Refractory Convulsive Status Epilepticus Provoked by Intoxication with Dalfampridine in a Patient with Multiple Sclerosis and Depression Disorder: A Case Report and Literature Review.

PURPOSE: Dalfampridine (DFP) is used to improve motor functions in patients with multiple sclerosis (MS). Overdose of DFP can occur for a variety of reasons and can lead to a state of epilepsy. CASE REPORT: A 24-year-old woman with MS was admitted to hospital with severe sweating and delirium after attempting suicide by overdosing on DFP. At the time of hospitalization, she developed a tonic-clonic seizure that did not respond to immediate intravenous (IV) diazepam injection, followed by intravenous sodium valproate. Therefore, according to the hospital protocol of the neurology department, the patient was intubated and IV infusion of midazolam was started, Due to the persistence of seizures, sodium thiopental began and the patient was admitted to the intensive care unit (ICU). In the ICU, she received an infusion of sodium thiopental and intravenous sodium valproate, monitored by a daily electroencephalogram (EEG). The patient was discharged after four days due to her stable medical condition. CONCLUSION: Epilepsy in case of overuse of DFP should be considered as a life-threatening side effect and timely treatment should be done to prevent damage to the nervous system.

Comparison between the efficacy of ginger and sumatriptan in the ablative treatment of the common migraine.

Frequency and torment caused by migraines direct patients toward a variety of remedies. Few studies to date have proposed ginger derivates for migraine relief. This study aims to evaluate the efficacy of ginger in the ablation of common migraine attack in comparison to sumatriptan therapy. In this double-blinded randomized clinical trial, 100 patients who had acute migraine without aura were randomly allocated to receive either ginger powder or sumatriptan. Time of headache onset, its severity, time interval from headache beginning to taking drug and patient self-estimation about response for five subsequent migraine attacks were recorded by patients. Patients(,) satisfaction from treatment efficacy and their willingness to continue it was also evaluated after 1 month following intervention. Two hours after using either drug, mean headaches severity decreased significantly. Efficacy of ginger powder and sumatriptan was similar. Clinical adverse effects of ginger powder were less than sumatriptan. Patients' satisfaction and willingness to continue did not differ. The effectiveness of ginger powder in the treatment of common migraine attacks is statistically comparable to sumatriptan. Ginger also poses a better side effect profile than sumatriptan.

Association of chronotype and timing of interferon injection with severity of IFNß-induced flu-like syndrome in Multiple sclerosis (MS) patients.

BACKGROUND: Although Interferon-beta (IFNß) has long been approved as a disease-modifying therapy (DMT) for Multiple sclerosis (MS), flu-like syndrome (FLS) persists as a common adverse effect of interferon therapy. Given the importance of circadian rhythm in regulating physiological processes, we aimed to assess the relationship between patient's chronotype and time of interferon injection with FLS score in MS patients receiving IFNß. METHODS: A cross-sectional study was conducted on 118 MS patients who were referred to the clinic of neurology of Zanjan Vali-e-Asr Hospital for interferon injection. The included were invited to complete a morningness-eveningness questionnaire (MEQ) assessing patients' chronotype. The following data were extracted from patients' record: age, gender, duration of interferon treatment, type of interferon taken, time of interferon injection (morning/evening), FLS score, MS subtype, and usage of pain killers. All data found were imported and statistically analyzed in SPSS ver.26. RESULTS: According to the patients' record, 114 (96.6%) patients had experienced post-interferon injection FLS with different severities. Statistical analysis revealed no significant relationship between the patient's chronotype and FLS score. Nevertheless, the FLS score was significantly higher in those who had evening injections. CONCLUSIONS: Time of interferon injection was significantly associated with FLS score, with higher FLS score following evening injection. However, no significant relationship was found between the FLS score and the patient's chronotype. It is recommended that further studies assessing circadian rhythm using laboratory tests such as melatonin measurement need to be undertaken to investigate the association of circadian rhythm with post-interferon injection FLS.

Effect of add-on melatonin on seizure outcomes and quality of sleep in epilepsy with idiopathic generalized tonic-clonic seizures alone in adult patients: Cross-sectional, randomized, double-blind, placebo-controlled clinical trial.

BACKGROUND: Effective treatment of epilepsy is a major challenge in the field of neurology. Studies have suggested that melatonin can work in epilepsy with a good safety profile. OBJECTIVES: This study was performed to determine the effectiveness of melatonin in seizure outcomes, as well as the quality of sleep in patients with generalized epilepsy. METHODS: In this cross-over clinical trial study, 60 patients with epilepsy with idiopathic generalized tonic-clonic seizures alone (EGTCS) and under valproic acid treatment received either melatonin or placebo with a washout period of 2 weeks intermittently. Outcome variables included a reduction in the severity and frequency of epilepsy besides improvement in electroencephalogram (EEG) abnormalities and sleep quality. RESULTS: By adding melatonin, a decrease in the mean severity score of epilepsy (according to the Chalfont questionnaire) was 32.33 ± 9.24, while it was 5.58 ± 14.28 in treatment with placebo (p = .002). Evaluation of the number of attacks and EEG results did not disclose any therapeutic efficacy in treatment with melatonin versus placebo. The quality of sleep improved in 40% (first round) and 53.4% (second round) of subjects who received melatonin (p < .001). CONCLUSIONS: Considering that the addition of melatonin to routine anti-seizure treatment was effective in reducing the severity of epilepsy and improving sleep quality, it seems that melatonin can be useful as an adjunct therapy for EGTCS in well-defined circumstances.

The prevalence of metabolic syndrome parameters and their association with headache characteristics among migraineurs.

Background: Migraine is associated with metabolic syndrome (MetS). There are evidences that components of MetS are more prevalent among migraine patients than non-migraineurs. Since both migraine and MetS are associated with a high risk of cardiovascular events, it is likely that the parameters of MetS increase the occurrence of cardiovascular disease (CVD) in migraineurs. The present research project was conducted for the purpose of investigating the relationship between MetS parameters and different items of migraine headaches. Methods: This descriptive-analytical, cross-sectional study was performed on 240 migraineurs [according to International Headache Society (HIS) II criteria] within the 17+ age range. The participants were selected via consecutive and convenience sampling method. The evaluated parameters for each subject included 2 arms: migraine characteristics (intensity, frequency of attacks, subtype, duration, and treatment regimen) and indices of MetS according to the National Cholesterol Education Program's Adult Treatment Panel III (NCEP ATP-III) report criteria [high-density lipoprotein-cholesterol ( HDL-C), triglyceride (TG), fasting plasma glucose (FPG), height, waist circumference (WC), systolic and diastolic blood pressure (BP), and body mass index (BMI)]. All data were analyzed in SPSS software. Results: Total prevalence of MetS was 16.25% (39 patients). There was a statistically meaningful relationship between hypertriglyceridemia and gender (P = 0.021), hypertriglyceridemia and prophylactic antimigraine regimen (P = 0.022), hyperglycemia and age group (P = 0.010), hyperglycemia and the intensity of headache (P = 0.048), hyperglycemia and prophylactic treatment (P = 0.001), systolic hypertension and migraine subtype (P = 0.004), systolic hypertension and the duration of migraine disease (P = 0.005), diastolic hypertension and migraine subtype (P = 0.002), WC and gender (P = 0.001), WC and the intensity of headache (P = 0.028), WC and prophylactic medication (P = 0.017), HDL and gender (P = 0.001), HDL and the prophylactic regimen (P = 0.023), and MetS and gender (P = 0.005). The prevalence of MetS was increased with increase in the severity of migraine headache. Conclusion: Due to the relative increase in the prevalence of MetS in patients with more severe migraine, an evaluation of the mechanisms of MetS is recommended in this population.

Is There Any Relation between Serum Levels of Interleukin-10 and Neurophysiological Abnormalities in Bell's Palsy?

Background: Bell's palsy is the most common cause of peripheral facial palsy. The etiology and treatment of Bell's palsy are still controversial. Previous studies emphasize the role of herpes simplex and herpes zoster viruses in this ailment. The role of Interleukin-10 (IL-10) in Bell's palsy is yet unknown, and few studies have shed light on the matter. This study intended to assess the prognostic value of IL-10 and its relation to the intensity of electrodiagnostic abnormalities and evaluate its potential use as a factor for judging the need for medical or surgical interventions. Materials and Methods: 30 patients in the acute phase of Bell's palsy participated in this study. Peripheral blood samples were obtained for IL-10 assessment within the first 72 hours (before commencing treatment), and a nerve conduction study (NCS) was performed six days after symptom onset. Results: There was no significant correlation between IL-10 serum levels and the severity of nerve conduction pathology in Orbicularis oculi and Orbicularis oris muscles. Also, IL-10 serum levels did not show any meaningful relationships with participants' age, gender, or symptoms. Conclusion: The IL-10 serum levels are not relevant to the pathology of Bell's palsy, and the assessment of IL-10 serum levels cannot be used as an alternative to NCS for evaluating the severity of acute Bell's palsy.

Clinicogenetical Variants of Progressive External Ophthalmoplegia - An Especial Review of Non-ophthalmic Manifestations.

Progressive external ophthalmoplegia (PEO) is a slowly progressive myopathy characterized by extraocular muscles involvement, leading to frozen eyes without diplopia. The pattern of inheritance may be mitochondrial, autosomal dominant or, rarely, autosomal recessive. Sporadic forms were also reported. Muscular involvement other than extraocular muscles may occur with varying degrees of weakness, but this mostly happens many years after the disease begins. There are also scattered data about systemic signs besides ophthalmoplegia. This article aims to review non-ophthalmic findings of PEO from a clinicogenetical point of view.

Prevalence of Multiple Sclerosis (MS) in Zanjan Province of Iran.

BACKGROUND: The prevalence of multiple sclerosis (MS) varies in different geographical regions and has dramatically increased in Iran. Revealing the high prevalence rate draws the attention of policymakers and helps them allocate necessary resources. The aim of this study is to determine the prevalence of MS in Zanjan province of Iran. METHODS: We included all registered residents of Zanjan province with MS on the prevalence day (July 31, 2019). All cases met the McDonald criteria. All registered cases in Zanjan MS society were identified as index cases. Data regarding patient's national code, gender, age, age at the first symptom onset, city of residence, marital status, education level, occupation, ethnicity, family history of MS and the time span between symptom's onset and disease diagnosis were recorded. RESULTS: We identified 758 patients, 551 of whom (72.7%) were female. The mean age at the first symptom onset was 28.9 ±8.7 years old. The crude prevalence was 71.6 per 100,000 population (95% CI 66.6-76.9). The disease was most prevalent in Zanjan city (100.5 per 100,000). The gender-specific prevalence per 100,000 population was 105.4 for women (95% CI: 96.8-114.6) and 38.7 for men (95% CI: 33.6-44.1), with female to male ratio of 2.6. The standardized mortality ratio (SMR) was calculated as expected/observed for both men and women as 2.3 (207/88.2) (551/234.1). CONCLUSIONS: Our data confirm that the MS prevalence rate is high in Zanjan province of Iran.

Whole Exome Sequencing Revealed a Novel GJB1 Pathogenic Variant and a Rare BSCL2 Mutation in Two Iranian Large Pedigrees with Multiple Affected Cases of Charcot-Marie-Tooth.

Charcot-Marie-Tooth disease (CMT) is the most common hereditary neuropathy of the peripheral nervous system with a wide range of severity and age of onset. CMT patients share similar phenotypes which make it often impossible to identify the disease types based on clinical presentation and electrophysiological studies alone. In recent years, novel genetic diagnostic approaches such as whole exome sequencing (WES) has provided a ground for accurate diagnosis of CMT through identification of the disease-causing mutation(s). In the present study, that approach was effectively employed. Two unrelated large pedigrees with multiple affected cases of various pattern of inheritance (one autosomal dominant and one X-linked) were included. Clinical and electrophysiological data were obtained. DNA sample from each pedigree's proband was subjected to WES. Data analysis was performed using an in-house developed pipeline, adopted from GATK and ANNOVAR. Candidate variant segregation was evaluated by PCR-based Sanger sequencing. A known but extremely rare (unreported in the Middle Easterners) mutation in BSCL2 (c.C269T:p.S90L) as well as a novel hemizygous variant in GJB1 (c.G224C:p.R75P) were identified and segregations were confirmed by Sanger sequencing. This study supports effectiveness of WES for genetic diagnosis of CMT in undiagnosed families.

Clinical evaluation of vasomotor system functionality in type 2 diabetic Patients.

BACKGROUND: Autonomic neuropathy and vital organ dysfunctions are the known complications in type 2 diabetes Mellitus (DM). Genetic endowments involving individuals make subtle differences in physiological systems, particularly at the time of sickness. Hence, the presented study was designed to evaluate the vasomotor system in healthy people and type 2 DM cases for determining any functionality differences between the mentioned groups. METHODS: Sixty patients with type 2 diabetes (case group) and sixty healthy subjects (control group) matched for age and sex were enrolled in the study. Then, the performance of vasomotor system was assessed using valsalva maneuver, cold pressor, sustained hand-grip and mental arithmetic tests and the differences were determined via statistical methods. RESULTS: According to our findings, abnormal response to valsalva maneuver was found in the case group (P=0.028) and the same response was seen about mental arithmetic evaluations. In the case of cold pressor and sustained hand-grip tests, remarkable differences were not found in both groups. Important differences were also found among vasomotor dysfunction and the time of DM labeling. CONCLUSION: This study showed a higher incidence of vasomotor dysfunction in DM patients. However, revision in cold pressor and sustained hand-grip tests definition as well as methodology was recommended.

Association between abnormal serum free light chains ratio and known prognostic factors in lymphoma; a nephrology viewpoint.

Introduction: The serum immunoglobulin free light chain (FLC) assay quantities of free kappa (κ) and lambda (λ) light chains. This assay has prognostic value in plasma cell proliferative disorders. There are limited data on serum FLC in B-cell malignancies. Objectives: The aim of this study was to compare the known prognostic factors in non-Hodgkin's lymphoma of the type B-cell and Hodgkin disease with an abnormal secretion amount of light chains in these lymphomas. Patients and Methods: The association of pretreatment FLC and FLC ratio (κ:λ) with previously known prognostic factors for lymphoma such as the international prognostic index (IPI) and B symptoms were evaluated in 50 patients with Hodgkin's and non-Hodgkin's lymphoma. IPI is a prognostic score given based on the clinical variables including age, disease stage, serum LDH and extra-nodal involvement. Elevated FLC and an abnormal κ:λ ratio was defined based on the previous publications. Results: The prevalence of abnormal FLC ratio was 38% in all patients and 40.9% in patients with diffuse large B-cell lymphoma. Abnormal FLC ratio was significantly associated with IPI (P=0.04) and B symptoms (P=0.02) in both groups of the patients with Hodgkin's and non-Hodgkin's lymphoma. The stage of the disease in Hodgkin's lymphoma patients showed a significant relationship with the abnormal FLC ratio (P=0.04). Presence of the B symptoms in patients with Hodgkin's lymphoma had a modest but not statistically significant association with the abnormal FLC ratio (P=0.07). Conclusion: Abnormal FLC ratio as a new potent prognostic biomarker has a significant association with IPI which is the most common clinical tool used to predict outcome in lymphoma patients. Since there is a need for developing a reliable and quantitative prognostic biomarker for lymphoma, evaluation of the independent effect of the abnormal serum FLC ratio is suggested to be considered in future prospective studies. The result of these studies will also be useful for nephrologists, while serum immunoglobulin FLC is capable to damage kidney.