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    Brain MRI findings in two Turkish pediatric patients with aspartylglucosaminuria
    (SAGE PUBLICATIONS INC, 2016) Kartal, Ayse; Aydin, Kursad
    Aspartylglucosaminuria is a rare lysosomal storage disorder that occurs as a result of a deficiency of the aspartylglucosaminidase enzyme. Because the disease is commonly referred to as the Finnish disease heritage, it is underdiagnosed outside of Finland. To date, only three Turkish patients are described in the literature. Here we describe the clinical and brain magnetic resonance imaging findings in two Turkish cousins with aspartylglucosaminuria, which can raise the suspicion of this rare disease in clinical practice.
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    Epstein-Barr virus encephalitis: findings of MRI, MRS, diffusion and perfusion
    (TURKISH J PEDIATRICS, 2011) Ozbek, Orhan; Koc, Osman; Paksoy, Yahya; Aydin, Kursad; Nayman, Alaaddin
    Ozbek O, Koc O, Paksoy Y, Aydin K, Nayman A. Epstein-Barr virus encephalitis: findings of MRI, MRS, diffusion and perfusion. Turk j Pediatr 2011; 53: 680-683 Epstein-Barr virus is an infection that is known as infectious mononucleosis. Even though the central nervous system is not a primary region of involvement of this disease, neurological complications are reported rarely. In this case report, we evaluated a 15-month-old male who presented to the pediatric neurology clinic due to high fever and a neurologic attack. His serological tests and radiological examinations (magnetic resonance imaging (MRI), MR spectroscopy (MRS), diffusion-weighted imaging (DWI) and MR perfusion) were consistent with Epstein-Barr virus encephalitis, which is a very rare complication of infectious mononucleosis. Additionally, we discuss the MRI, MRS, DWI and MR perfusion findings of our case, which were different from other cases reported in the literature.
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    Fatal valproate overdose in a newborn baby
    (SAGE PUBLICATIONS LTD, 2007) Unal, Ekrem; Kaya, Ulkuhan; Aydin, Kursad
    Valproate is a widely used drug in the treatment of epilepsy in children and adults. However, it is not safe for patients under two years of age, especially during the newborn period. This study presents a case of fatal valproate overdose in a 26-day-old female newborn, who is the youngest patient in the literature.
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    High rates of malnutrition and epilepsy: two common comorbidities in children with cerebral palsy
    (TUBITAK SCIENTIFIC & TECHNICAL RESEARCH COUNCIL TURKEY, 2019) Aydin, Kursad; Kartal, Ayse; Keles Alp, Esma
    Background/aim: The aim of this study was to evaluate the nutritional status of children with cerebral palsy and determine the particular characteristics of the disorder. Materials and methods: The nutritional status of the children was assessed by the Gomez classification using weight-for-age. The Gross Motor Function Classification System was used to determine the gross and fine motor functions. Results: The study was conducted with 197 children (58.4% males) between the ages of 1 and 18 years old. Asphyxia (44.1%) was the primary etiological factor, and spastic quadriplegia (41.6%) was the most common type of cerebral palsy. Malnutrition was the most frequent comorbidity and the overall malnutrition rate was 76.6%. The most common type of malnutrition was severe malnutrition, which was seen in 70 patients (35.5%). Epilepsy was the second most common comorbidity, seen in 51.7% of the cases. Conclusion: Our results revealed a high rate of malnutrition and epilepsy in children with cerebral palsy. These two more common significant comorbidities that influence the outcomes of children with cerebral palsy should be carefully evaluated and successfully managed. Families of children with cerebral palsy and their physicians should be educated about the nutritional status in these children.
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    Kluver-Bucy syndrome in a boy with non-Hodgkin lymphoma
    (TAYLOR & FRANCIS INC, 2007) Unal, Ekrem; Koksal, Yavuz; Baysal, Tamer; Energin, Meltem; Aydin, Kursad; Caliskan, Umran
    Kluver-Bucy syndrome is a rare neurobehavioral condition characterized by visual agnosia, excessive oral tendency, hypermetamorphosis, placidity, altered sexual behaviors, and changes in dietary habits. The authors report a case of Kluver-Bucy syndrome in a 10-year-old boy with non-Hodgkin lymphoma after intratechal methotrexate administration. He was treated by risperidone without any sequels.
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    Plasma leptin, neuropeptide Y, ghrelin, and adiponectin levels and carotid artery intima media thickness in epileptic children treated with valproate
    (SPRINGER, 2012) Tokgoz, Huseyin; Aydin, Kursad; Oran, Bulent; Kiyici, Aysel
    Weight gain is a common side effect of valproate (VPA) treatment, although the mechanism is not clear. Abnormal weight gain and obesity are associated with dyslipidemia, hypertension, and atherosclerosis. Measurement of the common carotid artery intima media thickness (CAIMT) gives a picture of early arterial wall alterations and, currently, is considered a noninvasive marker of premature atherosclerosis. The aim of the present study was to evaluate plasma insulin, leptin, neuropeptide Y (NPY), ghrelin, and adiponectin levels in children with epilepsy treated with VPA and to evaluate these parameters for early atherosclerosis. Twenty prepubertal children with idiopathic epilepsy treated with VPA were enrolled in this study. Body mass index (BMI) and fasting insulin glucose ratio (FIGR) were calculated, and the plasma insulin, leptin, NPY, ghrelin, and adiponectin levels; the lipid profiles; and CAIMT were measured for all subjects before the treatment and after a follow-up period of 6 and 12 months. When pretreatment values were compared with those at the end of 6 and 12 months, the mean BMI values, plasma insulin, leptin, NPY levels, and FIGR were increased, whereas the plasma ghrelin and adiponectin levels, lipid profiles, and CAIMT did not change significantly at the end of 6 and 12 months. These results suggest that weight gain during VPA treatment may be related to increases in insulin, leptin, and NPY levels. Additionally, in this study, no increase in the risk for early atherosclerosis was determined by CAIMT in children with epilepsy treated with VPA.
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    Proton magnetic resonance spectroscopy findings and clinical effects of montelukast sodium in a case with Sjogren-Larsson syndrome
    (SAGE PUBLICATIONS INC, 2006) Pirgon, Ozgur; Aydin, Kursad; Atabek, M. Emre
    Sjogren-Larsson syndrome is a rare hereditary metabolic disorder characterized by congenital ichthyosis, mental retardation, and spastic diplegia or tetraplegia. This genetic disease is caused by fatty acid aldehyde dehydrogenase deficiency, leading to an accumulation of long-chain alcohols. The role of enzyme in the degradation of leukotrienes paved the way to the development of a new therapeutic strategy for Sjogren-Larsson syndrome, leukotriene antagonists. We describe a 3-year-old boy with Sjogren-Larsson syndrome who had a lipid peak on proton magnetic resonance spectroscopy despite normal findings on cerebral magnetic resonance imaging. He benefited from treatment with montelukast sodium, especially with respect to the agonizing pruritus.
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    Reversible posterior leukoencephalopathy and Adie's pupil after measles vaccination
    (B C DECKER INC, 2006) Aydin, Kursad; Elmas, Sefika; Guzes, Eylem Atilgan
    Reversible posterior leukoencephalopathy syndrome is an increasingly recognized disorder with typical radiologic findings of bilateral gray- and white-matter abnormalities in the posterior regions of the cerebral hemispheres. The majority of patients with reversible posterior leukoencephalopathy syndrome are adults, and it is rare in children. Previously reported associations of reversible posterior leukoencephalopathy syndrome include hypertension, vasculitis, nephrotic syndrome, severe hypercalcemia, hemolytic uremic syndrome, eclampsia, renal failure, and use of immunosuppressive drugs. Adie's pupil is described as the presence of a large unilateral or bilateral tonic pupil related to virus infections or trauma. In this article, we describe a case of reversible posterior leukoencephalopathy and Adie's pupil association that occurred after measles vaccination. To our knowledge, this association has not been reported.
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    Teachers' perceptions in central Turkey concerning epilepsy and asthma and the short-term effect of a brief education on the perception of epilepsy
    (ACADEMIC PRESS INC ELSEVIER SCIENCE, 2007) Aydin, Kursad; Yildiz, Hatice
    This study was aimed at assessing and comparing schoolteachers' perceptions of epilepsy and asthma in central Turkey. Two-hundred seventy-five schoolteachers completed a questionnaire on their knowledge, attitudes, and practice. There were 18 pairs of questions on the questionnaire (one question in each pair pertained to epilepsy, and the other to asthma). One month later, following a brief education program on epilepsy, participants completed the epilepsy section of the questionnaire a second time. Among the teachers, initial rates of acceptance of children with epilepsy were significantly lower than the rates for asthma. Children with epilepsy were less encouraged by teachers to play with others and were perceived as more aggressive. More teachers were worried about other parents objecting to having a child with epilepsy in the class. Following the brief education program, there was a significant positive change in the teachers' perception of epilepsy. (c) 2006 Elsevier Inc. All rights reserved.
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    A Turkish case of galactosialidosis with a new homozygous mutation in CTSA gene
    (SPRINGER/PLENUM PUBLISHERS, 2017) Kartal, Ayse; Aydin, Kursad
    Galactosialidosis is an autosamal reressive lysosomal storage disease caused by a combined deficiency of lysosomal beta-galactosidase and neuraminidase, due to a primary defect in protective protein/cathepsin A. Three subtypes are recognized: the early infantile type, the late infantile type, and the juvenile/adult type. We report here a female patient with early infantile galactosialidosis who was born at 35 weeks of gestation. After birth she remained at the neonatal intensive care unit. Physical examination revealed, coarse facial features, hepatomegaly, cardiac murmur and diffuse hypotonia. The patient's mother had a past history of fetal hydrops history. The diagnosis of galactosialidosis was confirmed by decreased activity of beta-galactosidase and undetectable neuraminidase activity in fibroblasts. Genetic examination revealed a new homozygous mutation (c.1284delG) in the CTSA gene.