mohammad barzegar

Guillain-Barré syndrome (GBS) involves the peripheral nervous system developed by infections or immune conditions. Cerebrospinal fluid (CSF) analysis and electrodiagnostic tests are essential diagnostic methods for GBS. However, limited data are available on how the findings from these methods relate to each other. This study aimed to evaluate changes in CSF analysis and electrodiagnostic tests in pediatrics with GBS.

The present study retrospectively evaluated electrodiagnostic tests and CSF changes in pediatrics with GBS who were admitted to Tabriz Hospital, Iran, from 2010 to 2020 due to CBS. Patients’ data, including age, gender, CSF analysis, and electrodiagnostic test results, were recorded from the patients’ files. Electrodiagnostic data included pace and amplitude of tibial, median, peroneal, sural, ulnar nerves, nerve conduction velocity (NCV), F-wave, and motor unit action potential (MUAP). The significance level was considered less than 0.05.

The mean age of patients was 4.83±2.72 years, and 54.6% were boys. The most common type of GBS involvement was demyelinating type. No gender differences were found between involved nerves unless the ulnar nerve was significantly more involved in girls (P-value: 0.012). The obtained findings indicated no significant relationship between electrodiagnostic tests and CSF protein (P-value: 0.439).

No association was observed between electrodiagnostic results and CSF changes in pediatrics with GBS.

Customer churn is one of the challenges of business management in today's complex competitive environment. For this purpose, the organization must have an efficient system to detect and analyze the factors influencing customer churn. To conduct this research, an attempt has been made to build a hybrid model based on data mining approaches from information related to 5830 customers of a chain store (demographic information and information based on customer purchase records) with 17 qualitative and quantitative characteristics. The features of higher importance were identified to build the model in the first stage using the logistic regression algorithm. In the second stage, the support vector machine algorithm, a critical supervised learning algorithm, was used to classify the customers and rank the essential features. Finally, the proposed model has been implemented as a case study in the chain store industry. The results indicate the optimal efficiency of the proposed analysis method. This research has been done to identify the influential factors in customer churn and focus on providing new solutions to reduce churn in the retail industry. Also, the results show that age, marital status, and average monthly income from the set of demographic features and how to get to know the store, the share of online shopping, and special sales from the set of features related to customer transaction records are among the most important factors affecting customer churn. In addition, practical suggestions have been presented that can be used for tactical and strategic planning of chain stores to attract and retain customers.

During the COVID-19 pandemic, there was a surge in the consumption of beverages containing methanol, as individuals have erroneously surmised that the ingestion of alcohol could potentially serve as a protective measure against the virus.

The aim of this systematic study was to investigate methanol toxicity and its clinical complications during the COVID-19 pandemic.

A comprehensive search for sources was conducted across multiple databases, including PubMed, Embase, Web of Science, and Scopus, as well as Iranian databases such as Magiran, Iran Doc, and SID. The search spanned from December 2019 to November 2022. The inclusion criteria for this review specified cross-sectional studies that examined the prevalence of methanol toxicity and its complications during the COVID-19 pandemic in Iran. Methodological quality was assessed for each study included in the review.

During the COVID-19 pandemic, the consumption of alcohol, including ethanol and methanol, increased. Men were more likely to consume alcohol compared to women, and a higher number of deaths were reported among young individuals. Visual impairment emerged as one of the most common complications of methanol poisoning, with an increase in its incidence during the disease outbreak.

Given the growing concerns about methanol poisoning, enhancing public awareness could significantly reduce both the prevalence of clinical complications and mortality rates. Therefore, it is recommended that greater emphasis be placed on improving education and raising awareness about methanol poisoning within Iran.

The aim of this study was To investigate the relationship between borderline personality traits and defense styles with marital adjustment. To achieve this goal, 300 individuals who referred to family counseling centers of Rafsanjan were selected by purposive sampling method. Then they were given the Leishnerling Personality Traits Questionnaire (1999), the Spanier Modified Marital Adjustment Questionnaire RDAS (1979) and the Defense Styles Questionnaire of  Andrews, Singh & Bond (1993). Then, the obtained data were analyzed by multivariate correlation coefficient and rRegression analysis. The results showed that borderline personality traits and immature defense style had a significant negative relationship with marital adjustment.Conflict of interest: According to the authors of the present article, there was no conflict of interest.Conflict of interest: According to the authors of the present article, there was no conflict of interest.

Guillain Barre Syndrome (GBS) is an immune mediated neuropathy following infection with a highly variable course and outcome. The aim of this study was to investigate the factors affecting the length of hospital stay of children with GBS.

In this retrospective descriptive-analytical study, the records of 324 children with GBS admitted in Tabriz Pediatric Hospital from June 2003 to December 2013 were studied. By carefully reviewing the existing information, the missing cases were carefully examined and any possible errors in record-keeping were eliminated as far as possible

175 patients (54%) were female and 149 patients (46%) were male. The age range was at least 6 months and maximum 16 years old and the mean was 5.13 ± 3.66 years. In this study, cases referring in summer were reported more (104 cases, 32.1%) than other seasons. In the treatment of patients with Guillain Barre, 272 (86.4%) patients were treated with IVIG, of which 206 (65.4%) received IVIG alone and 66 (21%) with IVIG also received methylprednisolone. 17 cases (5.4%) were treated with plasmapheresis and 26 cases (8.3%) received no treatment. Sensory and autonomic nerve involvements were observed in 46.6% and 16.4% of children, respectively. Cranial nerve involvement was reported in 127 children (39.4%) of which most of patients had involvement of 9th cranial nerve (89 patients, 34.4%).Of the children under study, only 31 (9.6%) needed mechanical ventilation, with a mean of 20.75 ± 21.25 days for these children. The mean duration of hospital stay was 10.07 ± 12.24 days, with more than half of the children (181 patients, 59.9%) being discharged from hospital within one week. 107 (35.4%) children were hospitalized for one to four weeks and 14 (4.7%) were hospitalized for more than four weeks.

The duration of hospitalization in children with GBS has a direct relationship with the type of treatment, autonomic and cranial nerve involvement, severity of illness and mechanical ventilation.

Given that deficiency in B vitamins can lead to the accumulation of homocysteine (Hcy), and hyperhomocysteinemia may have a role in migraine pathogenesis, the present prospective randomized doubleblinded placebo-controlled trial aimed to evaluate the effect of vitamin B-complex supplementation on the alleviation of migraine in children through a possible reduction in Hcy levels.

Ninety children under 15 years of age suffering from typical migraine were included in the present trial. They were randomly assigned into two groups (forty-five patients in each group) to receive either vitamin B-complex or a matching placebo for six months. Serum Homocysteine levels and headache characteristics were evaluated and compared before and after administering vitamin B-complex or placebo.

Unlike the placebo group, the monthly headache frequency, severity of headache, headache disability, and serum Hcy levels were significantly decreased after the vitamin administration. The headache duration was not significantly different before and after the treatment. In the vitamin group, there were significant positive correlations between the frequency and severity, frequency and disability, and severity and disability of headaches. Hcy also had significant positive correlations with the frequency and disability of headaches. In the placebo group, the only found significant correlation was between headache frequency and disability

The administration of vitamin B-complex might effectively relieve migraine severity in children by reducing serum Hcy. However, further studies are needed to confirm the results.

Oral health-related quality of life (OHRQoL) is the main aspect of children’s oral health. In this study, it was intended to explore OHRQoL and its pertinent factors in pre-school children. This cross-sectional study was directed on 352 mothers with children (aged 3–5 years) based on stratified random sampling in health centers in Tabriz, Iran. Data were collected using a self-report questionnaire completed by the caregiver, which includes socio-demographic data, OHRQoL (13 items), caregiver-reported children’s oral health status, and tooth brushing. The one-way ANOVA, independent samples t test, and linear regression were applied to define the predicators of OHRQoL using SPSS 16 at 95% significance level. Mothers’ mean age was 31.5 (SD = 4.9). It was 4.1 (SD = 0.82) for children. According to the results, the majority of the mothers (88%) were homemakers and 25% had higher education. The mean (SD) of OHRQoL was 18.8 (7.9) out of 65. The items most related to OHRQoL were feeling guilty (25%), dental pain (35%), and difficulty eating (14.8%). According to the results of multiple regression, OHRQoL had significant relationship with economic status (P = 0.046), caregiver-reported children’s oral health status (P = 0.0001), children’s frequency of tooth-brushing (P = 0.001), and their age (P = 0.0001). In total, these factors described 0.22% of the variance in children’s OHRQoL. OHRQoL did not have a statistically significant relationship with mothers’ education level or children’s gender. According to the findings, the key predictor of OHRQoL was children’s oral health status, tooth brushing, and age. Effective educational interventions could be designed based on these predictors to improve the OHRQoL of children.

Guillain-Barre syndrome (GBS) is an immune-mediated polyneuropathy and a common cause of acute ascending weakness in children. 

This study aims to report long-term disability and poor outcome predictors of GBS in children. Medline (via Pubmed), Embase, Cochrane Library, Web of Science, and Scopus database was searched for relevant studies until April 2022, with a designated search strategy, using MeSH terms and free keywords.

Studies evaluating functional outcomes of GBS in children with at least one year of follow-up were included. All studies achieved acceptable quality for inclusion. After selecting studies based on inclusion criteria, data were extracted based on a modified standardized Joanna Briggs Institute (JBI) data extraction tool, and the methodological quality of studies were reviewed using the Joanna Briggs Institute (JBI) critical appraisal tool.

Fourteen studies were included in this systematic review consisting of 1141 patients (647 males, 466 females, and 28 unclassified). Follow-up duration varied from one year to 11 years. The prevalence of the GBS subtype was as follows, acute inflammatory demyelinating polyneuropathies (AIDP) 46.6%, acute motor axonal neuropathy (AMAN) 30.2%, acute motor and sensory axonal neuropathy (AMSAN) 6.8% and Miller fisher 6.1%. The most commonly reported poor outcome after at least one year of follow-up was walking disabilities and gait disorders. Motor deficits and weakness, sensory complaints, including pain or paresthesia and fatigue were other prevalent residual symptoms. Axonal form of GBS was the most reported poor outcome predictor, followed by Hughes disability score >3, a delay in independent walking, artificial ventilation, and rapid progression of symptoms.

Despite the good prognosis of GBS in children, they could suffer long-term sequels, especially in walking abilities and gait. The axonal form is considered a crucial poor predictive factor.

Guillain-Barre syndrome (GBS) is an acute immune-mediated disease that affects both adults and children. Many infectious and non-infectious conditions may trigger this disease. Apolipoprotein E (APOE) is a glycosylated protein that has a variety of lipid and non-lipid related functions. The present study aimed to evaluate the serum levels of APOE in children with GBS compared to healthy control subjects to evaluate the diagnostic and pathologic effects of APOE in GBS.
In a cross-sectional design, 124 participants were divided into the GBS group (n=61) and the control group (n=63). Blood sampling and measurement of APOE were done according to the manual of the Human APOE ELISA kit. Demographic variables and further data about GBS patients were collected from patients' medical records. Weight and height were measured using SECA scales. The analyses were performed using SPSS Statistics 21.0 software and appropriate analytical tests. P-values of <0.05 were considered statistically significant.
Mean ±SD of APOE serum levels was 8.43±3.59 micrograms/ml in the GBS group and 35.28±11.18 micrograms/ml in the comparison group. The difference between the two groups was strongly significant (P<0.001). The mean level of protein in Cerebrospinal Fluid (CSF) in GBS patients was 184.36±19.09 mg/dl. There was not any significant difference in the demographic findings between the two groups.
Our study demonstrated that the serum levels of APOE in children with GBS are lower compared to healthy subjects.

The aim of this study was comparing of performance of Iranian children on the Bayley Scales of Infant and Toddler Development- third edition with the standardized norms of Iran and United State.

A total of 248 Iranian children at the ages of 1-42 months were participated in this study. The Bayley Scales of Infant and Toddler Development- third edition was administered to evaluate the cognitive, language and motor development of these children.

The scaled scores of the study group were higher based on the reference norms in receptive communication, fine motor, and gross motor subtests. The scaled scores were variable in expressive communication and cognitive subtests. The differences were significant for receptive communication and fine motor subtests (P<0.05). Using the reference norms instead of Iran norms resulted in under-referral regarding receptive communication and fine motor subtests. More children scored below 1 and 2 SD using Iran norms in comparison to those reported for using the reference norms.

Iran norms differ significantly from the reference norms over two subscales. It is recommended to use population-specific norms to identify children with developmental delay and early intervention

Subependymal Giant Cell Astrocytomas (SEGAs) are slow-growing glioneuronal tumors typically found around the ventricles of the brain, particularly near the foramen of Monro in 15%-20% of patients with tuberous sclerosis complex (TSC). Surgical resection is the standard treatment for these symptomatic tumors. The mTOR inhibitor everolimus can be regarded as an alternative treatment for SEGAs due to the complications of surgery. The present study primarily aimed to specify the effect of everolimus on SEGA volume change before and after treatment. The secondary objective was to determine the effect of this drug on renal angiomyolipoma (AML), skin lesions, and seizures in TSC patients.

This pre- and post-treatment clinical trial was performed on 14 children (eight females and six males with a mean age of 10 years) previously diagnosed with TSC based on the diagnostic criteria. The subjects received oral everolimus at a dose of 3 mg/m2 for at least six months.

Half of the patients had more than 30% of volume loss in SEGA, and in 28.5% of them, a ≥ 50% reduction in SEGA volume was observed (P=0.01). Moreover, 92.9% of the patients had a ≥ 50% decrease in the frequency of seizures (P=0.000). The response rate in AML and skin lesions was 14.2% and 50%, respectively.

Everolimus significantly reduced the seizure frequency and SEGA volume in the subjects; hence, it can be used as a potential alternative treatment for symptomatic SEGA in TSC patients.

Background :

Arterial ischemic stroke (AIS) is an important cause of neurological disabilities, such as hemiparesis, epilepsy, and cognitive dysfunction in children. This study aimed to describe the clinical presentation, risk factors, and neuroimages of pediatric patients with AIS.

In a cross sectional study all of 73 patients admitted to Tabriz children’s hospital with the diagnosis of Ischemic stroke between 2014 and 2019 were enrolled in the study and follow up for at least one year after discharge. Stroke was diagnosed based on clinical presentation and compatible Brain CT scan and MRI, or MRV/MRA and then Para clinical assessment were done.

Male predominance in the present have been observed with male/female ratio of 1.28. AIS was common in younger age (79.4% 0.05).

Conclusion :

With careful investigation, in most of the children underlying risk factors could be found. Based on the results, acute illness and cardiac disorders were the most frequent risk factors in pediatric stroke. However, some of these risk factors (such as anemia, infection, and dehydration) which are relatively common in the pediatric population were preventable.

Green space is effective in reducing noise pollution. The purpose of this research was to study the effect of tree species in the green space of Abadeh, as well as the number of tree plantation rows and planting width on decreasing the intensity of sound.

In this study, four stations in two areas was selected in Abadeh city including Shahid Chamran Blvd. and Northern Passageway and sampling was carried out in two turns in the morning and evening using the Cirrus sound level  meter CR: 303 in March, 2018 and 25 data in each station and 400 data in total was recorded.

The results of comparing the average sound intensity level measured in the two studied areas in the morning and evening with the Iranian national standard show that the average sound intensity level in both regions is higher than the standard; So despite the green space, there is noise pollution in both areas. The results of the study of sound intensity levels in Shahid Chamran Boulevard and along the northern passage in the morning and evening show that there is a negative correlation between the distance from the sound source (distance from the street) and the number of rows of trees planted and the average sound intensity level. This means that the greater the distance from the street and the number of rows of trees planted, the greater the amount of noise reduction.

The highest noise reduction in Shahid Chamran Boulevard is related to the distance of 54 meters from the edge of the street with 14 rows of planting with average sound intensity level for morning and evening of 65.04 and 60.96 dB, respectively, and next to the northern passage is related to the distance of 85 meters from the edge. The street was planted with 10 rows and there was a hill with an average level of sound intensity for the morning and evening of 65.04 and 64.08 dB, respectively.

Tuberous Sclerosis Complex (TSC), and Spinal Muscular Atrophy (SMA) are two inherited disorders while they are genetically independent. TSC is characterized by the formation of multiple hamartomas in nearly all organs. SMA is a destructive neurological disorder leading to progressive muscular weakness and atrophy.

The patient was an Iranian (Urmia, East Azerbaijan Province, Iran) 11-year-old daughter of non-consanguineous parents. She developed seizures as an infantile spasm at three months old. She had a delay in motor development. At 11 years old, the patient had proximal muscle weakness resulting in a characteristic waddling gait and Gowers’ sign, which was suspected of SMA. The SMA was then confirmed using molecular analysis. Clinical examination of the patient revealed angiofibromas, shagreen patch, and hypopigmented spots on the skin; cortical tubers, subependymal nodules, and subependymal giant cell astrocytoma in the brain; angiomyolipomas in the kidneys; and retinal hamartoma, which fulfilled the diagnostic criteria of TSC.

Although TSC and SMA are genetically independent disorders, they may rarely occur together in an individual, simultaneously. Further studies required to find the patterns of genetic inheritance of these diseases among the reported patient.

Determining the predictors of respiratory failure and duration of intubation in children with Guillain-Barre syndrome (GBS).

Children diagnosed with GBS at Tabriz Children’s Hospital were studied. Factors associated with and influencing respiratory failure as well as the duration of intubation were determined using both univariate and multiple analyses.

Overall, 324 children were enrolled in the study, 54.0% of whom were males. Thirty-one (9.6%) patients underwent mechanical ventilation, the patients under 5 years old were more prone to the requirement of mechanical ventilation (11.3% vs. 6.9%). Cases hospitalized in winter were more likely to need ventilation compared to those hospitalized in spring (OR =7.00; 95% CI:1.51-32.53). Also, autonomic involvement (OR=8.88, 95% CI:4.03-19.58; p<0.001) and cranial nerves involvement (OR=9.88, 95% CI:3.68 - 26.52; p<0.001) emerged as risk factors for mechanical ventilation requirement. Overall, 16.1% of patients with axonal electrophysiologic pattern required mechanical ventilation compared to 7.4% of those with demyelinating type (OR:2.15, 95% CI: 1.01-4.69). In univariate analysis, the only variable that showed a correlation with the duration of intubation was axonal electrophysiologic pattern (p= 0.028).

Approximately, 10% of the patients required mechanical ventilation. Season, cranial nerve involvement, autonomic dysfunction and electrophysiologic pattern were the most important variables in predicting respiratory failure and duration of mechanical ventilation.

Evaluating the state of clinical teaching is a very essential aspect of useful teaching.It helps in determining areas of strength and areas for improvement. Accordingly, the aim of this study was to evaluate clinical teaching activities in a pediatric hospital at the Tabriz University of Medical Sciences, based on standards provided by the Ministry of Health in Iran.

A cross-sectional study was conducted from October to December 2018 in the setting of a pediatric hospital of the Tabriz University of medical sciences. Participants were selected from this hospital: academic members and medical students who were present in the pediatric hospital during this investigation. The evaluation tool was a checklist of standards relating to clinical teaching settings. The outcome measures included four domains, assessed using mean standard scores: teaching round, morning report, journal club, and outpatient clinic. Analysis of variance (ANOVA) was used to compare scores on the checklist among academic members and medical students.

A total of 126 participants completed the checklist. Among the participants, 50 (39.6%) were 5th-year medical students, 51 (40.4%) were final-year medical students and 25(19.8%)were academic members. The highest and lowest mean standard scores were 85.3 and 34.5,the morning report and the teaching round by final-year medical students, respectively. No statistically significant differences were found among academic members and medical students’mean standard scores for teaching round (P = 0.983), morning report (P = 0.696), journal club (P= 0.952), and outpatient clinic (P = 0.060).

Considering both academic members and medical students, clinical teaching standards were not widely regarded as important in the pediatric wards of the Tabriz University of Medical Sciences. Some interventions are indicated to improve adherence to the Ministry of Health standards.

Designing and conducting residency exams have been the province of type I universities since 2005, based on the plans of the Medical Education Council. This study compared emergency medical residency pre-board and board exam results among the universities of medical sciences in Iran.

This cross-sectional descriptive study consisted of 600 questions from residency preboard exams and 150 questions from board exams in 2013-2014, as well as 600 questions from residency promotion exams and 150 questions from the board exam in 2013-2014 and 2014- 2015 at the Tabriz, Shahid Beheshti, Mashhad, and Tehran Universities of Medical Sciences. All questions were at the level I through III by Bloom’s taxonomy. Structural principles were evaluated using Millman’s checklist. All data were analyzed using SPSS 18 with chi-square tests.

The mean percent of more contextualized questions of the questions at Bloom’s taxonomy levels II and III in residency pre-board exam questions was 76.6% in 2013-2014 and 86.6% in 2014-2015 among the four universities. In terms of structural principles, the percentage of board exam questions that aligned with Millman’s structural principles was 100.0% and 99.3% in 2013-2014 and 2014-2015, respectively. For the residency pre-board exam in 2014- 2015, the mean discrimination index was 0.14 (low), and the mean of the difficulty factor was 0.64 (appropriate).

Questions at Bloom’s taxonomy levels II and III were higher in 2014-2015 in comparison to those in 2013-2014, and the percentage of the correctly-structured questions was high and did not significantly change from 2013-2014 in comparison to that of 2014-2015.

Background Tay-Sachs disease is an autosomal-recessive lysosomal storage metabolic disorder. The typical symptoms of the disease include ataxia, muscle weakness, and mental disorders. The severity of the clinical symptom relies on the enzymatic activity of residual Hexosaminidase-A. Case Presentation The patients were two Iranian (Tabriz city, East Azerbaijan Province, Iran) 22-month-old male identical twins of distant consanguineous parents with a high Apgar who score referred to Tabriz Children Hospital, Tabriz, Iran. Both twins had normal growth until 7 months of age. They started regression after 7 months of age and became hypotonic so that they could not keep their neck and control their head. The enzyme analysis of the patients showed the low-level activity of Hexosaminidase-A. A considerable delay in normal myelination process was discovered by brain Magnetic Resonance Imaging in the patients. Conclusion It can be determined that Tay-Sachs disease can occur in twins of distant consanguineous parents. Further studies are needed for detecting the mutations relating to the disease in the patients as well as their families.

Celiac disease (CD) is a systemic immune-mediated disorder caused by the dietary gluten in individuals who are genetically susceptible to the disease. In fact, CD is a T cell-mediated immune disease in which gluten-derived peptides activate the lamina propria CD4+ Teff cells, and these T-cell subsets can cause the intestinal tissue damages. Also, there are additional subsets of CD4+ T cells with suppressor functions. These subsets express the master transcription factor, FOXP3, and include Tr1 cells and CD4+CD25+ regulatory T cells (Tregs), which are the main population involved in maintaining the peripheral tolerance, preventing the autoimmune diseases and limiting the chronic inflammatory diseases such as CD. The suppressive function of Tregs is important to maintain the immune homeostasis. This paper examined the features and the basic mechanisms used by Tregs to mediate the suppression in CD.

Quantitative analyses of antiepileptic drugs are required in clinic and to rational dosage adjustment, the clinician needs the blood levels of these drugs. A highperformance liquid chromatography with spectrophotometric detection has been developed and validated for simultaneous determination of some antiepileptic drugs in plasma of patients with epilepsy.

A simple procedure based on deproteinization by acetonitrile was used for pretreatment of plasma samples. Liquid chromatographic analysis was carried out on a NovaPak® C18 analytical column, using a ternary mixture of potassium dihydrogen phosphate buffer (pH 6.0)-acetonitrile-2-propanol (63:22:15, v/v/v) as the mobile phase, at a flow rate of 1.0 mL min-1 .

Calibration curves were linear over a range of 1–40 µg mL-1 for phenobarbital, 1– 30 µg mL-1 for phenytoin, 0.3–15 µg mL-1 for carbamazepine and 0.5–6 µg mL-1 for carbamazepine epoxide.

The simple sample pre-treatment, combined with the fast chromatographic run was used for the determination of antiepileptic drugs for a large number of patient samples.

Modafinil (MDF) is used orally for the treatment of attention-deficit/hyperactivity disorder and narcolepsy. It holds low solubility and high permeability; therefore, improving its dissolution properties by preparing nanoformulations can be a promising approach to enhance its oral absorption. Our aims were to prepare and characterize MDF-Eudragit® RS100 (MDF-ERS) nanoparticles by electrospray technique. Electrosprayed nanoparticles were fabricated by varying MDF to ERS ratios and concentrations. The formulations were characterized by scanning electron microscopy (SEM), differential scanning calorimetry (DSC), powder X-ray diffraction (PXRD), and Fourier-transform infrared spectroscopy (FTIR). Release studies were performed on nanoparticles, physical mixtures, and raw MDF. The release data were fitted to different models to understand the mechanism of the drug release. Electrospraying of MDF and ERS solution resulted in the preparation of nonobeads or nanofibers, and the particulate characteristics of the obtained products were largely controlled by the polymer amount in the solution. PXRD and thermal analyses showed that MDF was an amorphous phase in the structures of nanoparticles. Using FTIR, no interaction was observed between MDF and ERS in nanoparticles. Nanoparticles showed biphasic release profiles and the order of dissolution rates was: nanofibers>MDF>nanobeads. The well-fitted model was Weibull model, indicating a Fickian diffusion as the main mechanism of release. The results suggest that by optimization of variables such as solution concentration of MDF-ERS nanofibers and nanobeads with higher dissolution rates can be made by electrospray. Electrospray deposition as a simple, continuous, and surfactant free method is an excellent choice for preparation of drug loaded polymeric nanoparticles.

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.

Valproic acid (VPA) is a well-established anticonvulsant drug that has been increasingly used in the treatment of many forms of generalized epilepsy. Although there are many reports of adverse effects of VPA, studies focusing on the concentration–response relationships of VPA and its metabolites in patients with refractory epilepsy are extremely limited. In this paper, a high efficient method was developed for the extraction, preconcentration, and determination of valproic acid and its main metabolite in plasma. For the extraction and preconcentration of the selected analytes, a volume of an extraction solvent was placed at the bottom of the microtube containing pretreated plasma. The mixture was rapidly withdrawn into a 1-mL syringe and then pushed-out into the tube in order to form a cloudy mixture. For the further turbidity, the mixture was shaken on a vortex agitator. This procedure was used to analyze plasma samples of Iran patients with epilepsy (n = 70). The results revealed that in most patient with low level of valproic acid relative to expected level of valproic acid, amount of 3-heptanone was high. The limits of quantification of the valproic acid and 3-heptanone were obtained 0.2 mg L–1 and 0.04 mg L–1, respectively. An acceptable precision was obtained for the concentrations of 2 mg L-1 each analyte (relative standard deviation ≤ 9 %). The obtained results showed that this method is simple, sensitive and reliable and can be used for analysis of the selected analytes in plasma sample of patient with epilepsy.

Alzheimer’s disease (AD) is an age-related and irreversible neurological disorder. The low efficacy of current therapeutic strategies is related to both poor drug potency and the presence of various obstacles in the delivery routes, such as blood-brain barrier (BBB) that limits the uptake of most drugs by the brain. Rivastigmine hydrogen tartrate (RHT) is used in mild to moderate forms of AD therapy. The present study described the use of Poly-lactic-co-glycolic acid (PLGA) nanoparticles (NPs), as effective delivery vehicles, to improve the therapeutic efficiency of RHT. RHT-loaded PLGA NPs were prepared using interfacial polymer deposition, following solvent displacement method with different ratios of polymer: Drug. The NPs were studied for entrapment efficiency, particle size, and surface morphology, using scanning electron microscopy (SEM), X-ray diffraction (XRD), Fourier transform infrared spectroscopy (FTIR), and differential scanning calorimetry (DSC). In vitro drug release from NPs was also assessed by a modified dissolution method. The entrapment efficiency of RHT in NPs was found to be between 27.71 ± 6.86 and 45.70 ± 11.06 and the average size was about 75.14 to 173 nm. The zeta potential was negative (-2.28 to -10.5 mV), as determined by dynamic light scattering (DLS). The drug released from NP formulations was between 69.98% and 89% upon 24 hours, which indicated improved sustained drug release characteristics. These results suggested the potential usefulness of PLGA NPs for the delivery of RHT in a sustained and controlled manner