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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.

Objectives: This is a critical review of anti-phospholipid antibodies (aPL). Most prior reviews focus on the aPL syndrome (APS), a thrombotic condition often marked by neurological disturbance. We bring to attention recent evidence that aPL may be equally relevant to non-thrombotic autoimmune conditions, notably, multiple sclerosis and ITP., Organization: After a brief history, the recent proliferation of aPL target antigens is reviewed. The implication is that many more exist. Theories of aPL in thrombosis are then reviewed, concluding that all have merit but that aPL may have more diverse pathological consequences than now recognized. Next, conflicting results are explained by methodological differences. The lupus anticoagulant (LA) is then discussed. LA is the best predictor of thrombosis, but why this is true is not settled. Finally, aPL in non-thrombotic disorders is reviewed., Conclusion: The current paradigm of aPL holds that they are important in thrombosis, but they may have much wider clinical significance, possibly of special interest in neurology.

Acute demyelinating disorders of the central nervous system represent some of the most formidable management and therapeutic challenges to clinicians. These disorders include acute relapses of multiple sclerosis, neuromyelitis optica, acute disseminated encephalomyelitis, optic neuritis, and transverse myelitis. This paper summarizes clinical manifestations of these disorders, as well as treatment strategies. Currently, immunosuppression with corticosteroids for the acute events is the mainstay of treatment. Other experimental therapies, which may be effective in certain patients, are discussed.

Fasting during Ramadan is mandatory for all healthy Muslim adults. During the fasting month, many physiological and biochemical changes occur that may be due to alterations in eating and sleeping patterns. A concern for Muslim multiple sclerosis (MS) patients is whether prolonged fasting might have an unfavorable impact on the course of their disease. The aim of this prospective study was to determine the effects of prolonged intermittent fasting on the course of MS in a cohort of patients who reside in Isfahan, Iran. The cohort consisted of 40 adult MS patients who fasted during Ramadan and 40 MS patients who did not fast. Only patients with mild disability (expanded disability status scale (EDSS) score 0.05). Fasting had no short-term unfavorable effects on the disease course in MS patients with mild disability. However, larger multi-center prospective studies of longer duration are needed to validate the results of this study., (Copyright 2009 S. Karger AG, Basel.)

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.

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.

We compared the relative efficacy of interferon beta (IFNbeta) products and azathioprine (AZA) in the treatment of relapsing- remitting multiple sclerosis (RRMS). Ninety-four previously untreated patients of short duration with RRMS were randomly allocated to the two treatment groups. The first group received IFNbeta products (Betaferon,Avonex or Rebif); the second group received AZA for 12 months. Response to treatment was assessed at 3, 6, and 12 months after starting therapy. The mean number of relapse during one year of the study was lower in the AZA group than in the IFNbeta products group (0.28 vs. 0.64, P < 0.05). After 12 months, 57.4% of patients receiving IFNbeta products remained relapse free compared with 76.6% of those given AZA. The Expanded Disability Status Scale (EDSS) decreased by 0.30 units in IFNbeta-treated patients (P < 0.05) and 0.46 in AZAtreated patients (P < 0.001). Treatment with IFNbeta products and AZA significantly reduces the relapse rate and EDSS score in patients with RRMS, while AZA is more effective than the IFNbeta formulations.

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.

Objective: To prospectively investigate the prevalence and clinical relevance of orthostatic hypotension (OH) in Parkinson`s disease (PD) in Isfahan, Iran., Methods: We investigated 150 consecutive patients with PD (42 women, 108 men) in Al-Zahra Hospital, Isfahan, Iran from January 2002 to January 2004. Blood pressure was measured first in a supine position following a rest of at least 10 minutes, and then after 3 minutes of active standing. Data concerning the age, gender, duration of disease, and drug consumption were recorded in a questionnaire., Results: Orthostatic hypotension in PD is more frequent in women, patients taking a higher dose of levodopa, in higher age groups, and patients with longer duration of the disease, however, a statistically significant difference was seen in the female group and patients taking a higher dose of levodopa., Conclusion: Orthostatic hypotension is mainly related to PD pathology and the clinical relevance of OH to gender, age, and disease duration may be due to the natural course for progression of human autonomic dysfunction during life. Higher doses of levodopa may increase the risk of OH.

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.

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 [corrected] existing symptoms were motor problems in all 92 (100%), cerebellar symptoms in 46 (50%), and cognitive impairment in only 6[corrected](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.

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.

Objectives: To compare the relative efficacies of Betaferon, Avonex, and Rebif in the treatment of relapsing-remitting multiple sclerosis (RRMS)., Methods: Ninety patients with RRMS were randomly allocated to the three treatment groups. The first group received Betaferon, the second group received Avonex, and the third group received Rebif for 24 months. Response to treatment was assessed at 6, 12, and 24 months after start of therapy., Results: Of the 30 patients treated with Betaferon, the mean (standard deviation, SD) of relapse rate decreased from 2.2 (0.7) to 0.7 (0.7) episodes. Correspondingly, in the 30 patients treated with Avonex, the mean (SD) of relapse rate decreased from 2.0 (1.2) to 1.2 (0.9) (P < 0.001). In the 30 patients treated with Rebif, the mean (SD) of relapse rate decreased from 2.4 (1.0) to 0.6 (0.9) (P < 0. 01). After 2 years, 43.3% of patients receiving Betaferon and 56.7% of patients receiving Rebif remained relapse-free compared with 20% of those given Avonex. Expanded Disability Status Scale (EDSS) decreased by 0.7 U in Betaferon-treated patients (P < 0.001), 0.3 U in Rebif-treated patients (P < 0.05), and remained stable in Avonex patients., Conclusion: Treatment with Betaferon, Avenox, and Rebif significantly reduce relapse rate and EDSS score in patients with RRMS.

Background: There are few treatment options for patients with secondary progressive and worsening relapsing-remitting multiple sclerosis. Mitoxantrone is an antineoplastic drug, recently approved for treatment of multiple sclerosis. Mitoxantrone is, however, associated with dose-related cardiotoxicity, which limits its use., Objective: To investigate the possible cardiotoxicity of mitoxantrone in multiple sclerosis., Methods: We studied 96 patients with worsening relapsing-remitting or secondary progressive multiple sclerosis, to evaluate cardiotoxicity within one year of mitoxantrone therapy. This study was performed in the Multiple Sclerosis Clinic of Isfahan University of Medical Sciences from October 2003 through October 2004. Analysis of mitoxantrone therapy (12 mg/m2), in terms of cardiac toxicity, was conducted on patients who received at least 4 doses. Cardiac assessment was carried out every 6 months with electrocardiogram, as well as a spectral and color-flow Doppler echocardiographic examination at the time of enrollment and 6 and 12 months later., Results: Ninety-six patients were assessed over 12 months. There was no evidence of clinically-significant cardiac dysfunction. Three patients had a left ventricular ejection fraction of <10% of the base-line value and three had <50%., Conclusion: Mitoxantrone (12 mg/m2) is effective and generally well tolerated by patients with worsening relapsing-remitting and secondary progressive multiple sclerosis. Our findings suggest that the risk for developing cardiotoxicity is low in patients with multiple sclerosis within one year of the treatment with mitoxantrone.

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.