Vitamin E and Niemann–Pick Disease Type C

How to Cite this Article: Rezayi AR. Vitamin E and Niemann–Pick Disease Type C. Iran J Child Neurol. 2015 Autumn;9:4(Suppl.1): 23.Pls see Pdf.

Analysis of the Urban Water Requisition Demand for the Purpose of Re-engineering and Water Network Optimization (Case Study: Tabriz' Eram Urban Area)

Water shortage is one of the most important challenges of this century and it would be a serious obstacle for the mankind. In the near future, drinking water supply and water resources management will be at the forefront of planning for urban managers, which is becoming a crisis due to the serious shortage of water resources. This problem is more critical in country of Iran, where water depletion is now receiving serious attention. Different water management programs have been started by government to conserve and manage water more efficiently, especially in some critical areas such as Tabriz megacity located in North East of the country. Eram town is one of the problematic areas of Tabriz. Hence, in this study, we tried to do reengineering for this town to offer some solutions for the problems related with water supplying of the implemented network in this region. Regarding this issue, the GIS (Geographic information system) data of the available water supply network was used and the collected field data such as consumption rates in current conditions were applied to the network. Then the calibration of model was performed. Eram Town water distribution network reengineering has been performed considering a 2043 plan, taking into account population growth and the increase in consumption. In conclusion, it is suggested that, the network will perform more efficiently by adding new pipes or modifying the existing pipes. Also, the problem of lack of pressure in some parts of the network which cannot fulfill the requirements of subscribers at peak water usage hours, could be solved

The Etiologic Profile of the Pediatric Seizure: An Epidemiological Study from Iran

Background: Seizures are one of the most common neurologic disorders in children. The aim of this study is to determine the main etiologies of seizure in children. Methods: Children with a complaint of seizure in Loghman Hakim hospital, from June 2014 to January 2016 were evaluated. The final diagnosis of seizure was made by a pediatric neurologist. The age, sex, type of seizure, associated fever, history of head trauma and other variables related to seizure in pediatric group were reviewed from the medical records.Results: A total of 200 children with a diagnosis of seizures were included in this study. A total of 59% were male patients. The age of the patients ranged from 3 months to 102 months with a mean of 23 months.About one-quarter of the patients had focal seizures while others had generalized seizures. Sixteen patients (8%) had a positive family history of epilepsy. Of total 126 patients (63%) experienced their first lifetime seizure. The body temperature of the patients ranged from 36.1 °C to 39.8 °C with a mean of 38.2 °C. The etiologies of seizures were febrile seizure (82%), vaccine-associated seizures (3%), hypoglycemia (6%), hypocalcemia (2%), hyponatremia (2%), encephalopathy (2%), hyperglycemia (1%), epilepsy (1%) and intracerebral hemorrhage (1%).Conclusion: While fever is the most common etiology of seizure in children, hypoglycemia should be taken into consideration as the second most common etiology of seizure. Although other etiologies such as hyponatremia and intracerebral hemorrhage are less common, they should be kept in mind due to their life-threatening complications. Hence, the results of this study underscores the importance of history and laboratory findings of the children with seizure.

Relationship Between Migraine and Abnormal EEG Findings in Children

How to Cite this Article: Nejad Biglari H, Rezayi A, Nejad Biglari H, Alizadeh M, Ahmadabadi F. Relationship Between Migraine and Abnormal EEG Findings in Children. Iran J Child Neurol 2012; 6(3): 21-24.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 children with migraine. The aim of this study was to evaluate EEG abnormalities in 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 revealed that there is an association between them. There was also a significant difference between EEG abnormalities in different types of aura. Migraine type was associated with the patient’s age. Sleep disorders were more common in patients with a positive family history of seizure.ConclusionOur study disclosed migraine as a common problem in children with abnormalities present in approximately 20% of the patients. Migraine and abnormal EEG findings are significantly associated.ReferencesOttman, R, Lipton RB, Comorbidity of migraine and epilepsy. Neurology 1994 Nov;44(11):2105-10.Haut SR, Bigal ME, Lipton RB. Chronic disorders with episodic manifestations: focus on epilepsy and migraine.Lancet Neurol 2006 Feb;5(2):148-57.Piccinelli P, Borgatti R, Nicoli F, Calcagno P, Bassi MT,Quadrelli M et al. Relationship between migraine and epilepsy in pediatric age. Headache 2006 Mar;46(3):413-21.Hauser WA, Annegers JF, Anderson VE. Epidemiology and the genetics of epilepsy. Res Publ Assoc Res Nerv Ment Dis 1983;61:267-94.Yankovsky AE, Andermann F, Bernasconi A.Characteristics of headache associated with intractable partial epilepsy. Epilepsia 2005 Aug;46(8):1241-5.The International Classification of Headache Disorders:2nd edition. Cephalalgia 2004; 24 Suppl 1:9-160.Forderreuther S, Henkel A, Noachtar S, Straube A. Headache associated with epileptic seizures:epidemiology and clinical characteristics. Headache 2002 Jul-Aug;42(7):649-55.Lewis DW, Diamond S, Scott D, Jones V. Prophylactic treatment of pediatric migraine. Headache 2004 Mar;44(3):230-7.Holguin J, Fenichel G. Migraine. J Pediatrics 1967 Feb;70(2):290-7.Chu ML, Shinnar S. Headaches in children younger than7 years of age. Arch Neurol 1992 Jan;49(1):79-82.Friedman E, Pampiglione G. Recurrent headache inchildren (a clinical and electroencephalographic study).Arch Neurobiol 1974;37 SUPPL:115-76.Kramer U, Nevo Y, Neufeld MY, Harel S. The valueof EEG in children with chronic headaches. Brain Dev1994 Jul-Aug;16(4):304-8.Schon F, Blau JN. Post-epileptic headache and migraine.J Neurol Neurosurg Psychiatry 1987 Sep;50(9):1148-52

The Best Time for EEG Recording in Febrile Seizure

How to Cite This Article: Karimzadeh P, Rezayi A, Togha M, Ahmadabadi F, Derakhshanfar H, Azargashb E, Khodaei F. The Best Time for EEG Recording in Febrile Seizure. Iran J Child Neurol. 2014 Winter; 8(1):20-25.ObjectiveSome studies suggest that detection of epileptic discharge is unusual during the first postictal week of febrile seizure and others believe that EEGs carried out on the day of the seizure are abnormal in as many as 88% of the patients. In thisstudy, we intend to compare early and late EEG abnormalities in febrile seizure.Materials & Methods EEG was recorded during daytime sleep, 24-48 hours (early EEG) and 2 weeks (late EEG) after the seizure in 36 children with febrile seizure (FS), aged between 3 months and 6 years. EEGs that showed generalized or focal spikes, sharp, spike wave complex, and slowing were considered as abnormal EEG.Abnormalities of the first EEG were compared with those of second EEG.ResultsThe most common abnormal epileptiform discharges recorded in the early EEG were slow waves (27.6%) and sharp waves in late EEG (36%). Distribution of abnormalities in early and late EEG showed no significant statistical difference.ConclusionThe early and late EEG recording had the same results in patient with febrile seizure. Reference:Hauser WA, Kurland LT. The epidemiology of epilepsy in Rochester, Minnesota, 1935 through 1967. Epilepsia 1975;16(1):1-66.Freeman JM. Febrile seizures: a consensus of their significance, evaluation, and treatment. Pediatrics 1980;66(6):1009.Waruiru C, Appleton R. Febrile seizures: an update. Arch Dis Child 2004;89(8):751-6.ILAE. Guidelines for epidemiologic studies on epilepsy, International League against Epilepsy. Epilepsia 1993;34(4):592-6.Annegers JF, Hauser WA, Shirts SB, Kurland LT. Factors prognostic of unprovoked seizures after febrile convulsions. N Engl J Med 1987;316(9):493-8.Berg AT, Shinnar S, Darefsky AS, Holford TR, Shapiro ED, Salomon ME, et al. Predictors of recurrent febrile seizures. Arch Pediatr Adolesc Med 1997;151(4):371-8.Nelson KB, Ellenberg JH. Predictors of epilepsy in children who have experienced febrile seizures. N Engl J Med 1976;295(19):1029-33.Anonymous. Practice parameter: the neurodiagnostic evaluation of the child with a first simple febrile seizure. American Academy of Pediatrics. Provisional Committee on Quality Improvement, Subcommittee on Febrile Seizures. Pediatrics 1996;97(5):769-72; discussion:773-5.Rosman NP. Evaluation of the child who convulses with fever. Paediatr Drugs 2003;5(7):457-61.Kanemura H, Mizorogi S, Aoyagi K, Sugita K, Aihara M. EEG characteristics predict subsequent epilepsy in children with febrile seizure. Brain Dev 2012;34(4):302-7.Yamatogi Y, Ohtahara S. EEG in febrile convulsions. Am J EEG Techno1 1990;30:267-80.Aicardi J, Chevrie JJ. The significance of electroencephalographic paroxysms in children less than 3 years of age. Epilepsia 1973;14(1):47-55.Tsuboi T. Seizures of childhood: a population-based and clinic based study. Acta Neurol Scand Suppl 1986;110:1-237.Maytal J, Steele R, Eviatar L, Novak G. The value of early postictal EEG in children with complex febrile seizures. Epilepsia 2000;41(2):219-21.Joshi C, Wawrykow T, Patrick J, Prasad A. Do clinical variables predict an abnormal EEG in patients with complex febrile seizures? Seizure 2005;14(6):429-34.Lennox-Buchthal M. Febrile convulsions: a reappraisal. Electroencephalogr Clin Neurophysiol 1973;32:Suppl:1-138.Frantzen E, Lennox-Buchtal MA, Nygraad A. Longitudinal EEG and clinical study of children with febrile convulsions. Electroencephalogr Clin Neurophysiol 1968;24(3):197-212.Kajitani T, Ueoka K, Nakamura M, Kumanomidou Y. Febrile convulsions and rolandic discharges. Brain Dev 1981;3(4):351-9.Sofijanov N, Emoto S, Kuturec M, Dukovski M, Duma F, Ellenberg JH, et al. Febrile seizures: clinical characteristics and initial EEG. Epilepsia 1992;33(1):52-7

Blood lead level and related factors in ADHD patients of Loghman Hakim Hospital in 2016- 2017

Introduction: Lead is a highly neurotoxic metal mainly in early life. In this study we investigate blood lead level (BLL) in children with attention deficit/ hyperactivity disorder (ADHD) and some related factors mainly opium exposure, as a source of lead exposure in recent years in Iran. Materials and Methods: In this cross-sectional descriptive study children & adolescents aged < 18 years in Child Neurology Clinic of Loghman Hakim hospital with ADHD criteria according to DMS-V in Tehran-Iran were studied. Lead Care II checked BLLs using 0.5-milliliter heparinzed venous blood. Demographics characteristic   and some   related factors such as old housing, parents’ job, pica, opium exposure were   asked and analyzed. Results: Fifty-one children and adolescents <18 years, 25.5% female and 74.5% male with mean ages of    71.4+30.3 months entered the study. Mean BLL was 6.34+2.63  µg/dl. The mean BLL in 100 normal children in Loghman Hakim hospital was 3.4 µg/dl. Mean BLL was 57/6 µg/dl in boys and60/6  µg/dl in girls, (p=0.973).  Also, the difference in mean BLLs were not significant in terms of living place, sex, age, pica and parents job.  Totally, 43 patients (84.3%) of the study samples had BLL ≥5 µg/dl. The highest blood lead level in our patient was 20.1 µg/dl. Eighteen (32.7%) of our patients have positive history for opium exposure in their family that BLL in this group was 5.84 µg/dl in comparison 6.95 µg/dl in cases with no opium exposure, that there were not statistically significant. (p=0.148) Conclusion: Based on the results of our study, clinicians are encouraged to take accurately attention about possible lead exposure and to rule out environmental hazards when evaluating for ADHD, particularly in young children and laboratory investigation for this toxin in high-risk cases and further researches recommende

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.

An uncommon presentation of mucopolysaccharidosis type IIIB similar to the landau-kleffner syndrome

Abstract Mucopolysaccharidosis type III (MPS III; Sanflilippo syndrome) is a metabolic disorder characterized by a lysosomal enzyme deficiency in the catabolic pathway of heparan sulphate. The patients with mucopolysaccharidosis type III usually present with declined neurocognitive functions such as speech and hearing loss. Subtle somatic features of patients with mucopolysaccharidosis type III can lead to diagnostic delay and consequently a greater neurocognitive deterioration may happen. Herein, we report a 9-year-old boy who had developed normally until the age of 4 years when his symptoms initiated with behavioral disturbances such as auditory agnosia and decreased verbal communication. Progression of his symptoms to seizure and ataxia, brain perfusion scan and electroencephalography features strongly suggested landau-kleffner syndrome; however, results of gene sequencing analysis and high urinary glycosaminoglycan excretion confirmed mucopolysaccharidosis type III as his final diagnosis. This case strongly recommends screening for metabolic disorders such as mucopolysaccharidosis type III in the patients diagnosed as having landau-kleffner syndrome.