A 10 Year-old Male Presenting with Generalized Edema

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Neuroimaging Finding of Organic Academia in Children

Organic academia are diverse group of disorders characterized by abnormal degredation of specific amino acid in the specific catabolism due to an enzymeactivity defect which mainly due to enzyme deficiency. The majority of the classic organic acid disorders are caused by abnormal catabolism of branched-chain amino acids like lysine. maple syrup urine disease (MSUD), propionic acidemia, methylmalonic acidemia (MMA), Ethylmalonic academia,glutaricacidemia type I and so on. The most common presentation is related to toxic encephalopathy which are as follows: vomiting, poor feeding,neurologic symptoms such as seizures and abnormal tone, and lethargy progressing to coma and the outcome may be loss of intellectual function, ataxia, psychiatric symptoms and other neurological deficit.Along with laboratory tests, Imaging have some characteristic findings in determination of these wide range of disease and can help in categorization and differentiation these form of brain metabolic disorders in conjunction by other investigative study. In this lecture trying to review imaging findings of more common organic academia in children will be done

A 10 Year-old Male Presenting with Generalized Edema

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Salivary and serum anti-desmoglein 1 and 3 ELISA and indirect immunofluorescence in pemphigus vulgaris: Correlations with serum ELISA, indirect immunofluorescence and disease severity

Anti-desmoglein (anti-Dsg) ELISA and indirect immunofluorescence (IIF) are used for the diagnosis of pemphigus vulgaris (PV). The value of salivary ELISA, serum ELISA, and IIF in the diagnosis of PV, and the correlation of salivary anti-Dsg1 and anti-Dsg3 ELISA with serum ELISA, serum and salivary IIF titers, and disease severity in patients with PV were evaluated. Fifty newly diagnosed patients with PV were enrolled in the study. Demographic data and disease-severity scores were recorded for each patient. Anti-Dsg1 and anti-Dsg3 ELISA and IIF were performed on both serum and salivary samples. Given the cut-off value of 20 RU/mL for Dsg1 and Dsg3, serum Dsg1 and Dsg3 ELISA were positive in 41 (82%) and 40 (80%) patients, and saliva Dsg1 and the Dsg3 ELISA were positive in 2 (4%) and 3 (6%) patients, respectively. Using the cut-off value of 13.4 RU/mL and 7.7 RU/mL for Dsg3 and Dsg1 salivary ELISA, 25 (50%) and 23 (46%) patients tested positive for Dsg3 and Dsg1, respectively. Serum IIF results were positive in 35 (70%) patients, and salivary IIF results were positive in 16 (32%) patients. Salivary anti-Dsg1 and anti-Dsg3 showed moderate correlations with the total pemphigus disease area index (PDAI) score (r=0.466, P&lt;0.001), (r=0.459, P&lt;0.001), respectively. A moderate correlation between serum IIF and salivary IIF was also detected (r=0.413, P&lt;0.001). Salivary anti-Dsg1 and anti-Dsg3 ELISA could be used as a safe and noninvasive method for the diagnosis of PV under certain circumstances, especially in children or elderly patients. Salivary ELISA is superior to salivary IIF. </p

Ultrasonographic measurement of subarachnoid space and frontal horn width in healthy Iranian infants

Objectiveultrasonography is among the most general evaluating methods for central nervous system (CNS) assessment, especially for detecting extra axial collection via anterior fontanel. There are few studies showing values of this technique in normal developing infants for detection of subarachnoid space width.Association between age and sex and cerebrospinal fluid (CSF) spaces are controversial. Therefore, we conducted this study to evaluate the relationship between subarachnoid space and sex and age in Iranian infants.Material & Methodswe used ultrasonography with a 7.5MHZ linear probe to evaluate 74 healthy infants who were referred to our departments for other reasons. Sinocortical width (SCW), craniocortical width (CCW),  interhemispheric width (IHW) and frontal horn width (FHW) were evaluated. Data was collected and analyzed using STAT 9.1 software.ResultsFifty four percent of the patients were male and 45% were female. Mean age of cases was 71 days. Mean SCW was 2.8 ± 1.33 mm (5% and95% were 1.2-5.8). Mean CCW was 2.52±1.37mm (5% and 95% were 1.1 and 5.2mm, respectively) and mean IHW was 4.39±2mm (5% and 95% were 1.7 and 8mm, respectively). Mean FHW was 2.9+/-1.09mm in females and 3.52±1.34mm in males (5% and 95% were 1.4 and 5mm in females & 1.7 and 5.8mm in males, respectively). There was no significant difference in subarachnoid space width between boys and girls except for FHW which was wider in males than females. (P=0.037). All space diameters correlated with age and were wider in older infants.ConclusionAlthough our sample size was rather small for accurate conclusion, we found a normal range which was wider than western countries but similar studies conducted in China. Delayed maturation of arachnoid villi is one of the most important reasons of subarachnoid space widening in infants younger than one year which seems occur later in Iranian infants.Key words: Ultrasonography;subarachnoid space; infant

Neuroimaging Findings in First Unprovoked Seizures: A Multicentric Study in Tehran

How to Cite This Article: Molla Mohammadi M, Tonekaboni SH, Khatami AR, Azargashb E, Tavasoli A, Javadzadeh M, Zamani GR. Neuroimaging Findings in First Unprovoked Seizures: A Multicentric Study in Tehran. Iran J Child Neurol. 2013 Autumn; 7(4):24-31.ObjectiveSeizure is an emergency in pediatrics. It really matters to the parents of the involved child to have information about the causes, management and prognosis.First unprovoked seizures (FUS) are seizures that occur in patients without fever, trauma or infection. Due to the rapid improvement in diagnostic techniques in the last few decades, the etiology will be revealed and this term will no longer exist. This Study was designed to evaluate brain imaging findings in FUS patients. Materials &amp; MethodsNinety-six children with FUS, who were admitted in three major children’s hospitals in Tehran, underwent brain imaging and were enrolled into the study.The decision about the type of imaging (CT or MRI) was based on the patient’s medical and financial conditions. An expert radiologist in the field of pediatric neuroimaging interpreted the images. ResultsAltogether, 27.1% had abnormal findings of which 29.2% were in the brain MRI group and 14.3% were in the brain CT scan group.Abnormal results were gliosis (10.4%), hemorrhage (4.2%), dysgenesis (2.1%), dysmyelination (7.3%), encephalomalacy (1%), atrophy (5.2%) and infarction (2.1%). In some patients, the lesions were in 2 or 3 sites and some had more than one type of lesion.There was no association between the duration, age and type of seizure and imaging abnormlities. However, we found an association between the location of the lesion and the type of seizure. ConclusionWe recommend brain imaging in all patients with FUS and apart from some exceptions, brain MRI is superior to CT. ReferencesJohnston MV. Siezure in childhood. In: Kliegman RM, Behrman RE, editors. Nelson text book of pediatrics. 18th ed. Philadelphia: Saunders; 2010. p. 2457-70.Bluestein JS, Moshe SL. First unprovoked seizure. In: Maria BL, editor. Currents in management in child neurology. 3rd ed. Hamilton: BC Decker; 2005. p. 89-92.Khodapanahandeh F, Hadizadeh H. Neuroimaging in children with first afebrile seizures: to order or not to order? Arch Iran Med 2006 Apr;9(2):156-8.Alawaneh H, Bataineh HA. Urgent neuroimaging in children with first nonfebrile seizures. Middle East JFam Med 2008 Feb;6(1):24-6.Shinnar S, O’Dell C, Mitnick R, Berg AT, Moshe SL. Neuroimaging abnormalities in children with an apparent first unprovoked seizure. Epilepsy Res 2001 Mar;43(3):261-9.Kalnin AJ, Fastenau PS, deGrauw TJ, Musick BS, Perkins SM, Johnson CS, et al. Magnetic resonance imaging findings in children with a first recognized seizure. Pediatr Neurol 2008 Dec;39(6):404-14.King MA, Newton MR, Jackson GD, Fitt GJ, Mitchell LA, Silvapulle MJ et al. Epileptology of the first-seizure presentation: a clinical, electroencephalographic, and magnetic resonance imaging study of 300 consecutive patients. Lancet 1998 Sep 26;352(9133):1007-11.Pohlmann-Eden B, Beghi E, CarnfieldC, Carnfield P. The first seizure and its management in adults and children. BMJ 2006 Feb;332(11):339-34.Raman S, Susan K, Joyce W. Paroxysmal disorders.In: Menkes J, editor. Child neurology. 7th ed. Philadelphia: Lipincott; 2006. p. 857-942.Wical B. The first unprovoked seizure.Gillette Children’sSpecialty Healthcare. A PediatricPerspective 1999 Mar;8(3).Bano S, Yadav SN.Neuroimaging in epilepsy.Medi-Focus2010 Apr-Sep;9(3&amp;4):2-4.Rauch DA,Carr E, Harrington J.Inpatient brain MRI for new-onset seizures: utility and cost effectiveness.Clin Pediatr (Phila) 2008 Jun;47(5):457-60.Gaillard WD, Chiron C, Cross JH, Harvey AS, Kuzniecky R, Hertz-Pannier L et al. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia 2009 Sep;50(9):2147-53.Barkovich AJ. Techniques and methods in pediatric neuroimaging. 4thed. Philadelphia: Lippincott Williams &amp;Wilkins; 2005. p. 4-7.Doescher JS, deGrauw TJ, Musick BS, Dunn DW, Kalnin AJ, Egelhoff JC et al. Magnetic resonance imaging and electroencephalic findings in a cohort of normal children with newly dignosed seizures. J Child Neurol 2006 Jun; 21(6):490-5. 

Lived Experiences of Patients Suffering from Acute Old World Cutaneous Leishmaniasis: a Qualitative Content Analysis Study from Iran

Background: The aim of this study was to explore the experiences of patients who suffer from acute cutaneous leishmaniasis in Iran, focusing on quality of life.Methods: The study was conducted at two different sites in Iran in 2010–2011. Individual in-depth interviews were conducted with six men and six women parasitologically confirmed acute cutaneous leishmaniasis. Interviews were recorded, transcribed verbatim, and translated into English. Qualitative content analysis was used for data analysis.Results: The participants, aged 23 to 63yr, had mild to severe disease. Based on the analysis four main themes were developed. "Fearing an agonizing disease" reflects patients' experiences of disease development resulting in sadness and depression, "struggling to cope" and "taking on the blame" both illustrate how patients experience living with the disease, which included both felt and enacted stigma as major social concerns. "Longing for being seen and heard" refers to patients' experiences with healthcare as well as their expectations and demands from communities and healthcare to be involved in closing the knowledge and awareness gap.Conclusion: Mental and social dimensions of cutaneous leishmaniasis were complex and adversely affected patients' lives by causing psychological burden and limiting their social interactions. Health authorities have to plan programs to increase the disease awareness to prevent the existing stigma to improve patients' social condition and medical care.</p

Association of serum magnesium levels with risk factors, severity and prognosis in ischemic and hemorrhagic stroke patients

Background: Stroke is the third leading cause of mortality worldwide. One of the factors that affect the occurrence of stroke can be attributed to changes in the levels of trace elements. Accumulating evidence has been shown that magnesium, as an important element, is a new predictor of stroke. We aimed to determine the levels of Mg in ischemic stroke patients in comparison with those having the hemorrhagic type. Methods: This study was conducted on 447 stroke patients. Demographic characteristics of patients, stroke severity, and risk factors such as hypertension, ischemic heart disease, diabetes mellitus, and hyperlipidemia were recorded. Stroke was diagnosed based on the neurological examination and neuroimaging findings e.g. computed tomography (CT) or magnetic resonance imaging (MRI). The colorimetric technique was used to determine the concentration of Mg at 450 nm according to the commercial kit. Results: The mean of magnesium levels in ischemic patients was significantly higher than that in the hemorrhagic patients (P=0.001). Difference in magnesium status was associated with gender in thrombotic patients (P&#60;0.05), while hyperlipidemia was associated with the status of magnesium in embolic patients (P=0.012). Furthermore, magnesium levels were correlated with ischemic heart disease in embolic (P=0.011) and sub-arachnoid hemorrhagic (SAH) patients (P=0.012), and with diabetes mellitus in thrombotic patients (P=0.012). Magnesium status was associated with the severity of ischemic stroke at the time of discharge in ischemic patients (P&#60;0.001). Mg levels had the best area under curve (AUC) for the discrimination of ischemic patients from hemorrhagic ones. Conclusion: Magnesium levels were higher in ischemic patients compared to hemorrhagic ones, and these levels were associated with many risk factors contributing to a stroke. Magnesium may be used as a new predictor of stroke in ischemic patients as opposed to hemorrhagic ones. &#160

Twenty-three unsolved problems in hydrology (UPH) – a community perspective

This paper is the outcome of a community initiative to identify major unsolved scientific problems in hydrology motivated by a need for stronger harmonisation of research efforts. The procedure involved a public consultation through on-line media, followed by two workshops through which a large number of potential science questions were collated, prioritised, and synthesised. In spite of the diversity of the participants (230 scientists in total), the process revealed much about community priorities and the state of our science: a preference for continuity in research questions rather than radical departures or redirections from past and current work. Questions remain focussed on process-based understanding of hydrological variability and causality at all space and time scales. Increased attention to environmental change drives a new emphasis on understanding how change propagates across interfaces within the hydrological system and across disciplinary boundaries. In particular, the expansion of the human footprint raises a new set of questions related to human interactions with nature and water cycle feedbacks in the context of complex water management problems. We hope that this reflection and synthesis of the 23 unsolved problems in hydrology will help guide research efforts for some years to come