Estimation of Ambulation and Survival in Neurodegeneration with Brain Iron Accumulation Disorders.

BACKGROUND: Neurodegeneration with Brain Iron Accumulation (NBIA) disorder is a group of ultra-orphan hereditary diseases with very limited data on its course. OBJECTIVES: To estimate the probability of preserving ambulatory ability and survival in NBIA. METHODS: In this study, the electronic records of the demographic data and clinical assessments of NBIA patients from 2012 to 2023 were reviewed. The objectives of the study and factors impacting them were investigated by Kaplan-Meier and Cox regression methods. RESULTS: One hundred and twenty-two genetically-confirmed NBIA patients consisting of nine subtypes were enrolled. Twenty-four and twenty-five cases were deceased and wheelchair-bound, with a mean disease duration of 11 ± 6.65 and 9.32 ± 5 years. The probability of preserving ambulation and survival was 42.9% in 9 years and 28.2% in 15 years for classical Pantothenate Kinase-Associated Neurodegeneration (PKAN, n = 18), 89.4% in 7 years and 84.7% in 9 years for atypical PKAN (n = 39), 23% in 18 years and 67.8% in 14 years for Mitochondrial Membrane Protein-Associated Neurodegeneration (MPAN, n = 23), 75% in 20 years and 36.5% in 33 years for Kufor Rakeb Syndrome (KRS, n = 17), respectively. The frequencies of rigidity, spasticity, and female gender were significantly higher in deceased cases compared to surviving patients. Spasticity was the only factor associated with death (P value = 0.03). CONCLUSIONS: KRS had the best survival with the most extended ambulation period. The classical PKAN and MPAN cases had similar progression patterns to loss of ambulation ability, while MPAN patients had a slower progression to death. Spasticity was revealed to be the most determining factor for death.

Anti-inflammatory-antioxidant modifications and synbiotics improved health-related conditions in patients with progressive forms of multiple sclerosis: A single-center, randomized clinical trial.

BACKGROUND AND PURPOSE: There is growing evidence that dietary modification can improve clinical manifestations in multiple sclerosis (MS) patients. This study aimed to assess the impact of synbiotics and anti-inflammatory-antioxidant-rich diet on fatigue, pain, gut and bladder status, and sexual function in patients with progressive forms of MS. MATERIALS AND METHODS: In this single-center, single-blind, randomized, controlled clinical trial, seventy participants with three forms of progressive MS (primary-progressive, secondary-progressive, and progressive-relapsing) were randomly assigned to receive either synbiotics supplement and anti-inflammatory-antioxidant-rich diet or a placebo along with their usual diet for a duration of four months. Modified fatigue impact scale (MFIS), global pain scale (GPS), bladder control scale (BLCS), bowel control scale (BWCS), and sexual satisfaction scale (SSS) were assessed at baseline and at the end of the trial. RESULTS: Sixty-nine participants successfully completed the trial, resulting in a 98% adherence rate to the diet, and no reports of serious side effects. Significant mean changes were observed in fatigue (Δ for experimental group = -10.5 ± 10.8 vs. Δ for control group = -0.08 ± 4.1; P < 0.001), pain (-14.1 ± 19.0 vs. 0.9 ± 10.3; P < 0.001), bladder (-0.76 ± 2.1 vs. 0.3 ± 1.1; P = 0.013) and bowel (-6.6 ± 3.2 vs. -0.05 ± 2.3; P < 0.001) control, as well as sexual function (-1.0 ± 2.3 vs. 0.51 ± 0.21; P < 0.001). CONCLUSION: The anti-inflammatory-antioxidant-rich diet and synbiotics co-supplementation demonstrated improvements in fatigue, pain, sexual function, and bowel/bladder status among patients with progressive MS.

New Daily Persistent Headache (NDPH): Unraveling the Complexities of Diagnosis, Pathophysiology, and Treatment.

PURPOSE OF REVIEW: The current article aims to provide an overview of new daily persistent headache (NDPH), with a particular emphasis on its pathophysiology, evaluation, and current treatment options. RECENT FINDINGS: NDPH is an uncommon and heterogeneous condition associated with various comorbidities and is of great significance due to its prolonged duration and high severity. Variable causes and clinical aspects of NDPH may reflect differences in its underlying pathophysiological mechanisms, including genetics, environmental triggers, neuroinflammation, and brain changes. When assessing a patient with NDPH, potential triggers, past medical history, and differential diagnosis should be carefully considered. Non-pharmacological interventions aimed to improve diet, sleep patterns, and reduce consumption of caffeine and alcohol are recommended for all patients. Nerve blockade and nerve stimulation seem to be more efficacious in children than adults. Antiviral medications and neuroinflammation-targeting treatments may be helpful, particularly, when an infectious disease or severe inflammation is suspected. NDPH patients with concurrent affective disorders may benefit from treatment with serotonin reuptake inhibitors, serotonin/norepinephrine reuptake inhibitors, or benzodiazepines. Cerebrospinal-fluid-lowering medications may be useful for headaches started with a thunderclap or a Valsalva maneuver. Possible treatments for refractory NDPH include intravenous ketamine or lidocaine, onabotulinumtoxinA, and calcitonin gene-related peptide antibodies. Considering the variety of NDPH, it is critical to properly screen patients for correct diagnosis. Proper identification of potential mimics may enable precise therapy opportunities, yet there is no gold standard treatment for NDPH. Further well-designed studies are needed to elucidate the underlying mechanisms and develop effective treatment strategies for NDPH.

Third COVID-19 vaccine dose for people with multiple sclerosis who did not seroconvert following two doses of BBIBP-CorV (Sinopharm) inactivated vaccine: A pilot study on safety and immunogenicity.

Background: People with multiple sclerosis (pwMS) on anti-CD20 therapies (aCD20) and fingolimod have shown inadequate humoral responses to COVID-19 vaccines. Objective: The objective of the study was to pilot larger studies by demonstrating the safety and comparing the immunogenicity of different types of third doses in seronegative pwMS after two doses of BBIBP-CorV inactivated vaccine. Methods: In December 2021, subject to receiving their third dose, being COVID-19-naiive, and receiving no corticosteroid within two months, we measured the level of anti-SARS-CoV-2-Spike IgG in pwMS seronegative after two shots of BBIBP-CorV inactivated vaccine. Results: We included 20/29 pwMS who received adenoviral vector (AV), 7/29 who received inactivated, and 2/29 who received conjugated third doses. No serious adverse events were reported two weeks post-third dose. The pwMS receiving AV third doses showed significantly increased IgG concentrations, while only the ones not on aCD20 and fingolimod responded to inactivated third doses. An ordinal logistic multivariable generalized linear model indicated that age (per year ß: -0.10, P = 0.04), type of disease-modifying therapy (aCD20 ß: -8.36, P <0.01; fingolimod ß: -8.63, P = 0.01; others: reference), and type of third dose (AV or conjugated ß: 2.36, P = 0.02; inactivated: reference) are predictive of third dose immunogenicity among pwMS who remain seronegative after two shots of BBIBP-CorV vaccine. Statistical significance was not achieved for variables sex, MS duration, EDSS, duration of DMT, duration of third dose to IgG test, and duration from last aCD20 infusion to third dose. Conclusion: This preliminary pilot study highlights the need for further research to determine the optimal COVID-19 third dose vaccination strategy for pwMS living in areas where BBIBP-CorV vaccine has been used.

Effects of anti-Inflammatory-antioxidant-rich diet and co-supplemented synbiotics intervention in patients with progressive forms of multiple sclerosis: a single-center, single-blind randomized clinical trial.

BACKGROUND: Current evidence has demonstrated that patients with Multiple Sclerosis (MS) have dysbiotic gut microbiomes, and anti-inflammatory nutritional interventions can normalize this status. Therefore, we aimed to investigate the effects of dietary intervention in patients with progressive forms of MS. METHODS: Seventy patients with three forms of progressive MS (primary-progressive, secondary-progressive, and progressive-relapsing) were randomly assigned into intervention (daily synbiotics capsule plus anti-inflammatory-antioxidant rich diet) or control (placebo capsule plus dietary recommendations) groups for four months. Faecal calprotectin level, Impact of Vision Impairment (IVI), Gastrointestinal Symptom Rating Scale (GSRS), and anthropometric measurements were evaluated at baseline and trial cessation. Analysis of covariance was conducted and adjusted for age, gender, education level, family history & duration of MS, type of progressive MS, type of main drug, and physical activity. RESULTS: Sixty-nine participants were included in the final analysis (n of intervention = 34; n of control = 35). Synbiotics and dietary intervention significantly reduced Faecal calprotectin level after six months (110.5 ± 75.9-44.7 ± 49.3 É¥g/g, P < 0.001), and mean changes were statistically significant in comparison with control group. However, intervention did not elicit any change in the anthropometric measurements. CONCLUSION: Synbiotics supplementation and adherence to an anti-inflammatory-antioxidant-rich diet reduced intestinal inflammation and improved clinical manifestations in progressive forms of MS.Trial registration: Iranian Registry of Clinical Trials identifier: IRCT20141108019853N7..

Establishing a minimum data set for Parkinson's (PMDS) in Iran.

BACKGROUND: The minimum data set (MDS) is one of the important steps in the development of health care information systems. According to the Ministry of Health in Iran, a central and national registry along with Parkinson's MDS (PMDS) has not yet existed. So, this research was conducted to establish a PMDS in Iran. MATERIAL AND METHODS: This study was a descriptive-comparative method, which was done in 2019-2021 in four phases: (1) determining data elements related to Parkinson's disease in Iran and selected countries; (2) extracting and categorizing the data elements; (3) making a PMDS draft; (4) evaluating a draft by Delphi technique. The research population was the MDS in Australia, Canada, the United States of America, and Iran. After extracting the data elements of Parkinson's disease from various resources, the primary draft PMDS was developed. Then, the research group divided it into two categories (administrative and clinical). After that, it was sent to 50 healthcare professionals for validation by the Delphi method. RESULTS: Following the results of the two rounds of Delphi technique, Finally, PMDS was established including a total of 223 data elements in two categories: administrative and clinical with 72 and 151, respectively. Every category included 10 and 14 subcategories. CONCLUSION: The first and the most important step for standardization of data collection nationally is creating MDS. Due to the necessity of the existence of PMDS, a complete list of PMDS was established for collecting data on Parkinson's patients.

Prevalence, clinical, imaging, electroencephalography and laboratory characteristics of seizures in COVID-19.

BACKGROUND: COVID-19 is the cause of the recent pandemic. Viral infections could increase the risks of neurological impairments, including seizures. Here, we aimed to evaluate the prevalence, clinical, imaging, electroencephalography and laboratory characteristics of seizures in COVID-19. METHODS: This retrospective cross-sectional study was performed on cases of COVID-19 infection and seizure. The prevalence of seizures in patients with COVID-19 was calculated using the incidence of seizures in all patients. The collected data were age, sex, history of previous illnesses, the severity of COVID-19 disease, patients' medications, hospitalization, and the presence of electrolyte disorders in patients' tests and other tests such as blood gas. Those patients with their first seizure episodes were also divided into two groups of cases with COVID-19 associated seizures (N=38) and non-COVID-19 associated seizures (N=37) and the mentioned data were compared between the two groups. RESULTS: We assessed data of 60 patients with COVID-19-associated seizures (group 1), 40 patients with seizures not related to COVID-19 (group 2) and 60 patients with COVID-19 infection and no seizures (group 3). The prevalence of hypertension and diabetes mellitus were significantly higher in group 3 compared to group 1 (P=0.044 and P=0.009, respectively). Still, patients in group 1 had a higher prevalence of cerebrovascular accidents (CVA) compared to group 3 (P=0.008). The prevalence of abnormal EEG was significantly higher in cases with COVID-19 infection compared to the other group (P<0.001). Cases with their first seizure episode associated with COVID-19 had significantly higher creatinine levels (P=0.035), lower blood pH (P=0.023), lower blood HCO3 (P=0.001), higher ALT (P=0.004), higher blood urea nitrogen (BUN) (P=0.001), lower hemoglobin (Hb) (P=0.017), higher ESR (P=0.001), higher CRP (P<0.001) and higher mortality rates (P=0.004). CONCLUSION: Patients with COVID-19 infection and seizure have higher mortality rates and disturbed laboratory data.

Comparison of pramipexole and citalopram in the treatment of depression in Parkinson's disease: A randomized parallel-group trial.

Background: Depression is one of the most common neuropsychiatric symptoms in Parkinson's disease (PD). There is little evidence to guide depression treatment in these patients. The aim of this study was to compare citalopram and pramipexole in reducing depressive symptoms in patients with PD. Materials and Methods: In the present 8-week randomized trial, we compared the efficacy of pramipexole versus citalopram in the treatment of depression in PD patients. For this purpose, 44 PD patients with depression randomly received open-label oral citalopram tablets or pramipexole and their depression, quality of life, and daytime sleepiness scores were evaluated at baseline and after the 8-week trial period. Results: The median age of the patients was 64 years, and about 85% of them were male in both groups. The Beck Depression Inventory score, Parkinson's disease summary index (PDSI), and Epworth Sleepiness Scale were significantly decreased (P < 0.05) in both citalopram and pramipexole groups throughout this period and without significant difference (P > 0.05) between these two groups, except for PDSI score which showed significant improvement in pramipexole group compared with citalopram group (P < 0.0001, r = 0.319). There were neither serious adverse effects nor treatment discontinuation due to the adverse effects. Conclusion: The results indicated that both citalopram and pramipexole were effective in the alleviation of depression and improving the quality of life in PD patients; however, pramipexole was seemed to be slightly more beneficial on quality of life in these patients. Therefore, pramipexole seems to be an effective treatment for depression in addition to its benefits for motor symptoms of PD patients.

Can Early Neuromuscular Rehabilitation Protocol Improve Disability after a Hemiparetic Stroke? A Pilot Study.

BACKGROUND: The impairment of limb function and disability are among the most im portant consequences of stroke. To date, however, little research has been done on the early reha bilitation trial (ERT) after stroke in these patients. The purpose of this study was to evaluate the impact of ERT neuromuscular protocol on motor function soon after hemiparetic stroke. The sample included twelve hemiparetic patients (54.3 ± 15.4 years old) with ischemic stroke (n = 7 control, n = 5 intervention patients). ERTwas started as early as possible after stroke and included passive range of motion exercises, resistance training, assisted standing up, and active exercises of the healthy side of the body, in addition to encouraging voluntary contraction of affected limbs as much as possible. The rehabilitation was progressive and took 3 months, 6 days per week, 2-3 h per session. Fu gle-Meyer Assessment (FMA), Box and Blocks test (BBT) and Timed up and go (TUG) assessments were conducted. There was a significantly greater improvement in the intervention group com pared to control: FMA lower limbs (p = 0.001), total motor function (p = 0.002), but no significant difference in FMA upper limb between groups (p = 0.51). The analysis of data related to BBT showed no significant differences between the experimental and control groups (p = 0.3). However, TUG test showed significant differences between the experimental and control groups (p = 0.004). The most important finding of this study was to spend enough time in training sessions and provide adequate rest time for each person. Our results showed that ERT was associated with improved motor function but not with the upper limbs. This provides a basis for a definitive trial.

Artificial intelligence analysis to explore synchronize exercise, cobalamin, and magnesium as new actors to therapeutic of migraine symptoms: a randomized, placebo-controlled trial.

INTRODUCTION: Migraine is recognized as a complex neurological disorder that has imposed a social burden. We assessed the signaling pathways and molecular mechanisms based on the in silico analysis and predicted drug candidates by the biomedicine approach. Moreover, we evaluated high-intensity interval training and vitamin B12 + magnesium on women's migraine attacks and inflammatory status. METHODS: This study computed differential gene expression in migraine syndrome and the dimension network parameters visualized by software. Moreover, we proposed the functional mechanism and binding energy of essential micronutrients on macromolecules based on drug discovery. In this clinical trial, 60 cases were randomized to four groups, including applied high-intensity interval training (HIIT), cases consumed supplementation vitamin B12 and magnesium (Supp), cases applied high-intensity interval training, and consumed supplementation (HIIT + Supp), and migraine cases for 2 months. Serum levels of calcitonin gene-related peptide (CGRP) were measured at baseline and at the end of the study. In addition, migraine disability assessment score (MIDAS), frequency, intensity, and duration were recorded before and during interventions. RESULTS: In silico study revealed the association between inflammation signaling pathways and pathogenesis of migraine attacks as a remarkable pathomechanism in this disorder. Furthermore, serum concentrations of CGRP were significantly declined in the HIIT + Supp compared with other groups. In addition, MIDAS, frequency, intensity, and duration were reduced in the HIIT + Supp group compared with the other groups. CONCLUSION: We found that the synergistic effects of cobalamin and magnesium followed by regular exercise could silence the inflammation signaling pathway, and a combination of HIIT + Supp could ameliorate migraine pain. TRIAL REGISTRATION: This study was registered in the Iranian Registry of Clinical Trials; IRCT code: IRCT20170510033909N12. Approval Data: 2021/06/02.

SARS-CoV-2 serology among people with multiple sclerosis on disease-modifying therapies after BBIBP-CorV (Sinopharm) inactivated virus vaccination: Same story, different vaccine.

BACKGROUND: Various studies indicated blunted humoral responses to COVID-19 mRNA and viral vector vaccines among people with multiple sclerosis (pwMS) on sphingosine 1-phosphate receptor (S1PR) modulators and anti-CD20 therapies (aCD20); however, limited evidence was found regarding SARS-CoV-2 serology after inactivated virus vaccination. OBJECTIVE: To provide evidence regarding humoral response to COVID-19 inactivated virus vaccination among pwMS on disease-modifying therapies (DMTs). METHODS: A cohort study was carried out in Isfahan, Iran, enrolling DMT-exposed pwMS and unexposed (UX) healthy participants. Post-vaccination anti-SARS-CoV-2 Spike IgG serology testing was carried out among the participants and compared between participants based on their DMT exposure, using proper statistical tests. A multivariable logistic regression model was used to control for confounding. Association between the second vaccine dose-to-phlebotomy (vac2phleb) and the humoral response was investigated in each DMT-exposed cohort, using linear regression. Among the aCD20 cohort, the association of the last aCD20 infusion-to-first vaccine dose period with serostatus was investigated using an unpaired t-test. RESULTS: After enrolling 358 participants (144 pwMS and 214 healthy), blunted humoral responses were only observed in fingolimod (Log10 mean diff. [SE]: 0.72 [0.18], P = 0.001) and aCD20 (Log10 mean diff. [SE]: 0.75 [0.15], P < 0.001) cohorts compared to the UX cohort. Multivariable analysis confirmed the results. The study did not achieve enough statistical power to detect a significant association between the vac2phleb period and humoral responses. The last aCD20 infusion to first vaccination dose period was longer in the seroconverted pwMS on aCD20 (mean diff. [SE]: 8.43 weeks [2.57], P = 0.005). CONCLUSION: The results of this study mirrored the results of previous studies among mRNA- or viral vector-vaccinated pwMS on DMTs. Therefore, it can be concluded that mode of action contributes less than timing, to the efficiency of vaccination strategies among pwMS on DMTs - especially the ones on S1PR modulators and aCD20. Meanwhile, the mentioned pwMS should be advised to receive early boosters and remain vigilant until further data becomes available and more efficient vaccination strategies are crafted.

The effects of Mediterranean diet on severity of disease and serum Total Antioxidant Capacity (TAC) in patients with Parkinson's disease: a single center, randomized controlled trial.

Background: Parkinson's disease (PD) as one of the most common neurodegenerative disorders may be affected by healthy dietary pattern. The aim of this study was to investigate the effects of the Mediterranean Diet (MeD) on serum Total Antioxidant Capacity (TAC) and disease severity in PD patients.Materials & Methods: In this single-center randomized clinical trial, patients with idiopathic PD (n = 80) were selected randomly allocated to either MeD or control group (Iranian traditional diet); an individualized dietary plan based on the MeD was designed. Serum TAC and the motor & non-motor disease aspects using the Unified Parkinson's Disease Rating Scale (UPDRS) were evaluated in two groups. Statistical Analysis of data was performed using SPSS 24.Results: 70 PD patients with a mean age of 58.96 ± 8.7 and UDPRS of 41.66 ± 20.19 were analyzed in this study. MeD significantly increased serum TAC (P < 0.001). UPDRS score was also lowered in MeD group (P < 0.05).Conclusions: Mediterranean diet seems to have some benefits in PD. as well, TAC levels can also be affected by MeD. Anyway, further studies are needed to confirm the mentioned outcomes.Trial registration: Iranian Registry of Clinical Trials identifier: IRCT20141108019853N4.

Evaluation of the effectiveness of methylphenidate and modafinil in the treatment of daily drowsiness in patients with refractory epilepsy and their comparison with the control group.

BACKGROUND: Various articles show the high prevalence of sleep disorders and especially excessive daytime sleepiness (EDS) in patients with refractory epilepsy and the importance of personal and social burden of this complication on individuals. Considering the insufficient evidence to draw efficacy and safety of modafinil and methylphenidate to treat EDS in the patient with intractable seizures, we decided to compare the effect of methylphenidate and modafinil with the control group. It is hoped that this study will pave the way for further studies. METHODS: This study is a clinical trial (IRCT20171030037093N22) (URL: https://www.irct.ir/trial/42485). The study population was patients with refractory epilepsy referred to the neurology clinic of Al-Zahra Hospital, Isfahan, Iran, from 2019 to 2020. The patients were randomly divided into three groups. The first group was treated with methylphenidate, the second group was treated with modafinil, and the third group was not received any medication such as modafinil and methylphenidate. Methylphenidate dosage was 10-20 mg/day. The patients were treated with modafinil at a dose of 200-600 mg/day. EPWORTH sleepiness scale (ESS) and Total Sleep Time (TST) were calculated before and 8 weeks after the intervention for the patients. RESULTS: 47 patients were included and divided into 3 groups, methylphenidate (10 males and 9 females), modafinil (7 males and 13 females), and control (4 males and 4 females). There was no significant difference among the groups based on ESS before and after intervention and TST after the intervention (P>0.05), but the mean of TST was significantly lower in the control group than in methylphenidate and modafinil groups before the intervention (P=0.003). The change of ESS and TST before compared to after intervention in the methylphenidate and modafinil group were significant (P<0.001), but the changes of ESS and TST in the control group were not significant (P>0.05). The frequency of complications (P=0.74) and outcomes (P=0.07) were similar in both groups. CONCLUSION: Modafinil and methylphenidate are two effective and safe drugs to increase the quality of sleep in the patients. Additionally, ESS and TST scores are better in the patients who used modafinil and methylphenidate.

The effect of the Mediterranean diet on cognitive function in patients with Parkinson's disease: A randomized clinical controlled trial.

OBJECTIVES: Parkinson's disease (PD) is the second most common neurodegenerative disorder. It is proposed that adherence to the Mediterranean diet might have a beneficial effect on the prevention and treatment of PD and its complications. Thus, the aim of this study was to investigate the effects of the Mediterranean diet on cognitive function in patients with PD. DESIGN: The study was a single-center, randomized clinical trial. Eighty patients with idiopathic PD were randomly allocated to the Mediterranean diet (n = 40) or control (n = 40) group. Patients in the intervention group received an individualized dietary plan based on Mediterranean diet for 10 weeks. The Persian version of Montreal Cognitive Assessment (MoCA) test was used to assess the cognitive function at baseline and the end of the study. RESULTS: Thirty-five PD patients with a mean age of 59.3 ±â€¯8.3 and 35 patients with a mean age of 58.6 ±â€¯9.3 finished the study in intervention and control groups, respectively. After the intervention, the mean score of the dimensions of executive function, language, attention, concentration, and active memory and the total score of cognitive assessment significantly increased in the intervention compared with the control group (p < 0.05, for all). Nevertheless, the mean of the other scores including spatial-visual ability, memory learning task, and navigation versus time and place did not significantly change in both intervention and control groups. CONCLUSIONS: The findings of this study showed that adherence to the Mediterranean diet remarkably increased the dimensions of executive function, language, attention, concentration, and active memory and finally the total score of cognitive assessment in PD patients.

The effect of fampridine on the risk of seizure in patients with multiple sclerosis.

BACKGROUND: Fampridine was first approved by the US Food and Drug Administration (FDA) to improve walking in multiple sclerosis (MS) patients, which was demonstrated by an increase in their walking speed. Nevertheless, the medication has been reported to possess an epileptogenic effect since it blocks the voltage-gated potassium channels in neural fibers. Several studies have indicated that the risk of seizure among fampridine consumers is not substantially higher than that in the general MS population, however. The objective of this study is to describe 97 MS patients for whom fampridine was prescribed and to assess the incidence of post-medication seizures. METHODS: This cohort study included 97 MS patients with gait problems who referred to the Isfahan Clinic of MS from August 2017 to September 2019. The exclusion criteria were a previous or family history of seizure or a history of renal impairment. Fampridine was prescribed for all the patients at a dose of 10 mg twice daily (12 hours apart). RESULTS: three patients (with an approximate incidence rate of 0.015 per 100 patient-years) presented with generalized tonic-clonic seizures, 7, 9, and 14 months after initiating fampridine consumption. The radiological findings revealed significant cortical and subcortical lesions in the three patients. Further, two of them consumed baclofen or fingolimod simultaneously with fampridine. CONCLUSION: The reported incidence rate is relatively higher than that in the general MS population. The extensive (sub) cortical lesions and the concomitant medications probably have an important role in the epileptogenesis, regardless of fampridine. However, the potential pro-convulsant properties of fampridine should not be overlooked.

Tongue Protrusion Dystonia in Pantothenate Kinase-Associated Neurodegeneration.

BACKGROUND: Tongue protrusion dystonia is an uncommon focal dystonia involving the lingual muscles. Causes of tongue protrusion dystonia include tardive dystonia, posthypoxic dystonia, neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, and Lesch-Nyhan syndrome. METHOD: We summarize three children with pantothenate kinase-associated neurodegeneration and tongue protrusion dystonia. All three patients underwent careful neurological examination, brain magnetic resonance imaging, and genetic testing. RESULTS: Tongue protrusion dystonia was a prominent and disabling symptom in all three patients. Brain magnetic resonance imaging revealed a typical eye of the tiger sign in all patients. Two patients had the same genetic mutation (c.1168 A>T mutation, p.I390F). CONCLUSIONS: Tongue protrusion dystonia may be a clue to the underlying etiology of dystonia, including hereditary forms of dystonia. Among them, pantothenate kinase-associated neurodegeneration is an important cause, especially in children.

Association of dietary patterns with systemic inflammation, quality of life, disease severity, relapse rate, severity of fatigue and anthropometric measurements in MS patients.

Background: Multiple sclerosis (MS) is associated with changes in quality of life, disability, fatigue and anthropometric measurements. The important relationship of dietary patterns with such clinical manifestations was not completely investigated. Aims: The goal of this study was to define the dietary patterns and their association with systemic inflammation, Health-Related Quality Of Life, disease severity, Relapse Rate, severity of fatigue and anthropometric measurements in MS subjects. Methods: This cross-sectional study was conducted in 261 MS patients (mean age 38.9 ± 8.3). Dietary patterns were explored by a Food Frequency Questionnaire. Serum hs-CRP, Multiple Sclerosis Quality Of Life-54 item questionnaire, Extended Disability Status Scale, Fatigue Severity Scale and Visual Analog Fatigue Scale, Relapse Rate, Height, Weight and Deurenberg Equation were also used as tools. Data were analyzed by SPSS24, and using ANOVA, Tukey, Chi-square and ANCOVA tests. Results: Fruits, Vegetables, Low fat dairy-based pattern and Mediterranean-Like pattern were associated with lower serum hs-CRP (F = 6.037, P adjusted < 0.01), higher Physical and Mental Health Composite Scores (P adjusted < 0.001), lower attacks (F = 4.475, P adjusted < 0.05), lower acute and chronic fatigue (F = 5.353 and F = 7.011, respectively, P adjusted < 0.01), lower BMI (F = 7.528, P adjusted < 0.01) and Percent Body Fat (F = 6.135, P adjusted < 0.01); but no difference was observed about EDSS across the patterns. Conclusions: Adherence to healthy dietary patterns may reduce systemic inflammation, severity of fatigue, MS attacks, improved quality of life and balance weight especially body fat in MS patients.

Clinical outcomes of transverse myelitis with myelin oligodendrocyte glycoprotein antibody versus negative cases among adults in Isfahan, Iran: A comparative study.

Background: The aim of this study was to evaluate the status of anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in patients with transverse myelitis (TM) and compare the clinical and imaging characteristics of MOG immunoglobulin G (IgG)-positive with negative cases. Methods: This cohort study enrolled 71 patients diagnosed with new-onset of TM who were being followed at a referral university clinic in Isfahan, Iran, from November 2016 to January 2019. Magnetic resonance imaging (MRI) images and blood samples for anti-MOG, anti-aquaporin 4 (anti-AQP4) (using the cell-based technique), and vasculitis-related antibodies were collected from patients. Outcomes were assessed by the evolution of the Expanded Disability Status Scale (EDSS) score and brain and spinal cord imaging findings within three months. All patients underwent imaging and clinical assessment during a mean period of one year as a follow-up. We compared the characteristics of clinical and radiological outcomes in MOG-IgG-positive and negative cases. Results: Of the total population studied, there were 26.8% men and 73.2% women, with a mean age of 33 ± 10 years. 12 (16.9%) patients were seropositive for MOG antibody and 17 (89.5%) were positive for anti-AQP4 antibodies. There was no significant association between anti-MOG antibody seropositivity and age, gender distribution, the presence of other autoimmune diseases, and number and interval of relapses. However, the involvement site of the spine at first imaging was significantly different between seronegative and seropositive patients. Conclusion: In patients with MOG antibody disease (MOG-AD) TM, the MRI findings suggest a preferential involvement of the cervical-thoracic section in seropositive cases which may help differentiate from non-MOG demyelination TM.