mahmoudreza ashrafi
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Objectives
Acute Flaccid Paralysis (AFP) in children can stem from a diverse array of potential diagnoses.
Materials & MethodsThis retrospective study sought to diagnose children referred to a referral pediatric emergency unit with AFP between 2011 and 2016. The study gathered clinical observations, conducted stool and cerebrospinal fluid analyses, and assessed electrophysiological and imaging data.
ResultsThe present study enrolled 118 fully immunized children with a mean age of 6.09 ± 3.60 years. The most prevalent diagnoses included Guillain-Barré Syndrome (GBS-80 cases), acute viral myositis (20 cases), Transverse Myelitis Syndrome (TMS) (TMS-6 cases), and Vaccine-Associated Paralytic Poliomyelitis (VAPP) (VAPP-6 cases). All these six patients had primary immunodeficiency. Notably, all patients tested negative for poliovirus in stool analyses. This study encountered a unique case of a 2.5-monthold male patient who presented with acute limb motor weakness, along with fever, irritability, new-onset hypotonia, and generalized decreased deep tendon reflexes. Notably, no signs of upper motor neuron involvement were found. The Cerebrospinal Fluid (CSF) analysis was compatible with the diagnosis of viral meningitis. Moreover, among the 60 brain and spinal imaging series performed, five were indicative of GBS, six cases showed evidence of TMS, and one revealed a spinal mass. Besides, clinical investigations pointed toward acute viral myositis as a secondary etiology of AFP in 20 patients in this study.
ConclusionIn this hospital-based study, the most frequent diagnoses for children arriving at a third-level pediatric Emergency Room (ER) with acute flaccid paralysis AFP were GBS, acute viral myositis, TMS, and VAPP). These findings suggest a distinct pattern of AFP causes compared to those found in community-based epidemiological studies. Additionally, notably, unusual conditions, such as viral meningitis, can rarely present with AFP-like symptoms. Assessment for primary immune deficiency should be considered in cases of VAPP. Lastly, this research has implemented a pediatric AFP Management Protocol: A Local Practical Approach
Keywords: Acute Flaccid Paralysis, Guillain–Barre´ Syndrome (GBS), Acute Transverse Myelitis -
Background
Nowadays, it is generally accepted that focusing on in-service training can lead to organizational progress and improvements in the training process.
ObjectivesConsidering the high prevalence of epilepsy in children and the use of new technologies in education, the purpose of this study was to conduct an electronic educational course on epilepsy classification guidelines based on the guidelines of pediatric neurologists and to evaluate the effectiveness of the course using the Kirkpatrick model.
MethodsThis study was conducted in 2020. The study population comprised pediatric neurologists from universities of medical sciences throughout the country. Inclusion criteria included participation in the course during the semester, availability, and willingness to participate in the study. Participants entered the study according to their personal preference and provided informed consent, and those who were absent from two or more sessions related to these topics were excluded from the study. The study involved participation in the e-learning course on epilepsy classification according to the guidelines (ILAE 2017). The sample size was calculated using Cochran's formula, which resulted in 74 participants. Nineteen of them did not meet the inclusion criteria, and a total of 55 physicians were included in the study. Reaction and learning were the levels considered to evaluate the efficacy of the Kirkpatrick model. To analyze the data, paired t -test and linear regression were used at a significance level of 0.05, using SPSS version 22 statistical software.
ResultsThe results showed that most participants were satisfied with the e-course. The mean learning and behavior level score was 27.51. The findings indicated that the e-learning of epilepsy classification guidelines according to the guidelines (ILAE 2017) improved the performance of pediatric neurologists (P-value ˂ 0.001).
ConclusionsThe results indicate that e-learning guidelines for the classification of epilepsy according to the guidelines (ILAE 2017) can be an effective tool in improving the clinical performance of pediatric neurologists. Applying newer methods such as the Kirkpatrick model would be an effective approach in other fields of health as well. It is recommended to use up-to-date and efficient models, such as the Kirkpatrick model, in the field of health sciences.
Keywords: Effectiveness, Kirkpatrick Model, Guideline, Pediatric Neurologist, E-Learning -
Background
Altered concentrations of cerebrospinal fluid (CSF) and blood amino acids may be related to epilepsy or the severity of the seizure. In the present study, we assessed the concentrations of amino acids in CSF and plasma in neonates with refractory seizures.
MethodsIn this prospective cohort study, 27 neonates aged 1 to 56 days with refractory seizures were included. Blood and CSF samples were collected from each neonate within 24 hours after receiving 2nd antiseizure medications. All plasma and CSF samples were sent to the laboratory to measure amino acid concentrations. The associations between CSF and plasma amino acid levels with different variables were evaluated.
ResultsExcept for leucine (P=0.15) and isoleucine (P=0.07), the levels of all amino acids were significantly higher in plasma than CSF. Significant associations were observed between types of seizure and plasma citrulline (P=0.008) and leucine concentrations (P=0.04). The mean of CSF glutamic acid was also statistically different among neonates with different EEG results (P=0.02).
ConclusionOur findings indicate that several plasma and CSF amino acids could be candidate biomarkers for neonatal refractory seizures. Further studies with larger sample size are to confirm our findings.
Keywords: Amino acid, Cerebrospinal fluid, Plasma, Seizure -
Evaluation of the effectiveness of risperidone in the treatment of breath holding spells in childrenObjectives
Breath holding spells (BHS) are a type of syncope in children that is commonly seen in the first years of life. Although these attacks do not cause serious damage to the child's brain, in severe or
repeated cases, they expose the brain to hypoxia and cause a lot of stress in parents. In these cases, the clinician should consider therapy. The purpose of this study is to investigate the effectiveness of Risperidone in the treatment of BHS in children visiting the neurology clinic of the Children's Medical Center Hospital.Materials & MethodsIn this randomized clinical trial, the statistical population included patients with the history of Breath Holding spells grades 2 to 3 (after ruling out of seizure disorders) over one year old, visiting the neurology clinic of Tehran Children's Medical Center in 2019. The subjects were randomly selected from patients visiting the clinic using a table of random numbers from the admit ion list. After providing the necessary explanations and obtaining informed consent from their parents, they were treated with low-dose Risperidone (maximum 1 mg) for three months. The patients were observed for three months in terms of frequency and severity of spells monthly.
ResultsIn this study, the median (25th and 75th percentile) age of subjects was 2.3 (2.0-2.6) years. Both the number and severity of seizures in the subjects had a statistically significant decrease in the period after treatment with Risperidone (P<0.001). The studied boys and girls experienced statistically significant decrease in terms of both the frequency of spells (P-value of 0.002 and 0.039) and intensity of seizures (P=0.016) and P=0.008), respectively after treatment with Risperidone. Also, in the studied children under 2 years old and over 2 years old, both the frequency (P=0.021 and P=0.004) and intensity of spells (P=0.008) 0.016 for subjects under and over 2 years, respectively) had a statistically significant decrease after treatment with risperidone compared to pretreatment.
ConclusionAccording to the results of this study, it seems that both the frequency and the intensity of spells in children (regardless of gender and age group), experienced a statistically significant decrease after treatment with Risperidone. We suggest conducting a more comprehensive study considering a larger sample size in order to estimate this issue more correctly.
Keywords: Breath holding spells, grade of BHS, Risperidone, gender, children -
Objectives
Seizure is a common sign in neonates hospitalized in the neonatal intensive care units (NICU) that may lead to morbidity and mortality. Most neonatal seizures are subclinical. Conventional EEG (cEEG) is the gold standard for detecting and monitoring seizures but is not widely available. Amplitude-integrated electroencephalography (aEEG) has been used for over a decade to evaluate infants with seizures. In this study, we tried to determine the efficacy of aEEG as
a widely available diagnostic tool in diagnosing seizures.Materials & MethodsAll cases with seizures or suspicious seizures were admitted to the NICU of the Children’s Medical Center for one year. cEEG and aEEG were performed for these infants. aEEG was recorded for at least six hours with a description of the tracing. Clinical information, outcomes, and questionnaires (patient information) were recorded in detail. The obtained data were analyzed with the SPSS version 24 software.
ResultsEleven out of twenty-five aEEG recordings were abnormal; other patients showed normal aEEGs. The most common clinical and neurological manifestations were seizure (68%) and hypotonia (28%); the mortality rate was 12%. No significant correlation was observed between aEEG findings and gender, age, familial relation, outcome, ultrasound result, type of seizure, and underlying disease.
Keywords: aEEG, seizures, neonates -
Background
Limb-girdle muscular dystrophy (LGMD) is a bothersome muscle disease associated with weakness of the shoulder and pelvic girdle.
ObjectivesThe study aimed to determine the genetic diversity and relative frequency of various forms of LGMDin Iranian children.
MethodsIn this descriptive research, 60 children referred to the neurology or emergency department of the Pediatric Medical Center were studied from April 2019 to April 2020. Additional tests (muscle biopsy and genetic testing) were performed to confirm the diagnosis of LGMDs. Quantitative data such as disease level, motor, respiratory, and cardiac functions, and molecular data underwent statistical analysis.
ResultsA total of 41 patients with a mean age of 11.1 were studied. Twenty-two patients were diagnosed with genetic tests and 19 with muscle biopsies. Also, there were 26.8% cases of alpha sarcoglycanopathy, 24.4% beta sarcoglycanopathy, 17.1% gamma sarcoglycanopathy, 7.3% calpainopathy, 7.3% dysferlinopathy, 7.3% dystroglycanopathy, 7.3% titinopathy, and one case of laminopathy. Among genetically confirmed individuals, 27.3% had SGCB mutation, and 18.2% had SGCA mutation. A significant relationship was seen between the mutation type and creatine phosphokinase (CPK) levels (P < 0.05).
ConclusionsThe prevalence of alpha and beta sarcoglycanopathy phenotypes in the study population showed that the severity of clinical involvement may be predicted by SGCB gene mutation and sarcoglycan expression.
Keywords: Clinical Characteristics, Limb-Girdle Muscular Dystrophy, LGMD, Sarcoglycanopathy -
Background
COVID-19 infection and its neurological manifestations were seen in children although less common than adults. The aim of this study was to determine the frequency of different types of neurologic findings of hospitalized children with COVID-19. ]
MethodsThis retrospective study was performed on hospitalized pediatric patients aged≤18 years with confirmed SARS-CoV-2 at Children’s Medical Center Hospital. Neurological manifestations were defined as the presence of any of the following symptoms: seizure, altered mental status, behavioral/personality change, ataxia, stroke, muscle weakness, smell and taste dysfunctions, and focal neurological disorders.
ResultsFifty-four children with COVID-19 were admitted and their mean age was 6.94±4.06 years. Thirty-four of them (63%) were male. The most frequent neurological manifestation was seizure (19 [45%]) followed by muscle weakness (11 [26%]), loss of consciousness (10 [23%]), and focal neurological disorders (10 [23%]). Other neurological manifestations consisted of headache (n=7), movement disorders (n=6), behavioral/personality change (n=5), ataxia (n=3), and stroke (n=3). Twenty-nine percent of our patients had leukocytosis. A neutrophil count above 70% was seen in 31% of participants. Among our patients, 81% had a positive reverse-transcription polymerase chain reaction (RT-PCR) test for SARS-CoV-2.
ConclusionDuring the current pandemic outbreak, hospitalized children with COVID-19 should be evaluated for neurological signs because it is common among them and should not be under-estimated.
Keywords: Children, COVID-19, Neurological manifestations, SARS-CoV-2 -
Background
During the COVID-19 pandemic, educational programs have increasingly relied on virtual methods.
ObjectivesIn this study, we compared the effects of in-person and virtual electroencephalography (EEG) training on the knowledge of pediatric and adult neurology residents.
MethodsThe study participants consisted of 30 pediatric and adult neurology residents who were recruited nationwide via a virtual network. They were randomly divided into two groups for in-person and virtual education, respectively. Prior to the first workshop session, participants completed a pre-test comprising 19 knowledge-related questions. Two interactive training sessions focusing on benign variants in EEG were conducted, including two in-person workshops for eight residents and two online workshops for 22 residents. Each session lasted one hour, with one held every two weeks. After the second session, participants were asked to complete a post-test consisting of 24 questions (identical to the pre-test) and five workshop satisfaction-related questions.
ResultsOverall, the mean exam score after both the in-person and virtual workshops was significantly higher than the pretest score across all educational levels. The mean score differences in knowledge between the in-person and virtual groups after the workshop were similar, with no statistically significant difference. Approximately 90% of the participants believed that the workshop would be beneficial for their daily practice.
ConclusionsGiven the absence of a significant difference between in-person and virtual training methods in improving participants' knowledge and satisfaction with both approaches, we recommend integrating virtual EEG training into the educational protocol for pediatric and adult neurology residents. Nonetheless, larger sample size studies are required to further validate these findings.
Keywords: Electroencephalography, Virtual Education, COVID-19 -
Objectives
The SARS-CoV-2 pandemic is the most challenging crisis in the contemporary world. Besides severe pulmonary involvement, the disease also has several extrapulmonary manifestations, and new signs and symptoms are associated with it every dayThe present study aimed to inquire about the frequency of neurological manifestations and risk factors of COVID-19.
Materials & MethodsThis retrospective, descriptive study included patients with neurological involvement admitted to the Alborz University of Medical Sciences academic hospitals from March 2020 to July 2020 with confirmed COVID-19 infection. The data included in the analysis were the patient’s demographic information, underlying diseases, neurological manifestations, and laboratory findings.
ResultsThe study included ninety-five patients with a mean age of fiftynine. Neurological symptoms and signs were observed in 91.6% and 10.5% of the patients, respectively. The most frequently associated neurological symptoms of COVID-19 were fatigue (49.5%), headache (47.4%), and dizziness (45.3%). Furthermore, the most common neurological involvements included gait disorders (6.3%), cerebellar dysfunction (4.2%), and cerebrovascular accidents (3.15%). Positive troponin was shown to be the strongest predictor of neurological signs (OR=21, P=0.017), followed by WBC≥15,000 (OR = 20.75, P=0.018) and a history of respiratory disease (OR=7.42, P=0.007).
Keywords: COVID-19, Neurological Manifestations, SARS-CoV-2 -
زمینه و هدف
بخش عمدهی زندگی هر فرد، صرف اشتغال به کار میشود. فرسودگی شغلی یک نشانهی روان شناختی، پاسخ ممتد به فشارهای مزمن فردی و عاطفی موجود در محیط کار است. رزیدنتها به علل متعدد در معرض فرسودگی شغلی قرار دارند که سبب کاهش در کیفیت ارایه خدمات میشود.
روش بررسیاین مطالعه توصیفی- تحلیلی مقطعی، بر روی 57 نفر از رزیدنتهای کودکان در دانشگاه تهران انجام شد. پرسشنامهی فارسی شدهی ماسالاچ جهت تعیین میزان و شدت فرسودگی شغلی مورد استفاده قرار گرفت. جهت تجزیه و تحلیل دادهها ابتدا با استفاده از روشهای مبتنی بر آمار توصیفی شامل میانگین ± انحراف معیار مقادیر نمرات فرسودگی شغلی استفاده شد.
یافته هامیانگین شدت فرسودگی شغلی رزیدنتهای کودکان 55.45 هم چنین میانگین فراوانی فرسودگی شغلی رزیدنتهای کودکان 62.1 میباشد. در این مطالعه میان شدت و فراوانی فرسودگی شغلی با سن، وضعیت تاهل، سابقهی کاری ارتباط معنیداری یافت نشد. ولی بین شدت فرسودگی عاطفی با تعداد روز کار در هفته ارتباط معنیداری یافت شد. بین فراوانی فرسودگی عاطفی با محل سکونت خانواده ارتباط معنیدار یافت شد. بین شدت تهی شدن از ویژگیهای شخصیتی و داشتن فرزند نیز ارتباط معنیدار یافت شد.
نتیجه گیریبا توجه به نتایج این مطالعه، فرسودگی شغلی در رزیدنتهای کودکان در دانشگاه تهران در عدم موفقیت فردی از لحاظ فراوانی شدید و از لحاظ شدت میزان متوسط را نشان داده، در حالی که این متغیر در ابعاد فرسودگی عاطفی شدت کم و فراوانی متوسط و مسخ شخصیت شدت کم و فراوانی کم را گزارش کرده است. پیشنهاد میشود که تعدیل روزهای کاری هفتگی رزیدنتها با افزایش جذب رزیدنت ها و انتخاب دانشگاه محل تحصیل رزیدنت نزدیک خانواده خود در اولویت انتخاب رشته-محل قرار گیرد.
کلید واژگان: رزیدنت کودکان, فرسودگی شغلی, رضایت فردیBackground and ObjectiveMost of a person's life is spent in employment. Today, we see a trend and special attention to the issue of job burnout all over the world. Burnout is a psychological symptom, an ongoing response to chronic personal and emotional stress in the workplace. Pediatric resident physicians are exposed to job burnout for various reasons, which reduces the quality of service delivery. Therefore, due to the importance of paying attention to the issue of job burnout and also the lack of a studies in this field in the community of pediatric resident physicians of Tehran University of Medical Sciences, this study was conducted with the aim of investigating the job burnout of pediatric resident physicians of Tehran University of Medical Sciences based on Maslach questionnaire in 2020-2021.
Materials and MethodsThis descriptive-analytical cross-sectional study was conducted on 57 active in Pediatric resident physicians of Tehran University of Medical Sciences. To determine the level and intensity of job burnout, the Persianized Maslach questionnaire was used. To analyze the data, first descriptive statistics were used, including the mean standard deviation of burnout scores and their severity, and for comparison, a two-sample t-test or Mann-Whitney test was used. SPSS 26 software was used for data analysis.
ResultsThe average intensity of job burnout in pediatric resident physicians of Tehran University of Medical Sciences is 55.45 and also the mean frequency of job burnout in pediatric resident physicians of Tehran University of Medical Sciences is 62.1. In this study, there was no significant difference between the severity and frequency of job burnout with age, marital status, and work experience. However, a significant relationship was observed between the intensity of emotional burnout and the number of working days per week. A significant relationship was observed between the frequency of emotional burnout and living in a family. A significant relationship was observed between the severity of personality disorder and having children.
ConclusionBased on the results of this study, job burnout among pediatric resident physicians of Tehran University of Medical Sciences showed the level of severe personal failure in terms of frequency and in average level, while this variable in the dimensions of emotional exhaustion has low intensity and moderate frequency, and depersonalization has low intensity and frequency.
Keywords: Pediatric resident, Burnout professional, personal satisfaction -
Hereditary sensory and autonomic neuropathies (HSAN) are rare genetic disorders that often manifest during childhood in the form of absence of pain sensation or self-mutilation. Patients often present significant oral self-mutilation manifestations, and biting of the lips, tongue, and cheeks have been frequently reported. This case report describes a case of hereditary sensory and autonomic neuropathy with oral and cutaneous ulcers. Our patient was a 14-month-old girl with the chief complaint of a tongue ulcer, as stated by her parents, who were referred to our private dental clinic. Clinical examination revealed severe ulcers due to biting (Riga-Fede disease) on the ventral surface of the tongue and superficial ulcers on the dorsal surface of the tongue caused by the anterior maxillary teeth, along with some sores on fingers. The parents were healthy, with no congenital disease or familial history of a similar condition. The electrodiagnostic test revealed the absence of sensory nerve action potential response. However, the electromyographic findings and the compound muscle action potential of the tibial and ulnar nerves were normal. Oral ulcers such as trauma to the lips and tongue, and self-mutilation trauma to the fingers can be used for early detection of Hereditary sensory and autonomic neuropathies. A multidisciplinary approach involving a professional dental team and a regular treatment protocol are imperative to prevent complications of Hereditary sensory and autonomic neuropathies.
Keywords: Hereditary sensory, autonomic neuropathy, Oral ulcer, Self-mutilation -
Objective
Guillain-Barré syndrome (GBS) is an immune-mediated disease of the peripheral nervous system which affects all age groups through the world. Although the pathogenesis and optimal treatment of GBS are still not completely established, one of the most common infectious diseases to trigger the syndrome is cytomegalovirus (CMV) infection. GBS following CMV infections is rarely reported in childhood and there is no data on GBS with antecedent CMV infection in children in Iran. The aim of this study was to evaluate the association of CMV infection and GBS in children in Iran.
Materials & MethodsCase-control study design was used in 30 cases of Guillain-Barre syndrome, and 30 matched controls. All serum samples were tested for the presence of anti-CMV IgM and IgG antibodies using a commercially available ELISA (EUROIMMUN Medizinische, Germany).CMV viral DNA in the specimen was detected using polymerase chain reaction (PCR), (Cytomegalovirus PCR Detection Kit, CinnaGen company, Iran).
ResultsAnti–CMV immunoglobulin (Ig) G antibodies were detected in GBS patients and healthy controls in 97% and 93% of children, respectively. Anti-CMV IgM antibodies were demonstrated in 33% (n=10) of the healthy controls and 33% (n=10) of the children with GBS. The borderline level of anti-CMV IgM antibodies was found in 23% healthy controls (n=7) and 13% (n=4) in the children with GBS (p=0.57), respectively. None of the specimens from the both controls the cases with GBS was positive for CMV DNA by PCR.
ConclusionThese data demonstrated the presence of anti-CMV antibodies in a majority of both patients with GBS and controls and no relation between CMV infection and GBS was observed. However, further studies with larges sample sizes are highly recommended.
Keywords: Guillain-Barré syndrome, Cytomegalovirus, Children -
Introduction
Emotion can contribute to the severity of stuttering, although the underlying mechanism is unknown. Event-related Potential (ERP) could be very helpful for assessing emotional processing in persons with stuttering. Our study aimed at the investigation of phonological processing for emotional and neutral words in Children Who Stutter (CWS) by ERP.
Materials and MethodsTen CWS were given 120 emotional and neutral words to read. Phonological processing was assessed by aloud reading task, while simultaneously ERP was recorded. The results were analyzed as behavioral (reaction time and accuracy) and electrophysiological (amplitude and topography).
ResultsThere were significant differences in reaction time and accuracy between positive, negative, and neutral words (P<0.05). The electrophysiological data analysis showed significant differences for a minimum of amplitude in the left frontal area, for a maximum of amplitude in the right temporal area, and peak to peak distance in the left frontal area (P<0.05). Visual inspection suggested that recorded fluctuations have a bigger amplitude range for neutral words in all brain regions, except prefrontal, frontal and right frontal.
ConclusionValence would affect behavioral measures. Generally, emotion facilitates word processing by reducing activity in anterior brain areas in phonological processing time.
Keywords: Phonological processing, Emotional content, Developmental stuttering, Persian, ERP -
Background
Despite the great progress in the surgery of complex congenital heart diseases, there is still concern regarding adverse neurological events. We aimed to determine the pre- and on-pump modifiable factors that could predict the neurocomplications after pediatric cardiac surgery.
MethodsIn a prospective study, modifiable factors such as arterial blood gas, serum lactate, serum glucose, mean arterial pressure, and nasopharyngeal temperature were measured before and during cardiopulmonary bypass (CPB). Moreover, the CPB time, the aortic cross-clamp time, and the deep hypothermic circulatory arrest time were recorded. If there were adverse neurological complications, brain computed tomography scan was done.
Results435 patients with congenital heart diseases that underwent cardiac surgery, 364 patients at a mean age of 22 months were enrolled in the study. Thirty-three (9%) patients had adverse early-onset neurological events. Seizure and hemorrhage were the most common clinical and neuroimaging findings, respectively. Although the pre-pump oxygen saturation (P = 0.03), the blood CO2 level (P = 0.04), and the serum glucose level (P = 0.03) showed statistical significance in the univariate analysis, the only predictive variables of neurocomplications in the multivariate analysis of logistic regression were the on-pump serum glucose level (P = 0.001) and the nasopharyngeal temperature (P = 0.004).
ConclusionsAmong several modifiable factors exerting an influence on the neurological outcome of children undergoing cardiac surgery, special attention should be paid to the control of the intraoperative serum glucose level and the provision of the optimal cooling temperature. (Iranian Heart Journal 2020; 21(2): 48-56)
Keywords: Neurocomplications, Cardiac surgery, Cardiopulmonary bypass, Deep hypothermic circulatory arrest -
ObjectiveOne of the difficulties for conduct electroencephalography (EEG) in pediatric patient population is that they are not always cooperative during the procedure. Different medications have been used to induce sedation during EEG recording. In order to find a medication with least adverse effects and high efficacy, we aimed to compare clonidine and chloral hydrate as a premedication prior EEG performing in pediatric population. Materials & MethodsA prospective, randomized, single-blinded, controlled trial was carried out over 198 children (9 to 156 months) to investigate the sedative and adverse effects of clonidine and chloral hydrate. Patients, partially sleep-deprived the night before, were randomly divided in two groups of clonidine (100 patients) and chloral hydrate (98 patients), on an alternative day basis.Results The average sleep onset latency was significantly longer in the clonidine group than chloral hydrate group (Mann-Whitney test, p < 0.0001). Sleep duration ranged between 15-150 minutes and it was not significantly different between two groups (Mann-Whitney test p = 0.2). Drowsiness with chloral hydrate terminated faster than with clonidine. Drowsiness after arousal was seen in 58% and 26.1% of patients in the clonidine and chloral hydrate groups respectively that was significant (Mann-Whitney test, p = 0.058). EEG results were reported normal in 77 subjects in the chloral hydrate group (77%) and in 69 subjects (69%) in the clonidine group (p = 0.161). Generalized epileptiform discharges reported significantly in the clonidine group (Mann-Whitney test , p = 0.006).ConclusionThe results of this study showed that both chloral hydrate 5% (one ml/kg)and clonidine (4 μg/kg)could be administered as a pre medication agent for EEG recording in children , although drowsiness after arousal of clonidine is greater than chloral hydrate . However, the yield of generalized epileptiform discharges in the clonidine group was more than the chloral hydrate group.
Keywords: Premedication, Chloral hydrate, Clonidine, Electroencephalography (EEG), Sedation, Sleep -
Background
The purpose of treatment of children with autism is to help them to acquire functional skills in routine life and to control behaviors that are disruptive.
ObjectivesIn this study, the safety and efficiency of cerebrolysin was determined by childhood autism rating scale (CARS) on behavioral, verbal and nonverbal developments of children with autism referred to children’s medical hospital clinic of neurology.
MethodsIn a quasi-experimental study, 36 children with autism were enrolled and the effect of cerebrolysin on their behavioral, nonverbal and verbal development was determined by CARS questionnaire fulfilled before and one month after complete intervention.
ResultsAll evaluated items except level and consistency of intellectual response had favorable reduction in scores of CARS questionnaire (P = 0.001). The total score decreased from 40.6 to 36.1 showing 11.1% improvement. Despite the efficiency of cerebrolysin observed in this study, its partial use may be limited by the large number of intramuscular injections, i.e. nine per months. Sustained release formulation may alleviate this limitation, if this therapy proves to be beneficial in future studies.
ConclusionsAccording to the obtained results, the safety and feasibility of cerebrolysin administration could be considered and be effective on behavioral, nonverbal and verbal development in children with autism.
Keywords: Cerebrolysin, Behavioral, Nonverbal Verbal Development, Autism, Children -
Idiopathic neuralgic amyotrophy (INA) is a disorder presented with acute severe pain in the upper extremity, followed by muscle weakness, paralysis and atrophy. INA is rare in children and few reports are found in the literature. Here, we report a case of INA in an 8-year old boy following pharyngitis.Keywords: Neuralgic Amyotrophy, Child, Electromyography, Nerve conduction study
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Megalencephalic leukoencephalopathy with subcortical cysts (MLC), a demyelination leukodystrophy, is usually characterized by early-onset macrocephaly and gradually progressive motor and mild mental deterioration. Variable clinical expression has been reported in MLC especially in adult cases. In this study, a multi affected family with variable phenotypes including an asymptomatic adult individual is reported to expand the clinical spectrum of MLC. A seven-year old boy was referred to our hospital due to macrocephaly and gait disturbance. According to brain magnetic resonance imaging (MRI) findings, molecular studies were done to confirm the probable diagnosis of MLC in index case followed by family segregation analysis. A homozygous splice site variant, c.177+1G>T in MLC1 gene was found in proband, his mother and two aunts. Aunts were clinically affected but his mother had no clinical symptoms despite bi-allelic mutation in MILC1. The clinical data and available MRI findings were reviewed for these cases. A comprehensive searchwasconductedonclinical variations of MLC. Phenotypic variability and/or reduced penetrance are important phenomena in MLC.We extended phenotypic variation in MLC by reporting an asymptomatic adult case with a known pathogenic mutation in MLC1 gene.Keywords: MLC, Megalencephalic Leukoencephalopathy, Subcortical Cysts, Splice-Site Mutation
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BackgroundChildren with cyanotic Congenital Heart Disease (CHD) are at higher risk for delay in their growth and development due to more energy consumption during their activities. In addition, they are more prone to respiratory infection and hospitalization. Due to the nature of disease, these patients suffer from a chronic hypoxia and its impact on growth and development is not well investigated. This study was designed to find out which physical growth and neurodevelopmental parameters of these patients are affected by chronic hypoxia in comparison with acyanotic disease.Methods81 children with CHD (34 cyanotic and 47 acyanotic), aged between 6 months to 3 years from Children’s Medical Center affiliated to Tehran University of Medical Sciences in Tehran were recruited from January 2013 to January 2014. Growth parameters including weight, height, and head circumference were checked and then these indices were categorized into three groups of Failure To Thrive (FTT). Functional development was assessed by using modified Denver Developmental Screening Test (DDST II).ResultsIn acyanotic group, Ventricular Septal Defect (VSD) and in the cyanotic group, tetralogy of fallot (TOF) were the most prevalent disorders. Growth indices were low in 52% of patients (70% of cyanotic and 38.2% acyanotic), and also weight and height parameters were significantly lower in the cyanotic group (p= 0.009 and p= 0.05). 62% of cyanotic patients and 17% of acyanotic patients had delay at least in one of their neurological development indices (Gross motor, fine motor, speech or psychosocial behavior). This study also demonstrates an association between neurodevelopment delay and FTT in cyanotic patients, but not in acyanotic ones.ConclusionResults in this study suggest that children with cyanotic CHD are more prone to delay in their development besides their growth possibly due to the nature of their disease. Therefore, chronic hypoxia can be a risk factor influencing neurodevelopment of the patients and appropriate intervention is required to gain better outcome.Keywords: Chronic hypoxia, Congenital heart disease, Cyanosis, Neurodevelopmental delay
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WITHDRAWN: Megalencephalic Leukoencephalopathy with Subcortical Cysts: Presentation of an Asymptomatic Patient and Review of Literature
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BackgroundSodium valproate is one of the widely used medications in the treatment of children with epilepsy. The aim of this study was to evaluate the possible role of sodium valproate on enuresis and urinary frequency in epileptic children in comparison to carbamazepine.MethodsIn this prospective cohort study, we enrolled epileptic children aged 5 to 14 years who met the exclusion criteria and were under monotherapy with sodium valproate or carbamazepine. All the cases were assessed for urinary complications during a period of one-year follow-up. Patients with urinary complications were tested for urinary tract infection and also for any urogenital anatomical abnormalities to avoid possible false positive results.ResultsFrom 290 patients who met the inclusion criteria, 254 cases were studied. It was showed that among 127 patients in sodium valproate group, 14 (11.02%) cases had enuresis while in 4 cases it was accompanied by urinary frequency. Among these 14 cases, 10 were treatment naives (new cases) (symptoms appeared after 6.1 ± 1.4 weeks) and the other four only had their medication dosage increased (symptoms appeared after 3.2 ± 0.9 weeks). In three cases, the dosage was reduced and in one case a medication was replaced with another. In all patients, however, the symptoms subsided within 10 - 30 days. On the other hand, no urinary complication was found in the carbamazepine group.ConclusionsThe results demonstrated that in children with epilepsy, enuresis and day-urinary frequency are two side effects of valproate and carbamazepine therapy that should be taken into consideration.Keywords: Valproic Acid, Carbamazepine, Enuresis, Epilepsy
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The effect of emotion on word processing in Persian speaking childrenObjectivesEmotion is a key component in language processing, but it is controversy how emotional content words process in children. The aim of the present study is investigating the effect of arousal and valence on word familiarity, comprehension, use and emotional content recognition. As secondary goal, a list of emotional content words for this age was prepared in Persian.Materials And MethodsEmotional words, from adult emotional words list, were categorized in 5groups according their scores in arousal and valence. Evaluation of familiarity, use, comprehension and emotional content recognition of these words was conducted with a prepared checklist in 60 seven-year-old children by speech and language pathologist.ResultsNeutral words gained the highest score in familiarity, use, comprehension and emotional content recognition (the mean=0.74). Afterward, there were the emotional words with high valence, calm (the mean=0.64) and happy (the mean=0.52). Finally it was found the low score for valence emotional words, sad (the mean=0.46) anxious (the mean =0.43) in end of score rating. Interestingly, there was a significant difference between all word groups in four aspects (p-value0.001).ConclusionIt was shown that neutral words are better comprehended and expressed than emotional words. More importantly, valence is more effective than arousal in emotional words. It is noteworthy gender was not a determinant factor in all of the aspects. An emotional word list which is comprehensible for children in Persian language was prepared.Keywords: emotion, use, comprehension, familiarity, Persian language
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The primary aim of epilepsy treatment is seizure control, and the treatment is principally prophylactic. Although complete seizure control is the most important predictor of improved quality of life, antiepileptic drugs (AEDs) could cause severe side effects in the patients. Therefore, the risk-benefit ratio must be considered before the initiation of AED treatment. Accurate recognition and differentiation of epileptic and non-epileptic paroxysmal events and the diagnosis of the seizure type and epilepsy syndrome are essential procedures before AED treatment. It is often recommended that AED treatment start after two seizures, and being seizure-free for a minimum of two years is a prerequisite for treatment withdrawal. The AED treatment process must be initiated with a single drug at a low maintenance dose, along with further upward titration. Overall, the first attempt in AED treatment has been reported to effectively control seizures in 50-70% of the cases. Moreover, there is a consensus that being seizure-free for two years is the most valid approach to discontinue AED treatment. Approximately 50% of the children with epilepsy outgrow their disease. The present study aimed to provide a systematic method for the treatment and management of epilepsy in children.Keywords: Antiepileptic Drugs, Epilepsy, Quality of life
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BackgroundBrain is highly vulnerable to free radical damage due to a large quantity of mitochondria, a considerable amount of oxidizable polyunsaturated fatty acids, a ratio of high oxygen consumption, and less antioxidant capacity. The experimental and clinical data suggest a putative role of oxidative stress in the pathophysiology of seizures and epileptic syndromes.ObjectivesA case-control study was carried out to compare serum total antioxidant capacity in the newly diagnosed children with epilepsy and that of a control group of healthy children at the same age.
Patients andMethodsA total of 130 participants (65 in each group) aged between 1 and 17 years participated in this study. Serum total antioxidant capacity was compared between two groups before drug administration. The effect of antiepileptic therapy on the serum total antioxidant capacity also was studied in children with epilepsy before and 3 months after antiepileptic drug administration. Serum total antioxidant capacity values were measured based on Erel's method using an automated commercial kit. This method is based on the bleaching of the characteristic color of a more stable 2,2‑azinobis‑(3‑ethylbenzothiazoline‑6‑sulfonic acid) radical cation by antioxidants. The results were expressed in mmol Trolox equivalent/lResultsSerum total antioxidant capacity values was significantly lower in the patients group before drug administration [mean (SD): 1.31 (0.19) mmol/L] than that of the control group [mean (SD): 1.46 (0.21) mmol/L] (PConclusionReduced serum total antioxidant capacity, and an increased vulnerability to oxidative stress should be considered in the children with epilepsy.Keywords: Total antioxidant capacity, Children, Epilepsy, antiseizure medications, Oxidative stress
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