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 patiersity of Medical Sciences, Tehran, Iran. Ahmad Aoude Department of Neurosurgery, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Soheil Naderi Department of Neurosurgery, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Zeynab Najafi Department of Pediatrics, School of Medicine AND Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. Morteza Faghih-Jouibari Department of Neurosurgery, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2015 10 14 Background: Distinction between radiation necrosis and recurrence of intraparenchymal tumors is necessary to select the appropriate treatment, but it is often difficult based on imaging features alone. We developed an algorithm for analyzing magnetic resonance spectroscopy (MRS) findings and studied its accuracy in differentiation between radiation necrosis and tumor recurrence. Methods:   Thirty-three   patients   with   a   history   of intraparenchymal brain tumor resection and radiotherapy, which had developed new enhancing lesion were evaluated by MRS and subsequently underwent reoperation. Lesions with Choline (Cho)/N- acetyl aspartate (NAA) > 1.8 or Cho/Lipid > 1 were considered as tumor recurrence and the remaining as radiation necrosis. Finally, pre-perative MRS diagnoses were compared with histopathological report. Results: The histological diagnosis was recurrence in 25 patients and necrosis in 8 patients. Mean Cho/NAA in  recurrent  tumors  was  2.72,  but  it  was  1.46  in radiation necrosis (P < 0.01). Furthermore, Cho/Lipid was significantly higher in recurrent tumors (P < 0.01) with the mean of 2.78 in recurrent tumors and 0.6 in radiation     necrosis.    Sensitivity,    specificity,    and diagnostic accuracy of the algorithm for detecting tumor recurrence were 84%, 75% and 81%, respectively. Conclusion: MRS is a safe and informative tool for differentiating    between   tumor   recurrence   and radiation necrosis. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/576 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/576/226

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 14 1 2015 03 15 35 40 Siamak Abdi Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Shahram Oveis-Gharan Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Farnaz Sinaei Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Askar Ghorbani Department of Neurology, School of Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. 2015 10 14 Background: Serum troponin elevation, characteristic of ischemic myocardial injury, has been observed in some acute ischemic stroke (AIS) patients. Its cause and significance are still controversial. The purpose of this study is to find determinants of troponin elevation and its relationship with stroke severity and location. Methods:Between January 2013 and August 2013, 114 consecutive AIS patients confirmed by diffusion- weighted magnetic resonance imaging were recruited in this study. Serum troponin T level was measured as part of routine laboratory testing on admission. Ten lead     standard     electrocardiogram    (ECG)     was performed and stoke severity was assessed based on National Institutes of Health Stroke Scale (NIHSS). Results: Troponin T was elevated in 20 (17.6%) of 114 patients. Patients with elevated troponin were more   likely   to   have   higher   age,   higher   serum creatinine  and  ischemic  ECG  changes.  Troponin levels were higher in patients with more severe stroke measured  by  NIHSS  [7.96  (6.49-9.78)  vs.  13.59 (10.28-18.00)].  There  was  no  association  between troponin and locations of stroke and atrial fibrillation. There were 6 (5%) patients with elevated troponin in the presence of normal creatinine and ECG. Conclusion:  Stroke  severity,  not  its  location,  was associated   with   higher   troponin   levels.   Abnormal troponin levels are more likely, but not exclusively, to be due to cardiac and renal causes than cerebral ones. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/575 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/575/227

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 14 1 2015 03 15 41 46 Saeid Khosrawi Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Parisa Taheri Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. Seyed Hasan Hashemi Department of Physical Medicine and Rehabilitation, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. 2015 10 14 Background: H-reflex is a valuable electrophysiological technique for assessing nerve conduction through entire length of afferent and efferent pathways, especially nerve roots and proximal segments of peripheral nerves. The aim of this study was to investigate the relation between normal values of flexor carpi radialis (FCR) H-reflex latency, upper limb length and age in normal subjects, and to determine whether there is any regression equation between them. Methods: By considering the criteria of inclusion and exclusion, 120 upper limbs of 69 normal volunteers (68 hands of 39 men and 52 hands of 30 women) with the mean age of 39.8 ± 11.2 years participated in this study. FCR H-reflex was obtained by standard electrodiagnostic  techniques,  and  its  onset  latency was recorded. Upper limb length and arm length weremeasured   in   defined   position. The   degree   of association between these variables was determined with  Pearson correlation and  linear  regression was used for obtaining the proposed relaions. Results: Mean FCR H-reflex latency was found to be 15.88 ± 1.27 ms. There was a direct linear correlation between FCR H-reflex latency and upper limb length (r = 0.647) and also arm length (r = 0.574), but there was no significant correlation between age and FCR H-reflex latency (P = 0.260). Finally, based on our findings, we tried to formulate these relations by statistical methods. Conclusion: We found that upper limb length and arm length are good predictive values for estimation of normal FCR H-reflex latency but age, in the range of 20-60 years old, has no correlation with its latency. This estimation could have practical indications in pathologic conditions. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/574 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/574/228

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 14 1 2015 03 15 47 49 Fahmi Nasrallah Department of Biology, School of Medicine, Laboratory of Biochemistry, Rabta Hospital, Jebbari, 1007 Tunis, Tunisia. Hanene Benrhouma Department of Child and Adolescent Neurology, School of Medicine, Mongi Ben Hmida Institute of Neurology, 1700 Tunis, Tunisia. Ichraf Kraoua Department of Child and Adolescent Neurology, School of Medicine, Mongi Ben Hmida Institute of Neurology, 1700 Tunis, Tunisia. Gilbert Briand Department of Biochemistry and Molecular Biology, School of Medicine, Laboratory of Endocrinology, Metabolism-Nutrition, Oncology, Biology Pathology Center CHRU, 57039 Lille, France. Souheil Omar Department of Biology, School of Medicine, Mongi Ben Hmida Institute of Neurology, 1700 Tunis, Tunisia. Ilhem Turki Ben Youssef Department of Child and Adolescent Neurology, School of Medicine, Mongi Ben Hmida Institute of Neurology, 1700 Tunis, Tunisia. Naziha Kaabachi Department of Biology, School of Medicine, Laboratory of Biochemistry, Rabta Hospital, Jebbari, 1007 Tunis, Tunisia. 2015 10 14 Background: H-reflex is a valuable electrophysiological technique for assessing nerve conduction through entire length of afferent and efferent pathways, especially nerve roots and proximal segments of peripheral nerves. The aim of this study was to investigate the relation between normal values of flexor carpi radialis (FCR) H-reflex latency, upper limb length and age in normal subjects, and to determine whether there is any regression equation between them. Methods: By considering the criteria of inclusion and exclusion, 120 upper limbs of 69 normal volunteers (68 hands of 39 men and 52 hands of 30 women) with the mean age of 39.8 ± 11.2 years participated in this study. FCR H-reflex was obtained by standard electrodiagnostic  techniques,  and  its  onset  latency was recorded. Upper limb length and arm length were measured in  defined position.The degree  of association between these variables was determined with  Pearson correlation and  linear  regression was used for obtaining the proposed relations. Resalts: Mean FCR H-reflex latency was found to be 15.88 ± 1.27 ms. There was a direct linear correlation between FCR H-reflex latency and upper limb length (r = 0.647) and also arm length (r = 0.574), but there was no significant correlation between age and FCR H-reflex latency (P = 0.260). Finally, based on our findings, we tried to formulate these relations by statistical methods. Conclusion: We found that upper limb length and arm length are good predictive values for estimation of normal FCR H-reflex latency but age, in the range of 20-60 years old, has no correlation with its latency. This estimation could have practical indications in pathologic conditions. https://ijnl.tums.ac.ir/index.php/ijnl/article/view/573 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/573/229

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 14 1 2015 03 15 50 51 Abdolhamid Shariat Department of Neurology, Shiraz Neurosciences Research Center AND Clinical Neurology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. Ehsan Yaghoubi Department of Neurology, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran. Kamran Aghasadeghi Department of Neurology, Shiraz Neurosciences Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. Abbas Rahimi Department of Neurology, Clinical Neurology Research Center, Department of Neurology, Shiraz University of Medical Sciences, Shiraz, Iran. Reza Nemati Department of Cardiology, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran. Nahid Ashjazadeh Department Neurology, Shiraz Neurosciences Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. 2015 10 14 No Abstract https://ijnl.tums.ac.ir/index.php/ijnl/article/view/572 https://ijnl.tums.ac.ir/index.php/ijnl/article/download/572/230

Tehran University of Medical Sciences Current Journal of Neurology 2717-011X 14 1 2015 03 15 571 571