A case of Menkes disease caused by novel mutation in the ATP7A gene with infantile hypertrophic pyloric stenosis / 대한소아신경학회지

Menkes disease is caused by mutations in the ATP7A gene that lead to intracellular copper transport defects and characterized by brownish twisted (kinky) hair accompanied by growth retardation and intellectual disability. Reduced nitric oxide (NO) production contributes to infantile hypertrophic pyloric stenosis (IHPS) because NO plays an important role in smooth muscle relaxation. Here we describe a case of Menkes disease and IHPS in a 72-day-old male patient with severe persistent vomiting and convulsions with a novel ATP7A mutation.

Sleep Disturbances in Children Younger than 36 Months of Age with Iron Deficiency Anemia / 대한소아신경학회지

PURPOSE: Iron deficiency anemia (IDA) and sleep problems are prevalent in infancy and early childhood and are more associated with poor cognitive, motor, and social-emotional development. The aim of this study was to access the relationship between IDA and sleep disorders in a population of Korean children <36 months. METHODS: One hundred and ninety six children, who visited the outpatient clinic for a routine check-up were consecutively enrolled from March 2011 to March 2012. All parents answered a questionnaire about sleep patterns of their children using a modified expanded version of the Brief Infant Sleep Questionnaire. Among the subjects, 93 children with strong evidence of sleep disordered breathing were excluded. Hundred three children were then divided into the IDA group (n=23) and the control group (n=80). Sleep-wake parameters and prevalence of sleep disturbances were compared between the two groups. A multivariate analysis was performed to determine the independent risk factors for sleep disturbances in children. RESULTS: Children with IDA had more frequent nocturnal waking, restless sleep, and inconsolable crying during sleep than those in the control. Children with IDA also had more inappropriate sleep onset associations. No difference in sleep-wake parameters was observed between the two groups. The presence of IDA in children and maternal anemia were significant independent risk factors for sleep disturbances in children <36 months. CONCLUSION: The results suggest that prevention, early detection, and treatment of IDA would be important for good sleep in young children <36 months.

Impact of Sleep Duration on Emotional Status in Adolescents / 대한소아신경학회지

PURPOSE: South Korean adolescents have been identified as a chronic partial sleep-deprived population in several previous studies. This study was to identify the current nocturnal sleep duration by age in South Korean adolescents and to analyze the association of emotional status factors such as subjective happiness, depression and suicidal attempts, and health-risk behaviors with the nocturnal sleep duration. METHODS: The findings in this study are based on the data obtained from the 7th Korea Youth Risk Behavior Web-Based Survey (KYRBWS-VII), a cross-sectional, annual survey of the health-risk behaviors in a representative sample of South Korean middle- and high-school students aged 13-18 years, which was conducted in 2011. Out of 75,643 students from 800 schools across the nation, 75,205 students were selected by using the complex sampling design of the survey. We analyzed the relationships between the duration of nocturnal sleep and emotional status (subjective happiness, stress level, depression, and suicidal thoughts), health-risk behaviors (smoking, drinking), and sleep satisfaction. RESULTS: There was a significant relationship between the duration of sleep and the emotional status. In general, happy students had the longest nocturnal sleep duration, regardless of their grades. Self-rated stress levels and depression in adolescents were inversely proportional to nocturnal sleep duration. Health-risk behaviors such as smoking and alcohol consumptions were more common in sleep-deprived students (P<0.001). CONCLUSION: Appropriate nocturnal sleep duration is critical for emotional health as well as prevention of suicide in the adolescent population.

Depression and marital intimacy level in parents of infants with sleep onset association disorder: a preliminary study on the effect of sleep education / 소아과

PURPOSE: Sleep onset association disorder (SOAD) is a form of behavioral insomnia observed in children that is caused by inappropriate sleep training. SOAD typically disturbs the sleep of not only infants and children but also their parents. We investigated levels of depression and marital intimacy among parents of infants with typical SOAD, to understand the influence of SOAD on family dynamics, as well as examine ways for improving depression and marital intimacy through behavioral training. METHODS: Depression and marital intimacy were assessed using the Beck Depression Inventory (BDI) and Waring Intimacy Questionnaire (WIQ). These measures were administered to 65 parents of infants (n=50) diagnosed with SOAD. We conducted sleep education and behavioral training for the parents and compared levels of depression and marital intimacy after 2-6 weeks of training. RESULTS: The 65 parents consisted of 50 mothers and 15 fathers. Depressive symptoms were higher among mothers than fathers (P =0.007). Marital intimacy was negatively correlated with depressive symptoms. Twenty-six parents were assessed again after sleep training. We found that mothers' depressive symptoms and marital intimacy improved post training. CONCLUSION: SOAD can be detrimental to both infants and parents, especially for parents who sleep with their infants. For instance, disruption of sleep patterns in such parents can reduce marital intimacy. However, behavioral modification is an effective treatment for infants with frequent nighttime waking, as well as for diminishing the depressive symptoms of sleep-deprived parents.

A Case of Combined Acute Disseminated Encephalomyelitis and Acute Flaccid Paralysis associated with Enteroviral Infection / 대한소아신경학회지

Enterovirus infection is often aggravated and manifests as various neurological complications such as aseptic meningitis, brainstem encephalitis, poliomyelitis-like acute flaccid paralysis, transverse myelitis, and severe systemic diseases. There are a few reports indicating that enterovirus is associated with acute disseminated encephalomyelitis (ADEM), acute flaccid paralysis, or Guillain-Barre syndrome separately. However, none of these report ADEM and acute flaccid paralysis occurring simultaneously. Here, we present a case of combined ADEM and acute flaccid paralysis associated with enteroviral infection in a child and reviewed the relevant literature. A 5-year-old boy was admitted to the hospital for headache and vomiting with evident meningeal irritation signs. Despite of antibiotic therapy, his mental state rapidly declined to coma with abnormal upper motor neuron (UMN) signs. During 5 consecutive days of steroid pulse therapy, his abnormal UMN signs abruptly changed to flaccid paralysis. Suspected acute flaccid paralysis due to enterovirus prompted the addition of intravenous immunoglobulin (IVIG). On hospital day 33, he was discharged with full recovery of muscle tone and strength with an alert mental state.

Sleep patterns and school performance of Korean adolescents assessed using a Korean version of the pediatric daytime sleepiness scale / 소아과

PURPOSE: Korean adolescents have severe nighttime sleep deprivation and daytime sleepiness because of their competitive educational environment. However, daytime sleep patterns and sleepiness have never been studied using age-specific methods, such as the pediatric daytime sleepiness scale (PDSS). We surveyed the daytime sleepiness of Korean adolescents using a Korean translation of the PDSS. METHODS: We distributed the 27-item questionnaire, including the PDSS and questions related to sleep pattern, sleep satisfaction, and emotional state, to 3,370 students in grades 5-12. RESULTS: The amount of nighttime sleep decreased significantly with increasing age. During weekday nights, 5-6th graders slept for 7.95+/-1.05 h, 7-9th graders for 7.57+/-1.05 h, and 10-12th graders for 5.78+/-1.13 h. However, the total amounts of combined daytime and nighttime sleep during weekdays were somewhat greater, 8.15+/-1.12 h for 5-6th graders, 8.17+/-1.20 h for 7-9th graders, and 6.87+/-1.40 h for 10-12th graders. PDSS scores increased with age, 11.89+/-5.56 for 5-6th graders, 16.57+/-5.57 for 7-9th graders, and 17.71+/-5.24 for 10-12th graders. Higher PDSS scores were positively correlated with poor school performance and emotional instability. CONCLUSION: Korean teenagers sleep to an unusual extent during the day because of nighttime sleep deprivation. This negatively affects school performance and emotional stability. A Korean translation of the PDSS was effective in evaluating the severity of daytime sleepiness and assessing the emotional state and school performance of Korean teenagers.

Obstructive sleep apnea syndrome in children: Epidemiology, pathophysiology, diagnosis and sequelae / 소아과

The prevalence of pediatric obstructive sleep apnea syndrome (OSAS) is approximately 3% in children. Adenotonsillar hypertrophy is the most common cause of OSAS in children, and obesity, hypotonic neuromuscular diseases, and craniofacial anomalies are other major risk factors. Snoring is the most common presenting complaint in children with OSAS, but the clinical presentation varies according to age. Agitated sleep with frequent postural changes, excessive sweating, or abnormal sleep positions such as hyperextension of neck or abnormal prone position may suggest a sleep-disordered breathing. Night terror, sleepwalking, and enuresis are frequently associated, during slow-wave sleep, with sleep-disordered breathing. Excessive daytime sleepiness becomes apparent in older children, whereas hyperactivity or inattention is usually predominant in younger children. Morning headache and poor appetite may also be present. As the cortical arousal threshold is higher in children, arousals are not easily developed and their sleep architectures are usually more conserved than those of adults. Untreated OSAS in children may result in various problems such as cognitive deficits, attention deficit/hyperactivity disorder, poor academic achievement, and emotional instability. Mild pulmonary hypertension is not uncommon. Rarely, cardiovascular complications such as cor pulmonale, heart failure, and systemic hypertension may develop in untreated cases. Failure to thrive and delayed development are serious problems in younger children with OSAS. Diagnosis of pediatric OSAS should be based on snoring, relevant history of sleep disruption, findings of any narrow or collapsible portions of upper airway, and confirmed by polysomnography. Early diagnosis of pediatric OSAS is critical to prevent complications with appropriate interventions.

Sleep Problems in Children and Adolescents with Epilepsy and Chronic Headaches / 대한소아신경학회지

PURPOSE: Sleep is correlated with various neurological disorders. Epilepsy and chronic headaches(CH) are the most common causes of visiting children in neurology clinics. The aim of the present study is to evaluate the sleeping patterns of patients with epilepsy and CH. METHODS: Children and adolescents of between the ages 6-18 suffering from epilepsy or CH were assessed using the sleep questionnaires. Patients with motor disabilities, chromosomal abnormalities or neuromuscular diseases were excluded. RESULTS: Sleeping patterns of the 276 patients were analyzed(200 epilepsy and 76 CH). The mean age of the patients was 11.8+/-3.3 years. CH patients had more excessive daytime sleepiness, shorter total sleep time on weekday, later bedtime, and more trouble getting up in the morning than epilepsy patients(P<0.001). CH patients also had more mood instabilities, resistance to going to bed on time, and uncomfortable feelings in legs than epilepsy patients(P<0.05). On the contrary, epilepsy patients had more bed-wettings than CH patients(P<0.05). CONCLUSION: It is suggested that CH is more associated with sleep problems than epilepsy. The consideration and proper treatment for accompanying sleep problems are needed when we treat chronic headaches or epilepsy.

Pediatric Narcolepsy: Diagnosis and Treatment / 대한소아신경학회지

Narcolepsy is chronic devastating disease that characterized by excessive daytime sleepiness, cataplexy, which often precipitated by intense emotion or excitement, hypnagogic, or hypnapompic hallucinations, sleep paralysis and nocturnal disrupted sleep. In child onset narcolepsy, the presentations of narcolepsy can be very variable, making misdiagnosis as seizure disorders or delaying diagnosis as much as several years after disease onset. For the diagnosis of narcolepsy, overnight polysomnography(PSG) and multiple sleep latency test(MSLT) should be evaluated. Test for Cerebrospinal fluid hypocretin(orexin) concentration and human leukocyte antigens(HLA) would be great helpful to confirm the narcolepsy with cataplexy even in early stage of disease in children. The mainstays of treatment are that reducing the excessive daytime sleepiness, preventing the intrusion of the REM related phenomena including cataplexy and consolidating the nighttime sleep. Central nervous system stimulators such as methylphenidate or amphetamine decrease excessive daytime sleepiness and tricyclic antidepressant(TCA) or selective serotonin reuptake inhibitors(SSRI) can prevent cataplexy. Recently, new therapeutic agents such as modafinil and sodium oxybate are emerging in clinical practice with much effectiveness. Counseling for poor school performance, social isolation and depression should be provided. Early diagnosis and treatment can greatly improve the quality of life. Awareness of excessive daytime sleepiness in children or adolescent will allow pediatricians to effectively identify hypersomnia such as narcolepsy.

A pilot study of neuroprotection with umbilical cord blood cell transplantation for preterm very low birth weight infants / 소아과

PURPOSE: Preterm very low birth weight infant have high rate of adverse neurodevelopmental sequale. Recently, there have been lots of reports that human umbilical cord blood transplantation ameliorates functional deficits in animal models as hypoxic ischemic injury. This pilot study was undertaken to determine the clinical efficacy and safety of autologous umbilical cord blood cell transplantation for preventing neurodevelopmental sequale in perterm VLBW. METHODS: Subjects were 26 preterm infants whose birth weight are less than 1,500 g and delivered under the intrauterine period 34 weeks. Autologous umbilical mononuclear cells (about 5.87x10(7)/kg) were injected to neonate via the umbilical vein on the postnatal 24-48 hour. The therapeutic efficacy was assessed by numbers of nucleated RBC, urinary uric acid/creatinine ratio, concentration of neuron specific enolase (NSE), interleukin 6 (IL6), interleukin-1beta (IL-1beta), and glial cell derived neurotrophic factor (GDNF) in serum and cerebrospinal fluid on day 1 and 7. RESULTS: There were no significant differences in the numbers of the nucleated RBC, urinary uric acid/creatinine ratio, concentration of creatine kinase between the transplanted infants and controls. But the nucleated RBC is more likely to be rapidly discharged in the transplanted group. In the transplanted group, the concentrations of IL6, IL-1beta, and GDNF were no significant difference between day 1 and 7, although GDNF seemed to be elevated. Serum NSE concentration was significantly elevated after transplantation, but not in CSF. CONCLUSION: It is suggested that autologous umbilical cord blood transplantation in preterm very low birth weight infant is safe to apply clinical practice. Long term follow up study should be needed to evaluate the potential therapeutic effect of umbilical cord blood transplantation for neuroprotection.

A case of Spontaneous Transsphenoidal Encephalocele with Recurrent Bacterial Meningitis / 대한소아신경학회지

Encephaloceles are congenital defects in the skull through which menings and brain tissues herniated. Basal encephaloceles are found in 1 in 40,000 live births. Transsphenoidal basal encephaloceles are very rare, accounting for only 5% of all basal encephaloceles. Currently, most encephaloceles are diagnosed antenatally and present at birth. Postnatally, infants may present with CSF rhinorrhea, recurrent meningitis, headache, hypothalamic- pituitary dysfunction. Some, particularly trasnssphenoidal encephaloceles are often clinically occult and diagnosis of the disease may be postponed up to adulthood. Surgical indications and approaches for transsphenoidal encephaloceles have been remained controversial. We report a case of CSF rhinorrhea and recurrent meningitis caused by transsphenoidal encephalocele.

Physiology of sleep / 소아과

Sleep is a vital, highly organized process regulated by complex systems of neuronal networks and neurotransmitters. Normal sleep comprises non-rapid eye movement (NREM) and REM periods that alternate through the night. Sleep usually begins in NREM and progresses through deeper NREM stages (2, 3, and 4 stages), but newborns enter REM sleep (active sleep) first before NREM (quiet sleep). A period of NREM and REM sleep cycle is approximately 90 minutes, but newborn have a shorter sleep cycle (50 minutes). As children mature, sleep changes as an adult pattern: shorter sleep duration, longer sleep cycles and less daytime sleep. REM sleep is approximately 50% of total sleep in newborn and dramatically decreases over the first 2 years into adulthood (20% to 25%). An initial predominant of slow wave sleep (stage 3 and 4) that peaks in early childhood, drops off abruptly after adolescence by 40% from preteen years, and then declines over the life span. The hypothalamus is recognized as a key area of brain involved in regulation of sleep and wakefulness. The basic function of sleep largely remains elusive, but it is clear that sleep plays an important role in the regulation of CNS and body physiologic processes. Understanding of the architecture of sleep and basic mechanisms that regulate sleep and wake cycle are essential to evaluate normal or abnormal development of sleep pattern changes with age. Reduction or disruption of sleep can have a significant impact on daytime functioning and development, including learning, growth, behavior, and emotional regulation.

Correlation of serum total hydroperoxide levels and diseases of prematurity / 소아과

PURPOSE: Total hydroperoxide (TH), free radical-mediated oxidation product can be used as a measure of free radical injury. The aim of the present study was to see if preterm newborns are at increased risk for oxidative stress compared with term newborns, and to determine whether oxidative stress during postnatal first 1 week is associated with clinical outcomes in preterm infants. METHODS: Serum TH levels of preterm infants (n=39) were compared with those of term infants (n=24) on the postnatal day 1. Among the preterm infants, serum TH levels of uncomplicated group (n=23) were also compared with those of complicated group (n=16) who developed oxygen radical related diseases on the postnatal day 1 and 7. Retrospective analysis was performed to find out risk factors for oxygen radical injuries based on birth history, laboratory data, neuroimaging findings and clinical progress in two preterm groups. RESULTS: Serum TH levels on postnatal day 1 were higher in the preterm infant group than the term infant group. Serum TH levels on postnatal day 1 in the complicated preterm infant group were significantly higher compared with uncomplicated group, but there was no significant difference in serum TH levels on postnatal day 7. Also, there was no significant difference in serum TH levels between uncomplicated preterm infants and term infants. Serum TH level on postnatal day 1 was independently associated with higher morbidity after adjusting for gestational age, Apgar score (5 min), arterial blood gas analysis. CONCLUSION: Complicated preterm newborns are at increased risk for oxidative stress compared with uncomplicated newborns and term newborns. Oxidative injury during the prenatal or postnatal day 1 is associated with adverse outcomes in preterm infants. Elevated TH levels on postnatal day 1 may have a value to predict clinical outcomes in preterm infants.

Clinical Considerations of Panayiotopoulos Syndrome / 대한소아신경학회지

PURPOSE: Panayiotopoulos syndrome(PS) is a benign childhood focal seizure disorder characterized by seizures with emetic symptoms and other autonomic phenomena, and its EEG shows shifting and/or multiple foci, often with occipital predominance. More than 800 cases have been reported worldwide, but no case has been reported in Korea. This paper describes the characteristics of the patients that satisfy the diagnostic criteria of PS and gives its clinical considerations. METHODS: A total of 140 children with non-febrile seizures visited Bundang Cha General Hospital from June 2004 to June 2005. Among them seven patients satisfied the diagnostic criteria of PS. We analyzed their clinical findings and EEG results retrospectively. RESULTS: Boys predominated. The mean age at the first seizures was 6.21 years old. Nearly 57% of the cases had at least one seizure lasting more than 30 minutes. Seizures during sleep were more common than those in wakefulness. Seizures started with emesis while the children were usually fully conscious. Other conventional seizure manifestations such as loss of consciousness, eyeball deviation, and convulsions often ensued. The EEG records showed occipital spikes in four patients. Of the other three cases, two had extraoccipital spikes and one had a normal EEG. CONCLUSION: PS seems to be commonly observed in Korea, although it remains largely unknown in Korea. Since PS, in its early phases, is often indistinguishable from several serious diseases, a good knowledge of the condition can prevent unnecessary examinations and excessive treatment. Further studies are required to establish systematic practice parameter guidelines.

A Case of Thalamic Pain Syndrome after Meningoencephalitis / 대한소아신경학회지

Thalamic pain syndrome is a type of central pain caused by damage to both thalami and produces persistent and intractable pain; extreme burning or aching sensation by light touch, even without external stimuli. In adults, thalamic pain syndrome occurred in vascular diseases such as a stroke, but in children, it was reported that infectious diseases-upper respiratory infection or meningitis-were usually preceded by development of central pain syndrome. This paper reports on a 12 year old boy with meningoencephalitis followed by evolving to thalamic pain syndrome 10 days later, who suffered from hypersensitivity and severe generalized pain on his whole body. On the brain MRI, both medial temporal lobes showed increased signal intensity, worse on the right side. A combination therapy of intravenous ketamine, amitriptyline and gabapentine for intensive pain control markedly reduced the pain and hypersensitivity in about 1 month. On the following brain SPECT, a strong focal perfusion at the right temporo-parietal cortex area was nearly normalized compared to previous one.

Clinical Features of Benign Infantile Convulsions with Gastroenteritis / 소아과

PURPOSE: This study was performed to characterize clinical features of benign convulsions with gastroenteritis (CwG) in infants. METHODS: We reviewed clinical features of 67 episodes in 64 patients with afebrile seizure accompanied gastroenteritis admitted to Dept. of Pediatrics Bundang CHA hospital from January 2001 to June 2004. Patients with meningitis, encephalitis/encephalopathy or apparent history of epilepsy were excluded. RESULTS: There were 32 boys and 35 girls. The age of onset ranged from 1 to 42 months (18.5+/-6.1 months). The number of children admitted to the hospital with acute gastroenteritis was 2, 887 in the same period. The percentage of patients with CwG was 2.3. Seizure type was exclusively generalized tonic or tonic-clonic seizure. The average number of seizures during a single episode was 3.1 (range, 1-13). Two or more seizures occurred in 53 (79.1%) of the 67 episodes. Antiepileptic drugs were administered for 42 episodes. Seizure did not cease after the administration of one kind of antiepileptic drug in 23 episodes (54.7%). The seizures were rather refractory to initial antiepileptic treatment. There were no abnormalities in serum biochemistry test including glucose and electrolytes. Cerebrospinal fluid was normal in all 54 episodes. Stool cultures were negative in 49 episodes. Rotavirus was positive in stools in 51 (82.3%) of 62 episodes. Norovirus was positive in stools in 2 episodes and astrovirus in 1 of 18 episodes. CT and/or MRI were performed in 15 cases and demonstrated no neuroradiologic abnormalities. Of 73 Interictal EEG, initial 24 cases showed occasional spike or sharp wave discharges from the mid-line area during stage I-II sleep, which were apparently differentiated from vertex sharp transient or K-complexes. The mean follow-up period was 5.7 months (1-36 months). Three patients experienced a recurrence of CwG, but all patients exhibited normal psychomotor development at the last follow-up. CONCLUSION: Afebrile infantile convulsions with gastroenteritis are brief generalized seizure in cluster with normal laboratory findings and good prognosis. Therefore CwG is likely to be categorized as situation-related seizure of special syndrome. Recognition of this entity should lead to assurance of the parents and long-term anticonvulsant therapy is not usually warranted.

Methylenetetrahydrofolate Reductase C677T and A1298C Polymorphisms and Risk of Down Syndrome / 소아과

PURPOSE: The C677T polymorphism of the methylenetetrahydrofolate reductase(MTHFR) has been suggested as a risk factor of maternal meiotic nondisjunction for Down syndrome. Recently, a second genetic polymorphism in MTHFR at position 1298 was reported. However, a positive association between the A1298C MTHFR polymorphism and Down syndrome has not been reported. Therefore, this study was undertaken to determine which polymorphism of MTHFR gene was associated with the increased risk of a child suffering from Down syndrome(DS). METHODS: We enrolled 33 patients with Down syndrome and 100 healthy individuals and analyzed the MTHFR C677T and A1298C polymorphism by a PCR-restriction fragment length assay. RESULTS: Frequencies of MTHFR C677T genotypes(CC, CT, and TT) were 9(27%), 22(67%), and 2 (6%) in the DS patients and 24(24%), 55(55%) and 21(21%) in the control, respectively. The frequency of mutant 677TT was significantly low in the DS patients(OR : 0.14; 95% CI : 0.02-0.95; P= 0.04). For the MTHFR A1298C polymorphism, frequencies of genotypes(AA, AC, and CC) were 16(48 %), 15(45%) and 1(3%) in DS patients and 77(77%), 21(21%) and 2(2%) in the control, respectively. The frequency of mutant 1298AC was significantly increased in DS patients with an odds ratio of 3.3(95% CI : 1.39-7.82; P=0.007). CONCLUSION: Our results suggest that MTHFR mutant 677TT may have a protective effect against Down syndrome, but MTHFR mutant 1298AC may be an independent risk factor in Down syndrome.

The Levels of Zinc and Neuron-specific Enolase in Febrile Convulsion / 소아과

PURPOSE: To determine the role of zinc in febrile convulsion and to evaluate whether febrile convulsion causes neuronal damage, serum and cerebrospinal fluid(CSF), zinc and CSF neuron-specific enolase(NSE) levels were measured in patients with febrile convulsion, epilepsy and aseptic meningitis. METHODS: Three groups were formed as follows: group I:53 children with febrile convulsion; group II:34 children with epilepsy; and group III, 40 children with aseptic meningitis. Serum and CSF zinc and CSF NSE levels were measured in each groups. RESULTS: The serum zinc levels of groups I, II and III had a mean of 74.71+/-18.26 microgram/dL, 104.35+/-31.43 microgram/dL and 87.03+/-24.47 microgram/dL, respectively, and the values of group I were significantly lower than those of the other two groups. The CSF zinc levels of groups I, II and III were found to have a mean 27.72+/-17.93 microgram/dL, 44.73+/-26.72 microgram/dL and 54.44+/-28.43 microgram/dL, respectively. In group I, the CSF zinc levels were significantly lower than those of other two groups. The CSF NSE levels of groups I, II and III had a mean of 11.61+/-2.96 ng/mL, 16.51+/-5.46 ng/mL and 14.60+/-3.02 ng/mL respectively and the values of group I were significantly lower than those of others. CONCLUSION: We confirmed that low zinc levels in serum and CSF are participants in the pathogenesis of febrile convulsion, but we could not find out the evidence of neurologic damage in patients with febrile convulsion using NSE levels in CSF.

Comparison of Two-Minute Tidal Breathing Method and Five Vital Capacity Breathing Method for Bronchial Provocation Test in Children / 소아알레르기및호흡기학회지

PURPOSE: The results of the bronchial provocation test were different by the method of inhalation, but there was no data in children. We performed the provocation test by 2 different methods such as two-minute tidal breathing versus five vital capacity breathing and compared the datas. METHODS: Methacholine inhalation test was performed for the In 17 patients with suspected bronchial hyperresponsiveness in Pocheon university Bundang Cha hospital, under identical conditions, except for the method of inhalation. All the patients were performed two-minute tidal breathing (2 min-TB) in one test and five vital capacity breathing (5VCB) with interval 1 to 7 days. Baseline and postsaline FEV1 were also measured to compare effects by two inhalation methods. Methacholine PC20 was calculated by linear interpolation between the last two data parts on the dose-response curve, and the cut off value of bronchial asthma was 8 mg/mL. RESULTS: The rate of positive brochial provaction test in 5VCB was 88.24% which was not significantly lower than 70.59% in 2 min-TB. In 12 patients with positive bronchial provocation test in both inhalation methods, the mean PC20 in 5VCB (5VC-PC20) was 4.40 mg/mL, being significantly higher than 1.02 mg/mL in 2 min-TB (2 min-PC20) (P< 0.05). There was a significant positive correlation between 5VC-PC20 and 2 min-PC20 (R=0.76, P< 0.001). CONCLUSION: We conclude from this study that the results of bronchial provocation test in children are different according to methods of inhalation in using the same nebulizer. Therefore, when we analyze the result of bronchial provocation test, we need attention to factors which are nebulizer output and method of inhalation to influence the response to the test.

A Case of Ophthalmoplegic Migraine Developed in Infancy / 대한소아신경학회지

Ophthalmoplegic migraine is defined as recurrent unilateral headaches associated with extraocular muscle palsies. This disorder is more common in older children, but it may begin in infancy. Neuroimaging study should be performed to rule out conditions resulting in third nerve compression, such as intracranial aneurysm or mass lesions. Early treatment with corticosteroids could shorten the duration of the ophthalmoplegia and relieve the pain. We report a 8 year old boy who had severe headache with recurrent ipsilateral ophthalmoplegia that developed at the age of 2. The pediatrician should be aware that ophthalmoplegic migraine may occur in infancy and that early systemic steroid therapy is critical to prevent permanent sequelae.