Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 190 208 Marco Castori Department of Medical Genetics, San Camillo-Forlanini Hospital, Sapienza University of Rome and San Camillo - Forlanin, Rome, Italy. Nicol C Voermans Department of Neurology, Radboud University Medical Centre, Nijmegen, Netherlands. 2015 10 14 The term "Ehlers-Danlos syndrome" (EDS) groups together an increasing number of heritable connective tissue disorders mainly featuring joint hypermobility and related complications, dermal dysplasia with abnormal skin texture and repair, and variable range of the hollow organ and vascular dysfunctions. Although the nervous system is not considered a primary target of the underlying molecular defect, recently, increasing attention has been posed on neurological manifestations of EDSs, such as musculoskeletal pain, fatigue, headache, muscle weakness and paresthesias. Here, a comprehensive overview of neurological findings of these conditions is presented primarily intended for the clinical neurologist. Features are organized under various subheadings, including pain, fatigue, headache, stroke and cerebrovascular disease, brain and spine structural anomalies, epilepsy, muscular findings, neuropathy and developmental features. The emerging picture defines a wide spectrum of neurological manifestations that are unexpectedly common and potentially disabling. Their evaluation and correct interpretation by the clinical neurologist is crucial for avoiding superfluous investigations, wrong therapies, and inappropriate referral. A set of basic tools for patient's recognition is offered for raising awareness among neurologists on this underdiagnosed group of hereditary disorders. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/593 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/593/209

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 209 214 Ferdos Nazari Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Amirreza Azimi Department of Neurology, School of Medicine, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran. Siamak Abdi Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2015 10 14 Susac's syndrome (SS) is a clinical triad of encephalopathy, branch retinal artery occlusion and sensorineural hearing loss and maybe due to an immune-mediated endotheliopathy. Because of its rarity and some similarities to other common neurological conditions such as multiple sclerosis and acute disseminated encephalomyelitis, it is often misdiagnosed and therefore mistreated. To the best of our knowledge, there is only one case report from our country with this diagnosis. Here, we have a short discussion on this issue to introduce it to our colleagues and remind it as a differential diagnosis in patients with unexplained encephalopathy. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/592 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/592/210

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 215 219 Mahnaz Talebi Department of Neurology, Neurosciences Research Center (NSRC), School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran. Mohammad Ghertasi Department of Neurology, Neurosciences Research Center (NSRC), School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran. Aliakbar Taheraghdam Department of Neurology, Neurosciences Research Center (NSRC), School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran. Sasan Andalib Department of Neurology, Neurosciences Research Center (NSRC), School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran. Ehsan Sharifipour Department of Neurology, Neurosciences Research Center (NSRC), School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran. 2015 10 14 Background:  Gender  difference  has  been   reported  in stroke  risk factors  and  disease  history. The aim  of this study  was  to  compare  risk factors  and  the  severity  of ischemic stroke based  upon  modified Rankin Scale (mRS) and hospital mortality between two genders. Methods: In a  cross-sectional  study, 341  patients  (44% males and 56% females with a mean age of 68.94 ± 12.74 years) with ischemic stroke, who were hospitalized  in the neurology   wards  of  two  referral  university  hospital  of North-West Iran (Imam Reza and Razi Hospitals), from the beginning  to the end of 2011 were selected  and assessed. Gender difference in terms of demographic findings, vascular risk factors, 7th   day mRS, and  hospital  mortality (during admission) were evaluated. Results: In 2.6% of cases, mRS was found to be less than 2 (favorable) and in 97.4% of cases; mRS was 2-5 (with disability). No significant difference in ischemic stroke severity   based   on   mRS   was   observed   between  two genders. ere was a significant difference  in the  rate of hypertension (females = 72.3%, males = 59.3%, P = 0.010), diabetes   (females  =  28.8%,  males  =  18.7%,  P =  0.030), smoking (females = 6.3%, males = 35.3%, P < 0.001). No significant   difference   was   seen   in  other   risk  factors between two genders. There was no significant difference in the mortality rate, which constituted 8.9% and 4.7% in females and males respectively (P = 0.140). Conclusion:The   evidence   from   the   present   study suggests  that  despite  the  existence  of some  difference between risk-factors in two genders, there was no difference in terms of ischemic stroke severity and mortality rate between two genders. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/591 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/591/211

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 220 225 Gholam Ali Shahidi Department of Neurology, School of Medicine, Rasoule-Akram Hospital, Iran University of Medical Sciences, Tehran, Iran. Zeinab Ghaempanah Department of Psychology, School of Humanities, Tarbiat-Modares University, Tehran, Iran. Yasaman Khalili Department of Community Medicine, School of Medicine, Iran University of Medical Sciences, Tehran, Iran. Marzieh Nojomi Department of Community Medicine, School of Medicine, Preventive Medicine Research Center, Iran University of Medical Sciences, Tehran, Iran. 2015 10 14 Background:  Assessment  of quality-of-life (QOF) as  an outcome   measure   after  deep   brain  stimulation   (DBS) surgery in patients  with Parkinson’s disease  (PD) need  a valid, reliable and responsive  instrument. The aim of the current   study   was   to   determine   responsiveness   of validated  Persian  version  of PD questionnaire  with  39- items (PDQ-39) after DBS surgery in patients with PD. Methods: Eleven patients with PD, who were candidate  for DBS  operation  between May 2012 and  June  2013 were assessed.  PDQ-39 and  short-form  questionnaire  with 36- items (SF-36) were used. To assess responsiveness of PDQ- 39 standardized response mean (SRM) was used. Results: Mean age was 51.8 (8.8) and all of the patients, but just  one  were  male  (10 patients). Mean duration  of the disease was 8.7 (2.1) years. Eight patients  were categorized as moderate using Hoehn and Yahr (H and Y) classification. All patients  had a better  H and Y score compared  with the baseline evaluation (3.09 vs. 0.7 ). The amount  of SRM was above  0.70 for all domains  means  a large responsiveness for PDQ-39. Conclusion: Persian version of PDQ-39 has an acceptable responsiveness and could be used to assess as an outcome measure to evaluate the effect of therapies on PD. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/590 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/590/212

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 226 230 Ali Moghtaderi Department of Neurology, School of Medicine, Zahedan University of Medical Sciences, Zahedan, Iran. Sharareh Sanei-Sistani Department of Radiology, School of Medicine, Zahedan University of Medical Sciences, Zahedan, Iran. Ghassem Abdollahi Department of Radiology, School of Medicine, Zahedan University of Medical Sciences, Zahedan, Iran. Hamid Dahmardeh Department of Radiology, School of Medicine, Zahedan University of Medical Sciences, Zahedan, Iran. 2015 10 14 Background: Role of atherosclerosis  in the pathogenesis of ischemic and hemorrhagic  infarctions is still matter  of debate. Intima-media thickness (IMT) of the common carotid  artery (CCA) and  internal  carotid  artery (ICA) are markers of atherosclerosis. The aim of this study was to compare  CCA IMT and ICA IMT of patients  with ischemic and hemorrhagic infarction. Methods: Two equal groups of 80 patients  with small and large vessel ischemic stroke and 80 patients with non- traumatic  intracerebral  hemorrhage (ICH) who referred to our central teaching  hospital of Zahedan  were assessed  in this descriptive  study. IMT of four arteries  (right and  left CCA and  ICA)  were  measured,  and  collected  data  were analysis using Student’s t-test. Results: There were  137 males  (57.1%) and  103 (42.9%) female with mean age of 62.7 ± 11.7. Mean right CCA IMT of patients  with small vessel diseases  (SVD), large vessel diseases (LVD), and ICH were 0.564 ± 0.130, 0.623 ± 0.150, and 0.580 ± 0.140 mm, respecti ely (P = 0.032). Mean left CCA IMT   of  patients   with  SVD,   LVD,   and   ICH   were 0.569  ±  0.120,  0.618  ±  0.120,  and  0.573  ±  0.130  mm, respectively (P = 0.039). The above  ?ndings  for right ICA were 0.572 ± 0.120, 0.569 ± 0.140, and 0.522 ± 0.130 mm, respectively (P = 0.145). Those ?ndings for left ICA IMT were 0.525 ± 0.110, 0.554 ± 0.120, and 0.527 ± 0.120 mm, respectively (P = 0.257). Conclusion: Our findings showed  that by using CCA IMT, differentiation  between small and  large vessel infarctions and hemorrhagic infarctions can be made. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/589 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/589/213

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 231 236 Babak Bakhshayesh Department of Neurology, School of Medicine, Poursina Hospital, Guilan University of Medical Sciences, Rasht, Iran. Mozaffar Hosseininezhad Department of Neurology, School of Medicine, Poursina Hospital, Guilan University of Medical Sciences, Rasht, Iran. Seyed Mohammad Seyed Saadat School of Medicine, Guilan University of Medical Sciences, Rasht, Iran. Morvarid Hajmanuchehri Guilan Road Trauma Research Center, Guilan University of Medical Sciences, Rasht, Iran. Ehsan Kazemnezhad Department of Biostatistics, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran. Amir-Reza Ghayeghran Department of Neurology, School of Medicine, Poursina Hospital, Guilan University of Medical Sciences, Rasht, Iran. 2015 10 14 Background: Intracerebral  hemorrhage (ICH) is the  most fatal subtype  of stroke.  Despite  limited effective therapy, there  is no  accepted clinical grading  scale to  predict  in- hospital  mortality,  especially  in developing  nations.  The purpose  of this study was to assess the  predictors  of in- hospital mortality among a sample of Iranian patients  with spontaneous ICH for use at the time of the first evaluation. Methods: This prospective  study was carried from January 2010 to  the  end  of January  2011. Demographic, clinical, and   laboratory   data   of  ICH   patients   were   collected. Hematoma  volume and perihematoma edema  (PHE) were measured   on  brain  computed  tomography scan  using ABC/2 formula. Logistic regression analysis was performed to determine independent variables contributing to in- hospital mortality. Results:  Of  a  total   167  consecutive   ICH   patients,  98 patients  met inclusion criteria. Mean ± standard  deviation age  of  patients   was  70.16  ±  12.52.  Afte   multivariate analysis, five variables remained as independent predictors of   in-hospital    mortality    included:   age    [odds    ratio (OR)  =  1.12,  95%  confidence   interval  (CI)   =  1.03-1.23, P = 0.009], diabetes  mellitus (OR = 10.86, 95% CI = 1.08- 109.24, P = 0.009), National Institutes of Health Stroke Scale (NIHSS) score (OR = 1.41, 95% CI = 1.08-1.68, P ≤ 0.001), as well as volume of hematoma (OR = 1.1, 95% CI = 1.03-1.17, P  =  0.003),  and  PHE  (OR  =  0.75,  95%  CI  =  0.60-0.93, P = 0.010). Conclusion: Our results indicate  that  older age, diabetes mellitus,  higher   NIHSS,  as   well  as  larger   volume   of hematoma, and  smaller PHE on admission  are important predictors of in-hospital mortality in our ICH patients. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/588 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/588/214

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 237 240 Hamidreza Hatamian Department of Neurology, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran. Alia Saberi Department of Neurology, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran. Matin Pourghasem Department of Pediatrics, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran. 2015 10 14 Background: Several factors influence on the outcome  of ischemic stroke. The aim of this study was determination the relationship  between stroke mortality and red blood cell parameters. Methods: This cross-sectional  study was conducted  from 2011 July to June 2012. For all patie; for 2 months. After 2 months  of treatment, BICAMS was redone  and  changes  were  analyze .The significant change value on the before-after BICAMS points were considered  to be 8, 13, and 7 points for the symbol digit modality  test (SDMT),  the  California verbal  learning  test (CVLT),  and  the  brief visual-spatial  memory  test  revised (BVMT-R), respectively. Results:  The  patients’  mean  age  was 36.58     8.50  years. The mean duration of disease was 7.41    4.13 years. About 84.2% (n = 64) of the patients  was female. In the BVMT-R, 13 patients  (34.2%), who had  already taken  B. papyrifera, were shown to have significant improvement compared  to the  placebo  group  with  no  improvement  (P <  0.  001). About  12 and  8 patients  in the  treatment and  placebo groups in the SDMT, respectively (P = 0.200) and 17 and 12 patients  in the treatment and placebo groups in the CVLT, respectively (P = 0.170) had signi@cant change values. Conclusion: B. papyrifera showed significant improvement in  visuospatial  memory,  but   had   no  effect  on  verbal memory and information processing speed. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/604 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/604/198

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 3 2014 09 15 154 159 Aliasghar Molana Department of Neuroscience, School of Advanced Technologies in Medicine, Iran University of Medical Sciences, Tehran, Iran. Masoud Mehrpour Department of Neurology, Firoozgar Hospital, Iran University of Medical Sciences, Tehran, Iran. Nasim Vousooghi Department of Neuroscience, School of Advanced Technologies in Medicine AND Genetics Laboratory, Iranian National Center for Addiction Studies, Tehran University of Medical Sciences, Tehran, Iran. Mahmoud Reza Hajighasem Department of Neuroscience, School of Advanced Technologies in Medicine AND Brain and Spinal Cord Injury Research Center, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran. Mohammad Taghi Joghataei Department of Neuroscience, School of Advanced Technologies in Medicine, Iran University of Medical Sciences, Tehran, Iran. 2015 10 14 Background: Migraine is a chronic  neurological  disorder, characterized  by recurrent  moderate to severe headaches. Worldwide migraine a=ects nearly 15%. Studies suggest that genes involved in the production of nitric oxide (NO) may act as genetic  factors for migraine. NO synthase  3 (NOS3) by expressing  enzyme NOS regulates  endothelial  derived NO. One  class of  medications   used  as  first-line treatment in migraine prophylaxis is tricyclic antidepressants (TCAs). The aim of this study  was to determine  e=ects  of NOS3 gene Glu298Asp polymorphism  in  the  production  of  NO and response of patients to TCAs in migraine attacks. Methods: A total of 80 migraine patients  were invited to participate   in  the  study.  Patients  recorded   the characteristics of their migraine attacks such as frequency of attacks and intensity of headaches for the 1st  month  of the study. Then peripheral blood samples were taken from all subjects in order to determine patients’ genotype distribution,  mRNA  expression  level  of  NOS3 and   NO content  of plasma. Patients were then instructed  to use 25 mg nortriptyline at night before bed for 3 months. At the end of 3rd  month of the treatment patients  again recorded the  migraine characteristics  for  1  month   and   blood sampling was performed  in order to determine the level of plasma NO. Results: The patients’ genotype distribution  for TT,  GT, and GG was 9, 24, and 47 subjects, respectively. Mean NO level in patients  with TT genotype was less in comparison to GT and GG genotypes before and after use of TCAs (P < 0.05). Mean  intensity  of headaches in patients  with  TT genotype  was  lower  in  comparison   to   GT   and   GG genotypes before and after use of TCAs (based on verbal numerical   rating   scale).  Mean  frequency   of  migraine attacks after use of TCAs was significantly decreased in all genotypes of NOS3 Glu298Asp polymorphism particularly in TT genotype (P < 0.05). Conclusion: Presence of T allele of the Glu298Asp polymorphism may be a factor for TT genotype patients to produce less NO and is a favorable factor for better response to TCAs in reducing  migraine  attacks in comparison to GT and GG genotypes. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/603 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/603/199

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 3 2014 09 15 160 167 Roya Abolfazli Department of Neurology, School of Medicine, Amiralam Hospital, Tehran University of Medical Sciences, Tehran, Iran. Azam Elyasi Department of Clinical Pharmacy, School of Pharmacy, Tehran University of Medical Sciences, Tehran, Iran. Mohammad Reza Javadi Department of Clinical Pharmacy, School of Pharmacy, Tehran University of Medical Sciences, Tehran, Iran. Kheirollah Gholami Department of Clinical Pharmacy, School of Pharmacy, Tehran University of Medical Sciences, Tehran, Iran. Hassan Torkamandi Department of Pharmaceutical Care, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Mohammad Amir-Shahkarami Department of Neurology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Masoud Etemadifar Department of Neurology, Isfahan Neurosciences Research Center, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Zahra Nasr Department of Medicine, Medical Students' Research Center, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. 2015 10 14 Background:  Multiple  sclerosis  (MS) patients permanently confronted  with serious challenges from treatment regimen. Developing  a new  questionnaire in MS management, through  evaluation  of patients’ perspectives   and  knowledge   regarding   treatment  will help  to  identify the  sources  of tension, and  to  build  a therapeutic   alliance.   We   purposed   to   describe    MS patients’ understanding of their treatments. Methods: About 425 completed and returned questionnaire were assessed  of a total of 500 recruited  MS patients. The knowledge   of  correct  using  interferon-beta  (IFN-β)  and attitude   toward  medical  care  were  assessed   using  self- reported questionnaires consisted of 25 items with validity of multidisciplinary panel and pre-testing on 20 patients. Results:  Knowledge  about  IFN-β  therapy  was  very low; however, attitude  was at a high level. Female patients, self- injection ability, higher educational level, normal functional status, delay from the start of diagnostic workup to definite diagnosis, and being younger were related to a higher level of  knowledge.  Attitude  was  associated   with  functional status,  family  history  of  disease   and   the   summary  of knowledge variable. Conclusion: Developing educational  interventions  are needed for MS patients  regarding  to  their  low levels of knowledge. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/602 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/602/200

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 3 2014 09 15 168 171 Mahshid Hosseini Behbahani Department of Biochemistry, Payame Noor University, Tehran, Iran. Hamid Galehdari Departmanet of Genetic, School of Sciences, Shahid Chamran University, Ahvaz, Iran. Maryam Mohaghegh Departmanet of Genetic, School of Sciences, Shahid Chamran University, Ahvaz, Iran. 2015 10 14 Background:  Multiple  sclerosis  (MS) is a  chronic inflammatory  demyelinating  and  neurodegenerative disease  of central  nervous  system  with unknown  causes. Etiology of MS involves  both  genetic  and  environment factors.  The  interleukin  7   receptor   (IL7R)   gene   is  a promising candidate  for MS, because its involvement in the autoimmunity, regulation of the T-cell homeostasis, proliferation, and anti-apoptotic signaling. Methods: We investigated  the association of the IL7R gene polymorphism  rs6897932  in MS patients   in a  case  and control study. In this case and control study participating, 127  relapsing-remitting  MS (RRMS)  patients   (mean  age: 32.25, age range: 16-57) selected  according McDonald criteria, and 109 ethnically, sex and age matched  healthy control   (mean  age:  27.44,  age   range:  14-63)  with  no personal  or family history of autoimmune diseases  were studied. DNA was extracted  from whole blood using high pure  polymerase  chain reaction  template preparatio  kit from  Roch  Company.  Amplification refractory  mutation system method was applied to define the genotyping C/T within exon 6 of the IL7R gene among individuals. Results: Evaluation   of  the   IL7R   gene   polymorphism revealed  that  the  T allele and the  C/T and T/T genotypes are present  in 53.5%, 42.5%, 4.0%, and 68.8%, 26.6%, 4.6% in  MS patients   and   controls,  respectively.  Comparison between alleles and  genotypes in the  MS patients  and healthy controls show significant differences (P = 0.038). Conclusion: The distribution of  the rs6897932 polymorphism  is significantly different in our case/control study  in Khuzestan   Province.  This  single  nucleotide polymorphism  causes alternative  splicing in exon 6 of the IL7R gene with possible influence of the autoimmunity. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/601 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/601/201

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 3 2014 09 15 172 176 Samia Younes Department of Endocrinology and Internal Medicine, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Yousra Cherif Department of Endocrinology and Internal Medicine, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Mouna Aissi Department of Neurology, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Wafa Alaya Department of Endocrinology and Internal Medicine, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Olfa Berriche Department of Endocrinology and Internal Medicine, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Amel Boughammoura Department of Neurology, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Mahbouba Frih-Ayed Department of Neurology, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Baha Zantour Department of Endocrinology and Internal Medicine, School of Medicine, University of Monastir, Al Munastîr, Tunisia. Mohamed Habib Sfar Department of Endocrinology and Internal Medicine, School of Medicine, University of Monastir, Al Munast&