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 patients with ischemic stroke in middle  cerebral  artery  (MCA)  territory, the  cell blood count test was performed. We recorded  their mortality on the 1st  week and the 1st  month  after ischemic stroke. Data analysis was performed  using t-test, χ2, Mann–Whitney U- test,  logistic  regression  and  receiver  operating characteristic curve in SPSS for Windows 19.0. Results: A total  of 98  subjects  (45.9%  men  and  54.1% women)  with  the  mean  age  of 71.0 ±  13.9 years  were assessed,   while   67.3%   of   them    were   anemic.   The prevalence  of 1st  week mortality among  anemic and non- anemic  patients  was 40.9% and  34.4% (P = 0.534). The prevalence  of mortality  after  1st   week till 1st   month  was 19.6% and  21.0% respectively  (P = 0.636). In univariant analysis, only 1st month mortality had a significant relationship  with red  blood  cell (RBC)  count  (P = 0.022). However, the  result of logistic regression  model  showed that RBC (P = 0.012) and mean corpuscular volume (MCV) (P = 0.021) remained  as predictors of the 1st  week and the 1st  month  mortality (P = 0.011 and P = 0.090 respectively). The best  cutoB point  of RBC for the  prediction  of the  1st week mortality with 44.7% speciCcity and 69.5% sensitivity was  estimated   4.07   million/μl  and   for  the   1st    month mortality with 46.6% speciCcity and 72.2% sensitivity was estimated 4.16 million/μl. Conclusion: The RBC  count  and  MCV  are  independent predictors of ischemic stroke short-term mortality. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/587 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/587/215

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 241 244 Shahnaz Miri Department of Neurology, School of Medicine, Downstate Medical Center, State University of New York, Brooklyn, New York, NY, USA. Mohammad Rohani Department of Neurology, School of Medicine, Rasool Akram Teaching Hospital, Iran University of Medical Sciences, Tehran, Iran. Mansoureh Vahdat Department of Obstetrics & Gynecology, School of Medicine, Rasool Akram Teaching Hospital, Iran University of Medical Sciences, Tehran, Iran. Maryam Kashanian Department of Obstetrics & Gynecology, School of Medicine, Akbar Abadi Teaching Hospital, Iran University of Medical Sciences, Tehran, Iran. Elaheh Sariri Department of Obstetrics & Gynecology, School of Medicine, Rasool Akram Teaching Hospital, Iran University of Medical Sciences, Tehran, Iran. Babak Zamani Department of Neurology, School of Medicine, Rasool Akram Teaching Hospital, Iran University of Medical Sciences, Tehran, Iran. Gholam Ali Shahidi Department of Neurology, School of Medicine, Rasool Akram Teaching Hospital, Iran University of Medical Sciences, Tehran, Iran. 2015 10 14 Background: Restless legs  syndrome  (RLS)  is the  most common movement disorder in pregnancy, which can be idiopathic  or secondary. There are  limited  comparative data  regarding  these  two forms of RLS.  The aim of this study was to compare  clinical features  of idiopathic  and secondary RLS in pregnant women. Methods: Over a period  of 3 months, 443 women  who admitted for delivery in two clinical centers were screened for RLS using four diagnostic  criteria of the  international RLS study group. A total of 79 subjects diagnosed with RLS were  consecutively  enrolled  in the  present  study.  All of them were interviewed for medical history and complaints during pregnancy  and responded to self-administer international RLS rating scale. Results: Ten subjects  (12.9%) out of 79 pregnant women with  RLS  had  idiopathic  form, and  their  mean  age  was significantly higher than patients  with secondary RLS (30.6 ± 7.3 years vs. 26.4 ± 4.6 years, P = 0.0260). Compared with women  with secondary  RLS, sleep duration  in pregnancy was   significantly   decreased   in   idiopathic   RLS   group (P = 0.0460), whereas RLS severity score was similar in both groups.  No significant difference  was observed  between  the  two  groups  in terms  of other  sleep  complaints, the positive family history of RLS, parity, duration of pregnancy, or frequency of cesarean section (P > 0.0500). Conclusion: Idiopathic and secondary RLS have relatively similar courses and features  during pregnancy. However, the  idiopathic  form may have more negative  impact  on sleep in pregnancy. Careful screening  and effective treatment of idiopathic RLS before pregnancy  is recommended to limit these disturbances. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/586 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/586/216

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 245 249 Behnaz Sedighi Department of Neurology, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran. Kaveh Shafiee Department of Neurology, School of Medicine, Neurology Research Center, Kerman University of Medical Sciences, Kerman, Iran. Rostam Seifaldini Department of Neurology, School of Medicine, Kerman University of Medical Sciences, Kerman, Iran. As'ad Abdi Department of Neurology, School of Medicine, Neurology Research Center, Kerman University of Medical Sciences, Kerman, Iran. 2015 10 14 Background: Restless legs  syndrome  (RLS)  is the  most common movement disorder in pregnancy, which can be idiopathic  or secondary. There are  limited  comparative data  regarding  these  two forms of RLS.  The aim of this study was to compare  clinical features  of idiopathic  and secondary RLS in pregnant women. Methods: Over a period  of 3 months, 443 women  who admitted for delivery in two clinical centers were screened for RLS using four diagnostic  criteria of the  international RLS study group. A total of 79 subjects diagnosed with RLS were  consecutively  enrolled  in the  present  study.  All of them were interviewed for medical history and complaints during pregnancy  and responded to self-administer international RLS rating scale. Results: Ten subjects  (12.9%) out of 79 pregnant women with  RLS  had  idiopathic  form, and  their  mean  age  was significantly higher than patients  with secondary RLS (30.6 ± 7.3 years vs. 26.4 ± 4.6 years, P = 0.0260). Compared with women  with secondary  RLS, sl ep duration  in pregnancy was   significantly   decreased   in   idiopathic   RLS   group (P = 0.0460), whereas RLS severity score was similar in both groups.  No significant difference  was observed  between the  two  groups  in terms  of other  sleep  complaints, the positive family history of RLS, parity, duration of pregnancy, or frequency of cesarean section (P > 0.0500). Conclusion: Idiopathic and secondary RLS have relatively similar courses and features  during pregnancy. However, the  idiopathic  form may have more negative  impact  on sleep in pregnancy. Careful screening  and effective treatment of idiopathic RLS before pregnancy  is recommended to limit these disturbances. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/585 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/585/217

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 250 252 Sara Momtaz Department of Audiology, School of Rehabilitation, Arak University of Medical Sciences, Arak, Iran. Fahimeh Hajiabolhassan Department of Audiology, School of Rehabilitation, Tehran University of Medical Sciences, Tehran, Iran. Mansoureh Togha Iranian Center of Neurological Research, Neuroscience Institute, Tehran University of Medical Sciences, Tehran, Iran. Shohre Jalaie Department of Biostatistics, School of Rehabilitation, Tehran University of Medical Sciences, Tehran, Iran. Amir Almasi Department of Epidemiology, School of Health, Arak University of Medical Sciences, Arak, Iran. 2015 10 14 Background: Neurotologic signs and symptoms, especially vestibular symptoms are common in migrainous patients. Involvement of the visual system in migrainures has received a great deal of attention in recent years, but the oculomotor part of the visual system has been largely ignored. The goal of this study was to investigate some parts of the central vestibular system using the oculomotor part of videonystagmographic evaluation, including spontaneous nystagmus, gazeevoked nystagmus, smooth pursuit, saccade and optokinetic ystagmus interictally in migrainous patients. Methods: In this case–control study, 30 patients with migraine and 38 healthy volunteers within the age range of 18-48 years old were included spontaneous nystagmus; gaze-evoked nystagmus in right, left and up sides, smooth pursuit, optokinetic nystagmus using three different velocities and saccade test performed in both groups. The data were analyzed using SPSS for Windows 18.0. Results: Some parameters of gain and phase and also morphology of  he smooth pursuit, velocity of the saccade and slow phase velocity of optokinetic were significantly different in migrainures, although the statistical differences of these parameters were not clinically important as they were in the normal range of a defined device. Conclusion: These results may suggest the presence of subtle otoneurologic abnormalities in migrainous patients that is probably due to the efficiency of oculomotor function with vestibulocerebellar origin. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/584 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/584/218

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 253 254 Güliz Yılmaz Department of Radiology, School of Medicine, Near East University, Nicosia, North Cyprus, Turkey. Süha Akpınar Department of Radiology, School of Medicine, Near East University, Nicosia, North Cyprus, Turkey. 2015 10 14 No Abstract https://ijnl.tums.ac.ir/index.php/ijnl/article/view/583 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/583/219

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 13 4 2014 12 15 255 256 Deepak Jain Department of Medicine, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India. Hari Krishan Aggarwal Department of Medicine, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India. Shivraj Goyal Department of Medicine, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India. Ansul Mittal Department of Medicine, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India. 2015 10 14 No Abstract https://ijnl.tums.ac.ir/index.php/ijnl/article/view/582 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/582/220