Taner Sezer

Chloral hydrate versus hydroxyzine HCL for sedation prior to pediatric sleep EEG recording

Objective: The sleep electroencephalogram (EEG) can reveal certain epileptiform activity patterns and facilitate localization of the focus. Sedation is often required for sleep EEG recording in pediatric patients, but there is no consensus on the optimal sedative. Hydroxyzine HCL (HH) and chloral hydrate (CH) are popular sedatives, but HH is rarely used for pediatric sleep EEG recording. The goal of this prospective study was to compare CH to HH for sleep induction efficacy, safety and effects on pediatric sleep EEG pattern. Research design and methods: A total of 282 children (age 4−9 years) referred to our sleep EEG laboratory and requiring sedation were randomly assigned to two groups: the CH Group (n = 141) received 50 mg/kg CH and the HH group (n = 141) received 1 mg/kg HH. If sedation was unsatisfactory, a second equal dose of the same sedative was administered 30 min later. Results: Sleep induction was less successful in the HH group compared to the CH group (p < 0.001). Sleep onset latency was significantly longer in the HH group (p < 0.01) and the proportion of HH group patients requiring a second sedative dose significantly higher (p < 0.01). There was no significant difference in the proportion of patients exhibiting epileptiform activity on the EEG or in adverse event rate between groups. Conclusion: CH was a superior sedative compared to HH owing to more rapid and successful sleep induction with no increase in adverse events.

Hyperventilation during routine electroencephalography: are three minutes really necessary?

OBJECTIVE Hyperventilation induces absence seizures in children with absence epilepsy, and routine electroencephalography studies include three minutes of hyperventilation. We studied the duration of hyperventilation required to provoke a first absence seizure to determine whether three minutes of the procedure are indeed necessary. METHODS Electroencephalography records of children who experienced absence seizures during hyperventilation were reviewed. The time from hyperventilation onset to a first and further seizure(s) was measured, and the occurrence of absences during the posthyperventilation phase was also noted. RESULTS Sixty-two studies were evaluated. Mean time from hyperventilation onset to a first absence was 52 seconds (median 32 seconds). The vast majority (85.5%) had an absence within 90 seconds. Most (68%) children sustained a single event. All eight children with posthyperventilation seizures had experienced at least one event during hyperventilation. CONCLUSIONS Our findings suggest that current guidelines for routine pediatric electroencephalography recording requiring three minutes of hyperventilation may not be clinically necessary. We found that the vast majority of children referred for suspected absence seizures experience a seizure less than 90 seconds after hyperventilation onset, and even more so by 120 seconds. Hence, a larger prospective study is warranted to establish more accurate hyperventilation duration parameters. We also suggest that once an absence seizure has been recorded at any time during hyperventilation, this procedure could be stopped, thus reducing the amount of discomfort for the child.

A New Approach to the Prophylaxis of Cyclic Vomiting: Topiramate

Background/Aims The aim of this study was to compare the efficacy and tolerability of topiramate and propranolol in preventing pediatric cyclic vomiting syndrome. Methods A retrospective medical-record review of patients who underwent prophylaxis after receiving a diagnosis of cyclic vomiting syndrome was performed. Patients who completed at least 12 months of treatment were included in the analysis. Responder rate, and adverse-event rates were also calculated from all patients. Response to treatment was assessed as the total number of vomiting attacks per year. Patients in whom the frequency of vomiting attack reduced greater or equal to 50% were defined as responders, and the remaining patients were classified as nonresponders. Results A total of 38 patients who were treated prophylactically with either topiramate (16 patients) or propranolol (22 patients) were identified. Fifty-nine percent of the patients in the propranolol group and 81% of the patients in the topiramate group reported freedom from attacks. A decrease of more than 50% in attacks per year occurred in 23% of patients in the propranolol group and 13% of patients in the topiramate group. The responder rates were 81% for propranolol group and 94% for topiramate group (P = 0.001). Despite minor adverse effects (drowsiness, nervousness, and dizziness) observed in a few patients, the adverse event rates were not significantly different between the 2 groups (P = 0.240). Conclusions The efficacy of topiramate was superior to propranolol for the prophylaxis of pediatric cyclic vomiting syndrome.

Serum S100B levels in children with simple febrile seizures

PURPOSE Recent studies have found that S100B is a useful marker for astroglial activation seen in various neurologic disorders. The purpose of this study was to evaluate whether simple febrile seizures (SFS) was associated with an elevation in serum S100B levels. METHODS In this study the samples consisted of 39 patients with SFS ranging from 6 to 36 months of age, and age-matched and sex-matched controls including 30 patients with fever and 30 healthy subjects. Two serum samples were obtained for S100B from the study group at 0-1h and 6-24h following seizure. Serum samples were drawn once in the control group. The serum samples were then analyzed using ELISA. RESULTS In the study group, the mean values of the serum S100B concentrations at 0-1h and 6-24h were 32.6±7.8pg/ml and 32.1±5.8pg/ml, respectively, while the concentrations were 32.1±8.8pg/ml and 29.5±7.8pg/ml in the control groups. No significant differences were detected in serum S100B levels at 0-1h or 6-24h in the study when compared to the control groups. CONCLUSIONS These results suggest that SFS do not raise serum S100B concentration above the normal range.

A randomized trial comparing amitriptyline versus topiramate for the prophylaxis of chronic daily headache in pediatric patients

Objective: the goal of this prospective and double-blind study was to compare the efficacy of amitriptyline and topiramate for the prevention of pediatric chronic daily headache (CDH). Research design and methods: fifty-seven children (aged 9−16 yr) diagnosed with CDH were randomly assigned to two groups: group A (n = 29 patients) received amitriptyline 0.5 mg/kg/d and group B (n = 28 patients) received topiramate 25 mg/d increasing up to 100 mg/d according to patient response. Treatment response was monitored for at least 4 months. Results: fifty-five percent of the patients in group A responded to amitriptyline and 61% of patients in group B responded to topiramate as defined by a reduction of more than 50% in monthly headache frequency. There was no significant difference in responder rate or adverse event rate between the two groups (p > 0.05). By the end of the 4-month treatment period, there were no significant differences in the final average severity and monthly frequency of headaches between treatment groups. Conclusion: these results suggest that the efficacy and tolerability of topiramate is equivalent to that of amitriptyline for reducing the frequency of headache in pediatric CHD patients.

The Prevalence of Celiac Disease in Children With Arterial Ischemic Stroke.

Objective: The association between arterial ischemic stroke (AIS) and celiac disease (CD) has been described in only a few cases in adults and children. We aim to determine the prevalence of CD in children and adolescents with AIS. Study Design: We investigated serum levels of tissue transglutaminase antibody immunoglobulin (Ig)A and total IgA from 76 children with AIS and in a healthy control group of 102 children. Study participants who were positive for tissue transglutaminase IgA antibodies underwent a duodenal biopsy. Results: A total of 2 patients in the AIS group (2.26%) and 2 in the control group (1.96%) had positive serum tissue transglutaminase antibody (P=0.89; 95% confidence interval, −5.05 to 6.89). Duodenal biopsy confirmed CD in only 1 patient who had AIS. Conclusions: In the present study, children with acute arterial stroke did not exhibit a higher prevalence rate of CD compared with healthy controls. Therefore, the screening test for CD is not a necessary part of the management of AIS in children. However, cases of recurrent AIS could be examined for CD.

Is Celiac Disease an Etiological Factor in Children With Migraine

To determine the prevalence of celiac disease in children and adolescents with migraine, the authors investigated serum levels of tissue transglutaminase antibody immunoglobulin A and total immunoglobulin A from 81 children with migraine and in a healthy control group of 176 children. Study participants who were positive for tissue transglutaminase immunoglobulin A antibodies underwent a duodenal biopsy. Two patients in the migraine group (2.5%) and 1 in the control group (0.57%) tested positive for serum tissue transglutaminase immunoglobulin A antibodies (P > .05). Duodenal biopsy did not confirm celiac disease in both groups, and these patients were considered “potential celiac” cases. In the present study, children with migraine did not exhibit a higher prevalence rate of celiac disease compared with healthy controls. Therefore, the screening test for celiac disease is not a necessary part of the management of migraine in children.

Novel Deoxyguanosine Kinase Gene Mutations in the Hepatocerebral Form of Mitochondrial DNA Depletion Syndrome

Deoxyguanosine kinase (DGUOK) gene mutations have been identified in the hepatocerebral form of mitochondrial DNA depletion syndromes. We report here clinical and laboratory features of 3 infants with novel DGUOK gene mutations, c.130G>A (Glu44Lys), c.493G>A (Glu165Lys), and c.707+3_6delTAAG.

A Case of Shwachman–Diamond Syndrome who Presented with Hypotonia

We present a patient with failure to thrive and severe hypotonia, who was initially suspected of having a neurometabolic disease but later diagnosed as Shwachman-Diamond syndrome (SDS), which was genetically confirmed. SDS is a multisystemic disease, which is characterized by exocrine pancreatic deficiency, bone marrow dysfunction with increased risk for malignant transformation, and skeletal abnormalities. It should be included in differential diagnosis of patients with failure to thrive and unexplained neurodevelopmental delay with neutropenia.

Neonatal cerebral sinovenous thrombosis: two cases, two different gene polymorphisms and risk factors

Turan Ö, Anuk-İnce D, Olcay L, Sezer T, Gülleroğlu K, Yılmaz-Çelik Z, Ecevit A. Neonatal cerebral sinovenous thrombosis: Two cases, two different gene polymorphisms and risk factors. Turk J Pediatr 2017; 59: 71-75. Cerebral sinovenous thrombosis (CSVT) is a rare disease in the neonatal period and also the greatest risk of neonatal mortality and morbidity. In this report, we presented two cases with CSVT and different risk factors. One of these cases had methylenetetrahydrofolate reductase (MTHFR) C677T homozygous polymorphism and the other case had both MTHFR A1298C homozygous polymorphism, plasminogen activator inhibitor-1 (PAI-1) 4G/ 5G polymorphism and elevated lipoprotein a. Early diagnosis and prompt initiation of therapy of neonatal CSVT may prevent neonatal mortality and poor long-term neurodevelopmental outcomes.