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Aim: The purpose of this study was to examine the effects of antiepileptics on thyroid function tests and to compare these effects among different antiepileptics. Materials and Methods: Two hundred and twenty patients (102 female and 118 male) aged 1-17 years indicated for antiepileptic drug (AED) therapy for epilepsy were enrolled in this study which was performed in a child neurology clinic between January 2014 and January 2018. Those patients using a single AED and with complete seizure control were included. In this study period, according to the local protocol, we measured free thyroxine (fT4) and thyroid-stimulating hormone (TSH) levels at the beginning of treatment and at the 12th month of AED therapy. Results: The mean age of the patients was 10.2 +/- 4.4 years. TSH elevation was observed in only eight patients. These eight patients' thyroid autoantibodies were negative and their thyroid ultrasonography were normal. Subclinical hypothyroidism (TSH: 5-10, fT4 normal) was present in three of these eight patients, and they were therefore not started on medication. The other five were started on L-thyroxine. Four of these were using valproic acid and one was using carbamazepine. We found no significant difference between TSH and fT4 levels measured before the start and at the 12th month of drug therapy, nor among the different AEDs used. Conclusion: AEDs have no marked effects on thyroid function, and may therefore be safely used from that perspective. WOS:000718894700005

It has been known for several decades that epilepsy and autism spectrum disorders (ASD) are related to each other. Epilepsy frequently accompanies ASD. The purpose of this study was to investigate relationship between clinical and electroencephalogram (EEG) findings in ASD patients and to identify EEG characteristics that may create a disposition to epilepsy in ASD by examining differences in clinical and EEG findings between patients diagnosed with ASD without epilepsy and ASD with epilepsy. A total of 102 patients aged 2 to 18 years and diagnosed with ASD based on Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) diagnostic criteria between January 2017 and June 2019 were included in the study. Patients were assigned into two groups: (1) ASD with epilepsy and (2) ASD without epilepsy. Clinical findings were retrieved from patients' files, and EEG findings from first EEG records in the EEG laboratory at the time of diagnosis. EEG findings were defined as central, parietal, frontal, temporal, or generalized, depending on the location of rhythmic discharges. The incidence of epilepsy in our ASD patients was 33.7% and that of febrile convulsion was 4%. Generalized motor seizures were the most common seizure type. Epileptic discharges most commonly derived from the central and frontal regions. These abnormalities, especially frontal and central rhythmic discharges, may represent a precursor for the development of epilepsy in ASD patients.

Aim: In this study, we aimed to investigate the etiological factors, electroencephalographic (EEG) findings, rates of response to iron therapy, and factors affecting response to iron therapy in children diagnosed with breath-holding spells (BHS). Methods: The study included 136 children aged 1 to 48 months who received iron therapy after a BHS diagnosis at our pediatric neurology clinic between November 2015 and No¬vember 2019. Patient medical records (physical examination, laboratory and EEG findings, medical history, and effectiveness of iron therapy) were reviewed retrospectively. Results: Of all patents, 81 (59.6%) exhibited partial response (partial remission) to iron therapy (50% decrease in BHS frequency), 52 (39%) responded completely (complete remission), and 2 were unresponsive. Comparison of the patients with complete and partial remission revealed a higher rate of complete remission in girls. In addition, patients with complete remission had higher levels of hemoglobin, MCV, and ferritin than those with partial remission. Complete remission rates were also higher in patients with normal EEG findings. Conclusion: BHS in childhood is a benign, recurring, and non-epileptic disorder and its differentiation from epilepsy is important. Children with BHS respond well to iron therapy, which can be recommended even if the serum iron and ferritin levels are normal.

Background Increasing evidence suggests that vasoactive neuropeptides such as pituitary adenylate cyclase-activating polypeptide-38 (PACAP-38), substance P, calcitonin gene-related peptide, and vasoactive intestinal peptide are involved in the pathophysiology of migraine in adults, but their role in pediatric migraineurs remains unclear. We prospectively investigated plasma levels of these vasoactive neuropeptides in pediatric migraine patients without aura and compared the results with those of age-matched healthy controls. Methods Thirty-eight children aged 6–18 years with migraine without aura and 20 age-matched control subjects were included in the study. Neuropeptides in plasma samples from the controls, and in either the ictal or interictal periods in pediatric migraine without aura, were measured using ELISA. Results PACAP-38 and vasoactive intestinal peptide levels in both ictal and interictal plasma were higher in the patients with pediatric migraine without aura than in the controls ( p Conclusions This study demonstrates increased plasma PACAP-38 and vasoactive intestinal peptide levels, but not calcitonin gene-related peptide and substance P levels, in pediatric patients with migraine during both attack and attack-free periods. The study findings suggest that PACAP-38 and vasoactive intestinal peptide may be implicated in the pathophysiology of migraine, particularly in pediatric migraineurs.

The aim of this study was not only to emphasize the role of clinical signs as well as ophthalmologic evaluation for accurate and differential diagnosis of papilledema (PE), but also to present an instructive algorithm that would help to eliminate unnecessary examinations and treatments. The files of 43 patients (ages 0–18) diagnosed with PE were retrospectively reviewed. The study included 25 patients from our pediatric neurology outpatient clinic, who were thought to have PE, and 18 patients, who were referred from the external centers to our hospital with a pre-diagnosis of PE. Of the 43 patients, 28 had PE, 8 had pseudopapilledema (PPE), and 7 had optic nerve pathologies (ONP). For patients who applied directly to our pediatric neurology unit, a margin of error of 8% was detected based on only a simple ophthalmologic examination and an evaluation of clinical findings. For the patients who were forwarded to our pediatric neurology unit from the external centers without examining any clinical findings and with no details, the margin of error was 72%. For patients with suspected PE, advanced ophthalmologic opinion is a necessary requirement before invasive radiological examinations are used. When the ophthalmologic evaluation is properly elaborated, the distinction can be made more clearly by using noninvasive methods. In order to determine the gold standard in terms of the methods used in the evaluation of patients who are not clinically diagnosed, new prospective studies with more patients should be planned.

Background Decreased vitamin D levels lead to an increase in infectious diseases, including urinary tract infections (UTIs). Objective To assess serum vitamin D levels in children with renal parenchymal injury secondary to UTIs. Methods Forty-three upper UTI patients and 24 controls, aged 1–15 years, were included. Vitamin D levels and other laboratory tests were obtained when they first admitted to hospital. 99mTc-labeled dimercaptosuccinic acid (DMSA) scans were performed to evaluate renal parenchymal injury. Results Mean serum 25-hydroxyvitamin D (25(OH)D) was lower in the upper UTI group compared to the control group [18 (SD 9) vs. 23 (SD 10.6) ng/mL, respectively; P=0.045]. The upper UTI group was sub-divided into two groups, those with 22 (51.1%) and without 21 (48.8%) renal parenchymal injury. Mean 25(OH)D was significantly lower in patients with renal parenchymal injury [15.1 (SD 7.1) vs. 21 (SD 9.9) ng/mL, respectively; P=0.03]. The renal parenchymal injury cases were further sub-divided into two groups: 8 patients (36.3%) with acute renal parenchymal injury and 14 (63.6%) with renal scarring (RS), but there was no significant difference in 25(OH)D between these two groups [12.5 (SD 8.9) vs. 16.6 (SD 5.7) ng/mL, respectively; P=0.14). Conclusion Decreased vitamin D is associated with renal parenchymal injury in children with upper UTIs. However, vitamin D is not significantly decreased in renal scarring patients compared to acute renal parenchymal injury patients.

Hot water epilepsy (HWE) is a subtype of reflex epilepsy in which seizures are triggered by the head being immersed in hot water. Hot water or bathing epilepsy is the type of reflex epilepsy most frequently encountered in our clinic. We describe our patients with HWE and also discuss the clinical features, therapeutic approaches, and prognosis. Eleven patients (10 boys, 1 girl), aged 12 months to 13 years, admitted to the pediatric neurology clinic between January 2018 and August 2019, and diagnosed with HWE or bathing epilepsy based on International League Against Epilepsy (ILAE)-2017, were followed up prospectively for similar to 18 months. Patients' clinical and electroencephalography (EEG) findings and treatment details were noted. All 11 patients' seizures were triggered by hot water. Age at first seizure was between 2 months and 12 years. Seizure types were generalized motor seizures, absence, and atonic. EEG was normal in two patients, but nine patients had epileptiform discharges. Magnetic resonance imaging of the brain was performed and reported as normal (except in one case). Histories of prematurity were present in two patients, unprovoked seizures in one, and low birth weight and depressed birth in the other. Patients with HWE have normal neuromuscular development and neurological examination results, together with prophylaxis or seizure control with a single antiepileptic drug, suggesting that it is a self-limited reflex epilepsy. WOS:000571792200003 2-s2.0-85091574351 PubMed: 32294767

The aims of our study were to refer to the complex relationship between idiopathic intracranial hypertension (IIHT) and cerebral sinovenous thrombosis (CSVT), and to determine the differences and commonalities between the patients with and without CSVT in their etiology, along with documenting the uncertainties in concluding on the diagnosis and treatment of these patients.IIHT was diagnosed according to Dandy criteria, while CSVT was screened for by way of a cranial magnetic resonance imaging for all patients and cranial magnetic resonance venography only if the magnetic resonance imaging was nebulous or there was a family history.We retrospectively evaluated a total of 26 patients (9 of whom had CSVT) diagnosed with IIHT between 2014 and 2018. A total of 9 patients with concurrent CSVT were described as suffering from vascular IIHT, while the remaining 17 were described as suffering from other IIHT. Demographic characteristics were similar in both groups (mean age: 12 vs. 11; male/female ratio: 2/7 vs. 5/12 in vascular IIHT and other IIHT, respectively). Clinical findings, cerebrospinal fluid-opening pressure values, and pathologies of etiology were also similar (vitamin D deficiency: 66% vs. 52%; vitamin B12 deficiency: 11% vs. none; iron deficiency: 22% vs. 11%; obesity: 22% vs. 23%). A mixture of acetazolamide, topiramate, anticoagulant therapy, and acetylsalicylic acid were given according to the diagnoses.CSVT is a common clinical entity among the causes of IIHT, and it should be taken into consideration in this patient group. However, there is a need for a common guideline for laboratory and imaging methods to understand the etiopathogenesis of childhood IIHT and determine the patients at risk.

There is a paucity of data on the effects of hyperoxia-induced brain damage on learning and such psychosocial phenomenon as anxiety. Preterm infants encounter hyperoxia within a relatively early stage of life (leaving the intrauterine environment earlier than was expected) and are exposed to high-level hyperoxic stress due to the insufficiency of their antioxidant defense mechanisms. In an experimental rat model, we investigated the effects of early postnatal hyperoxia on learning, anxiety, and depression in the early adulthood period. Rat nestlings (n = 7) were exposed to about 80% oxygen for the first 5 days after birth to create a rat model of hyperoxia, and these nestlings and those of the control group (n = 7) were subjected to behavioral tests (Morris water tank, open-field test, elevated plus maze, and Porsolt test) at 30 days old. Video recordings of the tests were captured, and indices of the tests in the experimental groups were compared with the Mann–Whitney U-test. In the Morris water navigation task, the latency and distance required to locate the platform were greater (P = 0.018 and 0.025, respectively) in the hyperoxia group than in the control group, suggesting that exposure to hyperoxia during the development of the brain can exert a negative effect on the learning function. There was no difference in the time spent in the open center area of the open-field test (anxiety), while the rats in the hyperoxia group spent more time in the enclosed area in the elevated plus maze test, suggesting a higher level of anxiety (P = 0.048). In the Porsolt test, rats in the hyperoxia group moved faster (P = 0.013) and travelled a longer distance (P = 0.048). Although this finding suggests less depressive behavior in the mentioned group, which was contrary to the expectations, this may also explain the tendency of preterm infants to hyperactivity at later ages. Thus, it has been demonstrated experimentally that exposure of neonates to oxygen exceeding physiological needs may cause behavioral problems, such as impaired learning, anxiety, and hyperactivity.

Aim: Migralepsy is a clinical entity that occasionally represents a diagnostic problem. An apparent history and clinical manifestation of migraine may mask the epileptic attack accompanying migralepsy. The aim of this study is to present our experience with clinical and electroencephalography (EEG) findings and treatment of our patients diagnosed with childhood migralepsy disease.Methods: We documented six patients who were initially followed-up with a diagnosis of migraine, subsequently observed to have epileptic seizures, and then diagnosed with migralepsy.Result: Our patients became asymptomatic by giving good responses to antiepileptic therapy based on clinical and electroencephalography (EEG) findings.Conclusions: This case series shows that EEG recording can be useful in all stages of migraine for long-term, safe monitoring. Identifying patients with possible migralepsy will enable them to receive antiepileptic treatment.

The aim of this study is to examine the factors affecting seizure recurrence in pediatric patients diagnosed with epilepsy. Three hundred patients presenting to the pediatric neurology clinic between 2015 and 2018 and diagnosed with epilepsy and treated with single antiseizure drug were included in the study. Medical histories and clinical and laboratory findings were retrieved retrospectively from the hospital data system. The combined and adjusted effects of risk factors on seizure recurrence were evaluated using multivariate binary logistic regression analysis. Boys had a higher rate of seizure recurrence than girls. Seizure recurrence was also higher in patients with abnormal neurological examinations at the time of diagnosis compared to those with normal neurological examinations. Seizure recurrence was significantly higher in patients with global growth retardation. Epilepsy patients with abnormal MRI findings also had a higher rate of seizure recurrence than patients with normal neuroimaging findings. In addition, seizure recurrence was significantly higher in epilepsy patients with comorbidities such as cerebral palsy and autism spectrum disorders compared to patients without comorbidities. No significant association was observed between seizure recurrence and the first drug, perinatal asphyxia history, localization of epileptiform discharges on EEG, family history of epilepsy, family history of febrile seizures, history of stay in the neonatal intensive care unit during the perinatal period, or preterm delivery. Abnormal neurological examination, abnormal neuroimaging and accompanying comorbidities, and global growth retardation at the time of diagnosis are important factors affecting seizure recurrence in pediatric patients with epilepsy.

Amaç: ACTH tedavisi alan West sendromlu hastalarımızın ileri dönemdeki nörogelişimsel prognozu ve nöbet prognozunun saptanması ve bunlara katkı sunan faktörlerin belirlenmesidir.Gereç ve Yöntem: Kliniğimizde 3-24.aylarında West sendromu tanısı alarak ACTH tedavisi uygulanan 34 hastanın demografik faktörleri, nöbet şekilleri, öncesindeki nöbet öyküleri, ACTH kullanımı ve yanıt süreleri, etyolojik faktörlerini belirledik. Bunların nörolojik gelişim ve nöbet prognozuna etkisini araştırdık.Bulgular: Hastaların tanı öncesinde nöbeti olmasının ilerideki nöbet prognozu ile ilişkisini anlamlı bulduk. Ayrıca semptomatik etyolojili hastalarda ACTH yanıtını daha geç; nörogelişim ve nöbet prognozunu ise daha kötü saptadık. Tüm hastaların %76’ sında global developmental delay, % 62’sinde antiepileptik ilaclara rağmen devam eden nöbetler belirledik.Sonuç: West sendromunda semptomatik etyolojik faktörler nörogelişimsel süreci ve ileriki dönemdeki nöbet prognozunu olumsuz etkilemektedir., Objective: To determine neurodevelopmental and seizure prognoses in our patients with West syndrome receiving adrenocorticotropic hormone (ACTH) therapy, and to identify the factors affecting these.Materials methods:We determined the demographic factors, previous seizure histories, ACTH use and response times, and etiological factors of 34 patients diagnosed with West syndrome in our clinic at 3-24 months and receiving ACTH therapy. We also investigated their neurological development and its effect on seizure prognosis.Results: We found a significant relationship between patients experiencing seizures before diagnosis and subsequent seizure prognosis. We also found a later response to ACTH and poorer neurodevelopmental and seizure prognoses in patients with symptomatic etiologies. Global developmental delay was determined in 76% of all cases, and seizures persisted despite antiepileptic drugs in 62%.Conclusions: Symptomatic etiological factors in West syndrome adversely affect the neurodevelopmental process and subsequent seizure prognosis.

Aim: Preterm infants encounter hyperoxia relatively early on as they leave the intrauterine environment earlier than expected, while also being exposed to a higher level of hyperoxic stress due to insufficiencies in their antioxidant defense mechanisms. With that in mind, we investigate whether running exercises performed during pregnancy can contribute to the development of tolerance to neonatal hyperoxic brain damage. Method: While two female rats maintained a sedentary pregnancy, one female rat performed the mandatory running exercise for 30 minutes for five days a week throughout the pregnancy. Following delivery, the sedentary rats and the exercised rat were kept together with their offspring for five days at oxygen concentrations above 80 percent in order to induce brain damage. The offspring were sacrificed on postnatal Day 7 and brain/body ratio measurements were obtained. Results: The brain/body ratios in the control, hyperoxia and exercise-hyperoxia groups were found to be median (IQR) 0.074(0.68-0.77), 0.065(0.06-0.067) and 0.064(0.060-0.068), respectively. The brain/body ratios of the offspring of the mothers in the hyperoxia group were found to be significantly lower than the control group (p=0.002), irrespective of exercise (p=0.007). No statistically significant difference was noted between the offspring of the sedentary and the exercised mothers in the hyperoxia group (p=0.94). Conclusion: Hyperoxia was found to result in lower brain mass relative to total body mass. This finding, which indicates the presence of microcephaly, reflects the negative effects of hyperoxia on brain development. Contrary to expectations, exercises performed during pregnancy had no significant effect on the brain/body weight ratio of the offspring.

The purpose of this study was to determine the etiologic factors, clinical characteristics, seasonal distributions, family history, response to corticosteroid therapy, recurrence and residual paralysis rates, and factors affecting these in pediatric facial palsy. Patients aged

This study evaluates the role of obesity, overweight and vitamin D deficiency in primary headaches in childhood. This retrospective observational study included pediatric patients aged 5–17 years admitted to the pediatric neurology clinic with headaches between January 2015 and August 2018 and diagnosed with primary headache based on ICHD III-beta criteria. The control group consisted of healthy children without headache admitted to the pediatric outpatient clinic for check-ups before engaging in athletic or school activities. The control and patient groups were at the same risk of low 25(OH)D3 levels. The study population was divided into three groups—patients with migraine (group A), patients with tension-type headache (TTH) (group B) and the control group (group C). Participants’ demographic data, medical histories, physical examination findings and laboratory results were retrieved retrospectively from the patient charts. BMI was significantly higher in patients with primary headache, the risk of primary headache increasing in patients with a BMI in excess of 25. Comparison of the patients with primary headache and the control group revealed lower 25(OH)D levels in the primary headache group, although the difference was not statistically significant. Girls with primary headache had significantly lower 25(OH)D levels than boys. A relationship may be present between overweight, obesity and primary headache, while female gender may be suggested as a negative factor for primary headache. Patients should be advised to lose weight if BMI indicates overweight or obesity.

Purpose: The objective of this study was to determine risk factors for epilepsy and drug-resistant epilepsy (DRE) development in children with cerebral palsy. Method: Two hundred twenty-nine patients presenting to the pediatric neurology clinic and diagnosed as having cerebral palsy between November 2016 and November 2019 were included in the study. Medical histories and clinical, laboratory, and radiological findings were examined retrospectively from patient records in the hospital data system. Results: Girls represented 103 patients (45%) and boys 126 (55%). The patients' mean age was 8.39 +/- 4.54 years. Epileptic seizures were present in 120 (52.4%) patients and drug-resistant seizures in 64 (27.9%). The risk of epilepsy was significantly higher in patients with motor or speech impairment, with hearing impairment, or undergoing first seizure in the neonatal period. We also observed a higher risk of epilepsy in patients with psychiatric comorbidity, particularly autism spectrum disorder. The risk of epilepsy was also higher in patients with microcephaly or quadriplegic cerebral palsy and in patients with focal and generalized epileptiform abnormality on electroencephalograms (EEGs). However, no significant difference was identified when all these factors were evaluated in terms of the risk of developing DRE. Conclusion: Patients with cerebral palsy have high comorbid epilepsy rates. We think that the risk of epilepsy may be higher in patients undergoing first seizure in the neonatal period, with microcephaly, with quadriplegic type cerebral palsy, and with additional psychiatric comorbidity. The rate of DRE development was very low in patients with normal EEG findings or with only background rhythm abnormalities on first EEGs during neonatal seizures. This may be regarded as a good prognostic factor for nondevelopment of DRE. (C) 2020 Elsevier Inc. All rights reserved. WOS:000588004200031 2-s2.0-85089799848 PubMed: 32858364

Aim: Research into the different treatment methods based on the intrauterine lipopolysaccharide (LPS)-induced Periventricular leukomalacia (PVL) model, as one of the main causes of morbidity in preterm infants still continues to be relevant. The present study investigates the effect on PVL of salidroside obtained from Rhodiola Rosea (golden root, orpin rose), which is a plant with known for its medicinal qualities. Method: To develop an induced PVL model, a 500 microgram/kg dose of LPS (Escherichia coli, serotype 055:B5, Sigma) was applied to two pregnant rats intraperitoneally on day 18, day 19 and day 20 of gestation. One of the LP applied rats was given 25 mg/kg Salidroside (250 mg Rhodiola root extract capsules, which include 3 mg Salidroside) by oral gavage (LPS+Salidroside), and a physiological saline solution was given to the control group. After delivery, 10 offspring of the LPS-applied mother, nine offspring of the LPS+Salidrosideapplied mother and seven offspring of the control mother were sacrificed on postnatal Day 7 with ether anesthesia. The caspase enzyme located in apoptosis pathways of 10 percent neutral-buffered formalin fixed brain tissue was stained immunohistochemically, and apoptotic cells were counted. Results: No statistically significant difference was noted between the LPS+Salidroside group and the control group, while a statistically significant difference was noted between the LPS and LPS+Salidroside groups. It was observed that Salidroside reduced LPS induced apoptosis. Conclusion: The intended experimental neuroprotective effect of Salidroside usage was provided through the inhibition of apoptosis in a PVL-damaged brain.

Aim: The effects of gestational exercise on potential pathological conditions is not known yet. Therefore, in the present study, it was aimed to evaluate the effects of forced running exercise on LPS-induced brain damage in pregnant rats. Method: Pregnant females in the experimental group were forced to exercise 30 min daily for five days a week. Lipopolysaccharide (LPS) induced brain injury model was created by administering 500 µg/kg body weight of LPS on gestational days 18 and 19. To evaluate injury histopathologically, brain tissues were fixed at the postnatal day seven through transcardial perfusion (n=4 pups/group). When the remaining pups reached 30-day of age, Morris water maze test (MWM) was performed to assess memory and learning, open filed (OP) and elevated plus maze (EPM) for testing anxiety, and Porsolt test (PT) for evaluating depression. The groups were defined as brain injury group (BI, n=13) and exercise+brain injury group (E+BI, n=7). Results: The results obtained from MWM test indicated that animals found the platform in a shorter duration and distance at the day five compared to the day three. However, there was no significant difference between the groups. No significant difference was found in OP test regarding the distance traveled, time spent at the margins, movement at the center and the time spent as immobile. However, in the EPM test, the offspring at the BI group displayed higher mobility and increased number of entry to the open arms compared to the E+BI groups (p=0.01). There was no significant difference regarding mobility duration and total distance traveled in the PT test. Conclusion: In the present study, we tested the impact of gestational exercise using the brain injury model. The results of the EPM test suggests that the gestational exercise can suppress the stress factors in the pregnant females with brain injury leading to the prevention of hyperactivity-induced negative learning behavior.

Aim: To determine the relation of echocardiographic subepicardial adipose tissue (SAT) thickness with anthropometric and clinical parameters in pubertal obese children. Subjects and methods: A total of 52 obese pubertal subjects (13.1 +/- 1.56 yr, 27 male patients) and 39 age- and gender-matched lean pubertal subjects (13.0 +/- 1.28 yr, 16 male patients) were included in the study. Serum glucose, lipid profile, and insulin levels were measured during the fasting state. Each subject underwent a transthoracic echocardiography and the SAT thickness was measured during end-diastole from the parasternal long-axis views. Results: The obese pubertal subjects had significantly higher SAT, triceps skin fold (TSF) thickness (mm), waist (WC) and mid-arm circumference (MAC) values (cm) compared with lean pubertal subjects group (p0.05). As an optimal cut-off point, a SAT thickness of 5.25 mm determined IR with 92% sensitivity and 62.1% specificity. Conclusions: Our study showed that SAT thickness in obese pubertal children shows a good correlation with age, SDS-BMI, BMI, WC, MAC, TSF, and HOMA-IR. In addition, our results suggest that SAT thickness might be used as a supportive data for risk stratification of metabolic syndrome in obese children. (J. Endocrinol. Invest. 33: 715-719, 2010) (C) 2010, Editrice Kurtis

The aim of this study was to investigate possible correlations of the cognitive impairment with abnormalities of regional cerebral blood flow and electroencephalogram in children with (Down's Syndrome) DS. Nine patients with DS were evaluated by single photon emission computed tomography (SPECT) in combination with clinical findings, electroencephalography (EEG), and magnetic resonance imaging (MRI). In cases with IQs below 40, there were one or more findings of abnormal EEG/MRI and brain perfusion SPECT. In 6 cases (66.7%) EEG findings were normal, but 3 (33.3%) had abnormal EEG findings. Perfusion abnormalities were most pronounced in the fronto-parieto-temporal region in the form of hypoperfusion (n = 5) and in the right hemisphere (n = 5) than the left hemisphere (n = 1). These findings suggest that the children with DS had varying levels of structural, perfusion, and electrophysiological abnormalities in the brain and these abnormalities were reflected by measurable alterations of the cognitive functions.

Background: Sandifer Syndrome is an uncommon clinical entity characterized by gastroesophageal reflux, irritability and abnormal movements of the body and contortions of the neck. The majority of paroxysmal cases, in particular, tend to show an association with epilepsy. Methods: The clinical, laboratory and 6-month observation results of the four patients (two boys, two girls) have been presented. Results: The 6-month prospective observation/treatment of four patients aged between 2 and 14 months (mean age, 6.5 ± 5.2 months) with a diagnosis of Sandifer Syndrome has been investigated. Due to paroxysmal extensor jerks, two of the patients were misdiagnosed with infantile spasm and they were treated accordingly. In the clinical observations of the patients, abnormal neurobehavioral attacks (irritability, crying, head/eye version, torticollis, extensor spasm and dystonic posture) 5–10 times daily were observed. In two of the patients, motor growth retardation was observed, in one patient, bronchospasm attacks were observed, and in all the patients iron deficiency anemia was observed. The electroencephalograms of the patients which were taken during the routine, sleepless and paroxysmal behaviors were normal; the gastroesophageal scintigraphies were positive in the manner of reflux. Management of the infant with gastroesophageal reflux disease, in addition to nonpharmacological interventions pharmacologic therapy, including metoclopramide HCl and Fe (6 mg/kg per day, oral) was useful for the patients, and their paroxysmal attacks decreased dramatically (0–2 attacks/day). Conclusion: These findings suggest that infants or children with these atypical movements should be evaluated for Sandifer Syndrome. Expensive and comprehensive neurologic examination may be unnecessary. Early diagnosis permits prompt treatment and relief of the problem. Medical management is usually successful.

Fragile X syndrome is an inherited disorder caused by a defective gene on the X chromosome. It is associated with developmental or behavioral symptoms and various degrees of mental retardation. Morphologic abnormalities and altered perfusion of various brain areas can underlie these functional disturbances. The aim of this study was to investigate the cerebral perfusion state in patients with fragile X syndrome using single-photon emission computed tomography (SPECT). Structural and functional assessment was also performed by magnetic resonance imaging (MRI) and electroencephalography (EEG). Eight boys with cytogenetically confirmed fragile X syndrome (mean age 8.8 ± 4.4 years, range 5–18 years), were included. All patients had mental retardation, with a mean IQ of 58.9 ± 8.8 (range 40–68), and additional neurobehavioral symptoms. SPECT revealed cerebral perfusion abnormalities in six patients (75%), most commonly in the frontoparietotemporal area and prominent in the right hemisphere. The SPECT and EEG findings were concordant: hypoperfused areas in SPECT corresponded to regions of persistent slow-wave paroxysms on EEG. On the other hand, cranial MRI was abnormal qualitatively only in two patients (25%) showing cerebellar and vermal hypoplasia and cerebral hemispheric asymmetry. Our results indicate that cerebral perfusion abnormalities, which are correlated with electrophysiologic findings but not necessarily with anatomic abnormalities, can underlie the pathogenesis of the clinical findings observed in fragile X syndrome.

Segmental myoclonus is described as the involuntary contractions of contiguous muscles innerved by the brain stem or by spinal cord. The underlying causes of segmental myoclonus in children are demyelinating diseases and intrinsic tumors. Here, we report a case who was presented with segmental myoclonus on his left arm and later diagnosed as atypical monosymptomatic presentation of acute disseminated encephalomyelitis (ADEM). The case represents the first in the literature in which ADEM is considered as the possible cause of segmental myoclonus. Our findings demonstrate that: (i) in focal movement disorders such as segmental myoclonus, a careful neuroradiological examination of the neuroanatomical region for the possible presence of organic lesions might be rewarding, (ii) ADEM might be one of the potentially reversible causes of myoclonus.

Acute hemorrhagic leukoencephalitis (AHL) is a rare and usually fatal disorder characterized by an acute onset of neurological abnormalities. It may occur in association with a viral illness or vaccination. Radiology and brain biopsy are essential for the diagnosis. We present a case of AHL mimicking or manifesting as intracerebral hemorrhage associated with herpes simplex virus. A 3-year-old boy was admitted to our hospital with cerebral hemorrhage. The findings of cerebrospinal fluid, MRI and electroencephalogram indicated acute hemorrhagic leukoencephalitis associated with herpes simplex virus involving the left parietal cortex (hemorrhage) and the right temporal lobe (encephalitis). The patient received intravenous dexamethasone (1.5 mg/kg/24 h divided q6 (six times a day) for 5 days, tapered slowly to discontinuation over the next 5 days) and aciclovir (15 mg/kg/every 6 h for 14 days). His clinical condition markedly improved, and after 3 weeks he was discharged from the hospital with moderate right hemiparesis. Mild hemiparesis with normal psychometric testing was determined when he was checked 6 months later. AHL remains misdiagnosed as viral encephalitis because it produces different protean clinical forms. Pediatric patients admitted with cerebral hemorrhage and infarct should be evaluated for presence of hemorrhagic encephalitis-causing agents, especially for herpes simplex. Since, as in our case, treatment with corticosteroid and acyclovir markedly improves conditions of herpes simplex-caused AHL, we suggest that early diagnosis and treatment is of importance for the prognosis.

Although controversial, protective and therapeutic effects of valproic acid in various types of cellular injury suggest a potential role for this agent in hypoxic-ischemic brain injury. We therefore investigated the effects of valproic acid in an experimental model of neonatal hypoxic-ischemic brain injury. To examine the effect of valproic acid in this condition, hypoxic-ischemic brain injury was induced in 7-day-old rat pups by ligation of the right common carotid and then the pups were exposed to 1 hour of hypoxia in 8% oxygen. Low (200 mg/kg/day) and high (400 mg/kg/day) doses of valproic acid were administered in a 5-day regimen. Neuropathologic evaluation was performed using the hippocampus, cerebral cortex, and basal ganglia in the coronal plane. The 5-day regimen of valproic acid administration resulted in some protective and therapeutic effects on the brain damage and neuronal apoptosis in both hemispheres in a dose-dependent manner. Administration of valproic acid also decreased the percentage of apoptotic neurons in the contralateral hemisphere ( P < .05). These results suggest that valproic acid can have therapeutic and protective effects in hypoxic-ischemic brain injury. ( J Child Neurol 2005;20:582—587).

PubMedID: 15885323 Immunologic studies in relation to clinical status might help to understand the pathogenesis of subacute sclerosing panencephalitis (SSPE) and the effect of treatment. We measured lymphocyte subsets and intracellular TNF? and interleukin-4 levels in peripheral blood in SSPE patients. Patients had elevated percentages of CD8+ cells compared to age-matched control children. Rapidly progressive course was associated with increased CD4+ cells. Treatment with interferons and inosiplex altered the percentage of CD3+, CD4+ and CD19+ cells. TNF? and interleukin-4 levels had no correlation with course or treatment. The proportions of lymphocyte subsets appear to have a role in the evolution or manifestations of SSPE, if not in the pathogenesis. © 2005 Elsevier B.V. All rights reserved. This study was supported by the research grant HEK 01/52 from Hacettepe University, Turkey. Banu Anlar did scientific consultancy for Newport Pharmaceuticals, Ireland.

Cortical visual impairment (CVI) most commonly occurs in children after hypoxic insults but may also occur as a sequel of meningitis, encephalitis, head trauma, and hydrocephalus. CVI secondary to hypoglycemia is rarely encountered. A 4-year-old girl was admitted to hospital with a history of bilateral visual impairment. She had several severe hypoglycemic attacks lasting a week in the neonatal period. Bilateral occipital gliotic lesions were defined on magnetic resonance imaging (MR). [99mTc]-hexamethyl propylene amine oxime (HMPAO) brain single-photon emission computerized tomography (SPECT) was performed to determine the cerebral blood flow (CBF). SPECT images showed an extended hypoperfusion area in the right parieto-temporal region with marked non-perfusion areas in bilateral occipital cortices. It should be noted that hypoglycemia has devastating effects on cerebral tissues including optical areas and thus resulting in CVI. We would like to emphasize the importance of early diagnosis and prompt trea...

To understand the viral etiology of acute childhood encephalitis in Elaziğ, Eastern Turkey, 36 children aged between 4 months and 14 years who were treated in a regional medical center between January 1995 and June 1999 were studied. Viral etiology was identified in 16 of 34 (47.1 per cent) cases and the most frequently detected pathogens was mumps (seven cases, 20.6 per cent). No specific etiology was found in 18 (52.9 per cent) cases. Among the survivors, mental and/or focal neurological deficits persisted in 18 (52.9 per cent). Two children died and 32 survived, of whom 16 were left with no neurological sequel, 10 had persistent neurological sequel, and eight recovered with some degree of handicap. Improvement in the general health and sanitation of the population, and the universal use and development of new vaccination will significantly reduce the incidence of viral encephalitis.

Background: Infantile spasm (IS) is an age-dependent epileptic encephalopathy of variable etiology. Although IS is well studied, its pathogenesis is unclear. Infantile spasm is usually considered a generalized epilepsy, but recent studies point to focal cerebral blood flow (CBF) abnormalities. Methods: In six symptomatic IS patients, single photon emission computed tomography (SPECT) with [99mTc]-HMPAO, electroencephalography (EEG), magnetic resonance imaging (MRI)/computed tomography (CT) and their correlation were evaluated. Results: Single photon emission computed tomography showed unifocal (regional) cerebral hypoperfusion in two infants, multifocal (diffuse) cerebral hypoperfusion in three infants and normal perfusion in the other infant. Electroencephalograms obtained in near-time of the SPECT studies showed diffuse abnormalities in five infants with hypoperfusion on SPECT. Cranial MRI/CT showed diffuse and/or localized cerebral lesions in all infants, all of whom had corresponding areas of hypoperfusion on SPECT. In one patient whose spasms were stopped with anticonvulsants, SPECT was normal, in two patients SPECT showed unifocal lesions, while in another three patients whose spasms were decreased but not stopped, SPECT showed multifocal hypoperfusion. Conclusions: This pilot study may indicate that there are CBF anomalies in symptomatic IS. The degree of CBF may be a predictor of prognosis and multifocal hypoperfusion may be a poor prognostic criteria in IS.

Although it is known that platelet count is altered in iron deficiency anaemia (IDA), the qualitative extent of this interference is not well documented. In the present study we investigated platelet aggregation (PA) by impedance and optic methods in IDA. Forty-seven patients (plasma group: 16 boys, 9 girls and whole blood group: 11 boys, 11 girls) with IDA and thirty-one healthy children (plasma group: 6 boys, 10 girls and whole blood group: 6 boys, 9 girls) were enrolled into the study. Template bleeding times were measured by the Ivy method in all children. In the control group whole blood count, serum iron levels, bleeding time and PA were determined. After basal PA was determined in the patients and controls, ferrous sulphate was orally administered to the patients at a dose of 6 mg/kg/24 h for three months. Then, PA tests were performed again in the IDA (test group) patients. Ristocetin-induced PA was suppressed in both plasma and whole blood groups. Inhibition by both collagen (p < 0.05) and ristocetin (p < 0.001)-induced PA was determined by the optic method. Similarly in PA measured by the impedance method a suppression to adenosine diphosphate (p < 0.001) and to ristocetin (p < 0.01) was found. However, no significant alteration was observed in the bleeding time. All defective responses were reversed by the iron supplementation therapy. In addition, a significant correlation was found between some parameters of PA and several haematological values. In conclusion, although defective PA responses cannot be clinically demonstrated in patients with IDA, this suppression of PA may be detected by laboratory examination. Therefore, it is advised that care should be taken when using anti-aggregant agents in IDA.

HizliS, Ozdemir O, Abaci A, Razi CH and Kabakus N. Relation of subepicardial adipose tissue thickness and clinical and metabolic parameters in obese prepubertal children. Background: The measurement of subepicardial adipose tissue thickness (SATT) has been found to be related to insulin resistance (IR) in adults. Until now, the association between SATT and IR has not been evaluated in obese prepubertal children. We aimed to determine the relation of SATT with clinical anthropometric and metabolic parameters and to provide cutoff value of SATT associated with IR in obese prepubertal children. Methods: Fifty-two obese (mean age: 9.5 ± 1.6 years, 29 female) and 31 lean prepubertal age- and gender-matched subjects (mean age: 9.2 ± 1.4 years, 12 female) were evaluated by echocardiography. SATT was measured by transthoracic echocardiography. Results: SATT (6.54 ± 1.38 mm) and homeostatic model assessment-insulin resistance (HOMA-IR) (3.2 ± 2) values of obese prepubertal subjects were significantly higher than those of the lean subjects (3.72 ± 0.57 mm and 1.6 ± 1) in the control group (both p < 0.001). Bivariate correlation analysis showed significant correlation between SATT, age, body mass index (BMI), waist circumference (WC), hip circumference (HC), waist-to-hip ratio (WHR), mid-arm circumference (MAC), triceps skin fold (TSF) thickness, insulin, and HOMA-IR (r = 0.547, r = 0.524, r = 0.543, r = 0.431, r = 0.289, r = 0.402, r = 0.400, r = 0.328, r = 0.289, p < 0.05, respectively). As an optimal cutoff point, an SATT of 4.33 mm determined IR with 93.3% sensitivity and 51% specificity. Conclusions: Our study on obese prepubertal children showed that SATT was significantly correlated with age, BMI, WC, HC, MAC, TSF, insulin, and HOMA-IR.

Objective To assess the clinical characteristics of patients with migraine. Methods The medical records of 76 patients diagnosed with migraine were reviewed using the ICHD–II 2004 diagnosis criteria. The patients were classified into three age groups: 3–6 yr olds (group I), 7–12 yr olds (group II), and 13–17 yr olds (group III). Results Migraine was the most common cause of headache in the patients of present pediatric neurology outpatient clinic (57.1%, 76/133). The mean age of patients was 11.08±3.27 (3.25–17) yrs. The number of girls as the age increased (groups II and III). The mean headache attacks rate was 2.5±1.5 per wk, which resulted in worsening of school performance (n=26, 34.2%). In the majority of patients (n= 54, 71.1%), there was a family history of migraine or headache in the close relatives. Prophylaxis was found effective for all given medications (flunarizine: 46/54, propranolol: 19/21, topiramate: 10/10, sodium valproate: 1/1). Conclusions These findings indicate that: (a) migraine is the most frequent cause of headache in pediatric patients, (b) it has negative effects on school performance and daily activities, (c) the family history is important for making the diagnosis and (d) prophylaxis is significantly effective.

The case of a 14-year-old girl with fatal interstitial nephritis and myocarditis as complications of mumps is reported. The illness began with parotitis; renal symptoms developed within a week. The patient's renal and cardiac status and clinical course rapidly deteriorated and the outcome was fatal. The post-mortem renal biopsy sample showed interstitial mononuclear cell infiltration, oedema, and focal tubular epithelial damage in biopsy material of kidney, confirming the clinical diagnosis. Myocarditis was determined by electrocardiographic and echocardiographic findings. Since it has been reported that fatal complications such as myocarditis, dilated cardiomyopathy, and nephritis may develop in the course of mumps, the patients with mumps, especially in complicated cases, should be followed closely because of the severe clinical conditions which may progress.

Fragile X syndrome (FXS) is known as the most common form of inherited mental retardation. In our study, brain perfusion single photon emission computed tomography (SPECT) was performed in a 6 year-old boy diagnosed with FXS. Diffuse bilateral uptake of Technetium-99m hexamethyl propylene amine oxime (99mTc-HMPAO) was noted in his orbits, as well as cortical perfusion defects (hypoperfusion in the right parietal and the left temporal lobe). Ophthalmologic examination showed no pathological findings. Magnetic resonance imaging (MRI) revealed no abnormality in the orbital structures, although hypoplasia of cerebellum and vermis was visualized. Since the patient was crying during the injection, the increased blood flow or the increased metabolism of the eyes and/or ocular muscles may be responsible for this orbital finding. Alternatively, the enhanced uptake of HMPAO in the orbits may reflect the pathology associated with FXS, because patients with FXS might have visual-motor abnormalities. To the best of our knowledge, there has been no report documenting such an orbital uptake of HMPAO. Moreover, the visualization of decreased cerebral perfusion, with the normal findings of MRI, indicates that brain SPECT imaging with HMPAO is helpful for detecting brain abnormalities in children with FXS.

This study was undertaken to investigate the effects of neonatal cerebral hypoxic–ischaemic brain injury (HIBI) in acute and early chronic phases in the rat. HIBI was induced in 7-day-old rat pups by ligation of the right common carotid and then the pups were exposed to 1 h of hypoxia in 8% oxygen. They were divided into two groups: 1-day (acute phase, in the first 24 h) and 5-day (early chronic phase, 120 h). Neuropathological evaluation was performed using the hippocampus, cerebral cortex and basal ganglia on the coronal plane. The following values were obtained: (i) the ratio of the infarcted area; (ii) hemispheric atrophy/asymmetry; (iii) patchy lesions confined to the thalamus, caudate and putamen; (iv) the ratio of damaged neurons to all neurons; and (v) the percentage of apoptotic neurons relative to the total neurons in all brain areas. HIBI-induced global cerebral damage and cellular damage findings did not significantly differ between the two groups. However, they showed a tendency to recover/deteriorate in both acute and early chronic phases. The ratio of ipsi- and contra-lateral hemisphere infarct areas (20.7 and 15.7% vs. 40.1 and 26.7%, respectively), basal ganglia patchy lesion ratio (27.5 vs. 36.7%) and hemispheric atrophy/asymmetry (92.4 vs. 84.7%) were found to be lower in the rat pups in the chronic phase than those in the acute phase. In contrast, increases in the ratio of damaged neurons (16.7 vs. 13.3% in the cerebral and dorsal hippocampus, respectively) and in the ratio of apoptotic neurons (ipsi-lateral: 18 vs. 6%; contra lateral hemispheres: 3.5 vs. 1.7%, respectively) were recorded. It is concluded that cellular damage tends to deteriorate (damaged and apoptotic neurons) while global damage (cerebral infarct and patchy damage) improves with the progression of HIBI. However, further studies are needed in order to elucidate this process. Copyright © 2005 John Wiley & Sons, Ltd.

Anthrax is primarily a disease of herbivores, but it also causes cutaneous, respiratory and gastrointestinal infections in humans. Bacillus anthracis is an uncommon cause of meningitis and generally produces a haemorrhagic meningoencephalitis. We present the CT and MR findings of anthrax meningoencephalitis due to the cutaneous form of anthrax in a 12-year-old boy. They showed focal intracerebral haemorrhage with leptomeningeal enhancement.

Objective: To induce experimental peripheral facial paralysis by inoculation of HSV1 and to compare the effects of steroid, acyclovir, lipoprostoglandin E2 and steroid + acyclovir treatments in terms of clinical recovery,electrophysiologically and histopathologically. Materials and methods: A total of 132 adult female rats were used in this study. HSV type 1 strain was inoculated at the back of the left ear by using 27 gauge needle. Of all animals, 70 (53%) rats which developed facial paralysis were divided into five groups (n = 14 for each group) as control, steroid + acyclovir, lipoprostaglandin E1, steroid only and acyclovir only. At the end of the 21 days period, the rats were clinically examined and electrophysiological tests were performed, then decapitated and the nerve specimens were obtained. Results: A modified electroneurography (ENoG) test was performed and the latencies and the amplitudes were compared. The findings of the intact side were better, but with no significant difference. Histopathologicaly edema was significantly smaller in all groups compared to the controls (p < 0.05). Similarly, no difference was seen in terms of vacuolar degeneration and Schwann cell hyperchromatisation among the groups and no significant difference in recovery period and rate of facial paralysis when all groups were compared. Conclusion: Facial paralysis induced by HSV1 recovered spontaneously within a week. In the treatment of facial paralysis, steroid alone, acyclovir alone, steroid + acyclovir, or lipoprostaglandin E1 all reduced edema in the infected facial nerve but there was no statistical difference in of the rate or degree of recovery.

Neurofibromas occurring in the breast are very rare. A 10-year-old boy with neurofibromatosis type 1 (NF1) presented with a palpable mass on his left breast. US showed a well-defined and hypoechoic solid mass within the subcutaneous fat tissue in the areolar area. Mass was isointense compared to muscle and hypointense compared to fat tissue on TIW and T2W MR images. Excisional biopsy was applied to the mass. The diagnosis of gynecomastia and neurofibroma was made on the basis of histopathological and immunohistochemical findings.

The effects of iron deficiency anemia (IDA) on nerve conduction and efficiency of iron therapy were investigated by peripheral nerve-electrophysiological measurements. Eighteen children (10 boys, eight girls; mean age 31 +/- 1.3 months) with IDA and 12 healthy children (six boys, six girls; mean age 29 +/- 1.3 months) were enrolled into the study. Nerve conduction velocity was measured in the median and posterior tibial nerve. After nerve conduction values were determined in the patients and controls, 6 mg/kg/24 h ferrous sulphate was given orally to the patients for 3 months and nerve conduction velocity tests were performed again. Median/motor and sensory nerve conduction velocity and tibial/motor nerve distal-amplitute values of children with IDA were lower than for the control group (p < 0.05, p < 0.01 and p < 0.001 respectively). With iron supplementation these values increased to the normal levels and even higher than control levels for some parameters. In correlation studies between whole blood parameters and nerve conduction velocity results, there was a correlation between median/sensory nerve conduction velocity values and serum iron levels. Additionally there was a correlation between some nerve conduction velocity values and age. In conclusion, the evidence from this preliminary study suggests that peripheral neuropathy may develop in children with IDA. Peripheral neuropathy symptoms in these patients may be improved by iron therapy.