January 2013 to July 2014. The 66 patients, who visited our clinic without language developmental delay, were included in the control group. The data were collected by using self-report questionnaires (media exposure time, contents, background media or foreground media, age of first exposure, and media exposure with or without parents), and analyzed through a t-test, Chi-square test, bivariate logistic regression model by using the SPSS-Version 21.0. RESULTS: The mean age of the language delay group was 33.6±10 months, while the male-to-female ratio was 2.6:1 in this study. In regard to media exposure time, 63% of the language delay patients were exposed to media for more than 2 hours a day, as compared to 16% of the control group (P<0.001). Among the language delay group, 90% of the patients were under 24 months old at the time of exposure to media, as compared to 58% of the control group (P<0.001). In addition, 79% of the language delay group watched media without anyone, as compared to 41% of the control group (P=0.001). CONCLUSION: Risk factors of language developmental delay were exposure to media more than 2 hours a day and toddlers under 24 months old at the time of exposure, as compared to the control group. In conclusion, longer exposure and earlier exposure to media would be risk factors in language developmental delay, and watching media alone may negatively influence the language development.

Effect of Media Exposure to Language Development / 대한소아신경학회지

PURPOSE: This study was performed in order to evaluate the association of media exposure with language developmental delay. METHODS: The sample consisted of 40 patients with language developmental delay who visited the pediatric clinic of Dongtan Sacred Heart Hospital from January 2013 to July 2014. The 66 patients, who visited our clinic without language developmental delay, were included in the control group. The data were collected by using self-report questionnaires (media exposure time, contents, background media or foreground media, age of first exposure, and media exposure with or without parents), and analyzed through a t-test, Chi-square test, bivariate logistic regression model by using the SPSS-Version 21.0. RESULTS: The mean age of the language delay group was 33.6±10 months, while the male-to-female ratio was 2.6:1 in this study. In regard to media exposure time, 63% of the language delay patients were exposed to media for more than 2 hours a day, as compared to 16% of the control group (P<0.001). Among the language delay group, 90% of the patients were under 24 months old at the time of exposure to media, as compared to 58% of the control group (P<0.001). In addition, 79% of the language delay group watched media without anyone, as compared to 41% of the control group (P=0.001). CONCLUSION: Risk factors of language developmental delay were exposure to media more than 2 hours a day and toddlers under 24 months old at the time of exposure, as compared to the control group. In conclusion, longer exposure and earlier exposure to media would be risk factors in language developmental delay, and watching media alone may negatively influence the language development.

Behavioral and intelligence outcome in 8- to 16-year-old born small for gestational age / 소아과

PURPOSE: We investigated behavioral problems, attention problems, and cognitive function in children and adolescents born small for gestational age (SGA). METHODS: Forty-six SGA children born at term and 46 appropriate for gestational age (AGA) children born at term were compared. Psychiatric symptoms were examined with reference to the Korean-Child Behavior Checklist, Korean-Youth Self Report, and Attention Deficit Hyperactivity Disorder Rating Scale (ADHD-RS). Cognitive function was estimated using the Wechsler Intelligence Scale. Sociodemographic data were recorded from interviews. RESULTS: SGA children had high scores on delinquent behavior, aggressive behavior, and the externalizing scale, and they also showed a propensity for anxiety and depression. The SGA group had a higher mean ADHD-RS score than the AGA group (10.52±8.10 vs.9.93±7.23), but the difference was not significant. The SGA group had a significantly lower verbal intelligence quotient (IQ) than the AGA group, but the mean scores of both groups were within normal limits. CONCLUSION: This study indicates marked behavioral problems, such as delinquency, aggressiveness, and anxiety and depression, as well as low verbal IQ in the SGA group than in the AGA group. Even in cases in which these symptoms are not severe, early detection and proper treatment can help these children adapt to society.

Plasma secreted phospholipase A2 in asthmatic children: correlation with leptin levels and exercise induced bronchoconstriction

PURPOSE: Dysregulated cysteinyl leukotriene (CysLT) synthesis is prominent in exercise-induced bronchoconstriction (EIB). Secreted phospholipase A2 (sPLA2) plays a key regulatory role in the biosynthesis of CysLTs. We previously found that serum leptin levels correlate with (EIB) in children with asthma. The aim of this study was to address the relationship between plasma sPLA2/leptin levels and EIB. METHODS: Sixty-seven prepubertal children between the ages of 6 and 10 years were included in the study. They were asthmatics with EIB (n=25), asthmatics without EIB (n=21), and healthy subjects (n=21). We measured the plasma sPLA2 and leptin levels. We also performed pulmonary function tests at baseline, after bronchodilator inhalation, and after exercise. RESULTS: The sPLA2 and leptin levels were significantly higher in asthmatics with EIB than in those without and control subjects. In addition, sPLA2 levels were significantly correlated with body mass index (Speraman correlation coefficient r=0.343, P=0.023) and leptin levels (partial correlation coefficient r=318, P=0.033). The maximum decrease in % forced expiratory volume in 1 second after exercise was significantly correlated with both PLA2 levels (r=0.301, P=0.041) and leptin levels (r=0.346, P=0.018). CONCLUSION: The sPLA2 and leptin levels were significantly higher in asthmatics with EIB than in asthmatics without EIB and control subjects. In addition, sPLA2 levels were significantly correlated with leptin levels and EIB in asthmatic children.

Screening for depression and anxiety disorder in children with headache / 소아과

PURPOSE: The purpose of this study was to investigate the importance of initial screening tests for depression and anxiety disorders in children with headache. In addition, this study evaluated whether the Children's Depression Inventory (CDI) and Revised Children's Manifest Anxiety Scale (RCMAS) are suitable for screening symptoms of depression and anxiety. METHODS: A retrospective chart review was conducted of 720 children aged 7-17 years who had visited a pediatric neurology clinic for headaches and were referred to a pediatric psychiatric clinic for psychiatric symptoms from January 2010 to December 2011. All patients completed the CDI and RCMAS. Among them, charts of patients with clinically significant total scores (cutoff>15) for psychiatric symptoms, as defined by the CDI and RCMAS scoring scales, were reviewed. RESULTS: Nineteen patients had headaches and clinically significant total scores for psychiatric symptoms. The mean age at headache diagnosis was 11.7 years, and 57% were male. Mean duration of headache was 11.5 months. Two point eight percent of the patients were diagnosed with psychiatric disorders including major depression (1.7%), generalized anxiety disorder (1.1%), and bipolar disorder (0.1%). Four patients (0.6%) were diagnosed with attention deficit/hyperactivity disorder (ADHD). Total mean CDI and RCMAS scores of patients referred to the psychiatric clinic were 18.8 and 22.2, respectively. There was no correlation between CDI or RCMAS total scores and headache frequency, duration, or severity. CONCLUSION: We recommend that all patients with headache should be screened for depression and anxiety by CDI and RCMAS scores.

Component-resolved diagnosis using microarray for diagnosing hypersensitivity to raw fruits in birch pollen sensitized children

PURPOSE: Recently, component-resolved diagnosis (CRD) using microarray technology has been introduced to the field of clinical allergy. This study was aimed to investigate the clinical usefulness of microarray-based IgE detection for diagnosing clinical raw fruit allergy in birch pollen-sensitized children. METHODS: Thirty-one children with allergic disease who had been sensitized to pollen were studied. A pollen-sensitized patient was defined as having an allergen-specific history with concomitant positive skin-prick tests (SPTs) to natural allergen extracts or positive allergen-specific IgE. All subjects underwent SPTs for pollen and fruit. In all subjects, specific IgE to pollen and fruit were measured by ImmunoCAP. Specific IgE antibodies to allergen components were determined by a customized allergen microarray (ISAC). RESULTS: Thirteen of the 31 patients (41.9%) had a history of fruit hypersensitivity with positive SPTs. Measuring IgE to allergen components by ISAC, all the 13 patients with fruit hypersensitivity were positive to at least one of Mal d 1, Pru p 1, Pru p 3, Act d 8, and Act d 2 compared to 12 of the 13 patients (92.3%) who had at least 1 positive IgE to fruits (apple, peach, and kiwi) using ImmunoCAP. The sensitivity of ISAC microarray was 100.0% for the diagnosis of fruit hypersensitivity, but its specificity was 27.7% (5/18). The sensitivity of ImmunoCAP was 92.3%, and its specificity was 83.3%. CONCLUSION: The sensitivity of allergen components tested using microarray for the diagnosis of clinical fruit hypersensitivity in children with pollen allergy was high; however, its specificity was low.

Wernicke's encephalopathy in a child with high dose thiamine therapy / 소아과

Wernicke's encephalopathy is an acute neurological disorder characterized by mental confusion, oculomotor dysfunction, and ataxia. It has been reported in individuals with alcohol dependence, hyperemesis gravidarum, and prolonged parenteral nutrition without vitamin supplementation. Here we present the case of a 13-year-old male patient with neuroblastoma and a history of poor oral intake and nausea for 3 months. After admission, he showed gait disturbances, nystagmus, and excessive dizziness; his mental state, however, indicated he was alert, which did not fit the classical triad of Wernicke's encephalopathy. A diagnosis of Wernicke's encephalopathy was made only after brain magnetic resonance imaging and serum thiamine level analyses were performed. The patient's symptoms remained after 5 days of treatment with 100-mg thiamine once daily; thus, we increased the dosage to 500 mg 3 times daily, 1,500 mg per day. His symptoms then improved after 20 days of replacement therapy. This case report describes a pediatric patient who was promptly diagnosed with Wernicke's encephalopathy, despite only 2 suspicious symptoms, and who completely recovered after high doses of thiamine were given intravenously.

Psychological Problems and Clinical Outcomes of Children with Psychogenic Non-Epileptic Seizures

PURPOSE: Our purpose was to investigate psychological problems and clinical outcomes in children with psychogenic non-epileptic seizures (PNES). MATERIALS AND METHODS: We retrospectively reviewed the data of 25 patients who were diagnosed with PNES between 2006 and 2012. RESULTS: Twenty-five children with PNES, aged 8 to 19 years (mean 13.82), were referred to psychiatrists for psychiatric assessment. On their initial visit, 72% of patients had comorbid psychological problems, including depression, anxiety, conduct disorder, adjustment disorder, Attention Deficit Hyperactivity Disorder, schizophrenia, and bipolar disorder. Among these, depression was the most frequent (36%). Predisposing and triggering factors included familial distress (40%), social distress (24%), and specific events (20%). The following treatment was advised based on the results of the initial psychological assessment: 3 patients regularly visited psychiatric clinic to assess their clinical status without treatment, nine underwent psychotherapy, and 13 received a combination of psychotherapy and psychopharmacological therapy. At the mean follow-up of 31.5 months after diagnosis, 20 patients (80%) were event-free at follow-up, three (12%) showed reduced frequency, and two (8%) experienced persistent symptoms. CONCLUSION: The outcomes of PNES in children are much better than those in adults, despite a high rate of psychological comorbidities.

Tumor Lysis Syndrome in a Child with Osteosarcoma / 임상소아혈액종양

Tumor lysis syndrome (TLS) is a metabolic derangement that may be seen after the initial treatment of cancer and usually occurs in patients with bulky, rapidly proliferating, or treatment-responsive tumors such as acute leukemia or Burkitt lymphoma. However, it is rare in the treatment of the solid tumor, especially osteosarcoma. An 11-year-old girl was admitted with a 3-months history of severe right leg pain. She was diagnosed as osteosarcoma and was treated with cisplatin and adriamycin. Three days after treatment, the values of potassium, phosphorus, uric acid were 6.4 mEq/L, 5.6 mg/dL and 8.9 mg/dL, respectively. At the same time, the value of calcium was 6.2 mg/dL. Although she was treated with proper conservative treatment including dialysis, she had significant complications such as renal failure, heart failure, hypoxic brain damage. We report a case of TLS in a girl with huge osteosarcoma.

Relationship between Catch-up Growth and Changes in Insulin, Leptin, IGF-I, Adiponectin, and Ghrelin in Infants Born Full-term Small for Gestational Age / 대한소아내분비학회지

PURPOSE: The aim of the study was to investigate postnatal changes in growth and insulin, leptin, IGF-I, adiponectin, and ghrelin, and to examine the relationship of these hormones with catch-up growth in full-term small for gestational age (SGA) infants. METHODS: SGA was defined as a birth weight less than the ten percentile. Weight and height were measured at birth, 1 month, 6 months, 1 year, and 2 years of age in 70 SGA infants (40 females and 30 males). The infants were subdivided according to their weight and height catch-up growth (CUG) at 2 years old. CUG is defined as reaching a standard deviation score (SDS) of > -2 SDS. Blood samples were serially taken for insulin, leptin, insulinlike growth factor (IGF)-I, adiponectin, and ghrelin. RESULTS: 1) Dramatic CUG for weight and height occurred during the first year of life; weight and height growth gain decreased thereafter. 2) Non-catch-up growth (NCUG) infants showed more decreased weight and height growth gain than CUG infants between the first and second year of life. 3) Weight CUG was 77.1% and height CUG was 75.8% in the SGA infants. 4) Weight CUG infants showed significantly higher leptin and ghrelin levels than in weight NCUG infants at the age of 1 year (P <0.05). 5) Height CUG infants showed significantly higher leptin and ghrelin levels than in height NCUG infants at the age of 1 year (P <0.05). CONCLUSION: CUG for weight and height occurred during the first year of life and growth velocity decreased thereafter. Significant corresponding changes occurred with regard to serum leptin and ghrelin.

Tumor Lysis Syndrome in a Child with Osteosarcoma / 임상소아혈액종양

Tumor lysis syndrome (TLS) is a metabolic derangement that may be seen after the initial treatment of cancer and usually occurs in patients with bulky, rapidly proliferating, or treatment-responsive tumors such as acute leukemia or Burkitt lymphoma. However, it is rare in the treatment of the solid tumor, especially osteosarcoma. An 11-year-old girl was admitted with a 3-months history of severe right leg pain. She was diagnosed as osteosarcoma and was treated with cisplatin and adriamycin. Three days after treatment, the values of potassium, phosphorus, uric acid were 6.4 mEq/L, 5.6 mg/dL and 8.9 mg/dL, respectively. At the same time, the value of calcium was 6.2 mg/dL. Although she was treated with proper conservative treatment including dialysis, she had significant complications such as renal failure, heart failure, hypoxic brain damage. We report a case of TLS in a girl with huge osteosarcoma.

Testicular Volume in Korean Boys / 대한소아내분비학회지

PURPOSE: Measurement of testicular volume is useful for the evaluation of puberty and testicular development in boys. Four mililiter of testicular volume means Tanner stage 2, the beginning of puberty, and its ethnic differences were reported. However, there is no reference value for Korean children. We tried to establish the standard testicular volume for Korean boys. METHODS: The subjects were selected among the children who visited the out-patient clinic of Kangdong Sacred Heart Hospital and Korea Cancer Center Hospital for the evaluation of growth from 2003 to 2008. Three hundred seventy two healthy Korean boys, 3 to 17 years of age, were included. We excluded the patients who were diagnosed as precocious puberty, delayed puberty, hypopituitarism, adrenal disorder and other endocrinologic disorder. The testicular volume was determined with Prader orchidometer. Clinical data were collected from patient's chart retrospectively. RESULTS: Right and left testicular volumes were 3.65 +/- 1.82 mL and 3.55 +/- 1.80 mL at 10 (10.0 - 10.9) year of age (n = 53), 6.17 +/- 3.60 mL and 6.13 +/- 3.69 mL at 11 (11.0 - 11.9) year of age (n = 52), respectively. There was no significant difference between right and left testicular volumes. CONCLUSION: The time when testicular volume reached 4 mL seemed to be not changed than previous Korean data. However, further study will be required to determine the secular trend of pubertal timing in Korean boys.