FEBRILE CONVULSION: ANOTHER LOOK AT AN OLD SUBJECT

Febrile convulsion (FC), an occurrence frequently encountered in everyday practice, is discussed in this article with a review of corresponding literature.Taking into account the extent of debate on the topic, from FC being considered a kind of epileptic seizure to its being viewed as a nonepileptic phenomenon, our aim is not to be judgmental regarding its nature in the present writing. Two distinct groups of children, who convulse with fever are described; one, the group whose neurological status is suboptimal and the other children who one who enjoy good health. In this review, the clinical aspects of management of fever, a forerunner of a seizure are emphasized. The other important aspect of handling a case of febrile convulsion consists of controlling the seizure, which should be done without any delay when it occurs. Nowadays, the drugs of choice are diazepines, used via the rectal, buccal or intranasal routes. The most important area of investigation is lumbar puncture in a child who has had a febrile convulsion, which will be discussed at the end

Intravenous Immunoglobulin PER SE or Combined with Intravenous Methylprednisolone in Children With GBS; Comparing the Effects

ObjectiveGuillain-Barre syndrome (GBS) is the most common cause of acute neuromuscular paralysis in children, its pathogenesis most probably involving an autoimmune response to Schwann cell or peripheral nerve myelin antigens. Steroid regimes improve demyelinating diseases such as chronic GBS. We assessed the benefit of high dose methylprednisolone (MP) combined with Intravenous immunoglobulin (MP-IVIG) and compared the effects with those of IVIG per se in children with GBS.Materials& MethodsThirty-six children, aged between 1-12 years were randomized to receive IV MP 20mg/kg/day combined with IVIG 400 mg/kg/day (MP-IVIG) or IVIG per se at same dose for 5 days. All patients were diagnosed by standard clinical criteria and entered the trial within less than 2 weeks of the onset of neurological symptoms. All patients were too weak to walk. Functional grade (FG) was at 3 or more (able to walk with support).ResultsIn the MP-IVIG group, FG improved at least one grade after 5 days of treatment. ConclusionThis study suggests that combined treatment with MP-IVIG in children with GBS does cause rapid improvement in the acute phase, but does not result in any significant difference in the long term outcome.

HIGH AND LOW DOSE IVIG THERAPY IN GUILLAIN-BARRE SYNDROME CHILDREN: A COMPARISON

Objective:Acute inflammatory demyelinating peripheral neuropathy (Guillain-Barre-Syndrome) is by far the most common cause of immune-medicated peripheral nervous system disease in children; with the near disappearance of poliomyelitis, GBS is responsible for the great majority of cases of acute flaccid paralysis. So far, in several controlled studies, corticosteroids, plasmapheresis and IVIG have been utilized in pediatric patients, afflicted with GBS. Regarding IVIG therapy, two methods have been used; the high dose (1 gr/kg/day for 2 days), and the low dose (400mg/kg/day for 5 days). Review of literature shows that a faster rate of recovery can be accomplished in patients who receive total dose of IVIG in 2 days as compared to the dose being given over 5 days.Materials & Methods:In this study we have compared these two types of treatment in an investigation, conducted in the Mofid Children Hospital on pediatric patients who had sudden onset of acute flaccid  paralysis, and were diagnosed as having GBS. Based on histories, physical examination and electrodiagnosis, subjects were divided in two groups, the high dose IVIG treatment, 1gr/kg/day for 2 days (experimental group), and the low dose IVIG treatment, 400 mg/kg/day for 5 days (control group). Statistical analyses were then carried out using the appropriate software.Results:Result of this study showed a faster rate of recovery for patients in the high dose IVIG group; in this group duration of weakness of limbs was shorter and returning of DTR was faster than in controls. In fact, in this type of treatment, the relationship between high dose IVIG therapy and drug side effects was not significant.Conclusion:Base upon the finding in the present study, we conclude that the high dose IVIG therapy is superior to low dose, in view of faster duration of recovery and shorter hospital stay. Also we may infer that shorter hospital stay could be a factor in reducing of more nasocomial infection. In conclusion, we suggest using high dose IVIG treatment of choice in GBS.Keywords:Gulliain Barre Syndrome-High dose, Low dose IVIGcomparison of two types of treatmen

Application of FFTBM with signal mirroring to improve accuracy assessment of MELCOR code

This paper deals with the application of Fast Fourier Transform Base Method (FFTBM) with signal mirroring (FFTBM-SM) to assess accuracy of MELCOR code. This provides deeper insights into how the accuracy of MELCOR code in predictions of thermal-hydraulic parameters varies during transients. The case studied was modeling of Station Black-Out (SBO) accident in PSB-VVER integral test facility by MELCOR code. The accuracy of this thermal-hydraulic modeling was previously quantified using original FFTBM in a few number of time-intervals, based on phenomenological windows of SBO accident. Accuracy indices calculated by original FFTBM in a series of time-intervals unreasonably fluctuate when the investigated signals sharply increase or decrease. In the current study, accuracy of MELCOR code is quantified using FFTBMSM in a series of increasing time-intervals, and the results are compared to those with original FFTBM. Also, differences between the accuracy indices of original FFTBM and FFTBM-SM are investigated and correction factors calculated to eliminate unphysical effects in original FFTBM. The main findings are: (1) replacing limited number of phenomena-based time-intervals by a series of increasing time-intervals provides deeper insights about accuracy variation of the MELCOR calculations, and (2) application of FFTBM-SM for accuracy evaluation of the MELCOR predictions, provides more reliable results than original FFTBM by eliminating the fluctuations of accuracy indices when experimental signals sharply increase or decrease. These studies have been performed in the framework of a research project, aiming to develop an appropriate accident management support tool for Bushehr nuclear power plant. 2016 Elsevier B.V. All rights reserved

New turbulence modeling for simulation of Direct Contact Condensation in two-phase pressurized thermal shock

Injection of Emergency Core Cooling System (ECCS) water into the primary loops of the Pressurized Water Reactors (PWRs) leads to rapid cooling of Reactor Pressure Vessel (RPV) inside wall after Loss Of Coolant Accident (LOCA). This condition, known as Pressurized Thermal Shock (PTS) intensifies the propagation of the RPV structural defects and would be considered as an ageing mechanism. For structural and fracture analysis of RPV wall, thermal-hydraulic analysis of PTS should be accomplished to obtain the steam/water flow characteristics in the downcomer. For this purpose, simulation of steam/water stratified flow (due to density difference) after the injection point should be done by Computational Fluid Dynamics (CFD) methods. In this region, steam condensation over water layer is considered as the only heat source and controlled by turbulence eddy motion near the steam/water interface. Based on Surface Renewal Theory (SRT), Heat Transfer Coefficient (HTC) would be calculated by evaluation of turbulence length and velocity. Therefore, prediction of turbulence characteristics plays a significant role for estimation of interfacial mass transfer and temperature profile. High gradient of velocity and Turbulence Kinetic Energy (TKE), and interfacial mass and momentum transfer at the steam/water interface needs some modifications for application of traditional turbulence models. Implementation of damping function is one of the common solutions to overcome the overestimation of TKE at the steam/water interface. Although, this function improves flow characteristics of smooth stratified flow, investigation of conservation equations and experimental data implies that the other source function is needed when the flow regime changes to wavy flow. In this paper, a new source function of TKE based on variations of turbulence characteristics is proposed for steam/water interface leading to a special boundary condition of turbulence. To investigate the effects of this modification, simulation of air/water and steam/water stratified flow in three different test facilities is performed. The results show that the implementation of the source function of TKE improves the prediction of turbulence characteristics at the interface of isothermal stratified flow. Also condensation rate and temperature gradient of steam/water stratified flow have a better agreement with experimental data

32 CASES OF PSEUDOTUMOR CEREBRI IN TWO PEDIATRIC HOSPITALS OF TEHRAN, IRAN

Objective:Pseudotumor Cerbri (PTC) is a clinical syndrome characterized by increased Intra-Cranial Pressure (ICP) without any evidence of a mass lesion or any obstructive process. The incidence is 0.9-2 per 100.000 people. It is more frequent in adults in the 20-30 years age group, especially obese women, and less common in pediatric age groups; 11-16 years old children may however suffer from PTC, with no difference in the rates of occurrence in either sex.Material & Methods:In this descriptive retrospective study we reviewed the files of 32 children with diagnosis of PTC admitted during the past 15 years in the neurology ward of the Mofid Children's Hospital (25) and Ali Asghar Children Hospitals (7) between the years 1988 and 2003.Results:The results of this study revealed that children in the 5-10 years age group, girls in particular, are more vulnerable.Conclusion:The most frequent complaints that brought these patients to physician included headache, vomiting and strabismus. Common findings of neurological examination were papilledema, abducent nerve palsy and ataxic gait. While all cases recovered with medical treatment, one did need surgical intervention. Abbreviations; Pseudotumor Cerbri = PTC; Intra Cranial pressure = ICP.Keywords :PTC , papilledema , ICP

100 CHILDREN WITH ACUTE ATAXIA; A SURVEY IN MOFID CHILDREN'S HOSPITAL

Objective:The term "Ataxia" refers to disturbances of body posture and movement that are normally controlled by the cerebellum, frontal lobes and the posterior columns of the spinal cord. The primary symptom and the most prominent feature of ataxia is abnormal gait which is characterized by lurching and wide base walking. Ataxia was considered acute, if it had occurred within the two preceding weeks. Knowing how frightening acute-onset Ataxia is for the family is not surprising that the condition prompts an immediate visit to the physician.Material & Methods:In view of the lack of information in our country, on the etiology of sudden-onset Ataxia, the authors enrolled 100 children with the chief complaint of acute loss of equilibrium, who came to the attention of the Pediatric Neurology Department over a two year duration (Sept.2001-Sept 2003); they were admitted to the Mofid Childrens' Hospital and all necessary investigations were carried out.Results & Conclusion:The results revealed that Acute Cerebellar Ataxia was the most common cause of the problem, the second most frequent being drug intoxication, which most commonly occurred in patients, 2- 4years old. The remaining causative factors in order of descending frequency consisted of infectious polyneuropathy, migraine, opsoclonus-myoclonus, brain tumor, acute disseminated encephalomyelitis, multiple sclerosis, and epilepsy.Key words:Acute ataxia, Children, Acute cerebellar ataxi

MYASTHENIA GRAVIS IN IRANIAN CHILDREN

ObjectiveThis study was undertaken to evaluate the clinical spectrum of myasthenia gravis in children and determine factors that help the clinician in his/her diagnosis and management.Materials & MethodsA retrospective review was performed on all pediatric patients suffering from myasthenia gravis (M.G) admitted in the department of pediatric neurology of the Mofid Hospital of the Shaheed Beheshti University, between 1994 and 2002.ResultsOf the thirty-two children with M.G. enrolled in our study, seven were suffering from the congenital type while the remaining (25 cases) had the juvenile M.G. Initial symptoms of congenital M.G were ptosis (7/7), limitation of eye movement (2/7) and mild generalized weakness (6/7). Although the Tensilon test was positive in 85% of congenital M.G cases, no myasthenia crisis or spontaneous remission was observed in any of them. In children with juvenile M.G, the age of presentation was 1.2 to 12.5 years, mean age 5.7+4.2 years (15 girls and 10 boys). The most common presenting symptoms in juvenile group were ptosis in 96% and generalized weakness in 76%. Eight of them (32%) had had at least one myasthenia crisis. EMG was diagnostic in 83% and one case the tensilon test was positive in 84%. One patient had hyperthyroidism and had already been diagnosed with hypothyroidism; two of them were epileptics. Eight patients underwent thymectomy microscopically; in specimens examined, five (62%) showed thymic follicular hyperplasia while in remaining three results were normal. One patient (12.5%) recovered completely after thymectomy with no need for medication during the follow up. Four patients (50%) showed relative  improvement and in three cases (37%) improvement was negligible.ConclusionThe results showed a female to male ratio of 1.5/1 which was correlated to adult M.G. The most common presenting symptoms consisted of ophtalmoplegia, with bilateral ptosis being the most significant. Although this study revealed that thymectomy lacks any remarkable prognostic influence, all patients had thymectomy after two years of disease onset. Some reports have indicated positive results if surgery was performed within two years of onset of disease.Key words: Myasthenia gravis, Children, Thymectomy, Congenita

Intractable Seizure Disorders: Efficacy of The Classic Ketogenic Diet

 ObjectiveThe ketogenic diet is a high-fat, low carbohydrate, adequate protein diet,developed in the 1920s for the management of intractable seizure disorders in children. To evaluate efficacy and tolerability of the classic  ketogenic diet, we analyzed records of the children started on the diet from 1999 to 2006 at the Mofid children's hospital.Materials & Methods The subjects were 87 children, mean age 55 months. Before initiation of the diet, 55% of the patients had seizures, at least 1-4 times per day, 36% - 5 or more per day and 9% - 2 to 4 times per week. Mean number of Anti Epileptic Drugs (AEDs) tried for them was 8 and 67% were receiving three or more drugs.Results The ketogenic diet showed drastic improvement, with at least 50% reduction in seizure frequency in 87% of our patients, 39% of whom showed complete seizure control in the third month. After one year, in 80% of the patients who returned, improvement  continued, with 26% of them being seizure free; besides, 23% had one AED decreased, 36% had two or three AEDs decreased, and 25% (one child) had all AEDs discontinued. Of the 30 improved cases, 20%, at the end of the first year, had improved behavior as  well, and 23% of them had become more alert. The median diet duration of the improved group was 15 months.Conclusion The improvement in our patients, low  side effects, and the duration of diet by families reveal that the ketogenic diet can still be a very useful alternative therapy in certain epileptic children.