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Introduction: This study aimed at investigating these models through evaluation of one psychological variable correlation (pain self efficacy) with pain intensity in M.S. Method: In this study, 249 patients were selected through convenient sampling. They were selected among those who referred to M.S assembly of Isfahan. The data were analyzed through Pearson product moment correlation, regression analysis, and MANOVA. Results: There is a significant negative correlation between pain self-efficacy and perceived intensity in M.S patients. The results of MANOVA also indicate that the highest correlation was reported to be between the highest rate of pain and the lowest rate of pain self-efficacy among the patients. Conclusion: This study provided empirical evidence supporting bio-psycho-social model and shows that psychological factors influence the perception and experience of pain intensity. [ABSTRACT FROM AUTHOR]

Background: Few studies are carried out on the epidemiology of amyotrophic lateral sclerosis (ALS) in Middle East with no reports from Iran. Objective: To determine the epidemiological and clinical features of ALS amongst the Iranian population living in Isfahan, Iran. Methods: Medical records of all hospitals with a neurology department and outpatient neurology clinics in Isfahan province from 2002 to 2006 were reviewed, and all patients with the diagnosis of ALS according to El Escorial diagnostic criteria were extracted and related demographic and clinical data were gathered and analyzed. Results: We found 98 new patients (66 men and 32 women) with definite, probable, or possible ALS. The average annual incidence was 0.42/100 000, with the highest incidence rate amongst those aged 70–74. On 21 March 2006, the crude prevalence was 1.57/100 000. Median survival from onset was 48 months (95% confidence interval 34–61) and survival rates for 1, 3,and 5 years after the onset were 94%, 66%, and 32%, respectively. Conclusions: The incidence and prevalence of ALS in the Iranian population seems to be lower compared to other populations and the survival of patients was longer than previously reported. [ABSTRACT FROM AUTHOR]

This study was designed to evaluate the efficacy of gabapentin (GBP) in the treatment of SUNCT syndrome on a relatively large sample of Persian patients. Eight patients with SUNCT syndrome underwent a 4-week, open-label, daily treatment of 600–900 mg GBP. The frequency, intensity and duration of attacks were compared before and after the trial. After 4 weeks of treatment, intensity, duration and frequency of headaches were significantly ( P < 0.05) reduced. In addition, five patients (62.5%) were completely relieved from headaches, and in the other three patients the mean intensity, frequency and duration of headaches were decreased notably. In this study, GBP was well tolerated and no unfavourable side-effects were reported. After the end of the trial all patients continued the medication, and after 3 months none reported undesired side-effects or return of the headaches to the pre-treatment status. Our patients had a significant response to GBP, and considering other case reports on the effectiveness of GBP in the treatment of SUNCT syndrome, we propose that, taking into account the good side-effect profile and lack of interactions of GBP, this drug could be considered as an option for the treatment of SUNCT. [ABSTRACT FROM AUTHOR]

Primary progressive multiple sclerosis (PPMS) is an uncommon form of multiple sclerosis (MS) in which the course of disease is progressive from onset. In a retrospective study amongst 1606 MS patients registered in Isfahan MS Society (IMSS) from April 2003 to 31 December 2005, 92 PPMS cases were identified. That means, the frequency of PPMS amongst all included MS patients would be 5.7% (95% CI: 6.7% and 4.7%). The mean expanded disability status scale (EDSS) for the group was 5.09 ± 1.3. The commonest mode of presentation was motor disturbance in 55 (59.8%), other modes of presentation were, vertigo in 15 (16.3%), visual problems in 12 (13%), sensory disturbances in six (6.5%), and diplopia in four (4.3%). The current existing symptoms were motor problems in all 92 (100%), cerebellar symptoms in 46 (50%), and cognitive impairment in only six (6.5%). Interestingly, two (2%) were affected by poliomyelitis during childhood and presenting symptom in both was limb weakness. Primary progressive form of MS is less common in Persian population and some of the rates observed in PPMS patients differ from those in other regions, these differences may be due to different ethnicity of Persian patients or to geographical differences. [ABSTRACT FROM AUTHOR]

There is an overall increase in the worldwide prevalence and incidence of multiple sclerosis (MS). Studies from several countries also demonstrated an increase of female/male ratio over time denoting an increase in the incidence of MS particularly in women. In this study we sought to assess the trends in MS incidence and prevalence in males and females over recent decades in Isfahan, Iran, which differs from other regions in terms of environmental and lifestyle changes. We determined female/male ratio by year of birth (YOB) in 1584 patients with MS registered with Isfahan Multiple Sclerosis Society (IMSS) from April 2003 to August 2007. A comparison of sex ratio of MS patients by YOB showed a significant, progressive, gradual increase, with an apparent interruption in the late 1960s. In this study year of birth is a significant predictor for sex ratio (p<0.001, χ2=17.130, Spearman's rank correlation r=0.893). Our findings show that there is a significant increase in the incidence of MS among females for the the last decades in the Isfahan province of Iran. This rapid increase may be related to changes in environmental interactions rather than genetic factors, and among them vitamin D insufficiency, enhanced diagnosis, and lifestyle changes appear to be more plausible causative factors. [ABSTRACT FROM AUTHOR]

Conjugal multiple sclerosis (MS) is a rare form of MS in which both spouses are affected, and at least one is affected after marriage. Among 1606 definite MS patients, 1076 were in marital relationship, among whom we identified six conjugal pairs, giving the conjugal rate of 0.5%. This rate is 12.5 times higher than the estimated risk of MS for the general population (0.04%). The observed conjugal rate suggests an increased risk of developing MS for MS patients' spouses, this could be due to transmission or, more likely, to the same environmental factors shared in adult life. [ABSTRACT FROM AUTHOR]

Background: Since data is limited on radiologically isolated syndrome (RIS) subjects in certain regions like the Middle East, we aimed to further explore the replicability and generalizability of previously suggested predictors among a cohort of Iranian RIS subjects and report the long-term clinically definite MS (CDMS) conversion rate in this cohort., Methods: We conducted a prospective 10-year cohort on our RIS participants, during which we collected the MRI, paraclinical, and demographic data of the subjects, and identified those who converted to CDMS., Results: Out of 35 participants, 10 (28.5 %) developed CDMS during an average of 5.58 ± 3.08 years (range: 4 months to 10.33 years). OCB positivity was the only definitive predictor for conversion to CDMS in this cohort (P-value = 0.006), but other previously reported risk factors such as spinal cord lesions or age lacked statistical significance (P-values > 0.05). We also reported the median survival time as 114 months, the proportion surviving after 14 months as 96.9 % ± 3.1 %, and the overall conversion rate as 0.05 cases per year., Conclusion: Our results highlight OCB as an important predictive factor of clinical conversion in RIS. The prominence of OCB suggests a need for routine CSF analysis in RIS subjects and could guide clinicians in deciding which RIS subjects benefit from DMTs., Competing Interests: Conflict of Interest The authors declare no conflicts of interest., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Characteristics of livers and spleens of people with multiple sclerosis (pwMS) could constitute good biomarkers of MS-related characteristics such as the disability status. To test the hypothesis "the gross anatomical features of livers and spleens, are not similar between pwMS with different disease characteristics" a cross-sectional study was conducted on pwMS seen at the Isfahan MS clinic, Iran, from February until December 2023. Definitive, otherwise-healthy, pwMS were enrolled after an initial laboratory evaluation. Presence/absence and grading of non-alcoholic fatty liver disease (NAFLD) and the span of spleen were determined by a radiologist using high-resolution abdominopelvic ultrasonography. 193 pwMS (160 women) were enrolled. Of whom, 143 (74.1%) were receiving first-line disease-modifying therapies (DMTs), 24 (12.4%) fingolimod, and 26 (13.5%) rituximab. The span of spleen was negatively associated with EDSS (adjusted β [SE] - 4.08 [1.52], p < 0.01), as well as 6 m-CDW (adjusted β [SE] - 6.94 [3.56], p = 0.05), unlike age, DMTs, and MS duration (all with p > 0.05). Receiver operating characteristic analysis showed, spleen span performs significant but poor in discrimination of EDSS > 1 from EDSS = 1 (area under curve [AUC] 0.62, SE 0.05, p < 0.01), yet, significant and fair in discrimination of presence from absence of 6 m-CDW (AUC 0.72, SE 0.06, p < 0.01). Other findings were unremarkable. Further longitudinal, prospective studies are warranted to confirm whether smaller spleens are predictive of higher disability accrual rate in pwMS. Particularly, findings require further validation in untreated/treatment-naïve pwMS, and ones with higher EDSS scores., (© 2024. The Author(s).)

Medication adherence is of utmost importance in achieving the desired therapeutic outcome and effectively managing seizures in patients with epilepsy (PWE). It is imperative to recognize self-esteem as a psychological determinant that potentially influences the optimal compliance with anti-seizure medications (ASMs) among PWE. The objective of this study was to explore medication adherence and its relationship with self-esteem among individuals diagnosed with epilepsy in Isfahan, Iran., Methods: This descriptive-analytical study was conducted in the year 2021, encompassing a cohort of 250 PWE who were referred to designated medical facilities in Isfahan, Iran, and were selected by the consecutive sampling technique. A 3-part instrument including demographic components, the Rosenberg Self-Esteem Scale, and the Morissky Drug Adherence Questionnaire employed for data collection., Results: The mean and standard deviation of adherence to the medicinal regimen in the participants were 6.9 ± 2.02, and 46.4 % had a low level of adherence to the medication regimen (total score 0-6). At the same time, the mean and standard deviation of self-esteem in these patients was 5.11 ± 2.11. There was a statistically significant and direct correlation between adherence to the prescribed drug regimen and self-esteem (rs = 0.464, p = 0.00)., Conclusion: Based on the findings of the study that showed a statistically significant and positive correlation between self-esteem and adherence to the medication regimen, it is advisable to enhance and advocate for these factors in PWE., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

To affirm the short-term safety of the BBIBP-CorV (Sinopharm) COVID-19 vaccine among people with multiple sclerosis (pwMS), 517 vaccinated and 174 unvaccinated pwMS were interviewed. 16.2% of the vaccinated pwMS reported at least one neurological symptom in their respective vaccine-related at-risk periods (ARP) - a period from the first dose until two weeks after the second dose of the vaccine. In a multivariable logistic regression model, the presence of comorbidities ( P  = 0.01), use of natalizumab ( P  = 0.03), and experiencing post-vaccination myalgia ( P  < 0.01) predicted the development of post-vaccination neurological symptoms. One MS relapse, one COVID-19 contraction, and one ulcerative colitis flare after the first dose, and four MS relapses after the second dose of the vaccine were the only reported serious adverse events during the ARPs. To show if the vaccine provoked MS relapses, we compared the relapse rate of vaccinated pwMS in the vaccine-related ARP with the annualized relapse rate of unvaccinated pwMS in the prior year-a measure of baseline MS relapsing activity in the respective time-using a multivariable Poisson regression model accounting for possible confounders, which failed to show any statistically significant increase ( P  = 0.78). Hence, subject to replication-as the vaccinated and unvaccinated pwMS differed in baseline characteristics-the BBIBP-CorV vaccine does not seem to affect short-term MS activity. Furthermore, as 83.33% of the unvaccinated pwMS reported fear of possible adverse events to be the reason of their vaccination hesitancy, provision of evidence-based consultations to pwMS is encouraged. Limitations of our study briefly included lack of data for self-controlled analysis of relapse rates, possible presence of recall bias, and lack of on-site validations regarding the clinical outcomes due to the remote nature.

Background: Even within the most populous countries in the Middle East, such as Iran, autoimmune encephalitis cases have been rarely reported., Objective: We aimed to describe the demographic, clinical, and paraclinical characteristics of Iranian patients with autoimmune encephalitis positive for anti-neuronal autoantibodies., Methods: This cross-sectional study included all patients diagnosed with autoimmune encephalitis and referred to our hospital, in Isfahan, Iran, from March 2016 to May 2020. Patients' demographic, clinical, laboratory, radiological, and electroencephalographic features were obtained from their medical records., Results: We identified a total of 39 (21 females, 53.8%) patients with autoimmune encephalitis (mean age = 34.9 ± 12.8 years). The most commonly detected antibody was anti-NMDAR (n = 26, 66.7%), followed by anti-GABA B R (n = 8, 20.5%), anti-Zic4 (n = 4, 10.3%), and anti-GAD65 (n = 1, 2.6%) antibodies, in descending order of frequency. Two anti-NMDAR-positive patients had a history of systemic lupus erythematosus (SLE), and four had a prior history of herpes simplex encephalitis. Clinical presentations in patients positive for anti-Zic4 antibodies included cognitive decline (n = 4, 100%), seizures (n = 3, 75%), parkinsonism (n = 1, 25%), and stiff-person syndrome (n = 1, 25%)., Conclusion: This was the first case series of Iranian patients with autoimmune encephalitis with some interesting observations, including SLE-associated anti-NMDAR encephalitis, as well as an unusual concurrence of anti-Zic4 antibody positivity and cognitive problems, seizures, parkinsonism, and stiff-person syndrome., (© 2021. Fondazione Società Italiana di Neurologia.)

Purpose: The coronavirus disease 2019 (COVID-19) pandemic has affected people globally, and people with chronic diseases are suffering more in maintaining their mental and physical health., Method: This cross-sectional, case-control study assessed the anxiety level in people with epilepsy compared with the general population., Results: The results showed that 13.5% of patients had experienced a severe level of anxiety, but the mean anxiety level between groups did not show significant difference., Conclusion: Although still many aspects of the pandemic on people with epilepsy are yet to be determined, active investigation of psychological sequels of the pandemic is demanded., Competing Interests: Declaration of competing interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Few data are available on the prevalence of midbrain lesions among NMOSD patients. The study aimed to address the frequency of midbrain lesions, especially dorsal midbrain involvement, among a group of NMOSD patients., Methods: The medical records of 108 NMOSD patients were reviewed, who were registered at the Al-Zahra MS Clinic, Isfahan, Iran. The patients´ information including sex, age, first recorded chief complaint, and midbrain lesion presence in the first brain MRI was collected., Results: Out of the 108 NMOSD patients, eight had midbrain lesions in their first brain MRI (7.4%). Of these patients, 50% were male and 50% were female, with the mean age being 35.25 ± 12.17 years. The most frequent first chief complaints included diplopia due to incomplete third nerve palsy and vertigo. The brain MRIs of the patients showed symmetric dorsal midbrain involvement in all the patients, and enhancement was discovered in only one of the brain MRIs., Conclusion: Among the patients, 7.4% presented midbrain involvement at onset. Therefore, midbrain lesions should also be considered as a possible core clinical manifestation in the NMOSD diagnostic criteria., Competing Interests: Declaration of Competing Interest The authors declare there is no conflict of interest., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system with unknown etiology, which typically is manifested in early to middle adulthood. Recently, genome-wide association studies have identified susceptibility of immune-related genes to be involved in MS predisposition. The goal of the current study was to investigate the association of single nucleotide polymorphisms (SNP) with the immunologically related genes responsible for the disease, composed of CD58 (rs2300747 A>G), CD226 (rs763361 C>T), and HLA-G (rs1611715 A>C), with MS susceptibility. In this case-control study, a total of 200 patients suffering from relapsing-remitting multiple sclerosis and 200 healthy individuals were recruited. DNA was extracted from blood and then all subjects were genotyped for the polymorphism within mentioned genes by high-resolution melting (HRM) real-time PCR method. Statistical analyses were performed using SPSS software (version 20; SPSS, Chicago, IL, USA). Our finding showed that there are significant differences in genotype and allele frequencies between two groups regarding rs763361 (P = 0.035, OR 0.64, CI 95% for C allele) and rs1611715 (P = 0.038, OR 1.57, CI 95% for AA genotype) polymorphisms within CD226 and HLA-G genes, respectively. Concerning rs2300747 polymorphism on CD58 gene, no significant differences were found between cases and controls. In general, results from the current study indicate that CD226 and HLA-G, but not CD58 genetic polymorphisms are associated with increased risk of MS in Isfahan population similar to European populations. However, to elucidate how these SNPs contribute to MS pathogenesis, functional studies are needed.

Background: Multiple sclerosis (MS) is known as a neuroinflammatory disease of the central nervous system (CNS). The neuroinflammation may induce pro-inflammatory cytokines such as Osteopontin (OPN). OPN plays an important role in the inflammation by modulating the T helper1 (Th1) and Th17 responses. Since the exact immune pathogenesis of MS is complex and not well defined and many factors are involved, the need to detect more contributing biomarkers may help in setting new therapeutic strategies. Objective: This study tried to compare plasma OPN levels in relapsing- remitting multiple sclerosis (RRMS) patients during the remission phase with healthy subjects in Isfahan province. Materials and methods: In a case-control study, plasma was collected from the 40 RRMS as well as 38 (age and sex matched) healthy individuals as a control group. PlasmaOPN level was measured and compared between the two groups by Enzyme-linked immunosorbent assays (ELISA). Result: Statistical analysis revealed that plasma OPN level was markedly higher in the case group (RRMS patients during the remission phase) compared with the control group ( P - value = 0.039). Our results also showed that there was no statistically significant difference in mean of plasma OPN level among RRMS patients who were treated with interferon (IFN)-ß and those who were not ( P - value = 0.332). There was also no correlation between OPN plasma level and EDSS score ( r  = 0.037, P - value = 0.835), age of onset ( r  = 0.161, P - value = 0.357) and duration of disease ( r  = 0.121, P - value = 0.490). Conclusion: Higher OPN plasma level in studied patients suggests that OPN increased in RRMS patients who were in remission phase. It could be hypothesized that plasma OPN level may be increased as part of the pro-inflammatory cytokine milieu taking place in MS patients. OPN may not be specific marker for MS, but targeting it might present promising therapeutic effect to MS patients.

Emotional intelligence is a psychological component that may affect happiness level in patients with epilepsy. Given the high prevalence of depression in patients with epilepsy, as well as the limitations of studies in this regard in Iran, the aim of this study was to investigate the effect of an emotional intelligence component program on happiness in patients with epilepsy., Methods: This randomized clinical trial study conducted on 70 patients with epilepsy who were randomly divided into two experimental and control groups of 35 patients. Emotional Intelligence Training Based on Bar-On Combined Model was provided in eight 90-minute sessions for eight weeks. Data were collected using a two-part questionnaire: demographic data and the Oxford Happiness Questionnaire (OHQ)., Results: The mean age of the subjects was 33.3 ± 10.4 years in the intervention group and 34.4 ± 9.3 years in the control group. The independent t-test results showed no significant difference between the two groups before the intervention (p = 0.195). The Mann-Whitney test results showed a significant difference between the two groups after emotional intelligence training (p < 0.001)., Conclusion: Overall, the findings of this study showed that emotional intelligence training led to improvement of happiness in patients with epilepsy. According to the results of the study, it is suggested that training based on emotional intelligence components be used as an approach to improve happiness level in patients with epilepsy., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS) with varied prevalence rates among populations with different ethnic backgrounds. Therefore, studies done on minorities have shed more light on the risk factors., Objective: Comparing MS prevalence in Georgian-based population immigrated to Iran and other Iranians., Methods: All records of MS patients enrolled in the two biggest registry systems were investigated. All of the patients born in Fereydunshahr and Buin va Miandasht (2 biggest cities with Georgian immigrants) were interviewed and their baseline characteristics were obtained. Patients' ethnic background information were obtained from the Iran National organization for civil registration., Results: Forty-one patients from Fereydunshahr and Buin va Miandasht were identified. The population of the two cities combined and the estimated number of Georgian-based patients in both cities were reported 59817 and 12000, respectively. The estimated ethnicity-adjusted prevalence among the Georgian-based individuals was 2.3 times higher than the non-Georgian ones. Baseline characteristics were also compared., Conclusion: There was a higher prevalence of multiple sclerosis among the Georgian minority of Isfahan. Due to the ethnic background of the Georgian minority, genetic risk factors should be considered more as a risk factor., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Introduction: Epilepsy is a common neurological disorder, and the patients with epilepsy are heavily influenced by the psychological and social aspects of the illness. Therefore, patients try to use coping strategies to control their stress and tension in this situation. Given the importance of the issue of adaptation and coping with stress in patients with epilepsy, as well as the different factors affecting coping strategies in these patients, the present study aimed to investigate the relationship between coping strategies and emotional intelligence in patients with epilepsy., Methods: This descriptive-analytic study conducted on 134 male and female patients with epilepsy referred to the Epilepsy Society of Isfahan, Iran. The consecutive sampling method was applied in this study. The data collection tool included a three-section questionnaire: the Demographic information, the Coping Inventory for Stressful Situations (CISS), and the Bar-On Emotional Quotient Inventory (EQ-i)., Results: The emotion-focused coping strategy was mostly used by 53.7% of the samples. The mean and standard deviation of the total score of emotional intelligence was 285.6 ± 39.5. Moreover, Pearson correlation test showed a significant difference between emotional intelligence variables and coping strategies (p < 0.001)., Conclusion: According to the relationship between emotional intelligence and coping strategies, it is suggested to consider ways to improve the emotional intelligence of patients with epilepsy in order to use more adaptive coping strategies., (Copyright © 2019 Elsevier Inc. All rights reserved.)

This study was designed to evaluate ALS progression among different subgroups of Iranian patients. Three hundred and fifty-eight patients from centres around the country were registered and their progression rate was evaluated using several scores including Manual Muscle Test scoring (MMT) and the revised ALS Functional Rating Scale (ALSFRS-R). Progression rate was analysed separately in subgroups regarding gender, onset site, stage of disease and riluzole consumption. A significant difference in MMT deterioration rate (p = 0.01) was noted between those who used riluzole and those who did not. No significant difference was observed in progression rates between male/female and bulbar-onset/limb-onset groups using riluzole. In conclusion, riluzole has a significant effect on muscle force deterioration rate but not functional scale. Progression rate was not influenced by site of onset or gender.

Background: Multiple Sclerosis (MS) is a chronic demyelinating disease of the central nervous system and its epidemiology has changed in recent years. Previously, Iran was considered to have a low MS prevalence. In this study, we aimed to update the prevalence and incidence of MS in Iran., Patients and Methods: This study was conducted based on the data derived from the new report of Iran's Ministry Of Health and Medical Education (MOHME) regarding the patients who have registered to obtain support for treatment in Iran till the end of 2013., Results: Among the 42,200 registered patients, 32,477 were female and 9,723 were male (sex ratio: 3.34: 1), and 4,545 were diagnosed in 2013. This resulted in a prevalence of 54.51 and an incidence of 5.87 per 100,000., Conclusion: According to our study results, Iran has a medium-to-high prevalence rate of MS. According to the previous epidemiologic studies from Iran, it seems that the prevalence of MS significantly has increased during recent years. This rate is similar to what is seen in many western countries., (© 2014 S. Karger AG, Basel.)

No large population-based survey has until now been carried out on the linkage of epilepsy (EP) and multiple sclerosis (MS) either in the middle-east or in Asia. In this retrospective population-based study (April 2003 to July 2010) on the total Isfahan cohort of 3,522 Iranian MS patients, we looked for EP/MS patients and compared their features with 1,665 non-EP/MS cases. We identified 105 cases, though, 24 were excluded for an implausible EP/MS link. Among 81 (2.3 %) eligible cases, EP occurred (1) within a mean duration of 5.6 ± 5.4 years after the development of MS in 64 cases (79 %); (2) at MS onset as the presenting symptom in five cases (6.2 %); and, (3) by a mean duration of 4.3 ± 4.3 years prior to onset of MS in 12 patients (14.8 %). The overall mean ages at MS onset and at the first seizure episode were 28.5 ± 11.2 years and 32.5 ± 14.0 years, respectively. Regarding the age at onset of MS, 12.3 % of EP/MS patients were classified as early-onset; 81.5 % as adult-onset; and 6.2 % as late-onset. Such frequencies were statistically different (P < 0.0001) from those of the 1,665 control non-EP/MS patients (5.9; 93.0 and 1.1 %, respectively). Regarding the pattern of MS, EP/MS patients were classified as relapsing-remitting, secondary progressive, and primary progressive in 60.5, 25.9, and 13.6 %, respectively. This configuration differed (P < 0.0001) from that of non-EP patients (87.9; 6.3 and 5.7 %, respectively). Our results are suggestive of differences between EP/MS and non-EP/MS cases as regards the proportion of MS patterns and age-at-onset classifications.

The aims of this study were to suggest the rate of primary progressive (PP) subtype of pediatric onset multiple sclerosis (MS) in Isfahan, Iran, and describe its clinical and paraclinical features. The data of patients were retrieved from Isfahan MS Society (IMSS) database from April 2003 to August 2011. Among 3,843 MS patients of Isfahan who have been registered in IMSS, 260 patients had onset symptom when younger than the age of 18 years, of whom, 11 patients had a PP course (4.23%). The mean age at onset in pediatric primary progressive multiple sclerosis (PPMS) was 16 years (range: 13 to 17) with female preponderance (2.66:1) and disease duration of 4.73 ± 3.03 years. Ataxia was the most frequent initial symptom (7/11). Additionally, the mean Expanded Disability Status Scale and progression index was 4.31 ± 0.60 and 1.50 ± 1.21, respectively. Cerebrospinal fluid analysis showed oligoclonal immunoglobulin G bands in seven patients. Magnetic resonance imaging (MRI) demonstrated periventricular lesions in all 11 patients and spinal lesions in 9 patients. Exposure to parental smoking was recorded in seven individuals. In conclusion, PPMS is an uncommon subtype of pediatric onset MS. Cerebral lesions are more common MRI findings in pediatric PPMS patients than that in adults. The course of PPMS seems to be more progressive in the pediatric population than in adults., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: Necrotizing (malignant) external otitis (NEO) is a life-threatening progressive infection of the external auditory canal, mastoid, and skull base. Here, we review the clinical findings for a series of NEO patients., Methodology: This case review study includes 18 patients with NEO (males: 11 (61.11%), females: 7 (38.88%), with a mean age of 65.11 years (range: 40-79)), hospitalized at Al-Zahra hospital in Isfahan province, Iran, during 2007-2011., Results: The most common presentations were otalgia 18 (100%), otorrhea 15 (83.33%), edema and erythema of the external ear canal 14 (77.77%), and hearing loss 11 (61.11%). We found osteomyelitis of the skull base that involved cranial nerves (most commonly the facial nerve) in 8 patients (44.44%), which seemed to be a higher incidence than reported in previous studies. Pseudomonas aeruginosa was found in ear specimen cultures in only 7 cases (38.88%), which could be attributed to inappropriate topical and oral use of antipseudomonal agents before proper diagnosis. Nine patients underwent surgical debridement. All cases responded to systemic antipseudomonal antibiotics with no mortality. However, recurrence occurred during the follow-up period in two cases; yet, the patients responded to retreatment., Conclusion: The life expectancy is increasing for diabetic and immunocompromised patients; thus, it is necessary to identify those who have a high risk for NEO, especially older diabetic patients who complain of otalgia and otorrhea that are resistant to routine treatment. Immediate diagnosis and proper treatment is crucial to prevent complications and mortality.

Background: Multiple sclerosis (MS) typically affects young adults; however, the first symptoms can occur after age 50 and is classified as late-onset MS (LOMS). , Methods: In this retrospective study, we extracted the records of 3522 MS patients (2716 females and 806 males) registered in the Isfahan MS Society (IMSS) from 2003 to 2010. Next, we searched for LOMS cases. We aimed to compare these cases with 1698 non-LOMS subjects also extracted from the IMSS database., Results: We found 48 LOMS patients (28 females and 20 males), which gave a crude frequency of 1.36%. The frequency by sex of LOMS in males (2.4%) was significantly greater than in females (1.0%, P = 0.002). The mean age at onset was 55.1 ± 4.3 years. The female to male ratio of 1.4:1 in these patients was significantly lower than in non-LOMS subjects (3.37:1, P = 0.003). The leading pattern of MS was relapsing-remitting (RR) in 62.5%, followed by primary progressive (PP) in 27.1%, and secondary progressive (SP) in 10.4%. Predominant presenting symptoms and signs were motor disturbances (35.4%), followed by brainstem (25%), optic neuritis (22.9%), and sensory related deficits (18.7%). The mean progression index (PI) in LOMS patients (0.88 ± 0.48) was significantly higher than in non-LOMS cases, 0.37 ± 0.17 (P < 0.0001)., Discussion: In comparing LOMS patients with the non-LOMS cohort, there was a higher frequency of the PP pattern and a higher PI in the LOMS group. In comparing other high-risk populations with the Isfahan cohort, LOMS formed a lower percentage of the total Isfahan MS population.

Early-onset multiple sclerosis (EOMS) is defined as the first presentation of symptoms in childhood (before the age of 16 years). EOMS occurs in about 0.4% to 10.5% of multiple sclerosis (MS) patients. In this retrospective population-based study we aimed to describe the clinical/paraclinical details and frequency of epileptic seizures in Iranian EOMS patients registered with the Isfahan Multiple Sclerosis Society (IMSS) from April 2003 to July 2010. EOMS cases were extracted from the Isfahan total MS cohort and included 3522 patients. A total of 117 EOMS patients (19 males and 98 females) with a mean age at onset of 14.2 ± 2.0 years (range: 7-16 years) were extracted from our database (3.3% of the total cohort). Of cases, ten (one male and nine females) had experienced at least two epileptic seizures, providing a crude prevalence of 8.5%. The frequency of epilepsy in EOMS patients (8.5%, 10/117) was significantly greater (P < 0.001) than that of non-EOMS cohort (2.0%, 71/3405). Epileptic seizures occurred before MS onset in two patients, after MS onset in seven, and at MS onset in one as the presenting symptom of the disease. Our findings mostly indicate an excessive prevalence of epileptic seizures in Iranian EOMS patients (8.5%), which is higher than any other report concerning seizures or epileptic seizures in a large MS series.

To date, there are no reports studying the rate of amyotrophic lateral sclerosis (ALS) in relatives of multiple sclerosis (MS) patients and vice versa. This study was designed to look into this issue using two population-based databases of MS and ALS in Isfahan province of Iran. We have searched for any first, second or third degree familial kinship between the Isfahan MS Society database and Isfahan ALS population. We compared the rate of ALS among the population of first degree relatives of MS patients, with the crude prevalence of ALS in the general population of Isfahan. On the other hand, a reverse analysis was carried out to compare the prevalence of MS in Isfahan with its rate amongst the first degree relatives of ALS patients. We found 10 families among which five had first degree kinship. The rate of the diseases was significantly higher in both comparisons among the family members (p < 0.00001) and an odds ratios of more than 67 in both calculations showed a several-fold increase of ALS occurrence in the first degree relatives of MS patients and vice versa. In our study relatives of MS patients were significantly more prone to ALS and vice versa. This could give clues about the common features that the two disease share. Both diseases have an environmental and genetic component and these results mostly point toward genetic similarities.

Exposure to heavy metals has been associated to a higher incidence of multiple sclerosis. In this work, we present a possible relationship between serum mercury levels and development of multiple sclerosis in Isfahan, the third largest city in Iran. Seventy-four patients affected by multiple sclerosis were retrieved from multiple sclerosis (MS) clinic in Isfahan, Iran. By matching sex and age, 74 healthy volunteers were chosen as control group. Blood samples were collected and serum mercury content was determined. Serum mercury level in MS patients was significantly higher than controls (9.6 ± 10.17 vs. 5.7 ± 8.6, P = 0.037). Concerning all MS patients, serum mercury value was significantly higher than the mercury concentration founded in control subjects {odd ratio: 2.39 (CI, 1.96-2.94), P = 0.00}. Serum mercury level is higher in MS patients with odd ratio equal to 2.39 compared with healthy individuals. It may reveal that high mercury levels in serum might help MS development in susceptible individuals. More studies with larger sample size are needed to confirm this hypothesis.

Background: Virchow-Robin spaces (VRSs) are perivascular pia-lined extensions of the subarachnoid space around the arteries and veins as they enter the brain parenchyma. These spaces are responsible for inflammatory processes within the brain., Objectives: This study was designed to shed more light on the location, size and shape of VRSs on 3mm slice thickness, 1.5 Tesla MRI scans of newly diagnosed MS patients in Isfahan, Iran and compare the results with healthy age- and sex-matched controls., Methods: We evaluated MRI scans of 73 MS patients obtained within 3 months of MS onset and compared them with MRI scans from 73 age- and sex-matched healthy volunteers. Three mm section proton density, T2W and FLAIR MR images were obtained for all subjects. The location, size and shape of VRSs were compared between the two groups., Results: The total number of VRSs was significantly more in the MS group (p<0.001). The distribution of VRSs were significantly more located in the high convexity areas in the MS group (p<0.001), while there was no significant differences in other regions. The round shaped VRSs were significantly more detected on MRI scans of MS patients, and curvilinear shapes were significantly more frequently observed in healthy volunteers, however there were no significant differences for oval shaped VRSs between the two groups. The number of VRSs with the size over than 2mm were significantly more observed in the MS groups compared to controls. We also observed some differences in the characteristics of VRSs between the genders in the MS group., Conclusion: The results of this study shed more light on the usefulness of VRSs as an MRI marker for the disease. In addition, according to our results VRSs might also have implication to determine the prognosis of the disease. However, larger studies with more advanced MRI techniques are required to confirm our results., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Background: The epidemiology of multiple sclerosis (MS) has changed in recent decades., Objectives: This study aimed to give an update on the prevalence and incidence of MS in Isfahan, Iran., Methods: The study population was all residents of Isfahan province during the period from April 2003 to July 2010. In April 2003, a registry of MS patients was created at the Isfahan MS Society (IMSS), which is the only referral center for MS patients in the province. Nearly all MS patients in Isfahan province are now registered with IMSS and were included in the analysis., Results: Among the 3522 registered patients, 2716 were female and 806 were male (sex ratio: 3.37 : 1), and 431 were diagnosed in 2009. This results in a prevalence figure of 73.3 (95% CI: 70.9-75.8) and an incidence of 9.1 (95% CI: 8.3-10.0) per 100,000., Conclusion: The reported prevalence and incidence figures in our study were higher than in our previous report of 2007, in which the prevalence and incidence of MS were reported to be 43.8 and 3.64 per 100,000, respectively. This dramatic increase in the prevalence of MS puts Isfahan amongst the regions with the highest prevalence of MS in Asia and Oceania and is mostly due to changing environmental factors, amongst which vitamin D deficiency seems an important factor in our population.

Aims: We aimed to study the smoking habits of multiple sclerosis (MS) patients and their healthy siblings in Isfahan province in Iran., Methods: MS patients registered with the Isfahan MS society database were compared to their healthy siblings who served as controls. Data regarding the smoking habits of patients and their siblings were collected using a specially designed questionnaire. Conditional logistic regression was adopted to analyze the association of smoking with the risk of MS, adjusting for age and sex., Results: There were 1,606 participants with 516 cases and 1,090 controls. After adjustments for age and sex, subjects who were ever-smokers had a significant risk of developing MS [odds ratio (OR) 2.67; 95% confidence interval (CI) 1.70-4.21; p <0.001]. Past smokers (OR 8.83; 95% CI 3.98-19.60; p < 0.001) and current smokers (OR 1.84; 95% CI 1.10-3.10; p = 0.021) had a significant risk for developing MS. Disease progression (current expanded disability status scale/disease duration) was not different between smokers and nonsmokers (0.54 ± 0.42 vs. 0.49 ± 0.48; p = 0.61)., Conclusion: Using a sibling pair method our data confirm the association between smoking and MS. A degree of confounding due to overmatching between siblings was unavoidable, but any bias would be conservative and should have lessened the effect of smoking., (Copyright © 2011 S. Karger AG, Basel.)

Nitric oxide is a signaling molecule that has various roles under normal conditions, notably in relation to vasodilation, neuronal function, and immune responses. Additionally, it has been implicated in some pathological states including multiple sclerosis. Multiple sclerosis is characterized by chronic inflammation and oligodendrocyte and axonal damage and ultimately death of neurons. The precise role of nitric oxide in pathogenesis is confounded by its opposing beneficial and deleterious effects. With respect to multiple sclerosis, increased nitric oxide metabolites have been well documented in the cerebrospinal fluid of patients. Reports on changes in the concentration of nitric oxide in the serum of patients have been inconsistent. Here, we report statistically significant increases in the concentrations of nitric oxide metabolites in the serum of multiple sclerosis patients not under medications from two subpopulations in Iran, supporting contentions that disease status does correlate with nitric oxide levels in this easily accessible body fluid. However, the tightness of the correlation appears to be insufficient to allow it to be used as an independent surrogate for assessment of disease status., (Copyright 2010 Elsevier B.V. All rights reserved.)

Swallowing disorders are commonly observed in multiple sclerosis (MS) patients. The complications of dysphagia are common causes of morbidity and death in late stages of MS. However, dysphagia in MS usually receives limited attention. The purpose of this study was to determine the prevalence of different kinds of swallowing disorders in MS patients with mild to moderate disability; and to identify possible associations between clinical and demographic features of patients and the presence of dysphagia. The swallowing functions of 101 consecutive MS patients were screened by the Northwestern Dysphagia Patient Check Sheet. This is a screening test which identifies patients with pharyngeal stage disorders, aspiration, oral stage disorders and/or pharyngeal delay. 'Dysphagia' was defined as having at least one of the above mentioned four disorders. Among 101 MS patients, 32 (31.7%) were classified as having dysphagia. Pharyngeal stage disorders were the most common observed impairment (28.7%) and aspiration, oral stage disorders, and pharyngeal delay were observed in 6.9%, 5%, and 1% of patients, respectively. Dysphagic patients had a significantly longer disease duration (p = 0.031) and more neurological impairment in cerebellar functional system (p = 0.04) when compared with non-dysphagic patients. Moreover, dysphagia was significantly more prevalent in patients with more neurological disability as measured by Expanded Disability Status Scale (EDSS) scores (p = 0.04). These results emphasize the importance of assessment and management of swallowing function in MS patients, particularly in patients with a high EDSS score; more severe cerebellar dysfunction, and long disease duration.

This study was designed to determine the frequency and clinical characteristics of myasthenia gravis (MG) in a large cohort of Persian patients with multiple sclerosis (MS) living in the province of Isfahan. We reviewed the case records of patients with definite MS (McDonald's criteria) registered in the Isfahan MS Society (IMSS) for associated MG. Of the 1,718 patients with MS in the registry, six patients were found to have both MS and MG. The prevalence after excluding one patient with insufficient data, was 291 per 100,000 (0.29%), a higher prevalence than the earlier reports. These results may support the hypothesis that MS and MG share some common immunopathogenic mechanisms.

Introduction: Co-occurrence of multiple sclerosis (MS) and neurofibromatosis type 1 (NF1) is rare., Case Reports: In this study, we describe the clinical and neuroimaging features of seven patients with NF1 and MS. In our patients, six patients with MS were women, in all of them history of NF1 existed. Three of our patients had primary progressive, one had secondary progressive MS, and three relapsing-remitting MS. Optic neuritis as presenting symptoms was seen in three patients, and motor manifestation as presenting symptom was observed in three patients. The risk of having both NF1 and MS seemed to be higher than would be expected based on the prevalence rates of the two diseases in the general population., Conclusion: The findings of this study suggest a possible casual relationship between MS and NF1, indicating higher risk of MS among patients with NF1.

Background: A variety of neurological disorders have been reported in association with celiac disease (CD) including epilepsy, ataxia, neuropathy and multifocal leucoencephalopathy. The purpose of this study was to assess the prevalence of CD among epileptic patients., Methods: Our study population consisted of 108 consecutive unexplained epileptic patients from Epilepsy Clinics. Patients who were able to give informed consent were invited to undergo screening for CD in a gastroenterology clinic. The diagnosis of CD was determined by IgA anti-tissue transglutaminase (t-TG) antibodies and by small intestine biopsy. Histopathologic changes were interpreted according to the Marsh classification., Results: A total of 108 consecutive epileptic patients (72 females, 36 males) ranging from 2-64 years (mean: 23.44, SD: 12.1) were studied. Positive IgA anti t-TG were detected in 4 of 108 epileptic patients (3.7%), while the known prevalence of CD in the study area was 0.6%.The intestinal biopsy confirmed the diagnosis of CD in three patients and was interpreted as Marsh I. In the other patient, small intestinal biopsy indicated only slightly increased number of intraepithelial lymphocytes. There was a significant difference between patients with CD and without CD for two symptoms: diarrhea and aphtous lesions (p<0.05)., Conclusion: Prevalence of CD was increased among patients with epilepsy of unknown etiology. It is important to investigate CD in any patient with idiopathic epilepsy even in the absence of digestive symptoms.

Background: Television as an external stimulation can precipitate epileptic seizures. Today this kind of epilepsy is known as television epilepsy. As children spend much of their time watching television, it is important to study this type of epilepsy in this age group. This study was designed to describe the clinical and some demographic characteristics of television epilepsy in Iranian children., Methods: Patients who were diagnosed as having television epilepsy with an age less than 12 years were recruited from outpatient neurology clinics in Isfahan, Iran, from September 2002 through September 2006. We collected the case-related information including electroencephalograms, radiologic findings, and patients' history., Results: Thirty patients with television epilepsy with the age less than 12 years were identified. Of whom 17 (56.7%) were females and 13 (43.3%) were males. The mean age at the onset of seizure was 9.9+/-2.1 years. Children had absence (3.3%), myoclonic (3.3%), and generalized tonic-clonic (93.3+/-) seizures in response to intermittent photic stimulations. Interictal epileptiform discharges in electroencephalograms were detected in 83.3%. In addition, neuroimaging findings were normal in 96.7% of the patients. In our study, 56.7% of the children had pure television epilepsy and 43.3% experienced other types of generalized seizure. During the follow-up period after initiation of variable drug treatments including valproic acid, carbamazepine, phenobarbital, clonazepam, ethosuximide, and lamotrigine all the patients had complete seizure remission., Conclusion: The clinical and demographic differences of our patients compared with other reports are probably due to genetic differences. In our study, it was demonstrated that carbamazepine could be used in children with television epilepsy because it had successfully terminated seizures in 43.3% of the patients.

Background: Although multiple sclerosis is considered as an inflammatory demyelinating disease of young adults, nearly 3% of patients manifest it under the age of 16 years. The aim of this study was to highlight the clinical and demographic features of early-onset multiple sclerosis in Isfahan, Iran., Methods: This prospective study concerned multiple sclerosis patients in whom the disease started before the age of 16 years and who were referred to the only clinic of multiple sclerosis in Isfahan from October 1997 through February 2003. All early-onset multiple sclerosis patients underwent magnetic resonance imaging. Magnetic resonance imaging findings were analyzed according to the Barkhof's criteria. All early-onset multiple sclerosis patients were followed for a mean period of 4.7 years., Results: Among 1,238 multiple sclerosis patients, 82 early-onset multiple sclerosis patients were identified. The female to male ratio was 4.5:1. The mean age of onset was 14.1 (range: 5 to 16) years. In 53 (65%) patients, the onset was monosymptomatic; in the remaining 29 (35%), it was polysymptomatic. Sixty-four (78%) patients presented a relapsing-remitting course, 14 (17%) had a secondary-progressive and 4 (5%) had a primary-progressive course. At the last evaluation, the expanded disability status scale score was > or =6 in only 8 (10%) patients. According to Barkhof's criteria, the characteristic magnetic resonance imaging findings were observed in 80 (98%) of early-onset multiple sclerosis patients., Conclusion: In our study, a high rate of childhood multiple sclerosis was observed that may be because of geographical or ethnic differences. Our study also showed that Barkhof's criteria, which is mostly used in adult patients, could be also applied to early-onset multiple sclerosis cases.

Background: This survey was planned to study the prevalence and incidence of multiple sclerosis (MS) in Isfahan, Iran and to describe the clinical features of MS in general and in specific subgroups of patients (early-onset, late-onset, familial, and conjugal cases) and to compare our results with other reports., Methods: A cross-sectional study was conducted from April 5, 2003 to July 31, 2006. All patients known to have definite MS according to McDonald's criteria, alive, resident within Isfahan (a large province of Iran) and members of Isfahan MS Society (IMSS) were included in the study. Demographic and case-related information were recorded. A total number of 1718 definite MS patients (388 men and 1330 women) were identified from IMSS database., Results: The overall period prevalence of MS was 43.8/100,000. Among men the prevalence was 19.2 (95% CI: 17.4-21.2)/100,000 and among women 69.6 (95% CI: 66-73.4)/100,000. A female preponderance of 3.4 existed among these patients. In the year 2005, 143 new cases were diagnosed, resulting in an incidence rate of 3.64/100,000. The mean age of onset was 25.36 +/- 8.6 years (range 5-63 year), and mean duration of disease was 7.1 (+/-5.2) years for men and 6.7 (+/-5) years for women. Sensory and visual disturbances were the most common initial presentations with a prevalence of 51.7% and 47.5%, respectively. Cases identified include: early-onset MS (less than 15 years old at onset) with 87 cases (5%), late-onset MS (over 50 years old at onset) with 20 cases (1.1%), familial MS with 209 cases (12.2%), and conjugal MS with 6 cases (0.5%)., Conclusion: Isfahan is a medium- to high-risk area for MS, with prevalence higher than what has previously been reported, possibly because of an increase in the incidence rate. Clinical and demographic characteristics were similar to other reports; however, some differences existed.

Background: Multiple sclerosis is a leading cause of disability in young adults. Mitoxantrone has recently been shown to be effective in ameliorating multiple sclerosis activity and reducing the relapse rate. This study aimed to assess the efficacy of mitoxantrone on disease activity and decreasing relapse rate in patients with multiple sclerosis in Iran., Methods: This was a clinical trial on patients who received intravenous mitoxantrone, 12 mg/m2 every 3 months. The study was performed at Isfahan Multiple Sclerosis Clinics, affiliated to Isfahan University of Medical Sciences. This clinical trial was conducted from October 2003 through April 2005. One hundred and forty-seven patients with worsening relapsing-remitting and secondary progressive multiple sclerosis received mitoxantrone, 12 mg/m(2) every 3 months. Clinical assessment was made every 3 months for one year., Results: Of the 147 patients, 129 (93 females and 36 males) could successfully complete the course of our study. A significant therapeutic effect (P < 0.0001) was detected for the attack rate before and after treatment. The Mean attack rate 12 months before treatment was 1.10 (SD = 0.95), which reduced to 0.09 (SD = 0.29) during treatment. The Mean expanded disability status scale at the beginning of the treatment was 4.32, which declined to 3.62 (P < 0.0001) after one year., Conclusion: Mitoxantrone was generally well tolerated and reduced progression of disability and clinical exacerbation in our patients. Physicians must be careful about the complications of mitoxantrone especially cardiotoxicity.

Background: The prevalence of multiple sclerosis (MS) shows considerable variability all over the world. According to Kurtzke, Iran is considered to have a low prevalence., Objective: To estimate the period prevalence and risk factors of MS in Isfahan, central part of Iran., Methods: A cross-sectional case register study conducted between 2004 and 2005. In the province of Isfahan, Iran, all patients known to have definite MS during 2004 and 2005, being alive and resident within Isfahan as well as being a member of the Isfahan MS Association were included in the study. Demographic and case-related information was recorded. 1,391 definite MS patients (308 men and 1,083 women) from the Isfahan MS Association, Iran, have been identified. The disease was confirmed using clinical information and MRI findings by a neurologist and radiologist. The patients were evaluated by interview and a questionnaire. Population data were obtained from the year 1999 Iran Census. The mean (SD) age of the participants was 32.5 (9.3) years with a mean (SD) duration of the disease of 6.4 (5.1) years for men and 6.9 (5.3) years for women., Results: The period prevalence of MS was 35.5 per 100,000 [95% confidence interval (CI) 33.6-37.3] in a population of 3,923,255, with a higher rate in women than men [54.5 (95% CI: 51.1-57.8) for women and 14.9 (95% CI: 13.3-16.6) for men]. The female/male ratio was 3.6 (95% CI: 3.2-4.1). The direct age-adjusted period prevalence was 59.5 per 100,000 (95% CI: 44.8-75.2) for women and 17.0 per 100,000 (95% CI: 8.9-25.1) for men. MS rates were highest among 30- to 39-year-olds and decreased with increasing age. Sensory and visual disturbances were the most common initial presentations with a prevalence of 51.1% (95% CI: 48.4-53.7) and 47.0% (95% CI: 44.4-49.7), respectively., Conclusion: Isfahan could be considered as an area with a medium to high risk of MS. This is in sharp contrast with the gradient hypothesis.