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Objectives Extra gastrointestinal symptoms in celiac disease (CD), such as neurological manifestations, might be common in pediatrics. The present study aimed to evaluate the frequency of CD in children with idiopathic epilepsy. Materials & Methods In a cross-sectional study, signs and symptoms of CD were evaluated in 40 children aged 2-14 years with idiopathic epilepsy who were referred to the Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd, Iran. Then, serum levels of tissue transglutaminase antibody (tTG) and total IgA were measured in them. Upper gastrointestinal endoscopy and small intestine biopsy were recommended for patients with abnormal serum IgA Anti-tTG. Results Eighteen girls and 22 boys with a mean age of 5.29±2.4 years were evaluated. In this study, only three patients (7.5%) with epilepsy had abnormal serum IgA Anti-tTG and serum Total IgA. Upper gastrointestinal endoscopy and pathological examination of duodenal biopsy of those three children reported total villous atrophy (Marsh type 3). The age of onset of seizures in children with CD was more than three years, while in children without CD, 62.2% of cases were less than three years. These results indicate that CD is associated with the age of onset of seizures in children. Conclusion Due to the accompaniment of celiac with neurological manifestations, patients with neurological symptoms and gastrointestinal symptoms should be examined for celiac. [ABSTRACT FROM AUTHOR]

Objective The imbalance of phenylalanine (PA) to tyrosine level and decreased dopamine brain level in patients with phenylketonuria (PKU) may have a role in their susceptibility of them to attention-deficit/hyperactivity disorder (ADHD). This study aimed to evaluate the frequency of ADHD in referred patients to PKU Clinic in Yazd, Iran. Materials & Methods In this cross-sectional analytical study, all patients older than three years with PKU who were referred to the PKU Clinic of Shahid Sadoughi Hospital, Yazd, Iran, in 2018 were evaluated, and ADHD symptoms in them were assessed via parent face-to-face interview. The patients were diagnosed with ADHD if they scored at least of 20 on ADHD diagnostic rating scale via parent interview based on DSMVI criteria. Results Fourteen boys and 21girls with a mean age of 9.55±1.8 years were evaluated. 51.5% of those diagnosed with PKU had ADHD. Accordingly, ADHD was more frequent in girls (77.8% vs. 41% in boys, P=0.03). The mean age of diagnosis of PKU was significantly higher in patients with ADHD (52.54±15.65 months vs. 29.75±9.65 months, P = 0.03). The mean of PA level in the last six months (15.59±5.95 vs. 8.72+5.18, P= 0.005) and mean of the last six PA levels (14.76±4.71 vs. 8.96±3.86, P= 0.03) were significantly higher in ADHD group. Conclusion The prevalence of ADHD in phenylketonuria patients in the present study was much more than in other studies. Late diagnosis of PKU and long-term high PA blood and brain level might be associated with increased neonatal screening. Hence, regular follow-up and continuous evaluation of patients with PKU for ADHD symptoms should be performed. [ABSTRACT FROM AUTHOR]

Objective The imbalance of phenylalanine (PA) to tyrosine level and decreased dopamine brain level in patients with phenylketonuria (PKU) may have a role in their susceptibility of them to attention-deficit/hyperactivity disorder (ADHD). This study aimed to evaluate the frequency of ADHD in referred patients to PKU Clinic in Yazd, Iran. Materials & Methods In this cross-sectional analytical study, all patients older than three years with PKU who were referred to the PKU Clinic of Shahid Sadoughi Hospital, Yazd, Iran, in 2018 were evaluated, and ADHD symptoms in them were assessed via parent face-to-face interview. The patients were diagnosed with ADHD if they scored at least of 20 on ADHD diagnostic rating scale via parent interview based on DSMVI criteria. Results Fourteen boys and 21girls with a mean age of 9.55±1.8 years were evaluated. 51.5% of those diagnosed with PKU had ADHD. Accordingly, ADHD was more frequent in girls (77.8% vs. 41% in boys, P=0.03). The mean age of diagnosis of PKU was significantly higher in patients with ADHD (52.54±15.65 months vs. 29.75±9.65 months, P = 0.03). The mean of PA level in the last six months (15.59±5.95 vs. 8.72+5.18, P= 0.005) and mean of the last six PA levels (14.76±4.71 vs. 8.96±3.86, P= 0.03) were significantly higher in ADHD group. Conclusion The prevalence of ADHD in phenylketonuria patients in the present study was much more than in other studies. Late diagnosis of PKU and long-term high PA blood and brain level might be associated with increased neonatal screening. Hence, regular follow-up and continuous evaluation of patients with PKU for ADHD symptoms should be performed. [ABSTRACT FROM AUTHOR]

Objective One-third of epilepsy of children is refractory, and this study was conducted to evaluate the efficacy and adverse events of levetiracetam as add-on therapy in the treatment of refractory epilepsy of children. Materials & Methods In this quasi-experimental study, seizures frequency and side effects of 314 children aged 1-14 years with refractory epilepsy were referred to the Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd, Iran, and treated with levetiracetam for six months, were evaluated. Results We evaluated 142 girls and 172 boys with a mean age of 6.78±2.12 years. At the end of six months of treatment with levetiracetam, 20% became seizure-free, 28% had more than 50% decrease in seizure frequency, 38% did not have a notable change in seizure frequency, and 14% experienced an increase in seizure frequency. Good response (stopping of all seizures or more than 50% reduction in seizure frequency) was seen in 51% of mixed types, 61% of myoclonic seizures, 64% of generalized tonic-clonic seizures, 69 % of partial seizures, 100 % of tonic seizures, and in 40 % of atonic seizures. Levetiracetam was significantly more effective in partial seizures, idiopathic epilepsies, and children with normal developmental status and normal brain MRI. Twelve children discontinued the treatment due to severe drowsiness, restlessness, and exacerbation of seizures. Transient and mild side effects, including somnolence, anorexia, fatigue, headache, ataxia, and diplopia, were seen in 9% (N=28) of patients. Conclusion Levetiracetam could be considered an efficient and safe adjunct therapy in treating refractory epilepsy in children. Keywords: Levetiracetam; Refractory Epilepsy; Children; Side effects [ABSTRACT FROM AUTHOR]

Objective Topiramate is effective in the prevention of pediatric migraine, and studies show that vitamin D3 supplementation might also be useful in the treatment of adult migraineurs with a normal vitamin D3 level. The present study aimed at comparing the efficacy and safety of topiramate plus vitamin D3 and topiramate alone in the prophylaxis of pediatric migraine. Materials & Methods In a single-blinded, randomized, clinical trial, 5-15-year-old children with migraine headaches, referred to the Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd, Iran from January 2016 to January 2017, were randomly allocated to receive 2 mg/kg/day of topiramate or 2 mg/kg/day of topiramate plus one 500,000 IU vitamin D3 pearl weekly for two consecutive months. Primary outcomes were the reduction of monthly frequency, severity, duration, and the disability score of migraine, and the secondary outcomes included a good response to treatment (more than 50% reduction in monthly headache frequency) and a lack of clinical adverse events. Results Totally, 31 female and 26 male children with the mean age of 10.02±2.11 years were evaluated. Both drugs were effective in the reduction of monthly frequency, severity, duration, and disability for headaches. Nevertheless, the combination of topiramate and vitamin D3 was more effective than topiramate alone in reducing the monthly headaches frequency (6.12±1.26 vs. 9.87±2.44 times, P=0.01) and disability score (19.24±6.32 vs. 22.11±7.91, P=0.02). Good response to treatment was observed in 60.7% and 75.9% of the subjects in the topiramate alone and topiramate plus vitamin D3 groups, respectively, and topiramate plus vitamin D3 was more effective (P= 0.01). Transient mild side effects were observed in 14.3% and 17.2% of the subjects in the topiramate alone and topiramate plus vitamin D3 groups, respectively (P=0.8). Conclusion A combination of Topiramate and vitamin D3 might be considered safe and more effective than Topiramate alone in the prophylaxis of pediatric migraine. [ABSTRACT FROM AUTHOR]

Objectives Midazolam at a dosage of 0.5 mg/kg induces anxiolytic effects in 90% of children. This study was performed to elucidate whether intravenous midazolam with maternal presence is more efficient than intravenous midazolam alone in the reduction of pain and anxiety of lumbar puncture (LP) in 6 to 24-month-old children. Materials & Methods In this not-blinded clinical trial, we included 60 children aged 6 to 24 months old undergoing LP in the Pediatric Ward of Shahid Sadoughi Hospital, Yazd, Iran, from September 2014 to March 2015. The participants were randomly assigned to two groups, and all of them received painless injection of 0.5 mg/kg midazolam five minutes before LP. In group I, LP was performed with maternal presence and in group II, the mothers were absent. The primary outcomes included anxiety and pain scores before LP and during needle insertion to the skin for LP. The secondary outcomes comprised of success rates in the reduction of anxiety (anxiety score of four and more) and pain (pain score of less than three) when the needle was inserted to the skin for LP. Results Twenty-eight girls and 32 boys were evaluated in the two groups. Maternal presence was found to be effective in the reduction of anxiety (2.7±0.65 vs. 3.83±0.87; P=0.001) and pain scores (3.8±1.75 vs. 6.1±1.63, P=0.001). In the maternal presence group, success rate in anxiety (76.7% vs. 16.6%; P=0.0001) and pain reduction (63.3 % vs. 6.7%; P=0.0001) was higher than in the midazolam alone group. Conclusion Maternal presence during lumbar puncture can reduce pain and anxiety among 6 to 24-month-old children. [ABSTRACT FROM AUTHOR]

Objectives We aimed to answer the question whether or not previous antiepileptic drugs with combination of omega-3 and risperidone are more efficient than previous antiepileptic drugs with risperidone alone in decreasing of seizures monthly frequency of children with refractory epilepsy and attention-deficit/hyperactivity disorder (ADHD). Material & Methods In a randomized clinical trial (IRCT201604212639N18), participants referred to Pediatric Neurology Clinic of Shahid Sadoughi Hospital, Yazd, Iran from Jun 2015 were distributed randomly into two groups. In group I, one capsule of omega-3 daily and 0.5 mg of risperidone was divided into two doses with previous antiepileptic drugs and in group II, 0.5 mg of risperidone was divided into two doses with previous antiepileptic drugs were given. The drugs use was continued for three months and the children were followed up monthly for three consecutive months. Primary outcomes included seizure monthly frequency and good response (more than 50% of reduction in seizures monthly frequency). Secondary outcome was clinical side effects. Results Overall, 23 girls and 33 boys with mean age of 9.24±0.15 yr (29 children in omega-3 group and 27 children in control group) were evaluated. Omega-3 therapy was effective in decreasing of seizures monthly frequency (10.41±3.92 times vs. 17.01±4.98, P=0.03). Good response was seen in three children (11.1%) in control (95% confidence interval: 8%-22.8%) and in 9 children (31%) in omega-3 (95% CI: 47.83%-14.17%) group, which showed that omega-3 was more effective in seizure control. (P=0.001). Frequency of side effects was not different in the two groups (14.8 % in control vs. 20.7% in omega-3 groups, P=0.5). Conclusion Omega-3 might be used as an effective and safe drug in seizures control of children with refractory epilepsy and ADHD. [ABSTRACT FROM AUTHOR]

Objective The aim of the present research was to compare the effectiveness and tolerability of melatonin and amitriptyline in pediatric migraine prevention. Materials & Methods In a parallel single-blinded randomized clinical trial, 5-15 yr old children with diagnosis of migraine that preventive therapy was indicated in whom and were referred to Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd-Iran from 2013-2014, were randomly allocated to receive 1 mg/kg amitriptyline or 0.3 mg/kg melatonin for three consecutive months. Results Forty one girls (51.3%) and 39 boys (48.7%) with mean age of 10.44±2.26 yr were evaluated. Good response was seen in 82.5% of amitriptyline and 62%.5 of melatonin groups and amitriptyline was statistically significant more effective (P=0.04). Severity, duration and Pediatric Migraine Disability Assessment score (PedMIDAS) of headache reduced with melatonin from 6.05±1.63 to 4.03±1.54 scores, from 2.06±1.18 to 1.41± 0.41 hours, and from 33.13±9.17 to 23.38±9.51 scores, respectively. Severity, duration and PedMIDAS of headache decreased with amitriptyline from 6.41±1.67to 2.25±1.21, from 2.55 ±1.85to 0.56±0.51h, and from 31.4±9.33 to 8.28 ± 3.75, respectively (All P < 0.05). Both drugs were effective in reduction of monthly frequency, severity, duration and disability of headache. Daily sleepiness was seen in 7.5% of melatonin group as a side effect and adverse events were seen in 22.5% of amitriptyline group including daily sleepiness in four, constipation in three and fatigue in two children and melatonin was safer than amitriptyline (value = 0.04). Conclusion Amitriptyline and melatonin are effective and safe in pediatric migraine prophylaxis but amitriptyline can be considered as a more effective drug. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Suitable magnetic resonance imaging (MRI) techniques from conventional to new devices can help physicians in diagnosis and follow up of Multiple Sclerosis (MS) patients. The aim of present research was to compare effectiveness of Fluid Attenuation Inversion Recovery (FLAIR) sequence of conventional MRI and Diffuse Weighted Imaging (DWI) sequence as a new technique in detection of brain MS plaques. MATERIALS & METHODS: In this analytic cross sectional study, sample size was assessed as 40 people to detect any significant difference between two sequences with a level of 0.05. DWI and FLAIR sequences of without contrast brain MRI of consecutive MS patients referred to MRI center of Shahid Sadoughi Hospital, Yazd, Iran from January to May 2012, were evaluated. RESULTS: Thirty-two females and 8 males with mean age of 35.20±9.80 yr (range = 11-66 yr) were evaluated and finally 340 plaques including 127(37.2%) in T2WI, 127(37.2%) in FLAIR, 63(18.5%) in DWI and 24(7.1%) in T1WI were detected. FLAIR sequence was more efficient than DWI in detection of brain MS plaques, oval, round, amorphous plaque shapes, frontal and occipital lobes, periventricular, intracapsular, corpus callosum, centrum semiovale, subcortical, basal ganglia plaques and diameter of detected MS plaques in DWI sequence was smaller than in FLAIR. CONCLUSION: Old lesion can be detected by conventional MRI and new techniques might be more useful in early inflammatory phase of MS and assessment of experimental treatments. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Reduction of pain of invasive procedures in neonates can prevent pain side effects. The purpose of present study was to evaluate the efficacy and safety of a single low dose of intravenous fentanyl in reducing of lumbar puncture (LP) pain in neonates. MATERIALS & METHODS: In this randomized clinical trial, registered with code number of 2014022616761N150, admitted neonates to Shahid Sadoughi Hospital, Yazd, Iran from August-April 2012 (45 cases) were randomly assigned into two groups to receive 2 μg/kg of intravenous fentanyl or 0.2 milliliter of normal saline, two min before LP. Primary outcome was success rate in reducing of pain during needle insertion to skin (pain score of less than three). Secondary outcomes were clinical side effects and serious adverse events. RESULTS: Forty-five neonates including 23 girls and 22 boys were evaluated. Pain reduction was obtained in 39.1% (9 of 23 neonates) of fentanyl group and in 4.5% (one of 22 neonates) of control group. Means of pulse rate (136.41± 9.16 vs. 148.9± 8.99) and pain score during needle insertion (3.41±1.31 vs. 5.8±1.12) were lower in fentanyl group. No severe adverse effects were seen in both groups. Side effects such as vomiting [9% (N=2) in control and 4.3% (N=1) in fentanyle group] and mild transient decrease in oxygen saturation in 8.7% (N=2) of fentanyle group were seen. Safety in two groups was not statistically different. CONCLUSION: Intravenous fentanyl might be considered as a safe and effective analgesic drug in LP in neonates. [ABSTRACT FROM AUTHOR]

Procedural sedation may be needed in many diagnostic and therapeutic procedures in children. To make pediatric procedural sedation as safe as possible, protocols should be developed by institutions. Response to sedation in children is highly variable, while some become deeply sedated after minimal doses, others may need much higher doses. Child developmental status, clinical circumstances and condition of patient should be considered and then pharmacologic and non-pharmacologic interventions for sedation be selected. Drug of choice and administration route depend on the condition of the child, type of procedure, and predicted pain degree. The drugs might be administered parenteral (intravenous or intramuscular) or non- parenteral including oral, rectal, sublingual, aerosolized buccal and intranasal. The use of intravenous medication such propofol, ketamine, dexmedetomidine, or etomidate may be restricted in use by pediatric anesthesiologist or pediatric critical care specialists or pediatric emergency medicine specialists. In this review article we discuss on non-parenteral medications that can be used by non-anesthesiologist. [ABSTRACT FROM AUTHOR]

Objective Early diagnosis and treatment of congenital hypothyroidism (CH) and the prevention of developmental retardation is the main goal of public health national screening programs. This study compares the cognitive ability of children with CH diagnosed by neonatal screening with a healthy control group (2007) in Yazd, Iran. Materials & Methods In a case-controlled study, the intelligent quotient (IQ) of 40 five-year-old children with early treated CH and good compliance were evaluated by the Wechsler preschool and primary scale of intelligent test and compared to 40 healthy age and gender matched children as controls. Results 22 boys (55%) and 18 girls (45%) in both groups were evaluated. In children with CH, 19 (47.5%) and 21 (52.5%) persons had transient and permanent hypothyroidism, respectively. Range of TSH and T4 level at the onset of diagnosis were 11.41-81 mu/l and 1.50-14.20 µg/dl, respectively. The intelligence levels of all children with CH were within the average or normal range and IQs ranged from 91-108. Children with CH had lower full-scale IQs (107.25 ± 2. 9 versus 110.50 ± 2.66, p=0.001), verbal IQ (106.95 ± 3.5 versus 109.90 ± 3.44, P-value=0.001) and performance IQ (106.3 ± 3.68 versus 108.87 ± 3.70) than the control group. However, no statistically significant differences were observed for mean IQ scores in permanent and transient CH. Conclusion Children with CH who had early treatment and good compliance had normal cognitive abilities, but may have a decreased IQ relative to the healthy control group. [ABSTRACT FROM AUTHOR]

Objective Seizures are the most common pediatric neurologic problem. Research of the association between iron deficiency and seizures has shown conflicting results. This study evaluates iron status of children with a first seizure attack (febrile seizure (FS) or first unprovoked afebrile seizure (FUS) and healthy control group. Materials & Methods In a cross sectional case control study, iron status of 6-60 month year old admitted children with first seizure to Shahid Sadoughi Hospital from August 2011-December 2012 were evaluated and compared with healthy control children that were referred to primary health care center of Azadshar, Yazd, Iran. Results 150 children were compared in three equal (FS, afebrile seizure, and control) groups. Hemoglobin levels in FUS (11.39 ± 1.07 g/dl) and FS (11.46 ± 1.18 g/dl) were lower than the control group (11.9 ± 0.89 g/dl) group. Serum iron levels in FS (38.52 ± 11.38 μg/dL) and FUS (42.68 ± 14.76 μg/dL) were lower than the control group (54.32 ± 13.46 μg/dL). Serum ferritin level in FUS (46.21 ± 27.63 ng/mL) and FS (48.91 ± 22.96 ng/ mL) was lower than the control group (75.13 ± 35.57 ng/mL). Iron deficiency (48% in FS, 44% in FUS and 28% in control group) and iron deficiency anemia (26% in FUS, 22% in FS, and 10% in healthy children) was more frequent in children with seizures. Conclusion Iron status should be evaluated in children with a first attack of febrile or afebrile seizures. [ABSTRACT FROM AUTHOR]

Objective Magnetic resonance imaging (MRI) is a useful diagnostic tool for the evaluation of congenital or acquired brain lesions. But, in all of less than 8-year-old children, pharmacological agents and procedural sedation should be used to induce motionless conditions for imaging studies. The purpose of this study was to compare the efficacy and safety of combination of chloral hydrate-hydroxyzine (CH+H) and chloral hydrate-midazolam (CH+M) in pediatric MRI sedation. Materials & Methods In a parallel single-blinded randomized clinical trial, sixty 1-7-year-old children who underwent brain MRI, were randomly assigned to receive chloral hydrate in a minimum dosage of 40 mg/kg in combination with either 2 mg/kg of hydroxyzine or 0.5 mg/kg of midazolam. The primary outcomes were efficacy of adequate sedation (Ramsay sedation score of five) and completion of MRI examination. The secondary outcome was clinical side-effects. Results Twenty-eight girls (46.7%) and 32 boys (53.3%) with the mean age of 2.72±1.58 years were studied. Adequate sedation and completion of MRI were achieved in 76.7% of CH+H group. Mild and transient clinical side-effects, such as vomiting of one child in each group and agitation in 2 (6.6 %) children of CH+M group, were also seen. The adverse events were more frequent in CH+M group. Conclusion Combinations of chloral hydrate-hydroxyzine and chloral hydrate-midazolam were effective in pediatric MRI sedation; however, chloral hydrate-hydroxyzine was safer. [ABSTRACT FROM AUTHOR]

Objective Infantile spasms (IS) are among the most catastrophic epileptic syndromes of infancy. The purpose of this study was to compare efficacy and safety of topiramate (TPM) and nitrazepam (NZP) as first-line drugs in the treatment of IS. Materials & Methods In a parallel single-blinded randomized clinical trial, 50 patients with IS referred to Pediatric Neurology Clinic of Shahid Sadoughi University of Medical Sciences, Yazd, Iran, were evaluated from September 2008 to March 2010. Patients were randomly assigned to two groups to be treated with TPM or with NZP for 6 months. The primary endpoint was efficacy in cessation of all spasms or reduction of more than 50% in weekly seizure frequency, which was evaluated before and 6 months after the drug use. Secondary outcome was clinical sideeffects of the drugs. Results Twenty boys (40%) and 30 girls (60%) with the mean age of 9.4±3.8 months were evaluated. Cessation of all spasms occurred in 12 (48%) infants in TPM group and 4(16%) in NZP group. Eight (32%) children in TPM group and 7 (28%) in NZP group had more than 50% reduction in spasms frequency. So, TPM was more effective. Side effects were seen in 32% of TPM and in 36% of NZP groups. Conclusion Topiramate is an effective and safe drug, which might be considered as the firstline drug for the treatment of ISs. [ABSTRACT FROM AUTHOR]

Objective: Hypothyroidism may be an exacerbating factor for primary headaches and migraine is one of the most common primary headaches in childhood. The purpose of this study was to evaluate the effect of treatment of subclinical hypothyroidism on children with migraine headache. Materials & Methods: In a quasi-experimental study, the severity and monthly frequency of headache of 25 migraineur children with subclinical hypothyroidism who were referred to the pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences,Yazd, Iran between January 2010 and February 2011 and were treated with levothyroxine for two months were evaluated. Results: Thirteen girls (52%) and 12 boys (48%) with the mean age of 10.2 ± 2.76 years were evaluated. In children with hypothyroidism, the monthly frequency of headache (mean ± SD: 17.64 ± 9.49 times vs. 1.2 ± 1.1 times) and the severity of headache (mean± SD: 6.24±1.8 scores vs. 1.33 ± 0.87 scores) were significantly decreased by treatment. Conclusion: Based on the results of this study, treatment of subclinical hypothyroidism was effective in reducing migraine headaches. Therefore, it is logical to check thyroid function tests in migraineur children. [ABSTRACT FROM AUTHOR]

Background: Migraine is the most frequent acute recurrent primary headache in childhood. Hypothyroidism may be an exacerbating factor for some primary headaches. The purpose of this study was to determine the frequency of subclinical hypothyroidism in children with migraine headache. Methods: In a cross-sectional analytic study, the thyroid function tests of 5- to 15-year-old migraineurs who were referred to the Pediatric Neurology Clinic of Shahid Sadoughi University of Medical Sciences from January 2010 to February 2011 in Yazd, Iran, were measured based on the second edition of the International Classification of Headache Disorders. Results: Forty-eight girls (46.2%) and 56 boys (53.8%) with mean age of 10.46±2.72 years were evaluated. Twenty-five (24%) children had hypothyroidism. The monthly frequency of headache (mean±SD, 14.75±8.9 vs. 20.12±9.49, p=0.04) and the duration of headache (mean±SD, 1.96±1.08 vs. 3.75±2.71 h, p=0.03) were more statistically significant in migraineur children with hypothyroidism, but the mean age, mean of onset age of migraine, sex distribution, and severity of headache were not statistically different in both groups. Conclusion: Based on the results of this study, subclinical hypothyroidism was as an exacerbating factor for migraine headache. Therefore, it is logical to check the thyroid function tests in migraineur children. [ABSTRACT FROM AUTHOR]

Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory autonomic neuropathies type IV (HSAN type IV) is an extremely rare autosomal recessive disorder initially described by Swanson in 1963. We report a 2.5-year-old boy with clinical features of CIPA as the first case in Iran. The symptoms included recurrent episodes of hyperthermia and unexplained fever that began in early infancy, anhidrosis (inability to sweat), profound lossof pain sensitivity, neurodevelopmental delay, unconscious self-mutilation of fingers, lips and tongue, corneal lacerations, palmar hyperkeratosis, non-painful fracture and joint deformities in the right ankle. Tearing, deep tendon reflexes and motor and sensory nerve action potentials were normal. Prenatal screening is the sole accessible option to prevent the birth of an affected child as no cure is available. Early recognition of CIPA patients and its orthopedic complications, prevention of accidental injuries, regular visual and eye follow-up and specific dental management could be useful in the reduction of frequency and severity of complications. [ABSTRACT FROM AUTHOR]

Objective The advent of computed tomography (CT) scan revolutionized the diagnostic evaluation of neurologic patients. The aim of this study was to evaluate brain CT results of epileptic children.Materials & Methods In a descriptive cross-sectional study, noncontrast brain CT scan of 150 consecutive 1-18 year old epileptic children whom were referred to pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences, from May 2008 to October 2010 in Yazd-Iran, evaluated. Results Sixty two girls and 88 boys with mean age of 6.6 ± 4.3 years were evaluated.In 38 (25.3 %) children, seizure onset age was under one year and 38 others had abnormal mental / developmental status. Fifty three children (35.3 %) and 97 (64.7%) had partial and generalized seizures, respectively. Partial seizures were more prevalent in children with seizure onset in < 1 year [41.5% (22/53) vs. 16.5% (16/97)] Result of CT was normal in 74 % (n=111). Among the patients with abnormal results, 18(46%) had brain atrophy, 10 (25.6%) structural CNS dysgenesia, six (15.4%) intracranial calcification, three (7.8%) hydrocephaly and two had (5.2%) brain tumor. Abnormal brain CT was more prevalent in patients with seizure onset in less than one year of age [60.5% (23 of 38) vs. 14.3% (16 of 112), p = 0.003], partial epilepsy [51% (27 of 53) vs. 12% (12/97)], and abnormal developmental status [ 81.5% (31 of 38) vs.7% (8 of 112]. Mean age of seizure onset in epileptic children with abnormal brain CT scan was less (M ± SD:1/17 ± 0.6 years versus 4.02±1.9 years). Conclusion Brain CT scan might be considered in evaluation of epileptic children with partial seizures, seizure onset in less than one year of age or neurodevelopmental delay. [ABSTRACT FROM AUTHOR]

Objective: Hypothyroidism may be an exacerbating factor for primary headaches and migraine is one of the most common primary headaches in childhood. The purpose of this study was to evaluate the effect of treatment of subclinical hypothyroidism on children with migraine headache. Materials & Methods: In a quasi-experimental study, the severity and monthly frequency of headache of 25 migraineur children with subclinical hypothyroidism who were referred to the pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences,Yazd, Iran between January 2010 and February 2011 and were treated with levothyroxine for two months were evaluated. Results: Thirteen girls (52%) and 12 boys (48%) with the mean age of 10.2 ± 2.76 years were evaluated. In children with hypothyroidism, the monthly frequency of headache (mean ± SD: 17.64 ± 9.49 times vs. 1.2 ± 1.1 times) and the severity of headache (mean± SD: 6.24±1.8 scores vs. 1.33 ± 0.87 scores) were significantly decreased by treatment. Conclusion: Based on the results of this study, treatment of subclinical hypothyroidism was effective in reducing migraine headaches. Therefore, it is logical to check thyroid function tests in migraineur children. [ABSTRACT FROM AUTHOR]

Objective Status epilepticus (SE) is the most common pediatric neurologic emergency with high mortality and morbidity. There is no consensus on the drug of choice in the treatment of children. The purpose of this study was to evaluate the clinical efficacy and safety of intravenous sodium valproate as a third-line drug in the treatment of generalized convulsive SE of children. Materials & Methods In a retrospective study, medical records of those children who were admitted to Shahid Sadoughi Hospital of Yazd due to refractory generalized convulsive SE and were treated by intravenous sodium valproate as a third-line drug from 2009 to 2011 were evaluated. Results Six girls and five boys with a mean age of 5.12 ± 1.2 years (range: 3 - 9.6 years) were evaluated. Intravenous valproate was effective for cessation of seizures in seven patients (63.6%). The mean dose of valproate for stopping seizures was 27.1 ± 1.4 mg/kg/day. Children whose seizures were controlled by sodium valproate were older than non - responsive children (mean ± SD: 4.8 ± 1.2 years vs. 3.1 ± 0.43 years, p = 0.03) and they also had shorter ICU stay days (mean ± SD: 2.6 ± 1.4 days vs. 5.6 ± 2.8 days, p= 0.01). Two children had mild and transient nausea and vomiting. None of them had cardiopulmonary or severe paraclinical side effects. Conclusion Intravenous sodium valproate may be used as an effective and safe third-line antiepileptic drug in the treatment of pediatric generalized convulsive status epilepticus. [ABSTRACT FROM AUTHOR]

Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory autonomic neuropathies type IV (HSAN type IV) is an extremely rare autosomal recessive disorder initially described by Swanson in 1963. We report a 2.5-year-old boy with clinical features of CIPA as the first case in Iran. The symptoms included recurrent episodes of hyperthermia and unexplained fever that began in early infancy, anhidrosis (inability to sweat), profound lossof pain sensitivity, neurodevelopmental delay, unconscious self-mutilation of fingers, lips and tongue, corneal lacerations, palmar hyperkeratosis, non-painful fracture and joint deformities in the right ankle. Tearing, deep tendon reflexes and motor and sensory nerve action potentials were normal. Prenatal screening is the sole accessible option to prevent the birth of an affected child as no cure is available. Early recognition of CIPA patients and its orthopedic complications, prevention of accidental injuries, regular visual and eye follow-up and specific dental management could be useful in the reduction of frequency and severity of complications. [ABSTRACT FROM AUTHOR]

Objective The advent of computed tomography (CT) scan revolutionized the diagnostic evaluation of neurologic patients. The aim of this study was to evaluate brain CT results of epileptic children.Materials & Methods In a descriptive cross-sectional study, noncontrast brain CT scan of 150 consecutive 1-18 year old epileptic children whom were referred to pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences, from May 2008 to October 2010 in Yazd-Iran, evaluated. Results Sixty two girls and 88 boys with mean age of 6.6 ± 4.3 years were evaluated.In 38 (25.3 %) children, seizure onset age was under one year and 38 others had abnormal mental / developmental status. Fifty three children (35.3 %) and 97 (64.7%) had partial and generalized seizures, respectively. Partial seizures were more prevalent in children with seizure onset in < 1 year [41.5% (22/53) vs. 16.5% (16/97)] Result of CT was normal in 74 % (n=111). Among the patients with abnormal results, 18(46%) had brain atrophy, 10 (25.6%) structural CNS dysgenesia, six (15.4%) intracranial calcification, three (7.8%) hydrocephaly and two had (5.2%) brain tumor. Abnormal brain CT was more prevalent in patients with seizure onset in less than one year of age [60.5% (23 of 38) vs. 14.3% (16 of 112), p = 0.003], partial epilepsy [51% (27 of 53) vs. 12% (12/97)], and abnormal developmental status [ 81.5% (31 of 38) vs.7% (8 of 112]. Mean age of seizure onset in epileptic children with abnormal brain CT scan was less (M ± SD:1/17 ± 0.6 years versus 4.02±1.9 years). Conclusion Brain CT scan might be considered in evaluation of epileptic children with partial seizures, seizure onset in less than one year of age or neurodevelopmental delay. [ABSTRACT FROM AUTHOR]

Objective Status epilepticus (SE) is the most common pediatric neurologic emergency with high mortality and morbidity. There is no consensus on the drug of choice in the treatment of children. The purpose of this study was to evaluate the clinical efficacy and safety of intravenous sodium valproate as a third-line drug in the treatment of generalized convulsive SE of children. Materials & Methods In a retrospective study, medical records of those children who were admitted to Shahid Sadoughi Hospital of Yazd due to refractory generalized convulsive SE and were treated by intravenous sodium valproate as a third-line drug from 2009 to 2011 were evaluated. Results Six girls and five boys with a mean age of 5.12 ± 1.2 years (range: 3 - 9.6 years) were evaluated. Intravenous valproate was effective for cessation of seizures in seven patients (63.6%). The mean dose of valproate for stopping seizures was 27.1 ± 1.4 mg/kg/day. Children whose seizures were controlled by sodium valproate were older than non - responsive children (mean ± SD: 4.8 ± 1.2 years vs. 3.1 ± 0.43 years, p = 0.03) and they also had shorter ICU stay days (mean ± SD: 2.6 ± 1.4 days vs. 5.6 ± 2.8 days, p= 0.01). Two children had mild and transient nausea and vomiting. None of them had cardiopulmonary or severe paraclinical side effects. Conclusion Intravenous sodium valproate may be used as an effective and safe third-line antiepileptic drug in the treatment of pediatric generalized convulsive status epilepticus. [ABSTRACT FROM AUTHOR]

Camptomelic Dysplasia (CMD) is a rare autosomal dominant congenital dwarfism characterized by shortness and bowing of long bones (camptomelia) and other severe skeletal and extra skeletal malformations. CMD is generally considered to be lethal and the majority of cases die in the neonatal period due to respiratory insufficiency. We hereunder report a term male neonate with characteristic clinical and radiological findings of CMD, hydrocephaly, no sex reversal, and a negative family history of skeletal problems who was born to non-consanguineous healthy parents and was admitted to Shahid Sadoughi Hospital, Yazd, Iran, immediately after birth due to respiratory distress. The patient required continuous mechanical ventilation support and all attempts to reduce respiratory support failed and the patient died on the 21th day of his life. Camptomelic Dysplasia is a terrible experience for parents; thus, prenatal diagnosis of CMD by ultrasound is essential and mandatory for a better therapeutic intervention. [ABSTRACT FROM AUTHOR]

Objective Infantile Spasms (IS) is one of the catastrophic epileptic syndromes of infancy. The purpose of this study was to evaluate clinical efficacy and safety of topiramate (TPM) as the first -line drug in the treatment of infantile spasms. Materials and Methods In a quasi- experimental study, efficacy and safety of TPM in treatment of forty children with IS who were referred to pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences in Yazd, Iran, from September 2008 to 2010 was evaluated. Results Twenty two girls (55%) and 18 boys (45%) with a mean age of 9.2 ± 3.9 months (range= 3-20 months) were evaluated. Ninety percent of the patients had symptomatic IS. At the end of three months of TPM treatment, 40% of the patients became seizure free, 27.5% had more than 50% reduction in seizure frequency, 27.5% had no notable change in seizure frequency and 5% had an increased frequency of seizures. Transient and mild side effects, which were seen in 32.5% of the patients, included drowsiness in 15%, hypotonia and hyperthermia (each one) in 7.5% and anorexia and weight loss in 2.5%. All side effects disappeared in two or three weeks of treatment. Conclusion Topiramate is an effective and safe drug in the treatment of IS and could be considered as the first line of treatment. [ABSTRACT FROM AUTHOR]

Camptomelic Dysplasia (CMD) is a rare autosomal dominant congenital dwarfism characterized by shortness and bowing of long bones (camptomelia) and other severe skeletal and extra skeletal malformations. CMD is generally considered to be lethal and the majority of cases die in the neonatal period due to respiratory insufficiency. We hereunder report a term male neonate with characteristic clinical and radiological findings of CMD, hydrocephaly, no sex reversal, and a negative family history of skeletal problems who was born to non-consanguineous healthy parents and was admitted to Shahid Sadoughi Hospital, Yazd, Iran, immediately after birth due to respiratory distress. The patient required continuous mechanical ventilation support and all attempts to reduce respiratory support failed and the patient died on the 21th day of his life. Camptomelic Dysplasia is a terrible experience for parents; thus, prenatal diagnosis of CMD by ultrasound is essential and mandatory for a better therapeutic intervention. [ABSTRACT FROM AUTHOR]

Objective Infantile Spasms (IS) is one of the catastrophic epileptic syndromes of infancy. The purpose of this study was to evaluate clinical efficacy and safety of topiramate (TPM) as the first -line drug in the treatment of infantile spasms. Materials and Methods In a quasi- experimental study, efficacy and safety of TPM in treatment of forty children with IS who were referred to pediatric neurology clinic of Shahid Sadoughi University of Medical Sciences in Yazd, Iran, from September 2008 to 2010 was evaluated. Results Twenty two girls (55%) and 18 boys (45%) with a mean age of 9.2 ± 3.9 months (range= 3-20 months) were evaluated. Ninety percent of the patients had symptomatic IS. At the end of three months of TPM treatment, 40% of the patients became seizure free, 27.5% had more than 50% reduction in seizure frequency, 27.5% had no notable change in seizure frequency and 5% had an increased frequency of seizures. Transient and mild side effects, which were seen in 32.5% of the patients, included drowsiness in 15%, hypotonia and hyperthermia (each one) in 7.5% and anorexia and weight loss in 2.5%. All side effects disappeared in two or three weeks of treatment. Conclusion Topiramate is an effective and safe drug in the treatment of IS and could be considered as the first line of treatment. [ABSTRACT FROM AUTHOR]

Objective The Lennox-Gastaut syndrome (LGS: the triad of intractable seizures of various types, a slow spike-wave pattern in EEG and mental retardation) is one of the most difficult epilepsy syndromes to treat. The aim of this study was to evaluate the efficacy and safety of zonisamide (ZNS) as add-on therapy in seizures of children with LGS. Materials & Methods In a quasi- experimental study, seizure frequency and side effects of 40 children with LGS who were referred to the pediatric neurology clinic of Shaheed Sadoughi University of Medical Sciences, Yazd, Iran, between September 2008 and November 2009 and were on ZNS for six months were evaluated. Results Twenty one boys and 19 girls with a mean age of 6.6 3.6 years were evaluated. At the end of six months of treatment with ZNS, 25% became seizure free, 25% had > 50% reduction in seizure frequency while 35% did not have a notable change in seizure frequency and 15% experienced an increase in seizure frequency. Drug was effective in 62.5% of the myoclonic and generalized tonic-clonic, 50% of the atonic, 43% of the mixed type and 33.4% of the tonic seizures. Transient side effects were seen in 25% of the patients: drowsiness in 10%, hyperthermia in 5% and irritability, fatigue, ataxia and anorexia (each one) in 2.5% of the patients. No serious side effects were reported. Conclusion ZNS could be considered as an add-on therapy in the management of intractable epilepsy in LGS. [ABSTRACT FROM AUTHOR]

The Lennox-Gastaut syndrome (LGS: the triad of intractable seizures of various types, a slow spike-wave pattern in EEG and mental retardation) is one of the most difficult epilepsy syndromes to treat. The aim of this study was to evaluate the efficacy and safety of zonisamide (ZNS) as add-on therapy in seizures of children with LGS. Materials & Methods In a quasi- experimental study, seizure frequency and side effects of 40 children with LGS who were referred to the pediatric neurology clinic of Shaheed Sadoughi University of Medical Sciences, Yazd, Iran, between September 2008 and November 2009 and were on ZNS for six months were evaluated. Results Twenty one boys and 19 girls with a mean age of 6.6 3.6 years were evaluated. At the end of six months of treatment with ZNS, 25% became seizure free, 25% had > 50% reduction in seizure frequency while 35% did not have a notable change in seizure frequency and 15% experienced an increase in seizure frequency. Drug was effective in 62.5% of the myoclonic and generalized tonic-clonic, 50% of the atonic, 43% of the mixed type and 33.4% of the tonic seizures. Transient side effects were seen in 25% of the patients: drowsiness in 10%, hyperthermia in 5 % and irritability, fatigue, ataxia and anorexia (each one) in 2.5% of the patients. No serious side effects were reported. Conclusion ZNS could be considered as an add-on therapy in the management of intractable epilepsy in LGS. [ABSTRACT FROM AUTHOR]

Objectives Febrile seizure is the most common problem in pediatric neurology that occurs in 3- 4 % of children. The purpose of this study was to determine febrile seizure recurrence frequency and to evaluate its risk factors. Materials & Methods In a descriptive prospective study, 139 children (6 months to 6 years) with first febrile seizure were admitted to Yazd Shaheed Sadoughi Hospital between March 2004 and August 2005 and were followed up for at least 15 months for febrile seizure recurrence. Results Seventy six boys and 63 girls with a mean age of 2.03 ± 1.21 years were followed up for 25.1±5.5 months. About 30% of them had complex febrile seizures and 37.4% had febrile seizure recurrence with a mean recurrence time of 6.7 ± 5.9 months. About 65% of the children younger than one year and 30% of those older than one year had febrile seizure recurrence. (P value= 0.0001) Recurrence of seizure was seen in 63% of those who had seizure within an hour from the onset of fever and in 33% of those who had seizure after one hour from the onset of fever. (P value = 0.005) Seizures in children younger than one year old and seizures occurring in association with a fever lasting less than an hour were risk factors of febrile seizure recurrence. Conclusion Febrile seizure is more disturbing in children younger than one year old. Antipyretic usage was not effective in preventing seizure recurrence but may reduce discomfort and is reassuring. [ABSTRACT FROM AUTHOR]