Intraparenchymal Pericatheter Cyst as a Complication of a Ventriculo-Peritoneal Shunt in a Premature Infant

A ventriculo-peritoneal shunt is a standard surgical management for hydrocephalus, but complications may impede the management of this disease. Obstruction of the catheter is one of the most common complications and manifests clinically in various ways. Intraparenchymal cyst development after shunt malfunction has been reported by several authors, but the underlying mechanism and optimal treatment methods are debatable. The authors report a case of intraparenchymal cyst formation around a proximal catheter in a premature infant after a ventriculo-peritoneal shunt and discuss its pathogenesis and management.

Clinical Manifestations and Treatment Outcome of Bacterial Meningitis in Neonates and Infants Under the Age of Three Months

PURPOSE: Bacterial meningitis in neonates and young infants is one of the most serious conditions that can lead to severe neurological sequelae despite the appropriate treatment. This study aimed at evaluating the clinical manifestations and treatment outcomes in patients under the age of three months, who had been diagnosed with bacterial meningitis. METHODS: A total of twelve patients with bacterial meningitis under the age of three months from January 1997 to June 2010 were retrospectively evaluated through a review of their medical records. Patients who showed positive culture results were included in the study. RESULTS: A total of 12 patients (6 males and 6 females, mean age 44.2+/-30.0 days) were enrolled in the study. All patients had fever upon admission. But most of them were unremarkable upon physical examination (75%). Streptococcus agalactiae was the most common organism cultured from CSF (7cases; 58.3%). Six cases showed positive results on CSF culture as well as on blood culture. Cefotaxime and ampicilin/sulbactam or cefotaxime and ampicilin were given as initial treatment with a mean treatment duration of 15.1+/-6.0 days. Neurological complications and sequelae included subdural effusion and hearing disturbance in two cases (16.7%). Nine cases (75%) showed excellent outcomes without neurological deficits, and none were left with a severe degree of sequelae. CONCLUSION: The study showed that neonates or young infants with bacterial meningitis almost always present with fever and that S. agalactiae was the most common causative organism. In addition, the final outcome for these patients may be improved with early and appropriate treatment.

Predictors of Suicidal Ideation in People with Epilepsy Living in Korea

BACKGROUND AND PURPOSE: The risk of suicide or suicide attempts is reported higher in people with epilepsy (PWE) than in the general population. Although epileptic, psychiatric, and psychosocial factors are known risk factors for suicide or suicide attempt, no studies have evaluated the predictors of the severity of suicidal ideation-which is a warning sign for suicide attempts-in PWE. Therefore, we measured the severity of suicidal ideation and its risk factors. METHODS: Consecutive PWE who were medicated with antiepileptic drugs (AEDs) and attended epilepsy clinic were included in the study. The subjects completed self-reported questionnaires, which included the Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Symptom Checklist-90-Revised (SCL-90-R), and Scale for Suicide Ideation-Beck (SSI-Beck). We compared the patients' demographic and clinical variables, and BDI, BAI, and SCL-90-R scores with their SSI-Beck score, and used our findings to determine the predictors for suicidal ideation. RESULTS: In total, 257 PWE were enrolled in the study. SSI-Beck scores correlated strongly with several seizure-related variables, duration of education, IQ, BDI and BAI scores, and nine domains of the SCL-90-R questionnaire. However, the strongest predictor for suicidal ideation was BDI score (beta=0.41, p<0.001), followed by several SCL-90-R domains, such as obsessive-compulsive (beta=-0.39, p<0.001), depression (beta=0.38, p<0.001), hostility (beta=0.22, p=0.002), paranoid ideation (beta=0.17, p=0.01), and IQ (beta=-0.10, p=0.017). These variables explained 59% of the variance in the SSI-Beck score. The seizure-related variables that influenced the BDI score were seizure frequency, duration of education, MRI abnormality, and number of AEDs. However, these variables explained only 18% of the variance in the BDI score. CONCLUSIONS: Major risk factors for suicidal ideation in PWE were depressive and psychiatric symptoms rather than seizure-related variables. Therefore, clinicians should focus on screening for depression and other psychiatric problems and treat them appropriately in order to reduce suicidal behavior in PWE. Since seizure-related variables also exhibited a minor role in determining depressive symptoms, stronger seizure-related risk factors for depression should be sought, such as seizure severity or psychosocial factors, to minimize suicidal behavior.

The effect of erythropoietin in neonatal rat model of hypoxic-ischemic brain injury / 소아과

PURPOSE: Perinatal asphyxia is an important cause of neonatal mortality and subsequent lifelong neurodevelopmental handicaps. Although many treatment strategies have been tested, there is currently no clinically effective treatment to prevent or reduce the harmful effects of hypoxia and ischemia in humans. Erythropoietin (Epo) has been shown to exert neuroprotective effects in various brain injury models although the exact mechanisms through which Epo functions are not completely understood. This study investigates the effect of Epo on hypoxic-ischemic (HI) brain injury and the possibility that its neuroprotective actions may be associated with iron-mediated metabolism. METHODS: HI brain injury was produced in 7-day-old rats by unilateral carotid artery ligation followed by hypoxia with 8% oxygen for 2 h. At the end of HI brain injury, the rats received an intraperitoneal injection of 5,000 units/kg erythropoietin. Random premedication with iron, deferoxamine, iron-deferoxamine, or saline were performed 23 d before HI brain injury. The severity of the brain injury was assessed at 7 d after HI. RESULTS: Single Epo treatment post-HI brain injury reduced the gross and histopathological findings of brain injury. Iron premedication did not increase the incidence or severity of the injury as measured by the damage score. Deferoxamine administration before HI brain injury improved the brain injury as compared to no treatment or Epo treatment. CONCLUSION: These findings indicate that Epo provides neuroprotective benefits after HI in the developing brain. These findings suggest that Epos neuroprotective actions may involve reducing iron in tissues that mediate the formation of free radicals.

Stigma in Patients with Drug Refractory Epilepsy: the Risk Factor, Psychiatric Comorbidities, and Quality of Life / 대한간질학회지

PURPOSE: Stigma is more likely to be reported by people with epilepsy with frequent seizures and associated with various physical and psychosocial factors. We determined risk factors associated with stigma, and investigated the impact of felt stigma on psychiatric comorbidities and quality of life (QOL) in patients with drug refractory epilepsy (DRE). METHODS: Patients with DRE of partial onset, who experienced a failure of at least two antiepileptic drugs (AEDs) and at least 1/month of seizure attack for recent 6 months, were enrolled in the study. We divided patients into two groups according to the presence of stigma. We compared demographic and clinical variables, mood, anxiety, psychiatric symptoms, and QOL between two groups. RESULTS: Among 75 patients with DRE of partial onset, 34 patients (45%) had stigma. Risk factors associated with stigma were age, history of psychiatric disease, duration of epilepsy, and duration of AEDs intake. However, seizure frequency was not associated with the occurrence of stigma. Mood, anxiety, and psychiatric symptoms were significantly higher in patients with stigma than those without stigma. QOL was significantly lower in patients with stigma than those without stigma. CONCLUSIONS: A longer duration of epilepsy with previous history of psychiatric diseases may be indispensable for the occurrence of stigma in patients with DRE. Early detection and appropriate treatment of psychiatric comorbidities can lessen the degree of stigma and improve QOL.

Assessment of parental understanding of epilepsy and effects of educational programs in an epilepsy camp / 소아과

PURPOSE: To assess parental knowledge and understanding of epilepsy including social stigma and evaluate the effects of educational programs on parents in an epilepsy camp. METHODS: We conducted an epilepsy camp on August 23-24, 2008, at Gyeongsan. Twenty families with an epileptic child each participated in the camp. We performed a survey before and after the camp to obtain data concerning parental understanding of epilepsy and the effects of the camp-based educational programs on the parents. The data were analyzed with SPSS 14.0 using frequency analysis. RESULTS: The parents were sufficiently knowledgeable about epilepsy before the camp, and their knowledge and understanding of epilepsy improved further after the camp. Both perceived stigma and enacted stigma against epilepsy were found in 30.0% of the parents. The perceived stigma decreased to 15.0% after the camp. CONCLUSION: This study suggests that social approaches such as epilepsy camps are effective in improving parental knowledge and understanding about epilepsy as well as decreasing their perceived social stigma.

Assessment of parental understanding of epilepsy and effects of educational programs in an epilepsy camp / 소아과

PURPOSE: To assess parental knowledge and understanding of epilepsy including social stigma and evaluate the effects of educational programs on parents in an epilepsy camp. METHODS: We conducted an epilepsy camp on August 23-24, 2008, at Gyeongsan. Twenty families with an epileptic child each participated in the camp. We performed a survey before and after the camp to obtain data concerning parental understanding of epilepsy and the effects of the camp-based educational programs on the parents. The data were analyzed with SPSS 14.0 using frequency analysis. RESULTS: The parents were sufficiently knowledgeable about epilepsy before the camp, and their knowledge and understanding of epilepsy improved further after the camp. Both perceived stigma and enacted stigma against epilepsy were found in 30.0% of the parents. The perceived stigma decreased to 15.0% after the camp. CONCLUSION: This study suggests that social approaches such as epilepsy camps are effective in improving parental knowledge and understanding about epilepsy as well as decreasing their perceived social stigma.

The Effect of Hyperthermic Pretreatment in a Neonatal Rat Model of Hypoxic-ischemic Brain Injury

PURPOSE: Perinatal asphyxia is an important cause of neonatal mortality and subsequent lifelong neurodevelopmental handicaps. Although many treatment strategies have been tested, there is currently no clinically effective treatment to prevent or reduce the harmful effects of hypoxia and ischemia in humans. In the clinical setting, maternal hyperthermia induces adverse effects on the neonatal brain, but recent studies have shown that hyperthermic pretreatment (PT) plays some role in hypoxic-ischemic (HI) injuries of the developing brain. The present study investigated the effect of hyperthermic PT on HI brain injuries in newborn rats. METHODS: HI was produced in 7-day-old neonatal rats by unilateral common carotid artery ligation, followed by hypoxia with 8% oxygen at 38degrees C for 2 hours. Twenty-four hours before HI, one-half of the pups were exposed to a 40degrees C environment for 2 hours. The severity of the brain injury was assessed 7 days after the HI. RESULTS: Hyperthermic PT reduced the gross and histopathologic findings of brain injury from 64.7 to 31.2% (P<0.05). There were no differences in location and severity of injury between the pretreated and control brains. CONCLUSION: These findings indicate that hyperthermic PT provides neuroprotective benefits on HI in the developing brain. Also, these findings suggest maternal hyperthermia may have protective effect on perinatal HI brain injuries.

Cognitive Effects of Antiepileptic Drugs

Antiepileptic drugs (AEDs) can adversely affect cognitive function by suppressing neuronal excitability or enhancing inhibitory neurotransmission. The main cognitive effects of AEDs are impaired attention, vigilance, and psychomotor speed, but secondary effects can manifest on other cognitive functions. Although the long-term use of AEDs can obviously elicit cognitive dysfunction in epilepsy patients, their cognitive effects over short periods of up to a year are inconclusive due to methodological problems. In general, the effects on cognition are worse for older AEDs (e.g., phenobarbital) than for placebo, nondrug condition, and newer AEDs. However, topiramate is the newer AED that has the greatest risk cognitive impairment irrespective of the comparator group. Since the cognitive impact of AEDs can be serious, clinicians should be alert to adverse events by evaluating cognitive function using screening tests. Adverse cognitive events of AEDs can be avoided by slow titration to the lowest effective dosage and by avoiding polytherapy.

A Case of Epilepsy Exhibiting a Severe, Dose-Dependent, Memory Deficit after Zonisamide Administration / 대한간질학회지

Zonisamide (ZNS) has been proven as a safe, effective, and well-tolerated antiepileptic drug. We report an epilepsy patient who had a severe, dose-dependent, memory deficit after ZNS administration. A 65-year-old man visited our epilepsy clinic due to the occurrence of nocturnal convulsions. Despite the absence of seizures, he developed a severe impairment of verbal and visual memory functions after the increment of ZNS dosage from 200 mg/day to 300 mg/day. We substituted 1,000 mg/day valproic acid for ZNS. His cognitive performances were returned to original levels.

Prognostic Value of Electrophysiologic Tests in Children with Facial Nerve Palsy

PURPOSE: This study was aimed to evaluate the value of electrophysiologic tests for determining prognosis in children with facial nerve palsy. METHODS: We retrospectively analyzed 37 children diagnosed as the facial nerve palsy at the pediatric neurology clinic, Kyungpook National University Hospital from January 1, 2000 to March 31, 2007. RESULTS: A total of thirty seven children were involved in the study(male to female 21:16, and the mean age 87.5 months). Among those twenty one had electrophysiologic tests. As compared with the normal values, the amplitude decreased by 54.5%(0.6+/-0.5 mV) and the latency was prolonged by 11.0%(3.6+/-0.5 msec) in electroneurography(ENoG). Early response(R1) was absent in 15 out of 21(71.4%) and ipsilateral response(R2) was absent in 19 children(90.5%). As compared with the children who had the decrease of amplitude 90% or less in ENoG, the children with the decrease of amplitude greater than 90% showed poor recovery(100% vs 60%, P<0.05) and longer duration of follow-up(43.7+/-30.0 days vs 184.00+/-196.8 days, P<0.05). All children who had R1 and R2 responses in the Blink test were completely recovered from the illness, but they were not statistically different from the other groups. CONCLUSION: Decrease of amplitude in ENoG and responsiveness in the Blink test can be important prognostic determinants in children with facial nerve palsy, but further studies are needed.

Comparison of Cognitive Effects of Lamotrigine and Oxcarbazepine in Epilepsy Patients

BACKGROUND AND PURPOSE: This study compared the cognitive effects of 1 year of treatment with lamotrigine (LTG) and oxcarbazepine (OXC) in epilepsy patients. METHODS: This retrospective study investigated 60 epilepsy patients undergoing neuropsychological tests who were either newly diagnosed or untreated in the preceding 6 months. The cognitive function in 30 patients receiving LTG monotherapy and 30 age-matched patients receiving OXC monotherapy was compared after 1 year. The neuropsychological scores at baseline and all of the epilepsy-relevant variables except seizure type did not differ between the groups. The mean daily dosages of LTG and OXC at 1 year were 93 mg and 825 mg, respectively. RESULTS: The posttreatment list-learning performance was better in the LTG group than in the OXC group (p<0.05). The incidence of cognitive complaints did not differ between the two groups. The list-learning performance and Trail Making Test scores were better in each group after treatment. CONCLUSIONS: LTG and OXC monotherapies have similar, slightly beneficial effects on cognitive function, and are probably not harmful.

Long-Term Efficacy and Safety of Zonisamide Monotherapy in Epilepsy Patients

BACKGROUND AND PURPOSE: Zonisamide (ZNS) is a useful antiepileptic drug with a broad therapeutic spectrum. However, there is limited information on the long-term use of ZNS as a monotherapy. This study investigated the long-term effects of ZNS as a monotherapy for the treatment of epilepsy. METHODS: We retrospectively analyzed the records of epilepsy patients treated with ZNS monotherapy at our clinic. We identified outcomes for patients treated with ZNS monotherapy for a minimum of 6 months. Efficacy was quantified as the percentage change in seizure frequency, and safety was assessed by the frequency and types of adverse events. RESULTS: Sixty patients who received ZNS for a minimum of 6 months were included. The mean duration of treatment was 19.8 months (range, 6-37 months), and the mean ZNS dosage was 255 mg/day (range, 100-500 mg/day). Twenty-seven patients (45%) were seizure-free, and an additional 20 patients (33%) had above 50% seizure frequency reduction at the last follow-up visit. Partial seizures with or without secondary generalization and generalized seizures were well controlled by ZNS, whereas complex partial seizures were not. Forty-eight patients (80%) reported mild-to-moderate adverse events, including memory loss (35%), attention deficit (27%), and weight loss (20%). CONCLUSIONS: Long-term ZNS monotherapy is effective at treating a broad spectrum of seizure disorders, except complex partial seizures. However, a specific adverse event, such as cognitive impairment, is common and long-lasting.

Cognitive Impairment in Juvenile Myoclonic Epilepsy

BACKGROUND AND PURPOSE: Cognitive impairments are frequent consequences of epilepsy, with intellectual ability reportedly being lower in patients with idiopathic generalized epilepsies than in the general population. However, neuropsychological investigations have been rarely performed in patients with juvenile myoclonic epilepsy (JME). We aimed to quantify the cognitive function in JME patients using various neuropsychological tests. METHODS: We compared cognitive function in 27 JME patients with that in 27 healthy volunteers using tests examining cognitive performance, such as the verbal and visual memory, frontal function, attention, IQ score, and mood. In the JME group, we examined risk factors for cognitive function such as age, sex, family history, education level, age at seizure onset, seizure frequency, EEG abnormality, disease duration, and previous intake of antiepileptic drugs. RESULTS: Verbal learning was significantly lower in JME patients than in controls, and attention and verbal fluency were impaired in JME patients compared with controls. However, general intellectual ability and mood did not differ between the groups. Early onset of seizure and long duration of disease were closely related to impaired cognitive function. CONCLUSIONS: JME patients may exhibit impaired cognitive function, in terms of memory and execution, despite having normal intelligence and mood.

A case of Dyke-Davidoff-Masson syndrome in Korea / 소아과

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.

Clinical significance of cerebrospinal fluid pleocytosis in pediatric refractory status epilepticus / 소아과

PURPOSE: Refractory status epilepticus(RSE) is a serious neurological emergency in children. The mortality is high and the neurological outcome is not good. This study aimed to evaluate the clinical significance of cerebrospinal fluid(CSF) pleocytosis in refractory status epilepticus in children. METHODS: From January 1999 to January 2006, 25 out of 37 children with refractory status epilepticus had spinal tapping. We retrospectively analyzed the data from these children's medical records. We compared the results between groups with and without CSF pleocytosis, and between a group with first seizure and a group with epilepsy . RESULT: Six out of 25 children had CSF pleocytosis. The group without CSF pleocytosis had a higher mortality rate and required higher doses of antiepileptic drugs as compared with the group with CSF pleocytosis. The group with CSF pleocytosis had much worse neurologic segualae. However, except for the children with CNS infection, the overall prognosis between the group with and without CSF pleocytosis was not significantly different. All children with CSF pleocytosis came in with first seizures. CONCLUSION: In children with RSE, a CSF study must be perfomed as soon as possible to exclude the possibility of CNS infection. A CSF study is even more important in cases of first seizure or CNS infection suspected. Mild CSF pleocytosis without evidence of infection does not seem to affect the prognosis, so physicians should therefore be more cautious in selecting antibacterial or antiviral agents for it.

Cognitive Profiles of Lamotrigine in Epilepsy Patients : A Comparative Study with Valproate / 대한간질학회지

PURPOSE: To identify cognitive effects of lamotrigine (LTG) compared with valproate (VPA) in epilepsy patients after 1 year of treatment. METHODS: Cognitive tests and subjective complaints of 22 patients with LTG monotherapy (50-200 mg/day) were retrospectively compared with those of 22 patients with VPA monotherapy (500-1300 mg/day) at 1 year of medication. RESULTS: LTG group did not show any significant difference in the performance of cognitive tests compared with VPA group. The incidence of cognitive complaints between two drugs were also not different. Both groups showed a better performance of list learning and Trail Making Test type A after antiepileptic drug medication. CONCLUSION: The impact of LTG and VPA monotherapy on cognitive functioning is similar. Both drugs may not be harmful or rather slightly beneficial for cognitive functions.

The Experience of the 1st Daegu-Gyeongbuk Multicenter Epilepsy Camp for Children and their Parents to improve the understanding of Epilepsy

PURPOSE:This study was undertaken to announce our experience of the 1st Daegu- Gyeongbuk Multicenter Epilepsy Camp for children and their parents to improve the understanding of epilepsy. METHODS:We held the camp on February 25th to 26th, 2006 at Gyeongju. 33 families with epileptic childlend joined the camp. At the end of the camp, we performed a survey with 37 questionnaires to obtain data concerning the parents' understanding of epilepsy and the satisfaction with the camp. Data was analyzed with SPSS 10.0 program using frequency analysis. RESULTS:Parents knew about epilepsy but for some degrel still wanted to get more information about epilepsy. Parents were satisfied with the educational programs but not with the recreation programs. CONCLUSION:This study suggests that more social approaches such as an epilepsy camp is needed to provide the families with more information fot the understanding about epilepsy.

Clinical Features of Children with Febrile Status Epilepticus

PURPOSE: Febrile seizures(FS) are the most common form of childhood seizures and their prognosis are favorable. Despite the fact, there are still some concerns that prolonged febrile seizures may cause brain damages or neurological sequelae, which may be associated with the development of mesial temporal sclerosis and intractable temporal lobe epilepsy in later life. The aim of this study was to evaluate the clinical characteristics and outcomes in children with febrile status epilepticus(FSE). METHODS: 39 children aged 6 months to 5 years were enrolled in this study, who presented with FSE at Kyungpook National University Hospital, from January, 2000 to December, 2004. They were compared with 250 children who presented with the first episodes of FS at the same period. We reviewed retrospectively the medical records for the clinical characteristics and outcomes of the two groups. RESULTS: There were no statistically significant differences between the two groups in terms of age, etiology, degree and duration of fever, type of seizure, family history of FS or epilepsy, EEG and MRI findings, and recurrence rates of FS. Children with FSE were more likely to have neurological sequelae(12.5% vs. 2.4%; P=0.008), to develop afebrile seizures(21% vs. 4%; P=0.00) and to take anticonvulsants chronically(33% vs. 2%; P=0.00) than children with FS. CONCLUSION: Our results indicate that prolonged febrile seizures are associated with unfavorable neurological outcomes compared with simple febrile seizures. However, long-term, and extensive follow-up studies are needed to find out the relationship between prolonged febrile seizures and subsequent mesial temporal sclerosis as well as intractable temporal lobe epilepsy.

Mental retardation and other neurodevelopmental disorders / 소아과

Mental retardation(MR) is one of the most common developmental disabilities, which is characterized by deficits in intellectual and adaptive functions. Most children with MR have cognitive limitation in the mild range. With respect to the etiology, it is believed that genetic and environmental factors are interrelated and show variable pictures. Most children with MR present with speech and language delay during the early years. The diagnosis can be made by clinical features and neuropsychological tests of intelligence and adaptive functioning. The treatment is limited, but many associated problems are amenable to multidisciplinary interventions. The article will review the recent advances in the management of MR and other neurodevelopmental disorders in children.