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Thongmee W, Padungkiatsagul T, Jindahra P, Khongkhatithum C, Thampratankul L, Vanikieti K. Clin Ophthalmol. 2020;14:4271–4278. Table 5 on page 4276, the 95% CI and the p-value for the Time from initial ocular symptoms (pain and/or visual loss) to IVMP in each affected eye (days) row are incorrect. Table 5 on page 4276, the 95% CI and the p-value for the BCVA at nadir better than CF row are incorrect. The correct Table 5 is shown below. Table 5 Univariate Analysis The authors apologize for these errors and advise they do not affect the conclusions of the study.

Watcharaporn Thongmee,1 Tanyatuth Padungkiatsagul,1 Panitha Jindahra,2 Chaiyos Khongkhatithum,3 Lunliya Thampratankul,3 Kavin Vanikieti1 1Department of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 2Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 3Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Kavin VanikietiDepartment of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Bangkok 10400, ThailandTel +662 201 1526Fax +662 201 2729Email Vanikieti.kavin@gmail.comObjective: We aim to identify prognostic factors for visual outcomes following a first episode of neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) in affected eyes.Materials and Methods: This was a single-center, retrospective study. The study included 50 individuals who were diagnosed with NMOSD-ON (63 affected eyes) in a tertiary institution over a 12-year period. Data regarding any second, or higher, episodes of NMOSD-ON in each eye were not taken into consideration. Medical records of included individuals were reviewed. Demographic data, clinical and magnetic resonance imaging characteristics, and treatment outcomes were collected. Main outcome measures of the study were prognostic factors for good visual outcome (best-corrected visual acuity (BCVA) ≥ 20/200) following an initial episode of NMOSD-ON in affected eyes.Results: Sixty-three affected eyes of 50 individuals (3 men and 47 women) were included. BCVA at nadir that was better than counting fingers (CF) (odds ratio 10.43, 95% confidence interval 1.04, 104.45, p = 0.046) and time from NMOSD-ON onset to intravenous methylprednisolone (IVMP), less than 21 days (odds ratio 10.73, 95% confidence interval 1.91, 60.01, p = 0.007), were significantly associated with good visual outcomes.Conclusion: BCVA at nadir that was better than CF and treatment with IVMP within 21 days of symptom onset were important prognostic factors of good visual outcomes following a first episode of NMOSD-ON in affected eyes.Keywords: optic neuritis, neuromyelitis optica spectrum disorder, prognostic factors, visual outcomes, Thai

Chanomporn Narongkhananukul,1 Tanyatuth Padungkiatsagul,1 Panitha Jindahra,2 Chaiyos Khongkhatithum,3 Lunliya Thampratankul,3 Kavin Vanikieti1 1Department of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 2Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 3Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Kavin Vanikieti Email Vanikieti.kavin@gmail.comPurpose: To compare demographic data, clinical and radiological characteristics, treatment, and long-term visual outcomes between myelin oligodendrocyte glycoprotein autoantibody-positive optic neuritis (MOG-IgG + ON) and aquaporin-4 autoantibody-positive optic neuritis (AQP4-IgG + ON) in Thailand.Patients and Methods: We included individuals who were diagnosed with either MOG-IgG + ON or AQP4-IgG + ON over an 11-year period. Demographic data, clinical and radiological characteristics at ON presentation, treatment, and long-term visual outcomes were retrospectively collected.Results: There were 16 patients (28 eyes) and 43 patients (59 eyes) in the MOG-IgG + ON and AQP4-IgG + ON groups, respectively. AQP4-IgG + ON occurred predominantly in female patients whereas MOG-IgG + ON-affected female patients and male patients equally (p < 0.001). Prior or concurrent non-ON demyelinating events were more often observed at AQP4-IgG + ON onset (p < 0.001). At ON presentation, bilaterality and the presence of optic disc edema were predominantly found in the MOG-IgG + ON group (bilaterality: 80% vs 8%, MOG-IgG + ON vs AQP4-IgG + ON patients, respectively (p < 0.001); presence of optic disc edema: 92.3% vs 36.6%, MOG-IgG + ON- vs AQP4-IgG + ON-affected eyes, respectively (p < 0.001)). There was no statistically significant difference in age at ON onset, nadir visual acuity (VA), presence of pain, segmental enhancement, and total enhanced segments of the anterior visual pathways. At the last follow-up, immunosuppressive drugs were used more often in the AQP4-IgG + ON group (43.7% vs 74.4%, MOG-IgG + ON vs AQP4-IgG + ON, respectively; p < 0.027). Remarkably better final VA was achieved in MOG-IgG + ON-affected eyes (median: 0.0 vs 0.4 logMAR, MOG-IgG + ON- vs AQP4-IgG + ON-affected eyes, respectively; p < 0.001).Conclusion: Compared with AQP4-IgG + ON, MOG-IgG + ON tended to present with bilaterality and optic disc edema and demonstrated better visual outcomes.Keywords: optic neuritis, myelin oligodendrocyte glycoprotein, aquaporin-4, neuromyelitis optica spectrum disorder, Thai

Purpose: This study aimed to translate and validate the Japanese Sleep Questionnaire for Preschoolers (JSQ-P) into a Thai version and to evaluate factors associated with sleep problems in preschoolers., Methods: The cross-cultural adaptation of JSQ-P into Thai version of JSQ-P (TH-JSQ-P) was performed including forward translation, reconciliation, backward translation, and final approval by the original creator. Children aged 2-6 years from community and clinical settings were enrolled. The TH-JSQ-P was completed by parents of preschool children twice within 10-14 days to assess its reliability and validity. Six validity tests including face validity, content validity, structural validity, construct validity, criterion validity and predictive validity were assessed. Demographic data, Epworth sleepiness scale (ESS), medical and behavior problems, and socioeconomic status were collected for analysis., Results: A total of 2613 participants completed the TH-JSQ-P, with 1207 completing it twice to assess test-retest reliability. The TH-JSQ-P had good internal consistency reliability and test-retest ability with Cronbach's alpha value of 0.898 and intraclass correlation coefficient of 0.814. The content validity was acceptable. The ESS had correlation with sleep problems in almost all subscales except for sleep habit., Conclusions: The TH-JSQ-P is a valid and reliable instrument for evaluating sleep problems in preschool children in Thailand. It has the potential to be extensively utilized to screen for sleep problems, thereby enabling healthcare professionals to identify them early and implement prompt management., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier B.V.)

Background: Sleep plays an important role in children's behavior, emotional control, and cognitive development. There is a higher prevalence of sleep problems in children with neurodevelopmental disorders (NDDs) compared to those without disease. There are a few studies regarding sleep problems among Thai preschoolers with NDDs. This study examined sleep problems in preschoolers with NDDs, compared with typically developing children (TD), and studied the association between sleep problems and behavioral problems among preschoolers with NDDs., Methods: Sleep problems in 140 children aged 2 to 6 years with NDDs; autism spectrum disorders, attention deficit/hyperactivity disorder, global developmental delay, and developmental language disorder were evaluated by using the Thai version of Japanese Sleep Questionnaire for Preschoolers (TH-JSQ-P) and compared with 285 TD children at the same age. Behavioral problems in the NDDs group were evaluated by using the Child Behavioral Checklist (CBCL). Sleep and behavioral problem scores were analyzed by adjusted age, sex, underlying diseases, and medications used and compared between the two groups., Results: The study found that 48 % of the TD group and 71 % of the NDDs group were identified as having sleep problems. The NDDs group had significantly higher total TH-JSQ-P score and in five subscales, including obstructive sleep apnea, restless leg syndrome-motor, parasomnias, daytime behaviors, and insomnia/circadian rhythm disorders. Total TH-JSQ-P score was found to have a moderate correlation with internalizing and externalizing behavioral problems., Conclusion: Findings suggest that sleep problems are more common in preschool children with NDDs than in TD children and are associated with internalizing and externalizing behavioral problems., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier B.V.)

Nonketotic hyperglycinemia (NKH) is in most cases a fatal inborn error of metabolism which usually presents during the neonatal period as encephalopathy and refractory seizures. The reported congenital anomalies associated with NKH included corpus callosal agenesis, club foot, cleft palate, and congenital heart disease. Here, we report a newborn who presented with encephalopathy without overt seizures, cerebral venous sinus thrombosis, and cleft palate. Electroencephalography showed a burst suppression pattern, which suggests the etiology could be due to a metabolic or genetic disorder. The amino acid analysis of plasma and cerebrospinal fluid showed elevated glycine. Whole exome sequencing identified a heterozygous c.492C > G; p.Tyr164Ter variant in exon 4 of the GLDC gene inherited from the patient's father. Further long-read whole genome sequencing revealed an exon 1-2 deletion in the GLDC gene inherited from the patient's mother. Additional analyses revealed no pathogenic variants of the cleft palate-related genes. The cleft palate may be an associated congenital anomaly in NKH. Regarding cerebral venous sinus thrombosis, we found a heterozygous variant (p.Arg189Trp) of the PROC gene, which is a common cause of thrombophilia among Thai newborns. A neonate with NKH could present with severe encephalopathy without seizures. A close follow up for clinical changes and further next generation sequencing are crucial for definite diagnosis in neonates with encephalopathy of unclear cause., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Thewamit, Khongkhatithum, Thampratankul, Kamolvisit, Khongkrapan and Wattanasirichaigoon.)

Background: Anti-N-methyl-d-aspartate receptor encephalitis (anti-NMDARE) is one of the most common types of autoimmune encephalitis. Most patients have no apparent immunologic triggers, which suggests a genetic predisposition. This study was conducted to identify human leukocyte antigen (HLA) class II alleles associated with anti-NMDARE in Thai children., Methods: This case-control study enrolled patients younger than 18 years who were diagnosed with anti-NMDARE between January 2010 and December 2020. A "good outcome" was determined as a patient with a modified Rankin scale score of less than 2 at any follow-up visit. HLA genotypes were determined at four-digit alleles using reverse sequence-specific oligonucleotide probe hybridization. The HLA class II allele frequency in patients was compared with that in a database of 101 healthy control Thai children., Results: Thirty-four patients were enrolled with a mean age of 12.8 ± 5.6 years (females 85.3%). The HLA-DRB1∗1502 allele frequency was significantly higher in patients than in controls (odds ratio, 2.32; 95% confidence interval, 1.11-4.8, P = 0.023). A good outcome was noted in 14 of 14 (100%) HLA-DRB1∗1502-positive patients (median time to a good outcome, 6 months) and 14 of 17 (82.3%) HLA-DRB1∗1502-negative patients (median time to a good outcome, 3 months). Two (11.8%) HLA-DRB1∗1502-positive patients had one relapse each, and six (35.3%) HLA-DRB1∗1502-negative patients had one to three relapses., Conclusions: HLA-DRB1∗1502 was significantly associated with anti-NMDARE in our patients. Most patients had good outcomes. HLA-DRB1∗1502-positive patients tended to require a longer time to achieve a good outcome but had less frequent relapses than HLA-DRB1∗1502-negative patients., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: The authors declare no conflict of Interest.

Glucose transporter type-1 deficiency syndrome (Glut1 DS) is a rare disorder with various manifestations. Early diagnosis is crucial because treatment with the ketogenic diet can lead to clinical improvement. Here, we report the cases of two siblings with Glut1 DS and one of them presented with sleep disorder which is a rare and atypical manifestation of Glut1 DS. Patient 1 was a 3.5-year-old boy who presented with paroxysmal loss of tone and weakness of the whole body with unresponsiveness after waking up. He also had excessive daytime sleepiness, insomnia, and restless sleep. His other clinical findings included focal seizures, paroxysmal exercise-induced dyskinesia (PED), ataxia, mild global developmental delay, and hyperactivity. Patient 2 was a 5.5-year-old boy who presented with drug-resistant focal epilepsy, global developmental delay, paroxysmal dystonia, and ataxia. A novel heterozygous nonsense variant of SLC2A1, c.1177G > T (p.Glu393*), classified as a pathogenic variant, was identified in both patients, but not in their parents' blood. After treatment with the modified Atkins diet, their neurological functions significantly improved. In conclusion, we reported two siblings with variable phenotypes of Glut1 DS with a novel nonsense mutation. Although sleep disorder and daytime somnolence were the nonclassical manifestations of Glut1 DS, the diagnostic evaluation of possible Glut1 DS in patients presented with daytime sleepiness, particularly in cases with the cooccurrence of seizures or movement disorders should be considered., Competing Interests: The authors declare no competing interests., (Thieme. All rights reserved.)

Neurocysticercosis is one of the most common parasitic infections in the central nervous system in children. The usual clinical manifestation is new-onset focal seizure. However, there are other multiple clinical manifestations, such as increased intracranial pressure, meningoencephalitis, spinal cord syndrome, and blindness. The diagnosis needs high index of suspicion with clinical history, physical examination, neuroimaging, and immunologic studies. Recent advances in neuroimaging and serology facilitate the accurate diagnosis. Management of neurocysticercosis should focus on critical symptoms first, such as the use of antiepileptic drugs and medical or surgical therapy for increased intracranial pressure., Competing Interests: Disclosure There is no potential conflict of interest related to this work., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Obstructive sleep apnea (OSA) during pregnancy leads to adverse maternal and perinatal outcomes. There have been limited studies evaluated the effect of intrauterine exposure to maternal OSA on childhood developmental outcomes. This study was aimed to evaluate the early development of children born to mothers with gestational OSA and the impact of continuous positive airway pressure (CPAP) treatment. METHODS: Children aged 6-36 months, born to high risk pregnant mothers who had overnight polysomnography performed, were invited to participate. The Ages and Stages Questionnaires, third edition (ASQ-3), age-specific parent-completed questionnaires determining five developmental domains (communication, gross motor, fine motor, problem-solving, and personal-social) were used for developmental screening. Children who had a score of at least one domain less than -1 SD of age cut-off were determined as having a risk of developmental delay (RDD). RESULTS: There were 159 children (47% male, mean age 18 months) enrolled. The maternal PSG showed non-OSA, mild OSA, and moderate OSA in 14%, 46%, and 40%, respectively. Forty-two children (26%) had RDD, and the most affected domains were fine motor and problem-solving. Maternal moderate OSA was significantly associated with RDD (adjusted OR 5.39, 95%CI 1.11-26.12, P 0.037). Subgroup analysis showed that maternal moderate OSA with no CPAP treatment was significantly associated with RDD (OR 6.43, 95%CI 1.34-30.89, P = 0.020) CONCLUSION: Gestational moderate OSA in high-risk pregnancy mothers likely had a negative effect on early childhood developmental outcomes, particularly the mothers who did not have appropriate CPAP treatment., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: Children with refractory epilepsy (RE) are associated with increased mortality rate, nonfatal injuries, disability, and diminished quality of life. Biomarkers for the early prediction of RE is still an unmet need., Methods: Eighteen children with RE and six age-matched unrelated controls were included in this study. Plasma samples were prefractionated by the optimized thermal treatment before proteomic analysis using 2DE-LC-MS/MS. Bioinformatic analysis was carried out using STRING protein network. Immunoassay of unprocessed plasma was applied to confirm changes of proteins of interest. P-value < 0.05 was considered statistically significant., Results: Proteomic analysis (n = 6 each group) revealed nine differentially expressed proteins, i.e., haptoglobin, S100A9, serpin B1, apolipoprotein A-I, apolipoprotein A-IV, apolipoprotein C-II, alpha-1-acid glycoprotein 1 and 2, and transthyretin. Western immunoblotting confirmed haptoglobin upregulation in the RE group. STRING protein network predicted the inflammatory cytokines, i.e., interferon gamma (IFN-γ), interleukin-1 beta (IL-1β) and tumor necrosis factor alpha (TNF-α), play roles in pathophysiology in RE patients. Cytokine immunoassay (n = 24, 18 RE vs. 6 controls) exhibited plasma IFN-γ was upregulated in RE patients as compared to the healthy individuals (median [IQR]; 2.9 [2.9, 4.9] vs. 1.32 [0.8, 1.5] pg/mL, p = 0.0013), and plasma IL-1β was significantly downregulated in patients (1.0 [0.2, 1.9] vs. 4.5 [1.9, 11.0] pg/mL, p = 0.01). TNF-α had no difference between groups. The results suggest that haptoglobin may be associated with oxidative brain damage, while IFN-γ and IL-1β may be involved with neuroinflammation., Conclusions: Alterations in plasma haptoglobin, IFN-γ, and IL-1β were associated with RE patients. Future studies using a combination of these candidate biomarkers may help predict the intractability of epilepsy in pediatric populations., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Epilepsy surgery is proven as a cost-effective treatment in developed countries, especially in adults with drug resistant epilepsy (DRE). This study is aimed to demonstrate the cost-effectiveness of epilepsy surgery in children and adolescents with DRE at three years compared with those who were eligible for surgery but received medical treatment. This study was conducted from January 2014 to December 2018. Clinical data were obtained from a retrospective chart review. Direct medical costs, including epilepsy surgery, inpatient and outpatient treatment were retrieved from the finance department. Direct non-medical costs were collected from the family interview. The effectiveness was determined by percent seizure reduction and quality of life assessed by EQ-5D scores. Decision tree analysis using TreeAge Pro® 2018 was deployed to determine the cost-effectiveness. Seventeen patients had epilepsy surgery and 19 were in the medical group. Seizure freedom was noted in 52% and 16% in the surgical and medical groups, respectively. Incremental cost-effectiveness ratio (ICER) was 743,040 THB (22,793 USD) per 1 QALY and 3302 THB (101 USD) per 1% seizure reduction. The study did not demonstrate cost-effectiveness of epilepsy surgery in the short term compared with Thailand's threshold (160,000 THB (4908 USD) per 1 QALY). Epilepsy surgery may be cost-effective if evaluated beyond three years., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Purpose: Phenytoin (PHT) is a common causative drug for severe cutaneous adverse drug reactions (SCARs) in children. SCARs, including drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), are associated with a variation in HLA genotypes. Blood screening for specific HLA allele before PHT prescription would help in the reduction of the incidence of PHT induced SCARs. This study was to investigate the association between variations of HLA genotypes and PHT induced SCARs in Thai children., Methods: Cases were Thai children aged between 0-18 years diagnosed with SCARs from PHT. Control groups were Thai children of corresponding age who had taken PHT for a least 12 weeks without any hypersensitivity reaction and healthy population controls. Blood samples from both groups were collected for HLA genotyping using a reverse-sequence specific oligonucleotide (SSO) probes method. Carrier rates of HLA alleles were compared between 22 cases (17 DRESS and 5 SJS-TEN), 60 tolerant controls and 649 population controls., Results: Two HLA alleles includingHLA-B*51:01 and HLA-C*14:02 were significantly associated with PHT induced DRESS (OR 5.83; 95 % CI 1.36-25.00, p = 0.022 and OR 5.85; 95 % CI 1.16-29.35, p = 0.039). HLA-B*38:02 was significantly associated with PHT induced SJS-TEN (OR12.67; 95 % CI 1.50-106.89, p = 0.044). Haplotype analysis demonstrated the association of HLA haplotype A*11:01-B*51:01-C*14:02 and PHT induced DRESS compared to tolerant controls and the healthy population control group (OR 8.92; 95 % CI 1.47-54.02, p = 0.019, and OR 10.2; 95 % CI 3.04-34.21, p = 0.002). HLA haplotype B*38:02-C*07:01 in PHT induced SJS-TEN was significantly higher than those in tolerant controls and the healthy population control group (40 % vs 3.3 % vs 0.3 %; OR 19.33; 95 % CI 1.98-188.59, p = 0.027 and OR 215.67; 95 % CI 22.40-2076.04, p = 0.0003. HLA-B*15:02 was not associated with PHT induced SCARs., Significance: An association betweenHLA-B*51:01 and HLA-C*14:02 and PHT induced DRESS and HLA-B*38:02 and PHT induced SJS-TEN has been demonstrated in Thai children., Competing Interests: Declaration of Competing Interest The authors declare that there are no conflicts of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

A high red cell distribution width (RDW) and low hemoglobin level increase the risk of arterial ischemic stroke (AIS), mostly in adults. The mechanisms related to AIS remain unknown. A total of 233 subjects (90 patients and 143 healthy controls [HC]) were enrolled. The mean(SD) age in patients and HC was 9.5(3.8) and 11.4(1.8) years, respectively. We found increased odds ratios (ORs) for large vessel and small vessel subtypes in patients without underlying diseases with a mean corpuscular volume (MCV) <80 fL (OR: 5.4, 95%CI 1.8-16.3 and 2.8, 95%CI 1.2-7.2), mean corpuscular hemoglobin levels <27 pg (OR: 2.9, 95%CI 1.0-6.7 and 2.6, 95%CI 1.0-6.7), and RDW >15% (OR: 5.5, 95%CI 1.3-24.5 and 2.7, 95%CI 1.0-7.3). RBC indices showed significant correlations with TM levels. Therefore, low MCV and MCH levels, and a high RDW were risk factors for AIS and associated with TM levels in this population., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Levetiracetam (LEV) is an antiepileptic drug which has good safety and efficacy in neonatal seizure (NS), a common incident in neonates with weight <1500 g. The pharmacokinetics for LEV in neonatal populations is yet to be clearly understood. In this study, we developed and validated a method for determination of LEV in plasma by liquid chromatography tandem mass spectrometry for the purpose of pharmacokinetic study., Methods: Plasma LEV was spiked with Lamivudine as internal standard before extraction by C18 solid-phase extraction (SPE) cartridge. Chromatography was performed using isocratic elution with mobile phase A: B (10: 90) for 2.0 min with flow rate 0.4 mL/min. The mobile phase was composed of 0.1% formic acid in 10.0 mM ammonium acetate (A) and 100% methanol (B). The injection volume was 1.0 μL and the total run time was 2.0 min. Multiple reaction monitoring (MRM) with electro spray in positive mode was used. The mass transition for LEV was 171.2/126.0 and 230.0/112.0 for IS with retention time of 0.73 and 0.72 min, respectively., Results: A calibration curve range from 0.50-80.0 μg/mL was obtained with a correlation coefficient >0.99 in the quadratic model. Precision and accuracy was within the acceptable range and the intra- and inter-day %CV for three concentrations of QCs were <10%., Conclusion: This method was reliable, accurate and applicable for LEV pharmacokinetic study in neonates with seizure., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: The incidences of thromboembolism (TE) in neonates were reported to be around 0.51 per 10,000 live births per year for overall TE and 24 per 10,000 NICU admissions per year. As the incidences of TE in children and adults are lower in Asian populations, the incidences, risk factors, and outcomes of neonatal TE may be different to those reports from other countries., Objectives: To determine the incidences, risk factors, and outcomes of neonatal TE in a tertiary care hospital in Thailand., Materials and Methods: A retrospective study between the years 1998 and 2015., Results: From a total of 2463 neonatal admissions, 28 patients were diagnosed with TE. The female/male ratio was 1:1.2. The breakdown of diagnoses of neonatal TE were arterial ischemic stroke (AIS; 36%), arterial TE (ATE; 29%), deep vein thrombosis (DVT; 14%), cerebral venous sinus thrombosis (CVST; 11%), renal vein thrombosis (RVT; 3%), and purpura fulminans (2%). Underlying diseases were identified 57.1% of patients. The most common thrombophilic risk factor was protein C (PC) deficiency (14.3%). The overall mortality rate was 14.3%., Conclusion: The most common TE was AIS. PC deficiency was the most prevalent inherited risk factor, especially in neonates without precipitating factors.

Neurostimulation can be an alternative treatment for medically intractable epilepsy, especially when the resective surgery could not be performed. The author reported a case of 19-year-old, right-handed male patient who had a history of intractable epilepsy for 11 years after post viral encephalitis associated with status epilepticus. Following the failure of antiepileptic medications and then resective surgery, anterior thalamic deep brain stimulation (DBS) was performed. Indirect targeting of anterior thalamic nuclei could not be used because of asymmetric brain shift from prior multilobar resections. Direct targeting of anterior thalamic nuclei from MRI T1 sequence, Short Tau Inversion Recovery (STIR) sequence combined neurophysiological mapping by microelectrode recording were used as a technique for implantation of DBS electrodes. The stimulation was turned on with 145 Hz, pulse width 90 microseconds, 5 volts with cycling mode 1 minute “on” and 5 minutes “0ff”. The antiepileptic medications continued the same as pre-operative state. Sixty percent seizure reduction was achieved in 24 months after surgery. There were no side effects of DBS during the follow-up period. Anterior thalamic DBS can be performed safely with satisfactory seizure outcomes. Direct targeting of anterior thalamic nuclei combination with microelectrode recording can be very helpful, especially when asymmetric basal ganglion structures were detected.

Background: Intravenous levetiracetam is an option for treatment of status epilepticus (SE) and acute repetitive seizures (ARS). However, there have been relatively few studies with children and adolescents. Also, an appropriate dosage has yet to be determined., Aim: This study investigated the safety and the efficacy of levetiracetam for intravenous treatment of convulsive status epilepticus and acute repetitive seizures in children and adolescents., Method: Retrospectively, the study reviewed the medical records of 19 male and 31 female patients under 18 years of age who had received intravenous levetiracetam treatment either for acute repetitive seizures or for convulsive status epilepticus. The patients were admitted between April 1st, 2010 and December 31st, 2011 to the Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. Data were collected on underlying illnesses, etiology of seizures, indication for levetiracetam therapy, initial dosage, rate of infusion, untoward effects during infusion and emerged complications. Efficacy of treatment was defined as the termination of seizure within 30 min of completing levetiracetam infusion and no seizure recurrence within 6 h of initial treatment., Results: The age range of the 50 patients was from one day to 18 years (mean 79.6 months). The analysis included 52 episodes of 34 acute repetitive seizures (63.4%) and 18 convulsive status epilepticus (34.6%). Infusion rates ranged from 2 to 66 mg/kg/min (mean 29.6). Cessation of seizure was obtained in 59.6% of 52 episodes. Patients with underlying drug resistant epilepsy did not respond to levetiracetam therapy as well as patients with other etiology of seizures. There were no adverse drug reactions or untoward effects observed during the therapy., Conclusion: Intravenous administration of levetiracetam is safe and effective for treatment of acute repetitive seizures and convulsive status epilepticus in children and adolescents. Failure of treatment may be related to underlying drug resistant epilepsy. Further study of appropriate initial dosage and pharmacokinetic variations in the patients is needed as possible explanation of the unresponsiveness., (Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Objectives: To describe sleep-disordered breathing (SDB) in young boys with Duchenne muscular dystrophy (DMD) and its relationship with pulmonary function tests (PFTs)., Study Design: This retrospective study examined diagnostic polysomnogram and PFT data of boys younger than 18 years with DMD and treated with steroids. Spirometry, respiratory muscle strength, body mass index (BMI), sleep architecture variables, and indices of SDB were analyzed. We examined the effect of PFT measures on the risk of each type of respiratory event using logistic regression and have reported results as OR (95% CI)., Results: Subjects included 110 boys with DMD, mean age 11.5 (5.6-17.9) years. Mean (±SD) percent forced vital capacity predicted was 79.5% ± 29.1%. Mean BMI for all subjects was 21.9 ± 7.0 kg/m(2), and mean BMI z-score was 0.65 ± 1.93. Seventy (63.6%) subjects had obstructive sleep apnea; 37 (33.6%) subjects had central sleep apnea; 18 (17%) subjects had hypoventilation. Median (IQR) Apnea Hypopnea Index was 2.9 (1.6-6.9) and median Obstructive Index was 1.5 (0.5-3.8). Obstructive Index during rapid eye movement sleep positively correlated with BMI (r = 0.33, P = .002), BMI z-score (r = 0.22, P = .04), and age (r = 0.31, P = .004). Lower forced vital capacity was associated with increased risk of hypoventilation (OR 0.8, P = .001)., Conclusion: SDB is common in young boys with DMD treated with steroids. It is manifest with rapid eye movement-obstructive sleep apnea, often severe, and strongly influenced by BMI., (Copyright © 2015. Published by Elsevier Inc.)

This retrospective study examined the efficacy and safety of zonisamide for Thai children and adolescents with intractable seizures. The medical records of 24 patients (13 male, 11 female), aged 2 to 18 years (median 11.5, mean 10.4) who received zonisamide were reviewed. The underlying illness, etiology of epilepsy, seizure types, previous and concomitant antiepileptic drugs, dosage, and adverse effects of the drug were collected. Zonisamide's efficacy was evaluated on the basis of seizure reduction rates. At final evaluation, 7 patients were still taking zonisamide from 4.7 to 10.3 mg/kg/d (median 8). One patient became seizure-free and the other 6 experienced favorable seizure control. The median duration of zonisamide therapy was 23.75 months (range 20.5-25 months). Minor adverse effects were reported in 41.6% of patients during the first 3 months of therapy. Zonisamide is an option for the treatment of intractable seizures with favorable seizure control in children and adolescents., (© The Author(s) 2014.)