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Objective: In this study, we aimed to evaluate cases of meningitis for epidemiologic, clinical, laboratory findings and the treatment response. Materials and methods: We evaluated ninety two pa­tients whose diagnosis were meningitis retrospectively between January 2010 and December 2011, for age, gen­der, presenting symptoms, physical examination, labora­tory findings and treatments. The patients who were hos­pitalized with central nervous system infection were diag­nosed as meningitis by examination of cerebrospinal fluid (CSF) under a microscope and evaluation of biochemical (glucose, protein, chloride) results and / or culture. Results: Fifty patients were male and 42 were female, mean age was 4.2 ± 4.1 years. The most common symp­toms were fever (91.3%), vomiting (87.0%), headache (68.5%), and convulsion (20.7%). On physical examina­tion in descending order neck stiffness (60.9%), Brudz­inski phenomenon (50%), Kernig sign (46.7%), altered consciousness (16.3%) and rash (2.2%) were detected. When patients were classified in terms of clinical and laboratory findings; bacterial meningitis was determined 53.4%, aseptic in 38% and tuberculous meningitis in 7,6%. CSF and blood culture were positive in 13 of and seven of the cases respectively. The most frequent com­plications were seizures, cranial nerve palsy, hydroceph­alus and the syndrome of inappropriate ADH. Mortality rate was determined as 2.2%. Conclusion: The patients especially with symptoms of fever, vomiting, headache and convulsions, meningitis should be considered primary disease., Amaç: Çalışmamızda menenjit olgularının epidemiyolo­jik, klinik, laboratuvar bulguları ve tedavi yanıtlarını değer­lendirilmesi amaçlandı. Gereç ve yöntem: 2010- 2011 tarihleri arasında menenjit tanısıyla izlediğimiz 92 olgunun yaş, cinsiyet, geliş yakın­maları, fizik muayene, laboratuvar bulguları ve uygulanan tedaviler açısından geriye dönük olarak değerlendirmeye alındı. Menenjit tanısı, hastaların beyin omurilik sıvılarının (BOS) mikroskobik ve biyokimyasal (glikoz, protein, klor) sonuçlarının değerlendirilmesi ve/veya kültürde etkenin üretilmesi ile konuldu. Bulgular: Olguların 50\'si erkek 42\'si kız olup, yaş orta­lamaları 4,2±4,1 yıl idi. Başvuru sırasındaki en sık yakın­malar ateş (%91,3), kusma (%87,0), baş ağrısı (%68,5) ve havale geçirme (%20,7), olarak tespit edildi. Fizik incelemede ense sertliği (%60,9), Brudzinski fenomeni (%50), Kernig belirtisi (%46,7), bilinç değişikliği (%16,3) ve döküntü (%2,2) mevcuttu. Klinik ve laboratuvar bulgu­ları açısından hastalar sınıflandırıldığında %54.3 bakte­riyel, %38.0 aseptik ve %7,6 tüberküloz menenjit olarak saptandı. Olguların 13\'ünde BOS ve yedisinde kan kültü­ründe üreme oldu. En sık gözlenen komplikasyonlar kon­vülziyon, kraniyal sinir tutulumu, hidrosefali ve uygunsuz ADH sendromu idi. Mortalite oranı %2,2 olarak bulundu. Sonuç: Özellikle ateş, kusma, baş ağrısı ve konvülziyon yakınmaları olan hastalarda tanıda öncelikle menenjit dü­şünülmesi gereken bir hastalıktır.

Moyamoya (MMD) is a disease that often involves the vascular structures of anterior cerebral circulation, par­ticularly the proximal segments of anterior and middle cerebral arteries. The etiology of the disease is unknown. MMD often presents with cerebral ischemia and rarely with cerebral hemorrhage. The pathology is termed Moy­amoya syndrome (MMS) when the pathological cerebral angiography findings are accompanied by meningitis, neurofibromatosis, neoplasm, Down syndrome or poly­cystic kidney disease. Autoimmune diseases including Graves\' disease, Behcet\'s disease and antiphospholipid syndrome might also lead to the development of MMS. In this manuscript, we presented an interesting case of MMD associated with antiphospholipid syndrome, which is quite a rare cause of acute cerebral infarction in child­hood., Moyamoya hastalığı (MMH) çoğunlukla ön serebral do­laşıma ait vasküler yapıları özellikle de ön ve orta sereb­ral arterlerin proksimal segmentlerini tutan bir hastalıktır. Etiyolojisi net olarak bilinmemektedir. Hastalık sıklıkla serebral iskemi, nadiren de serebral hemoraji ile ortaya çıkmaktadır. Serebral anjiografideki patolojik bulguların menenjit, nörofibromatozis, neoplazmlar, Down sendro­mu ve polikistik böbrek hastalığı gibi durumlarla bir arada bulunması halinde Moyamoya sendromu (MMS) olarak isimlendirilmektedir. Graves hastalığı, Behçet hastalığı ve Antifosfolipid sendrom (AFS) gibi otoimmün hastalıklar da MS gelişmesine neden olabilmektedirler. Kliniğimizde An­tifosfolipid sendrom ile beraber MMS tanısı alan ve çocuk­luk çağı akut serebral infarkt nedenleri arasında oldukça ender görülen bu hastalık ilginç bulunarak sunuldu.

31st International Epilepsy Congress -- September 05-09, 2015 -- Istanbul, Turkey WOS: 000365756500750 …

31st International Epilepsy Congress -- September 05-09, 2015 -- Istanbul, Turkey WOS: 000365756500753 …

31st International Epilepsy Congress -- September 05-09, 2015 -- Istanbul, Turkey WOS: 000365756500751 …

In order to evaluate compliance with oral antibiotic treatment in 297 pediatric out patients parents of patients with acute pharyngitis were interviewed and bottles or packages of remained medicine were determined by the interviewer at follow up appointments Compliance rates were directly related to the educational status of mothers being lower in mothers with low educational level and also to the number of daily doses of prescribed antibiotics p lt;0 0001 and p lt;0 0001 Compliance rate in patients receiving antibiotics 3 or 4 doses day was lower than those receiving 2 doses day Only 9 4 of patients had received antibiotics for the entire prescribed period Most commonly antibiotics were discontinued due to the disappearance of symptoms This was interpreted as healing of the child by parents 35 1 of children had received less than half of the prescribed medicine In 3 7 compliance could not be estimated as history was unreliable Our data demonstrate that incompliance is an important problem during oral antibiotic therapy in pediatric ambulatory patients Key words: Children Antibiotics Compliance, Oral antibiyotik tedavisine hasta uyumunu araştırmak amacıyla kontrol muayenesi için polikliniğe çağrılan akut farenjitli 297 hastanın ailesiyle görüşüldü Arta kalan ilaç miktarı ve hastanın kullandığı total ilaç dozu belirlendi Tedaviye uyum anne eğitim düzeyi ve ilacın günlük doz sayısı ile doğrudan ilişkili bulundu p lt;0 0001 ve p lt;0 0001 Günde 3 veya 4 doz antibiyotik alan hastaların tedaviye uyumu günde 2 doz alanlara göre daha düşük bulundu Hastaların ancak 9 4 ü reçetede belirtilen süre boyunca tedavilerine devam etmişti Tedaviyi erken sonlandırmanın en sık nedeni hastanın semptomlarının kaybolmasının anneler tarafından iyileşme olarak algılanmasıydı Hastaların 35 1 i planlanan dozun yarısından daha azını almıştı Olguların 3 7 sinde anamnezin çelişkili olması nedeni ile tedaviye uyum belirlenemedi Elde ettiğimiz sonuçlar çocuk yaş grubu poliklinik hastalarında oral antibiyotik tedavisine hasta uyumsuzluğunun önemli bir sorun olduğunu göstermektedir Anahtar kelimeler: Çocuklar Antibiyotik Tedaviye Uyum

In this study breast feeding patterns of 414 young mothers were evaluated Mothers who had babies aged between 18 24 months were interviewed Of 414 mothers 79 3 were breast feeding their babies for longer 6 months The main two reasons for terminating breast feeding were insufficiency of milk and the infants inadequate growth The most common two methods used for terminating breast feeding were sending the baby to one of relatives and application of bad tasting material on the breast nipple Mothers who used these two methods were found 41 of all subjects Mothers who fed their babies whenever it cried was 88 7 The most common supplementary initial foods were cow s milk rice flour and fruit juice The influence of older family members on breast feeding attitudes of mothers was prominent Key words: Breast Milk Breast Feeding, Bu çalışma Manisa ve çevresinde annelerin emzirme alışkanlıklarını saptamak amacıyla yapıldı Bu amaçla 18 ayını henüz doldurmuş bebeği bulunan 414 anne ile görüşüldü Annelerin 79 3 ü bebeğini 6 aydan uzun emzirmişti Emzirmeyi sonlandırmanın başlıca sebebi anne sütünün yetersizliği ve bebeğin yeterince gelişmemesiydi Emzirmeyi sonlandırmak için en sık başvurulan yöntemler: bebeğini akrabaya yollamak veya meme başına acı sürmekti Bu yöntemleri kullanarak emzirmeyi sonlandıran annelerin oranı 41 i oluşturuyordu Annelerin 88 7 si bebek ağladıkça emziriyordu İnek sütü pirinç unu ve meyve suyu ilk başlanan ek gıdalardı Emzirme konusundaki davdanışlara aile büyüklerinin etkisi büyüktü Anahtar kelimeler: Anne Sütü Emzirme

Kaybolan beyaz madde (vanishing white matter, VWM) lökoansefalopatisi ''merkezi sinir sistemi hipomiyelinizasyonu ile seyreden çocukluk çağı ataksisi'' olarak da bilinen otozomal çekinik geçişli bir hastalıktır. İlk defa 1997 yılında Van der Knaap ve ark. (1) tarafından tanımlanmıştır. En sık görülen şekli erken çocukluk döneminde serebellar ataksi, spastite ve zeka gerilemesi ile karşımıza gelir (2). Stresin hastalığın başlangıcında ve klinik alevlenmelerde, önemli bir etmen olduğu bilinmektedir (3). Hastalığa neden olan mutasyon ökaryotik translasyon başlangıç etmeni olan eIF2B'yi kodlayan toplam beş gendeki (ElF2B1,ElF2B2,ElF2B3,ElF2B4,ElF2B5) mutasyonları içermektedir. Başlangıçta çocukluk çağı için tanımlanan ve son derece geniş bir fenotipik değişkenliğe sahip bu hastalığın gün geçtikçe her yaşı etkileyebileceği görülmüştür (1,4). Nadir görülen bu hastalığın Türkiye'de ilk genetiği kanıtlanan olgu olması ve yaş grubu için nörodejenaratif hastalıklar içinde ayırıcı tanıda akla gelmesini vurgulamak için bu hastayı sunmayı uygun gördük., To the Editor Vanishing white matter VWM leukoencephalopathy is an autosomal recessive disease which is also known as 39; 39;childhood ataxia with central nervous system hypomyelinization rdquo; It was defined by Van der Knaap et al 1 in 1977 for the first time The most common type is manifested by cerebellar ataxia spasticity and mental regression in the early childhood 2 It is known that stress is a significant factor in the disease onset and clinical exacerbations 3 The mutation which causes the disease involves the mutations in the five genes ElF2B1 ElF2B2 ElF2B3 ElF2B4 ElF2B5 coding eIF2B which is the eukariotic translation initiation factor In time it has been recognized that this disease which was initially described for the chidlhood and which has a wide range of phenotypic variability can affect patients at any age 1 4 We wanted to present this patient because he was the first genetically proved case in Turkey and we wished to emphasize that this rare disease should be considered in the differential diagnosis of neurodegenerative diseases in the childhood Our patient who was a 8 year old male patient presented because of gradually increasing loss of motor abilities following an upper respiratory tract infection with fevet at the age of four for the first time The family who recognized his muscle weakness observed that the child had unsteady gait and that this increased in time His medical history revealed that his perinatal history and neurological development until the age of one were normal The mother and father were third degree relatives and familial history was found to be normal On physical examination the height and weight were found to be normal visual and auditory problems were absent and cognitive functions were compatible with his age Our patient had no dysmorphic charactersitic Ataxia intentional tremor in both upper extremities and dysdiyadochinesia were found on neurological examination Diffuse hypotonia and decrease in muscle strenght in all extremities were found Muscle strength was found to be 4 5 in the left arm right arm and left leg and 3 5 in the right leg Both Achilles reflexes and patella reflexes were found to be increased Clonus and Babinsky were found to be positive in both lower extremities Fundoscopic examination was found to be normal Examination of the other systems was found to be normal Regular screenings and metabolic tests were found to be normal in the laboratory findings On magnetic resonance imaging MRI of the brain diffusely increased density in the white matter in FLAIR sequences and locally cystic areas with the same intensity as the cerebrospinal fluid were observed VWM disease was considered with the clinical findings and brain MR findings in the patient in whom genetic analysis was planned Genetic mutation analysis revealed homozygous mutation in the EIF2B5: 943C gt;G DNA R315G protein gene The mother and father were found to be carriers for the same mutation Thus the diagnosis of VWM disease was confirmed Our patient is now receiving physiotherapy and individual education in a special education center Worsening in the acute motor functions was observed for two times in 2010 and 2011 Our patient had fever during both of these worsening attacks In the follow up the intellectual functions were found to be retarded for his age No auditory or visual problem developed The patient who had an articulation problem while speaking did never lost comprehensibility in the communication with the people around The patient whose muscle weakness increased is not able to perform daily activities by himself for the time being The disease is manifested by cerebellar ataxia and more rarely with spasticity The consciousness is usually preserved In the advanced period vision loss with optic atrophy is among the findings described 1 Vermeulen et al 3 emphasized that stress was an important factor both in the onset of the disease and clinical exacerbations Other factors which speed the progression include infections with fever cranial traumas and fear spells Outside these periods the disease has a slow clinical course but mortality is inevitable 2 3 It was learned that our patient had an upper respiratory tract infection with fever at the time the first symptoms appeared In the early childhood brain MRI is the most strong supporting tool for the diagnosis On MRI progressive decrease in volume and cystic degeneration are observed in the white matter MR spectroscopy reveals progressive decrease in all metabolites which can be tested Cerebellar and brain stem involvement is observed in the lost white matter In the later stages cerebral and cerebellar atrophy is observed 1 2 Cortical grey matter is always preserved 1 On cranial MRI of our patient enlargement in the lateral ventricles Figure 1 and cerebellar atrophy in the T2 weighted sections diffusely increased intensity in the white matter and cystic areas with the same intensity as the cerebrospinal fluid in FLAIR sequences were observed For the time being there is no treatment for the disease except for supportive treatment which is given to increase the quality of life In time it has been recognized that this disease which was initially described for the chidlhood and which has a wide range of phenotypic variability can affect patients at any age 1 5 In the prenatal form of the disease decreased fetal movements oligohydramniosis primary microcephaly growth retardation congenital contractures renal hypoplasia hepato splenomegaly pancreatitis cataract ovarian dysgenesis and leukoencephalopathy may be observed 6 Conclusively vanishing white matter disease is a rare genetic disease In the diagnosis a history of discontinuation and regression of motor functions which start in the childhood progressive ataxia spasticity and intermittent worsening attacks is important Mutation analysis which is performed as a result of the findings observed on cranial MR confirms the diagnosis Early diagnosis is very important in terms of genetic counseling and protecting the patient from the factors which worsen the disease Researches for the unknown molecular mechanisms of this disease which has a wide clinical spectrum are continuing We wanted this patient who was the first genetically confirmed case in our country to remind physicans to consider this disease in the differential diagnosis in patients presenting with motor retardation Ad shy;dress for Cor shy;res shy;pon shy;den shy;ce: Serhat Güler MD Bezmialem Foundation University Medical Faculty Division of Pediatric Neurology İstanbul Turkey E mail: serhatguler@hotmail com Re shy;cei shy;ved: 06 30 2012 Ac shy;cep shy;ted: 11 15 2012 References 1 Bugiani M Boor I Powers JM Scheper GC van der Knaap MS Leukoencephalopathy with vanishing white matter: a review J Neuropathol Exp Neurol 2010; 69 10 : 987 986 2 van der Knaap MS Kamphorst W Barth PG Kraaijeveld CL Gut E Valk J Phenotypicvariation in leukoencephalopathy with vanishing white matter Neurology 1998; 51: 540 ndash;547 3 Vermeulen G Seidl R Mercimek Mahmutoglu S Rotteveel JJ Scheper GC van der Knaap MS Fright is a provoking factor in vanishing white matter disease Ann Neurol 2005; 57 4 : 560 563 4 van der Knaap MS Leegwater PA Könst AA Visser A Naidu S Oudejans CB Schutgens RB Pronk JC Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter Ann Neurol 2002; 51 2 : 264 270 5 van der Knaap MS Leegwater PA van Berkel CG Brenner C Storey E Di Rocco M Salvi F Pronk JC Arg113His mutation in eIF2Bepsilon as cause of leukoencephalopathy in adults Neurology 2004; 62 9 : 1598 600 6 van der Knaap MS van Berkel CG Herms J van Coster R Baethmann M Naidu S Boltshauser E Willemsen MA Plecko B Hoffmann GF Proud CG Scheper GC Pronk JC eIF2B related disorders: antenatal onset and involvement of multiple organs Am J Hum Genet 2003; 73: 1199 207

Sütçocukluğunun vitamin B12 eksikliği, nörogelişimsel gecikme ve hastalıkların tedavi edilebilir nedenlerinden biridir. Çoğunlukla vitamin B12 eksikliği olan annelerden doğup, yalnızca ve uzun süre anne sütü ile beslenen bebek- leri etkiler. Bu vitamin eksikliği ülkemizde yaygındır. Gecikmiş tanı ve tedavi kalıcı nörolojik sonuçlara yol aça- bilir. Burada, kliniğimize kusma, yutma güçlüğü, hipotoni ve tremor ile gelen 12 aylık ilginç bir hasta sunduk, Infantile vitamin B12 deficiency is one of the treatable causes of neurodevelopmental delay and disorders. It mostly affects exclusively prolonged breastfed infants born to vitamin B12 deficient mothers. This vitamin defiency is common in our country. Delayed diagnosis and treatment may lead to permanent neurological outcomes. Herein, we reported an interesting case of a 12-month-old patient who was admitted to our clinic with vomiting, dysphagia, hypo- tonia and tremor

Biotinidaz eksikliği biotin metabolizmasının bir bozukluğu olup nörolojik belirtiler, deri bulguları ve organik asidüri ile karakterizedir. Bu çalışmada koma tablosunda başvuran ciddi biotinidaz eksikliği olan dört aylık erkek bebek sunulmaktadır. Biotinidaz eksikliği hem idrar organik asit analizi hem de serum biotinidaz aktivitesi ölçümü ile doğrulanmıştır, Biotinidase deficiency is a disorder of biotin metabolism and most symptomatic patients with biotinidase deficiency have both neurologic and cutaneous symptoms and typical organic aciduria. We report the case of a child with severe biotinidase deficiency presenting coma. Biotinidase deficiency was considered and was confirmed by both urine organic acid analysis and biotinidase activity measurement

Glutarik asidüri tip 1, glutaril-koenzim A dehidrogenaz eksikliğine bağlı olarak gelişen otozomal resesif bir hastalıktır. Glutarik asit ve 3-hidroksiglutarik asit birikimi ile ikincil karnitin eksikliğine neden olur. Çocuklarda özellikle hayatın ilk bir kaç yılı içerisinde ortaya çıkan; hipotoni, makrosefali, motor gerilik, distoni ve diskinezi başlıca klinik bulguları oluşturur. Semptomlar özellikle bazal ganglion tutulumuna bağlanmaktadır. Silvian fissürde genişleme, frontotemporal atrofi ve bazal ganglion tutulumu sıklıkla gözlenen nöroradyolojik bulgulardır. Glutarik asidüri tip 1 tanısı alan vakalarda nadiren temporal loblarda araknoid kistler bildirilmiştir. Bu çalışmada Glutarik asidüri tip 1 tanısı alan iki taraflı araknoid kist saptanan ve bazal ganglion tutulumu olan sekiz aylık kız hasta sunulmuştur, Glutaric aciduria type 1 is an autosomal recessive disorder resulting from a deficiency of glutaryl-CoA dehydrogenase. This leads to an accumulation of glutaric and 3-hydroxyglutaric acids and secondary carnitin deficiency. The clinical picture typically shows varying degrees of muscular hypotonia, macrocephaly, motor delay, dystonia, and dyskinesia beginning acutely or gradually in the first few years of childhood. The symptomatology is discussed, especially those resulting from lesion in the basal ganglia. Frequent neuroradiological findings include widened sylvian fissures, frontotemporal volume loss, basal ganglia lesions. Patients with glutaric aciduria type 1 a rare appear to have a bitemporal arachnoid cysts. Here, we present glutaric aciduria type 1 in a 8-month-old girl with bilateral temporal cysts and basal ganglia lesion

Akut poststreptokoksik glomerulonefrit çocuklardaki akut hipertansiyonun en sık nedenlerinden biridir. Reverzibl posteriyor ensefalopati sendromu PRES , sıklıkla bilinen hipertansiyon veya immunsupresif ilaç kullanımı durumlarında ortaya çıkan bir klinik-radyolojik tablodur. PRES’in karakteristik semptomları baş ağrısı, kusma, görmede bozukluklar, bilinç değişiklikleri ve nöbetlerdir. Nörolojik sistem görüntülemelerinde en sık görülen anormallik, beyin hemisferlerininin arka bölgelerinde, özellikle pariyetooksipital bölgede, ak ve gri maddeyi tutan bilateral ödemdir. Burada, akut baş ağrısı, bilinç değişikliği, nöbet ile başvuran ve APSGN’ye bağlı PRES tanısı alan 11 yaşında bir erkek hastayı sunduk., Acute poststreptococcal glomerulonephritis APSGN is one of the most common causes of acute hypertension in children. Posterior reversible encephalopathy Syndrome PRES is a clinico-radiological picture which emerges commonly in the setting of known hypertension or use of immune suppressive agents. Characteristic symptoms of PRES include headache, vomiting, visual disturbances, changes in consciousness and seizures. The most common abnormality on neuroimaging is bilateral edema involving the white and grey matter in the posterior regions of cerebral hemispheres, especially in the parietooccipital regions. We reported herein a 11-year male patient who presented with acute headache, change in consciousness, seizure and was diagnosed PRES -related APSGN