Factors related to abnormal neuroimaging in children with first unprovoked seizure

ObjectiveThe first attack of unprovoked seizure is more frequent than recurrent one and neuroimaging is one of the main parts of the evaluation of these attacks in order to demonstrate the cause and predict the prognosis. The aim of this study was to determine the incidence of abnormal neuroimaging and related factors in children with the first unprovoked seizure.Materials & MethodsA 7-year retrospective chart review was done on all children who were visited at Ali-Asghar Children's Hospital with the first unprovoked seizure and underwent neuroimaging including brain computed tomography or magnetic resonance imaging. The diagnostic criteria for the first unprovoked seizure in this study were based on the absence of any immediate or acute cause for the first seizure such as fever, head trauma, hypoglycemia, hypocalcemia, electrolyte imbalance and etc. We compared the rate of abnormal neuroimaging in patients according to different clinical and electroencephalographic (EEG) parameters.ResultsOne hundred and forty two patients (63 females, 79 males) were included in the study. Twenty eight patients (20%) had abnormal neuroimaging. CT scan and MRI were done in 63% and 37% of the patients, respectively. The most common abnormalities were cerebral dysgenesis (n=9) and cortical brain atrophy (n=6). Patients who were abnormal on neurologic examination had a higher rate of abnormal imaging in comparison with neurologically normal children (51% vs. 10%) (

The Efficacy and Safety of Oxcarbazepine as Add-On Therapy in Intractable Epilepsy in Children

Objective1-3% of the population suffer from epilepsy. Up to 30% of them develop refractory epilepsy and their seizures occur more than once per month despite receiving at least 2 first line antiepileptic drugs. In  this group, more efficacious antiepileptics are needed. This study was undertaken to evaluate the efficacy and safety of Oxcarbazepine as an adjunction therapy in children with refractory epilepsy.Materials & MethodsFrom Feb 2004 until Sep 2006, 30 patients with refractory epilepsy aged between 4 and 14 years were evaluated in a before and after type study.The patients had seizure ranging from once monthly to more than 10 times daily and none of them had used Oxcarbazepine previously. They received Oxcarbazepine 30 to 50 mg/kg/day orally in  combination with their current antiepileptic drugs and were regularly assessed for seizure frequency and side effects for 10 months.ResultsWith Oxcarbazepine adjunction therapy, 10% of the patients became seizure-free, 36.6% experienced more than 50% reduction in seizure frequency, and 13.3% had increasing seizures. The drug was  especially effective in the patients with partial seizures (77.7%). Brief and transient adverse effects were seen in 36.6% of the patients which disappeared with treatment continuation. Wilcoxon signed ranks test showed that oxcarbazepine was effective in the treatment of refractory seizures (P=0.003) and as shown by Fisher's exact test, it was more effective in partial seizures (P=0.0043).ConclusionThe results showed that Oxcarbazepine was a useful medication in the treatment of refractory epilepsy, especially the partial type, in children.

The role of routine transanastomotic T tube ostomy in jejunoilealatresia:A prospective, randomized study

Introduction: Jejunoileal atresia (JIA) is a common cause of neonatal intestinal obstruction and is a common surgical emergency. The aim of this study was to compare primary anastomosis without resection of the dilated segment with transanastomotic T tube jejunostomy (TATTO) versus resection of the dilated segment without T tube as the control group for treatment of patients with JIA.Material and Methods: During 2008 to 2013, 105 patients were diagnosed with JIA at the time of surgery and were recruited for this study. Data relating to efficacy and procedural complications were compared among patients. The criteria for exclusion were duodenal obstruction, colonic atresia, intestinal perforation, malrotation, and also JIA patients with associated other anomalies like meconium ileous, gastroschisis and anorectal malformation.Results: A total of 125 patients were diagnosed and 16 patients were excluded. Our patients were divided into a control group (n=52) and a TATTO group (n=57). The TATTO group had a significantly shorter postoperative hospital stay (12 vs. 23 days, p=0.001) and time to start feeding (9 vs. 13 days, p=0.003) compared with the control group. Sepsis (12.0% vs. 32.7% p=0.004), other complications (3.6% vs. 15.4% p=0.001) and the associated morbidity were significantly lower in the TATTO group.Conclusion: The findings suggest that the TATTO technique was a better method for the treatment of children with JIA, as study showed lesser morbidity and mortality rates.

Clinical and Para clinical Manifestations of Tuberous Sclerosis: A Cross Sectional Study on 81 Pediatric Patients

How to Cite this Article: Tonekaboni SH, Tousi P, Ebrahimi A, Ahmadabadi F, keyhanidoust Z, Zamani Gh, Rezvani M, Amirsalari S, Tavassoli A, Rounagh A, Rezayi A. Clinical and Para clinical Manifestations of Tuberous Sclerosis: A Cross Sectional Study on 81 Pediatric Patients. Iran J Child Neurol 2012; 6(3): 25-31.ObjectiveMigraine is a disabling illness that causes absence from school andaffects the quality of life. It has been stated that headache may representan epileptic event. EEG abnormality is a prominent finding in childrenwith migraine. The aim of this study was to evaluate EEG abnormalitiesin children with migraine.Materials & MethodsTwo-hundred twenty-eight children were enrolled into the study.Evaluation and following of cases was performed by one physician,paraclinical tests were used to increase the accuracy. The study wasconducted under the supervision of pediatric neurology masters and theselected cases were from different parts of the country.ResultsComparing EEG abnormalities in different types of migraine revealedthat there is an association between them. There was also a significantdifference between EEG abnormalities in different types of aura. Migrainetype was associated with the patient’s age. Sleep disorders were morecommon in patients with a positive family history of seizure.ConclusionOur study dosclosed migraine as a common problem in children withabnormalities present in approximately 20% of the patients. Migraineand abnormal EEG findings are significantly associated.RefrencesBundey S, Evans K. Tuberous sclerosis: a genetic study. J Neurol Neurosurg. Psychiatry 1969 Dec;32(6):591-603.Staley BA, Vail EA, Thiele EA. Tuberous sclerosis complex: diagnostic challenges, presenting symptoms,and commonly missed signs. Pediatrics 2011 Jan;127(1):e117-25.Thiele EA, Korf BR. Phakomatoses and allied conditions.In: Swaiman KF, Ashwal S, Ferriero DM. Swaimans pediatric neurology. 5th ed. China: Elsevier Saunders;2012. p. 504-9.Lendvay TS, Marshall FF. The tuberous sclerosis complex and its highly variable manifestations. J Urol2003 May;169(5):1635-42.Curatolo P, Jóźwiak S, Nabbout R; on behalf of the participants of the TSC Consensus Meeting for SEGA and Epilepsy Management. Management of epilepsy associated with tuberous sclerosis complex (TSC):Clinical recommendations. Eur J Paediatr Neurol 2012Jun;16(1):83-5.Jansen FE, Van Huffelen AC, Van Rijen PC, LeijtenFS,Jennekens-Schinkel A, Gosselaar P et al. Epilepsy surgeryin tuberous sclerosis: the Dutch experience. Seizure 2007Jul;16(5):445-53.Turgut M, Akalan N, Ozgen T, Ruacan S, Erbengi A. Subepandymal giant cell astrocytoma associated with tuberous sclerosis: diagnostic and surgical characteristics of five cases with unusual features. Clin Neurol Neurosurg1996 Aug;98(3):217-21.Coppola G, Klepper J, Ammendola E, Fiorillo M, dellaCorte, Capano G. The effects of the ketogenic diet in refractory partial seizures with reference to tuberous sclerosis. Eur J Pediatr Neurol 2006 May;10(3):148-51.Jozwia S, Kotulska K, Domanska-Pakiela D, LojszczykB, Syczewska M et al. Antiepileptic treatment before the onset of seizure reduces epilepsy severity and risk of mental retardation in infants with tuberous sclerosis complex. Eur J Paediatr Neurol 2011 Sep;15(5):424-31.Konishi Y, Ito M, Okuno T, Hojo H, Okuda R, Nakano Yet al. Tuberous sclerosis: early neurologic manifestations and CT features in 18 patients. Brain Dev 1979;1(1):31-7.Gerard G, Weisberg L. Tuberous sclerosis: CT findings and differential diagnosis. Comput Radiol 1987 Jul-Aug;11(4):189-92.Pompili G, Zirpoli S, Sala C, Flor N, Alfano RM, Volpi A et al. Magnetic resonance imaging of renal involvementin genetically studied patients with tuberous sclerosis complex. Eur J Radiol 2009 Nov;72(2):335-41.Fleury P, de Groot Wp, Delleman JW, Verbeeten B Jr,Frankenmolen-Witkiezwicz IM. Tuberous sclerosis: theincidence of sporadic cases versus familial cases. BrainDev 1980;2(2):107-17.Saadat M, Ansari-Lari M, Farhud DD. Consanguineous marriage in Iran. Ann Hum Biol 2004 Mar-Apr;31(2):263-9.Roach ES, Sparagana SP. Diagnosis of tuberous sclerosis complex. J Child Neurol 2004 Sep;19(9):643-9.

Clinical Survey of Cerebrovascular Disease in Children

Objective: Stroke is defined as the sudden occlusion or rupture of cerebral arteries or veins resulting in focal cerebral damage and clinical neurologic deficits. The risk factors for stroke in children are numerous and differ from those in adults. Identification of these factors can prevent subsequent strokes. The aim of this study is to determine the etiology of stroke in children. Methods: In this descriptive prospective study, children who were referred to pediatric neurology clinic in Mofid Children's Hospital due to acute hemiparesis during 2 years period (Sep 2003-Sep 2005) were evaluated and their diagnosis was cerebrovascular disorders, in the view of physical examination and brain imaging. Findings: The study group consisted of 40 children in age groups between 3 months to 14 years old. The most common age group at presentation was 2 to 5 years old. Occurrence was predominant during autumn and winter (70%). The most common clinical presentations were acute hemiparesis (85%) and seizures (40%). The other symptoms were as follow: 15% decreased level of consciousness, 12.5% fever, 7.5% VI nerve palsy, and 2.5% cerebellar signs. 20% of patients suffered from hemorrhagic and 80% had ischemic stroke. In 60% of the patients specific etiologic factors were identified and in the rest of the group, risk factors could be delineated. The most common etiologies were: 17.5% cardiac diseases or procedures and 10% hematologic disorders. Other etiologies included: 7.5% prothrombotic states, 7.5% CNS infection, 5% mitochondriopathy, 2.5% for each of the head trauma, migraine, serum lipid abnormality, hypertension and arteriovenus malformation. The most common risk factors consisted of 40% anemia and 20% infections. Conclusion: This study demonstrates that stroke in children is a multifactorial process. Identification of the underlying risk factors for cerebrovascular disorders is highly desirable because many of the risk factors can be prevented, resulting in reduction of the risk of subsequent strokes

Klippel-Trenaunay-Weber Syndrome with Hemimegalencephaly; Report of a Pediatric Case

Background: Klippel-Trenaunay-Weber Syndrome (KTWS) is a rare neurocutaneous syndrome. Hemimegalencephaly (HME) and seizure episodes have been reported previously in a few cases with KTWS. Case Presentation: We report here a 3 day-old girl with partial motor seizures, extensive port-wine staining and mild structural deformities in the feet, and a hemimegalencephaly. Conclusion: Occurrence of partial motor seizures in addition to bilateral lower extremities extensive portwine staining is a unique feature seen in our case

Can We Rely on Frozen Sections of a Rectal Biopsy for One-stage Trans-anal Pull-through Operation in Hirschsprung's Disease?

Objective: Successful results after one-stage trans-anal pull-through (OSTAPT) operation for Hirschsprung's disease (HD) depend on accurate identification of the aganglionic segment in intra-operative frozen section (FS). Misinterpretation of the findings of the rectal biopsy is an anxiety-evoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and permanent Section (PS) rectal biopsies in children with HD who were candidates for OSTAPT in a single-step operation. Methods: Subjects under the age of 14 years, admitted from March 2000 to July 2008 in a university-affiliated children's hospital for open rectal biopsy to diagnose HD were included in the study. All biopsies were taken 2-3 cm above the dentate line. 210 specimens of full-thickness rectal biopsy were obtained for both frozen section and permanent biopsy from all patients, examined by two well experienced pediatric pathologists for ascertaining the presence of ganglion cells, and the results were compared. Analysis was performed by SPSS Software version 11.5. Findings: Two-hundred one infants and children underwent FS rectal biopsy to exclude HD. Positive results were seen in 63.8% of the specimens examined as PS and in 58.3% of FS samples. 93.9% of positive results in FS studies were confirmed by PS studies. 6.1% of FS reports were false positive and 21.7% were false negative (P<0.001). The sensitivity of FS was 85.8% and specificity 90.2%. Positive predictive value (PPV) was 93.9% and negative predictive value (NPV) was 78.3% in FS studies (P<0.001). The accuracy of FS was 80.4%. Conclusion: Although FS of the rectal biopsy is useful in defining the aganglionic segment during operation, according to this study, it cannot be used as the sole base for performing primary pull-through operation before the results of the permanent section are on hand