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Publisher: John Wiley and Sons   (Total: 1579 journals)

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Showing 1 - 200 of 1579 Journals sorted alphabetically
Abacus     Hybrid Journal   (Followers: 13, SJR: 0.48, h-index: 22)
About Campus     Hybrid Journal   (Followers: 5)
Academic Emergency Medicine     Hybrid Journal   (Followers: 65, SJR: 1.385, h-index: 91)
Accounting & Finance     Hybrid Journal   (Followers: 47, SJR: 0.547, h-index: 30)
ACEP NOW     Free   (Followers: 1)
Acta Anaesthesiologica Scandinavica     Hybrid Journal   (Followers: 52, SJR: 1.02, h-index: 88)
Acta Archaeologica     Hybrid Journal   (Followers: 156, SJR: 0.101, h-index: 9)
Acta Geologica Sinica (English Edition)     Hybrid Journal   (Followers: 3, SJR: 0.552, h-index: 41)
Acta Neurologica Scandinavica     Hybrid Journal   (Followers: 5, SJR: 1.203, h-index: 74)
Acta Obstetricia et Gynecologica Scandinavica     Hybrid Journal   (Followers: 15, SJR: 1.197, h-index: 81)
Acta Ophthalmologica     Hybrid Journal   (Followers: 6, SJR: 0.112, h-index: 1)
Acta Paediatrica     Hybrid Journal   (Followers: 56, SJR: 0.794, h-index: 88)
Acta Physiologica     Hybrid Journal   (Followers: 6, SJR: 1.69, h-index: 88)
Acta Polymerica     Hybrid Journal   (Followers: 9)
Acta Psychiatrica Scandinavica     Hybrid Journal   (Followers: 35, SJR: 2.518, h-index: 113)
Acta Zoologica     Hybrid Journal   (Followers: 6, SJR: 0.459, h-index: 29)
Acute Medicine & Surgery     Hybrid Journal   (Followers: 4)
Addiction     Hybrid Journal   (Followers: 35, SJR: 2.086, h-index: 143)
Addiction Biology     Hybrid Journal   (Followers: 13, SJR: 2.091, h-index: 57)
Adultspan J.     Hybrid Journal   (SJR: 0.127, h-index: 4)
Advanced Energy Materials     Hybrid Journal   (Followers: 27, SJR: 6.411, h-index: 86)
Advanced Engineering Materials     Hybrid Journal   (Followers: 26, SJR: 0.81, h-index: 81)
Advanced Functional Materials     Hybrid Journal   (Followers: 51, SJR: 5.21, h-index: 203)
Advanced Healthcare Materials     Hybrid Journal   (Followers: 14, SJR: 0.232, h-index: 7)
Advanced Materials     Hybrid Journal   (Followers: 265, SJR: 9.021, h-index: 345)
Advanced Materials Interfaces     Hybrid Journal   (Followers: 6, SJR: 1.177, h-index: 10)
Advanced Optical Materials     Hybrid Journal   (Followers: 6, SJR: 2.488, h-index: 21)
Advanced Science     Open Access   (Followers: 5)
Advanced Synthesis & Catalysis     Hybrid Journal   (Followers: 17, SJR: 2.729, h-index: 121)
Advances in Polymer Technology     Hybrid Journal   (Followers: 13, SJR: 0.344, h-index: 31)
Africa Confidential     Hybrid Journal   (Followers: 21)
Africa Research Bulletin: Economic, Financial and Technical Series     Hybrid Journal   (Followers: 13)
Africa Research Bulletin: Political, Social and Cultural Series     Hybrid Journal   (Followers: 10)
African Development Review     Hybrid Journal   (Followers: 33, SJR: 0.275, h-index: 17)
African J. of Ecology     Hybrid Journal   (Followers: 15, SJR: 0.477, h-index: 39)
Aggressive Behavior     Hybrid Journal   (Followers: 15, SJR: 1.391, h-index: 66)
Aging Cell     Open Access   (Followers: 11, SJR: 4.374, h-index: 95)
Agribusiness : an Intl. J.     Hybrid Journal   (Followers: 6, SJR: 0.627, h-index: 14)
Agricultural and Forest Entomology     Hybrid Journal   (Followers: 16, SJR: 0.925, h-index: 43)
Agricultural Economics     Hybrid Journal   (Followers: 45, SJR: 1.099, h-index: 51)
AIChE J.     Hybrid Journal   (Followers: 31, SJR: 1.122, h-index: 120)
Alcoholism and Drug Abuse Weekly     Hybrid Journal   (Followers: 7)
Alcoholism Clinical and Experimental Research     Hybrid Journal   (Followers: 7, SJR: 1.416, h-index: 125)
Alimentary Pharmacology & Therapeutics     Hybrid Journal   (Followers: 33, SJR: 2.833, h-index: 138)
Alimentary Pharmacology & Therapeutics Symposium Series     Hybrid Journal   (Followers: 3)
Allergy     Hybrid Journal   (Followers: 51, SJR: 3.048, h-index: 129)
Alternatives to the High Cost of Litigation     Hybrid Journal   (Followers: 3)
American Anthropologist     Hybrid Journal   (Followers: 143, SJR: 0.951, h-index: 61)
American Business Law J.     Hybrid Journal   (Followers: 24, SJR: 0.205, h-index: 17)
American Ethnologist     Hybrid Journal   (Followers: 90, SJR: 2.325, h-index: 51)
American J. of Economics and Sociology     Hybrid Journal   (Followers: 28, SJR: 0.211, h-index: 26)
American J. of Hematology     Hybrid Journal   (Followers: 33, SJR: 1.761, h-index: 77)
American J. of Human Biology     Hybrid Journal   (Followers: 12, SJR: 1.018, h-index: 58)
American J. of Industrial Medicine     Hybrid Journal   (Followers: 16, SJR: 0.993, h-index: 85)
American J. of Medical Genetics Part A     Hybrid Journal   (Followers: 16, SJR: 1.115, h-index: 61)
American J. of Medical Genetics Part B: Neuropsychiatric Genetics     Hybrid Journal   (Followers: 4, SJR: 1.771, h-index: 107)
American J. of Medical Genetics Part C: Seminars in Medical Genetics     Partially Free   (Followers: 6, SJR: 2.315, h-index: 79)
American J. of Physical Anthropology     Hybrid Journal   (Followers: 37, SJR: 1.41, h-index: 88)
American J. of Political Science     Hybrid Journal   (Followers: 269, SJR: 5.101, h-index: 114)
American J. of Primatology     Hybrid Journal   (Followers: 15, SJR: 1.197, h-index: 63)
American J. of Reproductive Immunology     Hybrid Journal   (Followers: 3, SJR: 1.347, h-index: 75)
American J. of Transplantation     Hybrid Journal   (Followers: 17, SJR: 2.792, h-index: 140)
American J. on Addictions     Hybrid Journal   (Followers: 9, SJR: 0.843, h-index: 57)
Anaesthesia     Hybrid Journal   (Followers: 134, SJR: 1.404, h-index: 88)
Analyses of Social Issues and Public Policy     Hybrid Journal   (Followers: 10, SJR: 0.397, h-index: 18)
Analytic Philosophy     Hybrid Journal   (Followers: 18)
Anatomia, Histologia, Embryologia: J. of Veterinary Medicine Series C     Hybrid Journal   (Followers: 3, SJR: 0.295, h-index: 27)
Anatomical Sciences Education     Hybrid Journal   (Followers: 1, SJR: 0.633, h-index: 24)
Andrologia     Hybrid Journal   (Followers: 2, SJR: 0.528, h-index: 45)
Andrology     Hybrid Journal   (Followers: 2, SJR: 0.979, h-index: 14)
Angewandte Chemie     Hybrid Journal   (Followers: 193)
Angewandte Chemie Intl. Edition     Hybrid Journal   (Followers: 218, SJR: 6.229, h-index: 397)
Animal Conservation     Hybrid Journal   (Followers: 39, SJR: 1.576, h-index: 62)
Animal Genetics     Hybrid Journal   (Followers: 8, SJR: 0.957, h-index: 67)
Animal Science J.     Hybrid Journal   (Followers: 6, SJR: 0.569, h-index: 24)
Annalen der Physik     Hybrid Journal   (Followers: 5, SJR: 1.46, h-index: 40)
Annals of Anthropological Practice     Partially Free   (Followers: 2, SJR: 0.187, h-index: 5)
Annals of Applied Biology     Hybrid Journal   (Followers: 7, SJR: 0.816, h-index: 56)
Annals of Clinical and Translational Neurology     Open Access   (Followers: 1)
Annals of Human Genetics     Hybrid Journal   (Followers: 9, SJR: 1.191, h-index: 67)
Annals of Neurology     Hybrid Journal   (Followers: 47, SJR: 5.584, h-index: 241)
Annals of Noninvasive Electrocardiology     Hybrid Journal   (Followers: 2, SJR: 0.531, h-index: 38)
Annals of Public and Cooperative Economics     Hybrid Journal   (Followers: 9, SJR: 0.336, h-index: 23)
Annals of the New York Academy of Sciences     Hybrid Journal   (Followers: 5, SJR: 2.389, h-index: 189)
Annual Bulletin of Historical Literature     Hybrid Journal   (Followers: 13)
Annual Review of Information Science and Technology     Hybrid Journal   (Followers: 14)
Anthropology & Education Quarterly     Hybrid Journal   (Followers: 25, SJR: 0.72, h-index: 31)
Anthropology & Humanism     Hybrid Journal   (Followers: 17, SJR: 0.137, h-index: 3)
Anthropology News     Hybrid Journal   (Followers: 15)
Anthropology of Consciousness     Hybrid Journal   (Followers: 11, SJR: 0.172, h-index: 5)
Anthropology of Work Review     Hybrid Journal   (Followers: 11, SJR: 0.256, h-index: 5)
Anthropology Today     Hybrid Journal   (Followers: 91, SJR: 0.545, h-index: 15)
Antipode     Hybrid Journal   (Followers: 48, SJR: 2.212, h-index: 69)
Anz J. of Surgery     Hybrid Journal   (Followers: 7, SJR: 0.432, h-index: 59)
Anzeiger für Schädlingskunde     Hybrid Journal   (Followers: 1)
Apmis     Hybrid Journal   (Followers: 1, SJR: 0.855, h-index: 73)
Applied Cognitive Psychology     Hybrid Journal   (Followers: 70, SJR: 0.754, h-index: 69)
Applied Organometallic Chemistry     Hybrid Journal   (Followers: 7, SJR: 0.632, h-index: 58)
Applied Psychology     Hybrid Journal   (Followers: 198, SJR: 1.023, h-index: 64)
Applied Psychology: Health and Well-Being     Hybrid Journal   (Followers: 49, SJR: 0.868, h-index: 13)
Applied Stochastic Models in Business and Industry     Hybrid Journal   (Followers: 5, SJR: 0.613, h-index: 24)
Aquaculture Nutrition     Hybrid Journal   (Followers: 14, SJR: 1.025, h-index: 55)
Aquaculture Research     Hybrid Journal   (Followers: 31, SJR: 0.807, h-index: 60)
Aquatic Conservation Marine and Freshwater Ecosystems     Hybrid Journal   (Followers: 36, SJR: 1.047, h-index: 57)
Arabian Archaeology and Epigraphy     Hybrid Journal   (Followers: 11, SJR: 0.453, h-index: 11)
Archaeological Prospection     Hybrid Journal   (Followers: 12, SJR: 0.922, h-index: 21)
Archaeology in Oceania     Hybrid Journal   (Followers: 13, SJR: 0.745, h-index: 18)
Archaeometry     Hybrid Journal   (Followers: 27, SJR: 0.809, h-index: 48)
Archeological Papers of The American Anthropological Association     Hybrid Journal   (Followers: 15, SJR: 0.156, h-index: 2)
Architectural Design     Hybrid Journal   (Followers: 25, SJR: 0.261, h-index: 9)
Archiv der Pharmazie     Hybrid Journal   (Followers: 4, SJR: 0.628, h-index: 43)
Archives of Drug Information     Hybrid Journal   (Followers: 5)
Archives of Insect Biochemistry and Physiology     Hybrid Journal   (SJR: 0.768, h-index: 54)
Area     Hybrid Journal   (Followers: 12, SJR: 0.938, h-index: 57)
Art History     Hybrid Journal   (Followers: 243, SJR: 0.153, h-index: 13)
Arthritis & Rheumatology     Hybrid Journal   (Followers: 50, SJR: 1.984, h-index: 20)
Arthritis Care & Research     Hybrid Journal   (Followers: 26, SJR: 2.256, h-index: 114)
Artificial Organs     Hybrid Journal   (Followers: 1, SJR: 0.872, h-index: 60)
ASHE Higher Education Reports     Hybrid Journal   (Followers: 14)
Asia & the Pacific Policy Studies     Open Access   (Followers: 16)
Asia Pacific J. of Human Resources     Hybrid Journal   (Followers: 321, SJR: 0.494, h-index: 19)
Asia Pacific Viewpoint     Hybrid Journal   (Followers: 1, SJR: 0.616, h-index: 26)
Asia-Pacific J. of Chemical Engineering     Hybrid Journal   (Followers: 8, SJR: 0.345, h-index: 20)
Asia-pacific J. of Clinical Oncology     Hybrid Journal   (Followers: 6, SJR: 0.554, h-index: 14)
Asia-Pacific J. of Financial Studies     Hybrid Journal   (SJR: 0.241, h-index: 7)
Asia-Pacific Psychiatry     Hybrid Journal   (Followers: 4, SJR: 0.377, h-index: 7)
Asian Economic J.     Hybrid Journal   (Followers: 8, SJR: 0.234, h-index: 21)
Asian Economic Policy Review     Hybrid Journal   (Followers: 4, SJR: 0.196, h-index: 12)
Asian J. of Control     Hybrid Journal   (SJR: 0.862, h-index: 34)
Asian J. of Endoscopic Surgery     Hybrid Journal   (SJR: 0.394, h-index: 7)
Asian J. of Organic Chemistry     Hybrid Journal   (Followers: 5, SJR: 1.443, h-index: 19)
Asian J. of Social Psychology     Hybrid Journal   (Followers: 5, SJR: 0.665, h-index: 37)
Asian Politics and Policy     Hybrid Journal   (Followers: 12, SJR: 0.207, h-index: 7)
Asian Social Work and Policy Review     Hybrid Journal   (Followers: 5, SJR: 0.318, h-index: 5)
Asian-pacific Economic Literature     Hybrid Journal   (Followers: 5, SJR: 0.168, h-index: 15)
Assessment Update     Hybrid Journal   (Followers: 4)
Astronomische Nachrichten     Hybrid Journal   (Followers: 2, SJR: 0.701, h-index: 40)
Atmospheric Science Letters     Open Access   (Followers: 29, SJR: 1.332, h-index: 27)
Austral Ecology     Hybrid Journal   (Followers: 15, SJR: 1.095, h-index: 66)
Austral Entomology     Hybrid Journal   (Followers: 9, SJR: 0.524, h-index: 28)
Australasian J. of Dermatology     Hybrid Journal   (Followers: 8, SJR: 0.714, h-index: 40)
Australasian J. On Ageing     Hybrid Journal   (Followers: 6, SJR: 0.39, h-index: 22)
Australian & New Zealand J. of Statistics     Hybrid Journal   (Followers: 14, SJR: 0.275, h-index: 28)
Australian Accounting Review     Hybrid Journal   (Followers: 4, SJR: 0.709, h-index: 14)
Australian and New Zealand J. of Family Therapy (ANZJFT)     Hybrid Journal   (Followers: 3, SJR: 0.382, h-index: 12)
Australian and New Zealand J. of Obstetrics and Gynaecology     Hybrid Journal   (Followers: 47, SJR: 0.814, h-index: 49)
Australian and New Zealand J. of Public Health     Hybrid Journal   (Followers: 11, SJR: 0.82, h-index: 62)
Australian Dental J.     Hybrid Journal   (Followers: 7, SJR: 0.482, h-index: 46)
Australian Economic History Review     Hybrid Journal   (Followers: 4, SJR: 0.171, h-index: 12)
Australian Economic Papers     Hybrid Journal   (Followers: 29, SJR: 0.23, h-index: 9)
Australian Economic Review     Hybrid Journal   (Followers: 6, SJR: 0.357, h-index: 21)
Australian Endodontic J.     Hybrid Journal   (Followers: 3, SJR: 0.513, h-index: 24)
Australian J. of Agricultural and Resource Economics     Hybrid Journal   (Followers: 3, SJR: 0.765, h-index: 36)
Australian J. of Grape and Wine Research     Hybrid Journal   (Followers: 5, SJR: 0.879, h-index: 56)
Australian J. of Politics & History     Hybrid Journal   (Followers: 14, SJR: 0.203, h-index: 14)
Australian J. of Psychology     Hybrid Journal   (Followers: 18, SJR: 0.384, h-index: 30)
Australian J. of Public Administration     Hybrid Journal   (Followers: 406, SJR: 0.418, h-index: 29)
Australian J. of Rural Health     Hybrid Journal   (Followers: 5, SJR: 0.43, h-index: 34)
Australian Occupational Therapy J.     Hybrid Journal   (Followers: 72, SJR: 0.59, h-index: 29)
Australian Psychologist     Hybrid Journal   (Followers: 12, SJR: 0.331, h-index: 31)
Australian Veterinary J.     Hybrid Journal   (Followers: 20, SJR: 0.459, h-index: 45)
Autism Research     Hybrid Journal   (Followers: 34, SJR: 2.126, h-index: 39)
Autonomic & Autacoid Pharmacology     Hybrid Journal   (SJR: 0.371, h-index: 29)
Banks in Insurance Report     Hybrid Journal   (Followers: 1)
Basic & Clinical Pharmacology & Toxicology     Hybrid Journal   (Followers: 11, SJR: 0.539, h-index: 70)
Basic and Applied Pathology     Open Access   (Followers: 2, SJR: 0.113, h-index: 4)
Basin Research     Hybrid Journal   (Followers: 5, SJR: 1.54, h-index: 60)
Bauphysik     Hybrid Journal   (Followers: 2, SJR: 0.194, h-index: 5)
Bauregelliste A, Bauregelliste B Und Liste C     Hybrid Journal  
Bautechnik     Hybrid Journal   (Followers: 1, SJR: 0.321, h-index: 11)
Behavioral Interventions     Hybrid Journal   (Followers: 9, SJR: 0.297, h-index: 23)
Behavioral Sciences & the Law     Hybrid Journal   (Followers: 24, SJR: 0.736, h-index: 57)
Berichte Zur Wissenschaftsgeschichte     Hybrid Journal   (Followers: 9, SJR: 0.11, h-index: 5)
Beton- und Stahlbetonbau     Hybrid Journal   (Followers: 2, SJR: 0.493, h-index: 14)
Biochemistry and Molecular Biology Education     Hybrid Journal   (Followers: 6, SJR: 0.311, h-index: 26)
Bioelectromagnetics     Hybrid Journal   (Followers: 1, SJR: 0.568, h-index: 64)
Bioengineering & Translational Medicine     Open Access  
BioEssays     Hybrid Journal   (Followers: 10, SJR: 3.104, h-index: 155)
Bioethics     Hybrid Journal   (Followers: 14, SJR: 0.686, h-index: 39)
Biofuels, Bioproducts and Biorefining     Hybrid Journal   (Followers: 1, SJR: 1.725, h-index: 56)
Biological J. of the Linnean Society     Hybrid Journal   (Followers: 16, SJR: 1.172, h-index: 90)
Biological Reviews     Hybrid Journal   (Followers: 4, SJR: 6.469, h-index: 114)
Biologie in Unserer Zeit (Biuz)     Hybrid Journal   (Followers: 42, SJR: 0.12, h-index: 1)
Biology of the Cell     Full-text available via subscription   (Followers: 9, SJR: 1.812, h-index: 69)
Biomedical Chromatography     Hybrid Journal   (Followers: 6, SJR: 0.572, h-index: 49)
Biometrical J.     Hybrid Journal   (Followers: 5, SJR: 0.784, h-index: 44)
Biometrics     Hybrid Journal   (Followers: 36, SJR: 1.906, h-index: 96)
Biopharmaceutics and Drug Disposition     Hybrid Journal   (Followers: 10, SJR: 0.715, h-index: 44)
Biopolymers     Hybrid Journal   (Followers: 18, SJR: 1.199, h-index: 104)
Biotechnology and Applied Biochemistry     Hybrid Journal   (Followers: 45, SJR: 0.415, h-index: 55)
Biotechnology and Bioengineering     Hybrid Journal   (Followers: 160, SJR: 1.633, h-index: 146)
Biotechnology J.     Hybrid Journal   (Followers: 14, SJR: 1.185, h-index: 51)
Biotechnology Progress     Hybrid Journal   (Followers: 39, SJR: 0.736, h-index: 101)
Biotropica     Hybrid Journal   (Followers: 20, SJR: 1.374, h-index: 71)
Bipolar Disorders     Hybrid Journal   (Followers: 9, SJR: 2.592, h-index: 100)
Birth     Hybrid Journal   (Followers: 38, SJR: 0.763, h-index: 64)
Birth Defects Research Part A : Clinical and Molecular Teratology     Hybrid Journal   (Followers: 2, SJR: 0.727, h-index: 77)
Birth Defects Research Part B: Developmental and Reproductive Toxicology     Hybrid Journal   (Followers: 6, SJR: 0.468, h-index: 47)
Birth Defects Research Part C : Embryo Today : Reviews     Hybrid Journal   (SJR: 1.513, h-index: 55)
BJOG : An Intl. J. of Obstetrics and Gynaecology     Partially Free   (Followers: 241, SJR: 2.083, h-index: 125)

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Journal Cover Acta Neurologica Scandinavica
  [SJR: 1.203]   [H-I: 74]   [5 followers]  Follow
   Hybrid Journal Hybrid journal (It can contain Open Access articles)
   ISSN (Print) 0001-6314 - ISSN (Online) 1600-0404
   Published by John Wiley and Sons Homepage  [1579 journals]
  • Visual field defect after ischemic stroke—impact on mortality
    • Authors: K. M. Sand; H. Næss, L. Thomassen, J. M. Hoff
      Abstract: ObjectivesWe aimed to investigate the impact of visual field defects (VFD) on mortality in ischemic stroke patients.Materials and methodsAll patients with acute infarction and a clinically detected VFD from February 2006 to December 2013 in the NORSTROKE Registry (n = 506) were included and compared with ischemic stroke patients with normal visual fields (n = 2041). A record of patients who had died per ultimo April 2015 was obtained from the central registry at Haukeland University Hospital.ResultsPatients with VFD were significantly older (75.0 vs 69.8, P 
      PubDate: 2017-11-17T04:35:54.853255-05:
      DOI: 10.1111/ane.12870
  • Plasma copeptin levels in patients with restless legs syndrome
    • Authors: S. Topaloglu Tuac; S. Ozben, M. Koseoglu Bitnel, A. Koksal, E. Kucuksayan, A. Hanikoğlu, T. Ozben
      Abstract: ObjectivesCopeptin, the C-terminal fragment of antidiuretic hormone, is a new biomarker that has been found to be elevated in several disorders and could be related with prognosis. This study aimed to compare plasma copeptin levels in patients with restless legs syndrome (RLS) with healthy individuals and to investigate whether plasma copeptin levels were associated with the severity of disease.Material and methods41 patients with primary RLS, who were followed in Bakirkoy Psychiatry and Neurology Research and Training Hospital and 41 age- and sex-matched healthy individuals were included into the study. RLS patients were divided into subgroups as mild-moderate, severe, and very severe according to the severity of symptoms. Sleep quality and excessive daytime sleepiness were determined according to Pittsburgh Sleep Quality Index and Epworth Sleepiness Scale, respectively, and sleep quality scores were analyzed statistically among the groups divided according to disease severity. Copeptin levels in all the patients were compared to the controls. RLS subgroups were compared with each other to evaluate association between copeptin levels and disease severity.ResultsPlasma copeptin levels in RLS patients were significantly higher than controls (P 
      PubDate: 2017-11-17T04:31:16.524851-05:
      DOI: 10.1111/ane.12866
  • Late age onset of amyotrophic lateral sclerosis is often not considered in
           elderly people
    • Authors: E. Broussalis; S. Grinzinger, A. B. Kunz, M. Killer-Oberpfalzer, E. Haschke-Becher, H.-P. Hartung, J. Kraus
      Abstract: IntroductionAmyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease causing an upper and lower motor neuron loss. It is neurology textbook knowledge that the mean age of onset is about 60 years. However, recent investigations show an increasing incidence in older persons. We therefore evaluated whether ALS is potentially not considered in elderly people with ALS symptoms, respectively, not recognized.Materials and MethodsWe included retrospectively all patients with ALS diagnoses after work-up that were admitted to our neurological and geriatric departments from 2007 to 2010 and collected their clinical data. The diagnosis of ALS was based on the El Escorial criteria. Patients were grouped into three categories according to age (70), and differences in clinical and/ or biographical factors were investigated.ResultsWe identified 35 patients (18 men and 17 women) with a median age at onset of 71.5 years (range: 36-87 years). When establishing the diagnosis, 51% were older than 70 years, 40% (14/35) between 50 and 70, and only 9% younger than 50. Only in 46 per cent of patients who were sent to our departments with ALS symptoms ALS was considered by the referring physician.ConclusionLate age onset of ALS seems to be more common than formerly assumed and is presumably under-recognized in elderly patients. ALS needs to be considered as a differential diagnosis in older patients. Potential factors accounting for older people being underdiagnosed with ALS relate to frequent presentation with symptoms like dysphagia, frailty or general weakness for other reasons.
      PubDate: 2017-11-17T04:30:29.972107-05:
      DOI: 10.1111/ane.12869
  • Polyglucosan myopathy and functional characterization of a novel GYG1
    • Authors: C. Hedberg-Oldfors; A. Mensch, K. Visuttijai, G. Stoltenburg, D. Stoevesandt, T. Kraya, A. Oldfors, S. Zierz
      Abstract: ObjectivesDisorders of glycogen metabolism include rare hereditary muscle glycogen storage diseases with polyglucosan, which are characterized by storage of abnormally structured glycogen in muscle in addition to exercise intolerance or muscle weakness. In this study, we investigated the etiology and pathogenesis of a late-onset myopathy associated with glycogenin-1 deficiency.Materials and methodsA family with two affected siblings, 64- and 66-year-olds, was studied. Clinical examination and whole-body MRI revealed weakness and wasting in the hip girdle and proximal leg muscles affecting ambulation in the brother. The sister had weakness and atrophy of hands and slight foot dorsiflexion difficulties. Muscle biopsy and whole-exome sequencing were performed in both cases to identify and characterize the pathogenesis including the functional effects of identified mutations.ResultsBoth siblings demonstrated storage of glycogen that was partly resistant to alpha-amylase digestion. Both were heterozygous for two mutations in GYG1, one truncating 1-base deletion (c.484delG; p.Asp163Thrfs*5) and one novel missense mutation (c.403G>A; p.Gly135Arg). The mutations caused reduced expression of glycogenin-1 protein, and the missense mutation abolished the enzymatic function as analyzed by an in vitro autoglucosylation assay.ConclusionWe present functional evidence for the pathogenicity of a novel GYG1 missense mutation located in the substrate binding domain. Our results also demonstrate that glycogenin-1 deficiency may present with highly variable distribution of weakness and wasting also in the same family.
      PubDate: 2017-11-15T23:01:29.598916-05:
      DOI: 10.1111/ane.12865
  • Brain signal intensity changes as biomarkers in amyotrophic lateral
    • Authors: Juan F. Vázquez-Costa; Miguel Mazón, Joan Carreres-Polo, David Hervás, Jordi Pérez-Tur, Luis Martí-Bonmatí, Teresa Sevilla
      Abstract: ObjectivesTo evaluate the contribution of the demographical, clinical, analytical and genetic factors to brain signal intensity changes in T2-weighted MR images in amyotrophic lateral sclerosis (ALS) patients and controls.MethodsSusceptibility-weighted and FLAIR sequences were obtained in a 3T MR scanner. Iron-related hypointensities in the motor cortex (IRhMC) and hyperintensities of the corticospinal tract (HCT) were qualitatively scored. Age, gender, family history and clinical variables were recorded. Baseline levels of ferritin were measured. C9orf72 was tested in all patients and SOD1 only in familial ALS patients not carrying a C9orf72 expansion. Patients who carried a mutation were categorized as genetic. Associations of these variables with visual scores were assessed with multivariable analysis.ResultsA total of 102 ALS patients (92 non-genetic and 10 genetic) and 48 controls (28 ALS mimics and 20 healthy controls) were recruited. In controls, IRhMC associated with age, but HCT did not. In ALS patients, both HTC and IRhMC strongly associated with clinical UMN impairment and bulbar onset. The intensity/extent of IRhMC in the different motor homunculus regions (lower limbs, upper limbs and bulbar) were linked to the symptoms onset site. Between genetic and sporadic patients, no difference in IRhMC and HCT was found.ConclusionsIRhMC and HCT are reliable markers of UMN degeneration in ALS patients and are more frequent in bulbar onset patients, independently of the mutation status. Age should be considered when evaluating IRhMC. The regional measurement of IRhMC following the motor homunculus could be used as a measure of disease progression.
      PubDate: 2017-10-29T21:50:32.030542-05:
      DOI: 10.1111/ane.12863
  • Combining olfactory test and motion analysis sensors in Parkinson's
           disease preclinical diagnosis: a pilot study
    • Authors: C. Maremmani; F. Cavallo, C. Purcaro, G. Rossi, S. Salvadori, E. Rovini, D. Esposito, A. Pieroni, S. Ramat, P. Vanni, B. Fattori, G. Meco
      Abstract: ObjectivesPreclinical diagnosis of Parkinson's disease (PD) is nowadays a topic of interest as the neuropathological process could begin years before the appearance of motor symptoms. Several symptoms, among them hyposmia, could precede motor features in PD. In the preclinical phase of PD, a subclinical reduction in motor skills is highly likely. In this pilot study, we investigate a step-by-step method to achieve preclinical PD diagnosis.Material and methodsWe used the IOIT (Italian Olfactory Identification Test) to screen a population of healthy subjects. We identified 20 subjects with idiopathic hyposmia. Hyposmic subjects underwent an evaluation of motor skills, at baseline and after 1 year, using motion analysis sensors previously created by us.ResultsOne subject showed significant worsening in motor measurements. In this subject, we further conducted a dopaminergic challenge test monitored with the same sensors and, finally, he underwent [123I]-FP/CIT (DaTscan) SPECT brain imaging. The results show that he is probably affected by preclinical PD.ConclusionsOur pilot study suggests that the combined use of an olfactory test and motor sensors for motion analysis could be useful for a screening of healthy subjects to identify those at a high risk of developing PD.
      PubDate: 2017-10-29T21:45:49.186058-05:
      DOI: 10.1111/ane.12862
  • Contrast-enhanced sonothrombolysis in acute ischemic stroke patients
           without intracranial large-vessel occlusion
    • Authors: C. E. Kvistad; A. Nacu, V. Novotny, N. Logallo, U. Waje-Andreassen, H. Naess, L. Thomassen
      Abstract: BackgroundContrast-enhanced sonothrombolysis (CEST) leads to a more rapid recanalization in acute ischemic stroke caused by intracranial large-vessel occlusion (LVO). Animal studies have shown that CEST also may be safe and efficient in treating the ischemic microcirculation in the absence of LVO. The exact mechanism behind this treatment effect is not known. We aimed to assess safety and efficacy of CEST in acute ischemic stroke patients included in the Norwegian Sonothrombolysis in Acute Stroke Study (NOR-SASS) without LVO on admission CT angiography (CTA).MethodsNOR-SASS was a randomized controlled trial of CEST in ischemic stroke patients treated with intravenous thrombolysis within 4.5 hours after stroke onset. Patients were randomized to either CEST or sham CEST. In this study, patients were excluded if they had partial or total occlusion on admission CTA, ultrasound-resistant bone window, had received CEST with incorrect insonation as compared to stroke location on Magnetic resonance imaging (MRI), or were stroke mimics.ResultsOf the 183 patients included in NOR-SASS, a total of 83 (45.4%) patients matched the inclusion criteria, of which 40 received CEST and 43 sham CEST. There were no patients with symptomatic intracranial hemorrhage (sICH) in the CEST group. Rates of asymptomatic ICH, microbleeds, and mortality were not increased in the CEST group. Neurological improvement at 24 hours and functional outcome at 90 days were similar in both groups.ConclusionCEST is safe in ischemic stroke patients without intracranial LVO. There were no differences in clinical outcomes between the treatment groups.
      PubDate: 2017-10-25T05:00:29.174217-05:
      DOI: 10.1111/ane.12861
  • Point-of-care versus central laboratory testing of INR in acute stroke
    • Authors: R. Zenlander; M. von Euler, J. Antovic, A. Berglund
      Abstract: ObjectivesEffective anticoagulant therapy is a contraindication to thrombolysis, which is an effective treatment of ischemic stroke if given within 4.5 hours of symptom onset. INR above 1.7 is generally considered a contraindication for thrombolysis. Rapid measurement of INR in warfarin-treated patients is therefore of major importance in order to be able to decide on thrombolysis or not. We asked whether INR measured on a point-of-care instrument would be as good as a central laboratory instrument.Material and MethodsA total of 529 consecutive patients who arrived at the emergency department at a large urban teaching hospital with stroke symptoms were enrolled in the study. INR was measured with a CoaguChek and a Sysmex instrument. Basic clinical information such as age, sex, and diagnosis (if available) was recorded. INR from the instruments was compared using linear regression and Bland-Altman plot.ResultsOf 529 patients, 459 had INR results from both instruments. Among these, 3 patients were excluded as outliers. The rest (n = 456) showed good correlation between the methods (R2 = 0.97). In the current setting, CoaguChek was in median 63 minutes faster than Sysmex.ConclusionOur results indicate that point-of-care testing is a safe mean to rapidly acquire a patient's INR value in acute clinical situations.
      PubDate: 2017-10-25T04:55:39.104064-05:
      DOI: 10.1111/ane.12860
  • Disturbance of verticality perception and postural dysfunction in
           Parkinson's disease
    • Authors: K. A. Schindlbeck; W. Naumann, A. Maier, F. Ehlen, F. Marzinzik, F. Klostermann
      Abstract: ObjectivesVerticality perception is known to be abnormal in Parkinson's disease (PD), but in which stage respective dysfunctions arise and how they relate to postural disorders remains to be settled. These issues were studied with respect to different dimensions of the subjective visual vertical (SVV) in relation to clinical parameters of postural control.Materials & MethodsAll participants had to orientate a luminous line at random planar orientations to a strictly vertical position using an automated operator system. The SVV was analyzed in 58 PD patients and 28 control subjects with respect to (i) the angle between true and subjective vertical (deviation) and (ii) the variability of this across five measurements (variability). Results were referred to the subjective upright head position (SUH), the disease stage, and clinical gait/balance features assessed by the MDS-UPDRS and the Tinetti test.ResultsParkinson's disease patients had significantly higher SVV deviation and variability than controls. With respect to disease stage, deviation developed before abnormal variability. SVV variability was associated with poor balance and gait performance, as well as postural instability. Deficits in SUH and SVV deviation were correlated and mostly unidirectional, but did not correspond to the side of motor symptom dominance.ConclusionsVisual verticality perception in PD is deviated already in early stages, conceivably as a relatively static internal misrepresentation of object orientation. Variability about verticality perception emerges in more advanced stages and is associated with postural and balance abnormalities.
      PubDate: 2017-10-23T23:24:18.984488-05:
      DOI: 10.1111/ane.12859
  • Experience with MAD on children with epilepsy in Egypt after classic KD
    • Authors: O. F. El Rashidy; M. F. Nassar, Y. G. El Gendy, S. M. Deifalla, S. Gaballa
      Abstract: Background and aimModified Atkins diet (MAD) is a less restrictive type of ketogenic diet (KD) as compared to the classic one. The aim of this study was to evaluate the impact of 9 months MAD treatment on the growth and seizure control in patients with intractable epilepsy as well as the quality of life (QoL) of their mothers.Subjects and MethodsThe study included 15 patients with intractable epilepsy who could not tolerate their classic KD management plan. From the 15 recruited cases, only seven patients completed this nine months prospective study. After neurological reassessment, the patients were prescribed MAD tailored from the local Egyptian ingredients. Arabic translation of the WHO Quality of life Instruments (WHOQOL-BREF) scale was calculated for the mothers initially and 9 months later. Daily seizure frequency with severity assessment by Chalfont scale was recorded as well as monthly weight and length throughout the study period.ResultsMothers ‘QoL in all WHOQOL-BREF domains significantly improved (P 
      PubDate: 2017-10-16T06:21:09.797826-05:
      DOI: 10.1111/ane.12856
  • The HMGB1 is increased in CSF of patients with an Anti-NMDAR encephalitis
    • Authors: P. Ai; X. Zhang, Z. Xie, G. Liu, X. Liu, S. Pan, H. Wang
      Abstract: BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system (CNS). Interleukin (IL)-6 and IL-17A may play important roles in the pathogenesis of this disease. High-mobility group box protein 1 (HMGB1), a small but highly conserved ubiquitous protein, is recognized to be a potent innate inflammatory mediator that can activate the nuclear factor light chain enhancer of activated B cells and release cytokines such as IL-6 and IL-17A when released extracellularly. However, whether cerebrospinal fluid (CSF) HMGB1 levels are altered in anti-NMDAR encephalitis is still unclear.ObjectiveThe aim of this study was to determine whether a correlation exists between the CSF concentrations of HMGB1 and IL-6 and IL-17A in anti-NMDAR encephalitis patients. We also sought to assess whether HMGB1 influences the clinical outcomes in anti-NMDAR encephalitis patients.MethodsThirty-three patients with anti-NMDAR antibodies and 38 controls were recruited. CSF HMGB1 was measured using an enzyme-linked immunosorbent assay. The main clinical outcomes were evaluated using the modified Rankin scale (mRS). The data were extracted using microarray analysis software.Results and ConclusionOur results showed significant increases in CSF HMGB1, IL-6, and IL-17A (P 
      PubDate: 2017-10-12T03:45:46.048872-05:
      DOI: 10.1111/ane.12850
  • Intermittent gait disturbance in idiopathic normal pressure hydrocephalus
    • Authors: Y. Nikaido; Y. Kajimoto, A. Tucker, K. Kuroda, H. Ohno, T. Akisue, R. Saura, T. Kuroiwa
      Abstract: ObjectivesWe identified intermittent gait disturbance (IGD) observed in the mild stage of idiopathic normal pressure hydrocephalus (iNPH). The first purpose of this study was to clarify the temporal gait profile of IGD during long-distance gait. The second purpose was to confirm the difference in treatment effect after cerebrospinal fluid (CSF) shunting in patients with and without IGD.Materials and MethodsFourteen consecutive iNPH patients with mild gait disturbance with a timed up-and-go (TUG) of
      PubDate: 2017-10-11T20:45:48.678841-05:
      DOI: 10.1111/ane.12853
  • Efficacy of the Danish epilepsy surgery programme
    • Authors: E. Holm; M. T. Foged, S. Beniczky, B. Jespersen, J. Brennum, L. H. Pinborg
      Abstract: ObjectiveDespite optimal medical treatment, approximately one-third of patients with epilepsy continue to have seizures. Epilepsy surgery is widely accepted as a therapeutic option in the selected subset of patients with drug-resistant focal epilepsy. Here, we report the results of the Danish epilepsy surgery programme from 2009 to 2014.Material and methodsA total of 169 consecutive patients, operated at Rigshospitalet, were included. Information was gathered from digital patient records. Before 1-year follow-up, two patients were lost to follow-up and three were referred to new surgery.ResultsThe median years of drug resistance before operation were 11 years. At 1-year follow-up (n = 164), seizure outcomes were as follows: 65% Engel I (free from disabling seizures), 51% Engel IA (completely seizure free) and 9% Engel IV (no worthwhile improvement), and for patients operated in the medial temporal lobe (n = 114): 70% Engel I, 56% Engel IA, 5% Engel IV. The outcomes of the 53 patients needing intracranial EEG recording (ICR) were not significantly different from the patients only evaluated with surface EEG. None of the eight MRI-negative patients operated outside the medial temporal lobe after ICR were free of disabling seizures. 12% of MTLE patients developed de novo depression after epilepsy surgery despite good surgical outcome. Three patients required rehabilitation due to post-operative hemiplegia.ConclusionThe outcomes of the Danish epilepsy surgery programme align with international results found in recent meta-analyses. Serious complications to epilepsy surgery are seldom. In accordance with international recommendations, Danish drug-resistant patients should be referred to epilepsy surgery evaluation at an earlier stage of the disease.
      PubDate: 2017-10-10T04:20:38.041499-05:
      DOI: 10.1111/ane.12857
  • Self-assessments and determinants of HRQoL in a German MS population
    • Authors: S. Nickel; O. von dem Knesebeck, C. Kofahl
      Abstract: ObjectivesThe importance of health-related quality of life (HRQoL) in multiple sclerosis (MS) is widely acknowledged. In 2015, a multicenter study was launched to assess the HRQoL and coping styles of chronically ill persons in Germany. The aims of this paper were (i) to describe how persons affected by MS assess their HRQoL and (ii) find out sociodemographic and disease-specific determinants of HRQoL.Materials and methodsA quantitative survey (optionally per paper-pencil or online) was conducted between March and October 2015 in Germany. Recruitment ways were as follows: Associations of the German Multiple Sclerosis Society (DMSG), medical practices specialized in neurology/MS, hospitals, public events, social networks, and self-help clearinghouses. To measure HRQoL, the Multiple Sclerosis International Quality of Life (MusiQoL) questionnaire and the short form of the Fear of Progression Questionnaire (FoP-Q) were used.ResultsA total of 1220 individuals with MS participated in the study. Frequent problems reported were related to activities of daily living as well as sentimental and sexual life. Multiple regression analyses identified disease severity and comorbidity—in different directions (positive and negative)—as the strongest factors in predicting activities of daily living, symptoms, sentimental and sexual life, and reject. The demographic variables sex, age, education, and employment status also have different impact on the HRQoL.ConclusionsUsing HRQoL measures can be feasible outcomes in addition to clinical assessments of MS and other chronic diseases and can help better managing the care. Some challenges have to be taken into account involving patients and the limitations of our empirical study.
      PubDate: 2017-10-03T23:00:45.670623-05:
      DOI: 10.1111/ane.12854
  • APOL1, CDKN2A/CDKN2B, and HDAC9 polymorphisms and small vessel ischemic
    • Authors: R. Akinyemi; H. K. Tiwari, D. K. Arnett, B. Ovbiagele, M. R. Irvin, K. Wahab, F. Sarfo, V. Srinivasasainagendra, A. Adeoye, R. T. Perry, A. Akpalu, C. Jenkins, O. Arulogun, M. Gebregziabher, L. Owolabi, R. Obiako, E. Sanya, M. Komolafe, M. Fawale, P. Adebayo, G. Osaigbovo, T. Sunmonu, P. Olowoyo, I. Chukwuonye, Y. Obiabo, A. Onoja, J. Akinyemi, G. Ogbole, S. Melikam, R. Saulson, M. Owolabi,
      Abstract: ObjectiveWorldwide, the highest frequencies of APOL1-associated kidney variants are found in indigenous West Africans among whom small vessel disease (SVD) ischemic stroke is the most common stroke phenotype. The objective of this study was to investigate the association and effect sizes of 23 selected SNPs in 14 genes of relevance, including the APOL1 G1 variants, with the occurrence of SVD ischemic stroke among indigenous West African participants in the Stroke Investigative Research and Education Network (SIREN) Study.Materials and MethodsCases were consecutively recruited consenting adults (aged 18 years or older) with neuroimaging—confirmed first clinical stroke. Stroke-free controls were ascertained using a locally validated version of the Questionnaire for Verifying Stroke-Free Status (QVSFS). Logistic regression models adjusting for known vascular risk factors were fitted to assess the associations of the 23 SNPs in rigorously phenotyped cases (N = 154) of SVD ischemic stroke and stroke-free (N = 483) controls.ResultsApolipoprotein L1 (APOL1) rs73885319 (OR = 1.52; CI: 1.09-2.13, P-value = .013), rs2383207 in CDKN2A/CDKN2B (OR = 3.08; CI: 1.15-8.26, P –value = .026) and rs2107595 (OR = 1.70; CI: 1.12-2.60, P-value = .014) and rs28688791 (OR = 1.52; CI: 1.03-2.26, P-value = .036) in HDAC9 gene were associated with SVD stroke at 0.05 significance level. Polymorphisms in other genes did not show significant associations.ConclusionThis is the first report of a specific association of APOL1 with a stroke subtype. Further research is needed to confirm these initial findings and deepen understanding of the genetics of stroke in people of African ancestry with possible implications for other ancestries as all humans originated from Africa.
      PubDate: 2017-10-03T23:00:28.114745-05:
      DOI: 10.1111/ane.12847
  • Transcranial magnetic stimulation studies in complex regional pain
           syndrome type I: A review
    • Authors: R. Nardone; F. Brigo, Y. Höller, L. Sebastianelli, V. Versace, L. Saltuari, P. Lochner, E. Trinka
      Abstract: The sensory and motor cortical representation corresponding to the affected limb is altered in patients with complex regional pain syndrome (CRPS). Transcranial magnetic stimulation (TMS) represents a useful non-invasive approach for studying cortical physiology. If delivered repetitively, TMS can also modulate cortical excitability and induce long-lasting neuroplastic changes. In this review, we performed a systematic search of all studies using TMS to explore cortical excitability/plasticity and repetitive TMS (rTMS) for the treatment of CRPS. Literature searches were conducted using PubMed and EMBASE. We identified 8 articles matching the inclusion criteria. One hundred fourteen patients (76 females and 38 males) were included in these studies. Most of them have applied TMS in order to physiologically characterize CRPS type I. Changes in motor cortex excitability and brain mapping have been reported in CRPS-I patients. Sensory and motor hyperexcitability are in the most studies bilateral and likely involve corresponding regions within the central nervous system rather than the entire hemisphere. Conversely, sensorimotor integration and plasticity were found to be normal in CRPS-I. TMS examinations also revealed that the nature of motor dysfunction in CRPS-I patients differs from that observed in patients with functional movement disorders, limb immobilization, or idiopathic dystonia. TMS studies may thus lead to the implementation of correct rehabilitation strategies in CRPS-I patients. Two studies have begun to therapeutically use rTMS. This non-invasive brain stimulation technique could have therapeutic utility in CRPS, but further well-designed studies are needed to corroborate initial findings.
      PubDate: 2017-10-03T02:15:04.787285-05:
      DOI: 10.1111/ane.12852
  • Plasma level of club-cell (CC-16) predicts outcome in amyotrophic lateral
    • Authors: A. C. Pronto-Laborinho; M. Gromicho, M. Pereira, S. Pinto, M. do A. Barros, M. Swash, M. Carvalho
      Abstract: ObjectivesThe club cell protein (CC-16) is a biomarker associated with respiratory distress and pulmonary inflammation. We evaluated CC-16 as a candidate biomarker for respiratory failure in amyotrophic lateral sclerosis (ALS).Materials and MethodsWe studied 81 ALS patients and 30 matched controls. We used an ALS-related measure of functional capacity, and tested forced vital capacity (FVC) and the amplitude of the diaphragmatic response by phrenic nerve stimulation (PhrenAmpl). Plasma CC-16 levels were measured in venous blood. Kaplan-Meier survival curves were plotted to evaluate risk to non-invasive ventilation and death in patients with abnormal CC-16 levels.ResultsCC-16 levels were significantly raised in ALS patients (10.56 ng/mL ± 6.84 vs 8.34 ng/mL ± 3.10, P = .02), and in 17% of them, CC-16 level was above the upper cutoff value (mean + 2.5SD). CC-16 levels did not correlate with age, onset region, disease duration, functional status, FVC, and PhrenAmpl. In patients with increased CC-16 level, the risk of non-invasive was greater in the following 6 months (P = .01) and tended to have higher mortality in the following 30 months (P = .07).ConclusionsWe propose that increased CC-16 levels is a marker of lung inflammatory response that associated with ventilatory insufficiency are related to impending respiratory failure, not fully predicted by conventional respiratory tests. The latter are limited by the moment of testing.
      PubDate: 2017-10-01T22:40:31.203817-05:
      DOI: 10.1111/ane.12851
  • Restless legs syndrome and cerebrovascular/cardiovascular events:
           Systematic review and meta-analysis
    • Authors: A. H. Katsanos; M. Kosmidou, S. Konitsiotis, G. Tsivgoulis, A. Fiolaki, A. P. Kyritsis, S. Giannopoulos
      Abstract: ObjectiveWe performed a systematic review and meta-analysis to evaluate the proposed association of restless legs syndrome (RLS) with cerebrovascular/cardiovascular outcomes.MethodsWe calculated the corresponding odds ratios on the prevalence of cerebrovascular/cardiovascular risk factors and standardized mean differences on the reported mean age at baseline between RLS patients and controls. We also calculated the corresponding risk ratios and adjusted for potential confounders hazard ratios (HRsadjusted) on the reported outcomes of interest between RLS patients and controls.ResultsWe identified 8 eligible studies (644 506 patients, mean age: 60.2 years, 36.2% males; 3.3% with RLS). RLS patients were found to have significantly higher prevalence of hypertension (P = .002), diabetes (P = .003) and hyperlipidemia (P = .010) compared to controls. In the unadjusted analyses of prospective observational studies, RLS patients were found to have significantly higher risk for cerebrovascular ischaemia (P = .01) and all-cause mortality (P = .04) compared to controls during follow-up, while in the adjusted for potential confounders analyses RLS patients were only found to have a higher risk of all-cause mortality (HR adjusted=1.52, 95% CI: 1.17-1.97, P = .002).ConclusionsThe present report does not provide evidence for an increased risk of cerebrovascular and cardiovascular events in RLS patients, which highlights the vast presence of confounding factors.
      PubDate: 2017-09-25T22:20:52.154624-05:
      DOI: 10.1111/ane.12848
  • Motor vehicle accidents in Parkinson's disease: A questionnaire study
    • Authors: T. Ueno; T. Kon, R. Haga, H. Nishijima, M. Tomiyama
      Abstract: ObjectivesFew studies have investigated the risk factors for motor vehicle accidents (MVA) in individuals with Parkinson's disease (PD) in Japan.Materials and MethodsWe sent an anonymous questionnaire to 1417 patients with PD who had received medical care certificates for Intractable Diseases during the 2014 fiscal year from the Aomori Prefectural Government in Japan. Data from patients with PD who previously or currently held a driving license at the time of the survey were analyzed.ResultsComplete datasets were obtained from 384 patients with PD who were either past or present driving license holders. Fifty-seven patients had caused at least one MVA in the last 5 years before the survey. Logistic regression analyses revealed that ergot-dopamine agonist (DA) use and excessive daytime sleepiness (Epworth Sleepiness Scale score ≥ 10) were the best predictors of MVAs. Patients having caused non-sleep-related MVAs had significantly longer disease durations, more frequent ergot-DA use, and higher cognition and communication subscores on the Parkinson's Disease Questionnaire-39 than those without non-sleep-related MVAs (P 
      PubDate: 2017-09-25T22:20:25.538652-05:
      DOI: 10.1111/ane.12849
  • Leptomeningeal collateral status predicts outcome after middle cerebral
           artery occlusion
    • Authors: C. F. Madelung; C. Ovesen, C. Trampedach, A. Christensen, I. Havsteen, C. K. Hansen, H. Christensen
      Abstract: ObjectivesPerfusion through leptomeningeal collateral vessels is a likely pivotal factor in the outcome of stroke patients. We aimed to investigate the effect of collateral status on outcome in a cohort of unselected, consecutive stroke patients with middle cerebral artery occlusion undergoing reperfusion therapy.Materials and methodsThis retrospectively planned analysis was passed on prospectively collected data from 187 consecutive patients with middle cerebral artery occlusion admitted within 4.5 hours to one center and treated with intravenous thrombolysis alone (N = 126), mechanical thrombectomy alone (N = 5), or both (N = 56) from May 2009 to April 2014.Non-contrast CT (NCCT) and computed tomography angiography (CTA) were provided on admission and NCCT repeated at 24 hours. Collateral status was assessed based on the initial CTA. Hemorrhagic transformation was evaluated on the 24-hour NCCT and according to European Cooperative Acute Stroke Study (ECASS) criteria. Modified Rankin Scale score was assessed at 90 days, and mortality at 1 year.ResultsAt 90 days, median (IQR) modified Rankin Scale score in patients with poor collateral status was 4 (3-6) compared to 2 (1-4) in patients with good collateral status (P 
      PubDate: 2017-09-14T04:42:11.700271-05:
      DOI: 10.1111/ane.12834
  • Cognitive functioning following one-year natalizumab treatment: A
           non-randomized clinical trial
    • Authors: I. Rorsman; C. Petersen, P.C. Nilsson
      Abstract: ObjectivesCognitive impairment is common in multiple sclerosis (MS) and can have serious impact on social and occupational functioning. Natalizumab reduces relapse rates, magnetic resonance imaging (MRI) lesions, and progression of disability. Previous studies on cognitive functioning have not based inclusion on cognitive performance criteria. The aim of the present study was to determine any potential natalizumab-related cognitive effects on MS patients performing below normal limits on neuropsychological testing.Materials and methodsPatients starting natalizumab (n = 21) and a quasi-control group of stable MS patients (n = 13) on first line disease modifying treatment were included following neuropsychological assessment demonstrating subnormal cognitive performance. Assessment, using ten cognitive variables, was repeated after 12 months. Symptoms of fatigue, anxiety and depression were also examined. Raw scores on the cognitive tests were transformed into Z-scores based on published age-corrected normative data.ResultsBetween-group analyses on difference Z-scores (baseline - follow-up) yielded significant results on Paced Auditory Serial Addition Test-2 (PASAT-2) (P = .008), with the natalizumab group showing larger improvement than quasi control patients. On PASAT-2, 28,5% from the natalizumab group demonstrated>1 SD improvement, indicative of clinically meaningful change, compared with none in the quasi control group. Patients receiving natalizumab showed within-group improvements on six of the ten cognitive variables. There were no group differences in symptoms of fatigue, anxiety or depression.ConclusionsThe results demonstrate improvement in information processing speed following 12-months of natalizumab treatment. The results are interpreted as reflection of anti-inflammatory properties of natalizumab rather than retest- or long-term restorative effects.
      PubDate: 2017-09-13T05:46:22.555519-05:
      DOI: 10.1111/ane.12833
  • Role of mHealth in overcoming the occurrence of post-stroke depression
    • Authors: J. J. Miranda; M. G. Moscoso, M. Toyama, V. Cavero, F. Diez-Canseco, B. Ovbiagele
      Abstract: Depression associated with stroke affects roughly one-third of stroke survivors. Post-stroke depression (PSD) is thought to adversely influence functional outcome by limiting participation in rehabilitation, decreasing physical, social, and cognitive function, and affecting neuroplasticity thereby placing stroke survivors at high risk for future vascular events. PSD has also been associated with higher mortality rates after stroke. In Peru, a country where there is no national stroke program and mental health disorders are largely underdiagnosed and untreated, people with PSD are likely to be further challenged by dependency and impoverished conditions that will limit their use of ambulatory services, leading to inadequate clinical follow-up. In this scenario, mobile health (mHealth) technology offers a promising approach to extend access to high-quality and culturally tailored evidence-based psychological care to address PSD given that cell phone use, Internet connectivity, and digital health technology have met a rapid growth in the last years and thus contribute to the attainment of broader Sustainable Development Goals (SDGs). The limited evidence of the effectiveness of mHealth for PSD calls for researchers to fill a knowledge gap where Peru poses as an ideal setting because rapid expansion of digital technology and current mental healthcare reform could be leveraged to enhance post-stroke outcomes. This article proposes the rationale for a suitable evidence-driven, mHealth-based, PSD self-management intervention called iMOODS—Investigating the role of mHealth in overcoming occurrence of depression after stroke—that could be tested among recent stroke patients with PSD in resource constrained settings.
      PubDate: 2017-09-13T01:10:30.031949-05:
      DOI: 10.1111/ane.12832
  • Efficacy of ketogenic diet for infantile spasms: A systematic review
    • Authors: G. Prezioso; G. Carlone, G. Zaccara, A. Verrotti
      Abstract: The aim of this systematic review was to collect and analyze all the RCTs and observational studies investigating the efficacy of ketogenic diet (KD) in infantile spasms (IS) patients after a 1- to 6-month follow-up period, in terms of decrease in seizure frequency of>50% or a seizure-free interval. Moreover, the potential effect of gender, IS etiology, age at onset of IS, and age at start of KD have been investigated. Finally, we evaluated the seizure-free rate at 12 and 24 months of follow-up.In June 2016, a computer search was performed on MedLine (PubMed), EMBASE, and the Cochrane Library. Only, English language studies conducted after 1980 and those reporting in detail the variation in seizure frequency have been selected.Thirteen observational studies (341 patients) were included in the final analysis. A median rate of 64.7% of patients experienced a spasm reduction>50% (IQR: 38.94%). The median spasm-free rate was 34.61% (IQR: 37.94%). IS of unknown etiology seemed to have an increased probability of achieving freedom from seizures (RR: 1.72, 95%CI: 1.18-2.53). Long-time follow-up data revealed a median seizure-free rate of 9.54% (IQR: 18.23%).Although the literature is still lacking in high-quality studies, which could provide a stronger level evidence, our findings suggest a potential benefit of KD for drug-resistant IS patients.
      PubDate: 2017-09-06T02:05:48.430739-05:
      DOI: 10.1111/ane.12830
  • Midazolam vs diazepam in prolonged seizures in children: A
           pharmacoeconomic approach
    • Authors: E. Beghi; G. Capovilla, E. Franzoni, F. Minicucci, A. Romeo, A. Verrotti, F. Vigevano, E. Perucca
      Abstract: ObjectiveA previous European cost-utility study reported that use of buccal midazolam in the community setting for the treatment of prolonged seizures (ie, seizures lasting ≥5 minutes) in children was associated with an overall €12 507 399 reduction in annual costs charged to the Italian national health service compared with rectal diazepam. We re-evaluated these findings by applying a more conservative approach.MethodsThe Italian Delphi panel reconvened to apply a more conservative assessment of available reports. A decision-tree model was used, allowing for different treatment pathways depending on whether or not a caregiver administers treatment, an ambulance is required for transport of the child to hospital, and an inpatient stay is required. Direct medical costs were derived from Italian healthcare system data. Estimates of the annual number of prolonged tonic-clonic seizures expected in the country were based on studies which assessed seizure duration using video-EEG recordings and medical records.ResultsAlthough drug acquisition costs were greater for buccal midazolam than for rectal diazepam, the acquisition cost difference was outweighed by larger cost savings resulting mostly from a reduction in hospital admissions. Assuming that 1.2% of tonic and/or clonic seizures occurring in children and adolescents over a 12-month period are prolonged, the annual nationwide reduction in costs from preferring buccal midazolam to rectal diazepam was estimated at €3 577 587.9.ConclusionsIn this more conservative revised analysis, the high cost of buccal midazolam is still counteracted by greater cost savings compared with rectal diazepam, but cost reduction was less than previously estimated.
      PubDate: 2017-09-06T02:00:50.107817-05:
      DOI: 10.1111/ane.12814
  • Disrupted intrinsic and remote functional connectivity in
           heterotopia-related epilepsy
    • Authors: W. Liu; X. Hu, D. An, Q. Gong, D. Zhou
      Abstract: ObjectivesSeveral neuroimaging studies have examined neural interactions in patients with periventricular nodular heterotopia (PNH). However, features of the underlying functional network remain poorly understood. In this study, we examined alterations in the local (regional) and remote (interregional) cerebral networks in this disorder.MethodsTwenty-eight subjects all having suffered from PNH with epilepsy, as well as 28 age- and sex- matched healthy controls, were enrolled in this study. Amplitude of low-frequency fluctuation (ALFF) and seed-based functional connectivity (FC) were calculated to detect regional neural function and functional network integration, respectively.ResultsCompared with healthy controls, patients with PNH-related epilepsy showed decreased ALFF in the ventromedial prefrontal cortex (vmPFC) and precuneus areas. ALFF values in both areas were negative correlated with epilepsy duration (P 
      PubDate: 2017-09-06T02:00:45.85657-05:0
      DOI: 10.1111/ane.12831
  • Polymorphisms in dopamine-associated genes and cognitive decline in
           Parkinson's disease
    • Authors: D. Bäckström; M. Eriksson Domellöf, G. Granåsen, J. Linder, S. Mayans, E. Elgh, H. Zetterberg, K. Blennow, L. Forsgren
      Abstract: ObjectivesCognitive decline is common in Parkinson's disease (PD), but the underlying mechanisms for this complication are incompletely understood. Genotypes affecting dopamine transmission may be of importance. This study investigates whether genotypes associated with reduced prefrontal dopaminergic tone and/or reduced dopamine D2-receptor availability (Catechol-O-methyltransferase [COMT] Val158Met genotype and DRD2 C957T genotype) affect the development of cognitive deficits in PD.Materials and methodsOne hundred and 34 patients with idiopathic PD, participating in a regional, population-based study of incident parkinsonism, underwent genotyping. After extensive baseline investigations (including imaging and biomarker analyses), the patients were followed prospectively during 6-10 years with neuropsychological evaluations, covering six cognitive domains. Cognitive decline (defined as the incidence of either Parkinson's disease mild cognitive impairment [PD-MCI] or dementia [PDD], diagnosed according to published criteria and blinded to genotype) was studied as the primary outcome.ResultsBoth genotypes affected cognition, as shown by Cox proportional hazards models. While the COMT 158Val/Val genotype conferred an increased risk of mild cognitive impairment in patients with normal cognition at baseline (hazard ratio: 2.13, P = .023), the DRD2 957T/T genotype conferred an overall increased risk of PD dementia (hazard ratio: 3.22, P 
      PubDate: 2017-09-04T05:31:27.820619-05:
      DOI: 10.1111/ane.12812
  • Life situation 5 years after subarachnoid haemorrhage
    • Authors: H. C. Persson; L. Carlsson, K. S. Sunnerhagen
      Abstract: ObjectivesSubarachnoid haemorrhage (SAH) has high mortality and morbidity among survivors. SAH mainly affects young people and may result in long-term disabilities such as decreased Health-related Quality of Life (HRQoL), mental health and cognitive function.The aim of this study was to investigate the life situation 5 years after a SAH including physical/emotional status, participation and HRQoL.Materials & MethodsIn this cross-sectional descriptive study, a mail survey was sent to all persons treated at a neurosurgery unit in Gothenburg, Sweden, for non-traumatic SAH in 2009-2010, approximately 5 years post-SAH. The survey included questions regarding HRQoL; EuroQol 5-Dimensions (EQ-5D), the impact of the SAH; Stroke Impact Scale (SIS), Occupational Gaps Questionnaire and participation in society; Impact of Participation and Autonomy (IPA).ResultsForty-two 5 year survivors were sent the survey, of whom 26 (62%) responded (59 years old, range 33-85). The participants had generally low HRQoL and scored low in the domain of anxiety and depression. Many reported problems with emotions, fatigue, memory and executive function, but few problems with physical condition. However, nearly all participants reported to have an acceptable level of participation and 64% were independent in their daily life.ConclusionsIn this 5-year follow-up after SAH, the participants reported to have a greater number of hidden disabilities compared to physical problems, whereas most had acceptable participation in society. A yearly follow-up after a SAH could be suggested aiming to improving the cognitive and mental health.
      PubDate: 2017-09-04T05:13:35.111399-05:
      DOI: 10.1111/ane.12815
  • Beta-blocker therapy is not associated with mortality after intracerebral
    • Authors: M. Sykora; J. Putaala, A. Meretoja, T. Tatlisumak, D. Strbian
      Abstract: BackgroundBeta-blocker therapy has been suggested to have neuroprotective properties in the setting of acute stroke; however, the evidence is weak and contradictory. We aimed to examine the effects of pre-admission therapy with beta-blockers (BB) on the mortality following spontaneous intracerebral hemorrhage (ICH).MethodsRetrospective analysis of the Helsinki ICH Study database.ResultsA total of 1013 patients with ICH were included in the analysis. Patients taking BB were significantly older, had a higher premorbid mRS score, had more DNR orders, and more comorbidities as atrial fibrillation, hypertension, diabetes mellitus, ischemic heart disease, and heart failure. After adjustment for age, pre-existing comorbidities, and prior use of antithrombotic and antihypertensive medications, no differences in in-hospital mortality (OR 1.1, 95% CI 0.8-1.7), 12-month mortality (OR 1.3, 95% CI 0.9-1.9), and 3-month mortality (OR 1.2, 95% CI 0.8-1.7) emerged.ConclusionPre-admission use of BB was not associated with mortality after ICH.
      PubDate: 2017-09-04T05:13:31.882895-05:
      DOI: 10.1111/ane.12817
  • Anti-epileptic drug exposure and risk of foetal death in utero
    • Authors: F. J. E. Vajda; T. J. O'Brien, J. Graham, A. A. Hitchcock, C. M. Lander, M. J. Eadie
      Abstract: ObjectiveTo clarify whether anti-epileptic drug exposure during pregnancy is associated with an increased risk of intrauterine foetal death.MethodsAnalysis of data from 2064 pregnancies with known outcomes included in the Australian Register of Antiepileptic Drugs in Pregnancy, 170 of the pregnancies being unexposed to the drugs in at least the first half of pregnancy.ResultsThe relative risk (6.46; 95% C.I. 0.90, 46.22) of intrauterine death appeared higher, though not statistically significantly higher, in drug-exposed pregnancies compared with unexposed ones (3.44% vs 0.59%). There was no statistically significantly increased hazard associated with AED polytherapy as compared with monotherapy. Logistic regression analysis showed a statistically significantly increased and dose-related hazard of intrauterine death in relation to carbamazepine exposure.ConclusionsIntrauterine exposure to anti-epileptic drugs, particularly carbamazepine, may be associated with an increased risk of foetal death during pregnancy.
      PubDate: 2017-08-31T00:25:39.541502-05:
      DOI: 10.1111/ane.12816
  • The Gulf War era multiple sclerosis cohort: 3. Early clinical features
    • Authors: M. T. Wallin; W. J. Culpepper, H. Maloni, J. F. Kurtzke
      Abstract: ObjectivesTo present clinical features at diagnosis for a large nationwide incident cohort of multiple sclerosis (MS) among those serving in the US military during the Gulf War era (GWE).Materials & MethodsMedical records and databases from the Department of Veterans Affairs (VA) for cases of MS with onset in or after 1990, active duty between 1990 and 2007 and service connection by the VA, were reviewed for diagnosis and demographic variables. Neurological involvement was summarized by the Kurtzke Disability Status Scale (DSS) and the Multiple Sclerosis Severity Score (MSSS).ResultsAmong 1919 cases of clinically definite MS, 94% had a relapsing-remitting course and 6% were primary progressive at diagnosis. More males of all races and blacks of both sexes were progressive. At diagnosis, functional system involvement was pyramidal 69%, cerebellar 58%, sensory 55%, brainstem 45%, bowel/bladder 23%, cerebral 23%, visual 18%, and other 5%. Mean DSS scores were: white males, females 2.9, 2.7; black males, females 3.3, 2.8; and other-race males, females 3.2, 2.6. Mean and median MSSS were marginally greater in black males and other males compared to the other sex-race groups.ConclusionsIn this incident cohort, males and blacks had significantly higher proportions of primary progressive MS. DSS at diagnosis was significantly more severe in blacks and significantly less so in whites and in women vs men, but MSSS was only marginally greater in black males and other-race males. This morbidity assessment early in the course of MS provides population-based data for diagnosis, management, and prognosis.
      PubDate: 2017-08-22T21:26:12.92968-05:0
      DOI: 10.1111/ane.12810
  • Cognitive complaint in early Parkinson's disease: A pilot study
    • Authors: J. Dupouy; F. Ory-Magne, C. Mekies, V. Rousseau, M. Puel, K. Rerat, J. Pariente, C. Brefel-Courbon,
      Abstract: BackgroundSubjective cognitive complaint (SCC) is a criterion recommended by the Movement Disorder Society (MDS) task force for the diagnosis of mild cognitive impairment (MCI). Until now there were few specific tools for detecting SCC in PD. We sought to develop a new tool to assess SCC specifically dedicated for PD.Materials and MethodsWe set a group of experts in movements disorders and neurocognition to develop an easy-to-use tool based on a visual analogue scale (VAS) for five cognitive domains: memory, executive functions, spatial orientation, attention, and language. We use it to assess SCC twice (at a one-month interval) in PD patients with disease duration of less than 5 years. Comprehensibility of the VAS was assessed. Controls were assessed with the same VAS. Patients with PD also underwent neuropsychological testing.ResultsVAS was easily understandable by the 70 patients with PD. We found significant SCC for the patients with PD vs controls in three cognitive domains: executive functions (1.7 ± 1.9 vs 0.8 ± 1.1; P 
      PubDate: 2017-08-22T21:20:39.528866-05:
      DOI: 10.1111/ane.12808
  • Outcome of cancer-related seizures in patients treated with lacosamide
    • Authors: M. Toledo; A. Molins, M. Quintana, E. Santamarina, F. Martinez-Ricarte, E. Martínez-Saez, J. Salas-Puig
      Abstract: ObjectivesLacosamide is an antiepileptic drug (AED), which has proven to be effective to control seizures, including acute conditions such as status epilepticus. The aim of this study is to describe the clinical experience with lacosamide in neuro-oncological patients.Materials and methodsMulticenter retrospective study in patients with cancer-related seizures, who received lacosamide as an add-on therapy.ResultsForty-eight patients with benign and malignant tumors, including primary brain tumors, lymphomas, systemic cancer with central nervous system involvement, or paraneoplastic encephalitis, were included. Lacosamide was effective in the control of chronic seizures in patients with either benign or malignant tumors. The success rate was greater in malignant tumors, and drug-resistant epilepsies were more likely associated with benign tumors. Adverse events occurred in nearly 70% of patients, particularly in acute conditions and associated with the concomitant use of radio-/chemotherapy. Lacosamide-related adverse events were more likely somnolence and dizziness, which usually resolved after dose adjustment. After starting lacosamide, nearly half of the patients discontinued one of the baseline AEDs and decreased or discontinued dexamethasone. Fifteen patients with status epilepticus were treated with intravenous lacosamide, and 73% of them had their condition resolved without serious drug-related adverse events.ConclusionLacosamide is an AED to consider in cases of cancer-related seizures. Lacosamide pharmacodynamics and pharmacokinetics allow the achievement of responder rates over 50% with no serious adverse effects, amelioration of side effects from other AEDs or radio-/chemotherapy, and no significant drug interactions. Furthermore, the intravenous formulation shows clear benefits in acute conditions such as status epilepticus.
      PubDate: 2017-08-22T04:20:51.436273-05:
      DOI: 10.1111/ane.12809
  • Screening for late-onset Pompe disease in western Denmark
    • Authors: J. S. Hansen; E. G. Pedersen, D. Gaist, F. W. Bach, O. J. Vilholm, B. Sandal, L. Weitemeyer, K. Nielsen, F. E. Schlesinger, N. Preisler, J. Vissing, H. Andersen
      Abstract: ObjectiveLate-onset Pompe disease (LOPD) is a rare autosomal recessively inherited metabolic myopathy caused by reduced activity of the lysosomal enzyme alpha-glucosidase. In a previous screening study at two large neuromuscular university clinics in Denmark, three patients with LOPD were identified out of 103 patients screened. No systematic screening has been performed at the other neurological departments in the western part of Denmark. Thus, patients with a diagnosis of unspecified myopathy were screened for LOPD.Materials and methodsAt seven neurological departments in the western part of Denmark, medical records were evaluated for all patients registered with myopathy diagnosis codes (ICD 10 codes: G 71.0-71.9 and G 72.0-72.9) during the period January 1, 2002, to December 31, 2012. If no specific diagnosis has been reached, patients were invited for screening. Dried blood spot (DBS) test was used to analyze the activity of the enzyme alpha-glucosidase.ResultA total of 654 patients were identified. From the medical records, information was obtained concerning symptoms, family history, electromyography, muscle biopsy results and creatine kinase levels. Eighty-seven patients (13.3%) (males 61%) at a mean age of 53.3 years (SD 16.5) fulfilled the criteria for screening. A DBS test was performed in 47 (54%) patients. In all patients, the enzyme activity was within reference values.ConclusionNone of the screened patients had a reduced activity of the enzyme alpha-glucosidase. Although the cohort studied was small, our findings do not suggest that LOPD is underdiagnosed in patients with unspecified myopathy in western Denmark.
      PubDate: 2017-08-22T04:10:27.062741-05:
      DOI: 10.1111/ane.12811
  • Prehospital pathways of occipital stroke patients with mainly visual
    • Authors: S. Räty; K. Silvennoinen, T. Tatlisumak
      Abstract: ObjectivesOccipital ischemic strokes typically cause homonymous visual field defects, for which means of rehabilitation are limited. Intravenous thrombolysis is increasingly and successfully used for their acute treatment. However, recognition of strokes presenting with mainly visual field defects is challenging for both patients and healthcare professionals. We studied prehospital pathways of occipital stroke patients with mainly visual symptoms to define obstacles in their early recognition.Materials & methodsThis observational, retrospective, registry-based study comprises occipital stroke patients with isolated visual symptoms treated at the neurological emergency department of Helsinki University Central Hospital in 2010-2015. We analyzed their prehospital pathways, including time from symptom onset to admission at the neurological emergency department (ODT), the number of points of care, the percentage of patients with ODT≤4.5 hours, and factors associated with delay.ResultsAmong 245 patients, only 20.8% arrived within 4.5 hours and 6.5% received IV thrombolysis. Delayed arrival was most often due to patients' late contact to health care. Of the patients, 27.3% arrived through at least two points of care, and differential diagnostics to ophthalmologic disorders proved particularly challenging. ODT≤4.5 hours was associated with EMS utilization, direct arrival, and atrial fibrillation; a visit at an ophthalmologist and initial misdiagnosis were associated with ODT>4.5 hours. After multivariable analysis, only direct arrival predicted ODT≤4.5 hours.ConclusionsOccipital stroke patients with visual symptoms contact health care late, are inadequately recognized, and present with complex prehospital pathways. Consequently, they are often ineligible for IV thrombolysis. This presents a missed opportunity for preventing permanent visual field defects.
      PubDate: 2017-08-13T22:55:22.877561-05:
      DOI: 10.1111/ane.12807
  • A survey of epilepsy knowledge, attitudes and practice in school-aged
           children in Bangkok, Thailand
    • Authors: C. Limotai; T. Manmen, K. Urai, C. Limarun
      Abstract: ObjectivesTo estimate the level of knowledge about epilepsy, attitude towards PWE and practice in school-aged children in Bangkok, Thailand. Significant findings from this study will be employed to develop a relevant and effective tool to educate children.Materials and MethodsThis cross-sectional survey study was conducted in Bangkok, Thailand, from August 2014 to December 2015. Study population included school-aged children between 9 and 14 years (4th to 8th grade). A structured age-appropriate, Thai culture-adjusted and simple 20-item questionnaire was used for this survey. The questionnaire comprised three domains which were eight items for knowledge (K), seven items for attitudes (A), and five items for practice (P). Descriptive and analytic statistics including Pearson's correlation were used to find correlation among KAP domains and age with KAP scores.ResultsA total of 1040 students from 13 schools participated in our survey study. Mean age was 11.27 (SD 0.94). Some basic knowledge about epilepsy and practice of inserting objects into mouth of seizing persons account for a high magnitude of misunderstanding. Girls and older-aged children are associated with better positive attitudes towards epilepsy. It seems that educating children with knowledge of both epilepsy and first aid is necessary to improve positive attitudes among this age group.ConclusionsGiven the findings, our study suggests that a lack of knowledge in some aspects of KAP in children exists. Educational materials should contain basic knowledge about the simple pathogenesis of seizure, seizure types and seizure characteristics, and provide explanation as to why inserting objects into mouths of seizing persons is not recommended.
      PubDate: 2017-08-06T22:30:21.151559-05:
      DOI: 10.1111/ane.12805
  • Musicogenic reflex seizures in epilepsy with glutamic acid decarbocylase
    • Authors: M. Falip; L. Rodriguez-Bel, S. Castañer, J. Miro, S. Jaraba, J. Mora, J. Bas, M. Carreño
      Abstract: BackgroundMusicogenic reflex seizures (MRS) are a rare form of seizures described in patients with temporal lobe epilepsy (TLE), mainly of unknown etiology. Epilepsy with antibodies against glutamic acid decarboxylase (GAD-ab) is a form of autoimmune epilepsy for which no specific semiology has been described.Aim of the studyTo retrospectively review the incidence of MRS in the general epileptic population and in the series of patients with epilepsy and GAD-ab and to describe its clinical and paraclinical characteristics.MethodsPatients recorded between January 2010 and January 2016 in the Database of Bellvitge Hospital Epilepsy Unit were reviewed.ResultsFrom a group of 1510 epileptic patients, three reported MRS (0.0019%) (two patients with epilepsy and GAD-ab and one patient with cryptogenic TLE). The incidence of MRS in patients with epilepsy and GAD-ab was 2 of 22 (9%). Both patients had a normal magnetic resonance Imaging (MRI), but FDG-PET showed medial temporal lobe hypometabolism (unilateral or bilateral) in both and also in the insula in one of them. MRS (recorded via video-EEG[electroencephalography] in one patient) arose from the right temporal lobe.ConclusionsMRS may be a distinctive seizure type in patients with epilepsy and antiGADab. Determination of GAD-ab should be carried out in all cases of MRS, even those with normal structural MRI.
      PubDate: 2017-08-02T01:41:24.377899-05:
      DOI: 10.1111/ane.12799
  • Return to work predictors after traumatic brain injury in a welfare state
    • Authors: L. Odgaard; A. R. Pedersen, I. Poulsen, S. P. Johnsen, J. F. Nielsen
      Abstract: ObjectiveTo identify predictors of return to work (RTW) and stable labour market attachment (LMA) after severe traumatic brain injury (TBI) in Denmark.Materials & methodsPatients aged 18-64 years, admitted to highly specialized neurorehabilitation after severe TBI 2004-2012 were included and followed up for ≤6 years. Weekly LMA data were retrieved from a national register of public assistance benefits. Weeks without or with supplemental public assistance benefits were defined as LMA weeks. Time of RTW was defined as first week with LMA. Stable LMA was defined as weeks with LMA ≥75% first year after RTW. Multivariable regressions were used to identify predictors of RTW and stable LMA among preinjury characteristics, injury severity, functional ability and rehabilitation trajectories.ResultsFor the analyses of RTW and stable LMA, 651 and 336 patients were included, respectively. RTW was significantly associated with age (adjusted subhazard ratio 0.98, 95% CI 0.97-0.99), education (1.83, 95% CI 1.16-2.89), supplemental benefits (3.97, 95% CI 2.04-7.71), no benefits (4.86, 95% CI 2.90-8.17), length of stay in acute care (0.77, 95% CI 0.60-0.99) and time period of injury (1.56, 95% CI 1.15-2.10). The only significant predictor of stable LMA was age (adjusted odds ratio 0.97, 95% CI 0.95-0.99).ConclusionRTW after severe TBI was associated with several socio-economic factors, whereas maintaining LMA depended on age only. We suggest that RTW rates could be improved by extensive rehabilitation targeting people that are older and low-educated, as these were less likely to RTW.
      PubDate: 2017-07-31T04:10:38.797879-05:
      DOI: 10.1111/ane.12806
  • Depression and anxiety in multiple system atrophy
    • Authors: L.-Y. Zhang; B. Cao, Y.-T. Zou, Q.-Q. Wei, R.-W. Ou, B. Zhao, Y. Wu, H.-F. Shang
      Abstract: BackgroundIt has been noticed that the patients with multiple system atrophy (MSA) can accompany with depression and anxiety. This study aimed to establish the incidence and determinants of depression and anxiety symptoms in Chinese MSA patients.MethodsA total of 237 MSA patients were enrolled in the study. Neuropsychological assessment was performed using Hamilton Depression Rating Scale-24 items and Hamilton Anxiety Rating Scale.ResultsWe found that 62.0% and 71.7% patients had at least mild depression and anxiety symptoms, respectively. The severity of depression of MSA patients was associated with lower educational years (P=.024), longer disease duration (P
      PubDate: 2017-07-26T22:10:28.96273-05:0
      DOI: 10.1111/ane.12804
  • Diffusion tensor imaging in multiple sclerosis at different final outcomes
    • Authors: O. Andersen; A. Hildeman, M. Longfils, H. Tedeholm, B. Skoog, W. Tian, J. Zhong, S. Ekholm, L. Novakova, B. Runmarker, O. Nerman, S. E. Maier
      Abstract: ObjectivesMethods to evaluate the relative contributions of demyelination vs axonal degeneration over the long-term course of MS are urgently needed. We used magnetic resonance diffusion tensor imaging (DTI) to estimate degrees of demyelination and axonal degeneration in the corpus callosum (CC) in cases of MS with different final outcomes.Materials and methodsWe determined DTI measures mean diffusivity (MD), fractional anisotropy (FA), and axial (AD) and radial (RD) diffusivities in the CC of 31 MS patients, of whom 13 presented a secondary progressive course, 11 a non-progressive course, and seven a monophasic course. The study participants were survivors from an incidence cohort of 254 attack-onset MS patients with 50 years of longitudinal follow-up. As reference, we included five healthy individuals without significant morbidity.ResultsIn patients with secondary progression, compared to all other groups, the corpus callosum showed increased RD and reduced FA, but no change in AD. None of the parameters exhibited differences among non-progressive and monophasic course groups and controls.ConclusionIncreased RD was observed in secondary progressive MS, indicating significant myelin loss. Normal RD values observed in the clinically isolated syndrome and non-progressive groups confirm their benign nature. AD was not a characterizing parameter for long-term outcome. Demyelination revealed by increased RD is a distinguishing trait for secondary progression.
      PubDate: 2017-07-25T04:20:50.35224-05:0
      DOI: 10.1111/ane.12797
  • Finding of increased caudate nucleus in patients with Alzheimer's disease
    • Authors: K. Persson; V. D. Bohbot, N. Bogdanovic, G. Selbæk, A. Brækhus, K. Engedal
      Abstract: ObjectivesA recently published study using an automated MRI volumetry method (NeuroQuant®) unexpectedly demonstrated larger caudate nucleus volume in patients with Alzheimer's disease dementia (AD) compared to patients with subjective and mild cognitive impairment (SCI and MCI). The aim of this study was to explore this finding.Materials & methodsThe caudate nucleus and the hippocampus volumes were measured (both expressed as ratios of intracranial volume) in a total of 257 patients with SCI and MCI according to the Winblad criteria and AD according to ICD-10 criteria. Demographic data, cognitive measures, and APOE-ɛ4 status were collected.ResultsCompared with non-dementia patients (SCI and MCI), AD patients were older, more of them were female, and they had a larger caudate nucleus volume and smaller hippocampus volume (P
      PubDate: 2017-07-25T04:15:28.699542-05:
      DOI: 10.1111/ane.12800
  • Eslicarbazepine acetate as adjunctive treatment in partial-onset epilepsy
    • Authors: S. Lattanzi; C. Cagnetti, N. Foschi, A. Lorusso, L. Provinciali, M. Silvestrini
      Abstract: ObjectiveThe aim of the study was to assess the clinical response to eslicarbazepine acetate (ESL) as add-on therapy in adult patients with partial-onset epilepsy by means of the time-to-baseline seizure count method.MethodsWe retrospectively identified consecutive patients with partial-onset seizures, with or without secondary generalization, prescribed to ESL add-on therapy. The primary endpoint was the time-to-baseline monthly seizure count. Subgroup analysis was performed according to carbamazepine (CBZ)/oxcarbazepine (OXC) status (prior vs never use). Secondary outcomes were the rate of treatment-related adverse events (AEs) and the AEs affecting ≥5% of patients.ResultsOne-hundred and eighteen patients were included. The median time-to-baseline monthly seizure count was 46 (35-101) days in the overall study cohort. The number of concomitant anti-epileptic drugs (AEDs) was associated with the time-to-endpoint (adjusted hazard ratio [adjHR]=2.22, 95% CI 1.18-4.14, P=.013 for two AEDs vs one; adjHR=3.65, 95% CI 1.66-8.06, P=.001 for three or more AEDs vs one). Groupwise, the median times-to-baseline seizure count were 47 (35-97) and 43 (34-103) in patients with prior and never exposure to CBZ/OXC, respectively (P for log-rank test=.807). Adverse events occurred in 53.4% (63 of 118) of patients; the most frequently reported were dizziness (13.6%), somnolence (11.9%), nausea (6.8%), and fatigue (5.1%).ConclusionsAdd-on ESL improved seizure control and was overall well-tolerated in adult patients with partial-onset epilepsy.
      PubDate: 2017-07-25T04:01:35.813107-05:
      DOI: 10.1111/ane.12803
  • Wearables in epilepsy and Parkinson's disease—A focus group study
    • Authors: A. Ozanne; D. Johansson, U. Hällgren Graneheim, K. Malmgren, F. Bergquist, M. Alt Murphy
      Abstract: ObjectivesWearable sensors that measure movement and physiological variables are attractive for clinical evaluation of neurological diseases such as epilepsy and Parkinson's disease (PD). The aim of this study was to explore perceptions regarding the use of wearable technology in disease monitoring and management as reported by individuals with epilepsy and Parkinson's disease as well as health professionals working with these patient groups.Materials and MethodsSix patient groups (n=25) and two groups with health professionals (n=15) participated in this qualitative, descriptive study with focus group interviews. A manifest qualitative content analysis was used.ResultsFour categories and nine subcategories emerged from the analysis. Participants saw possible benefits for improved treatment effect and valued this benefit more than possible inconvenience of wearing the sensors. Discrete design and simplicity were considered as facilitators for improved usability. They emphasized the importance of interactive information between patients and health professionals. However, they were concerned about unclear information and inconclusive recordings and some fears about personal integrity were at odds with the expectations on interactivity.ConclusionsPatients need to feel well informed and find an added value in using wearables. Wearables need to be user-friendly, have an attractive design, and show clinical efficacy in improving disease management. Variations in perceptions regarding integrity, benefits, and effectiveness of monitoring indicate possible conflicts of expectations among participants. The engagement of end users, patients, and health professionals, in the design and implementation process, is crucial for the development of wearable devices that enhance and facilitate neurological rehabilitation practice.
      PubDate: 2017-07-16T22:25:19.293864-05:
      DOI: 10.1111/ane.12798
  • The cost-utility of sodium oxybate as narcolepsy treatment
    • Authors: K. Bolin; P. Berling, P. Wasling, H. Meinild, J. Kjellberg, P. Jennum
      Abstract: Aims and ObjectivesBased on class-I studies, sodium oxybate is regarded as a first-line treatment for both EDS and cataplexy. The cost-effectiveness of sodium oxybate is largely unknown, though. In this study, we estimate the cost-effectiveness of sodium oxybate as treatment for patients with narcolepsy as compared to standard treatment, by calculating incremental cost-effectiveness ratios (cost per quality-adjusted life year, QALY) for patients in a Swedish setting.Materials and MethodsCalculations were performed using a Markov model with a 10-year time horizon. The study population consisted of adult patients treated for narcolepsy with cataplexy. Healthcare utilization and quality-adjusted life years (QALYs) for each treatment alternative were calculated assuming no treatment effect on survival. Sensitivity analyses were performed for treatment effectiveness and healthcare cost parameters.ResultsThe cost per additional quality-adjusted life year was estimated at SEK 563,481. The cost-effectiveness measure was demonstrated to be particularly sensitive to the duration of the relative quality-of-life improvements accruing to patients treated with sodium oxybate.ConclusionsThe estimated cost per additional QALY for the sodium oxybate treatment alternative compared with standard treatment was estimated above the informal Swedish willingness-to-pay threshold (SEK 500,000). The estimated cost per additional QALY obtained here is likely to overestimate the true cost-effectiveness ratio as potentially beneficial effects on productivity of treatment with sodium oxybate were not included (due to lack of data).
      PubDate: 2017-07-05T03:05:39.183021-05:
      DOI: 10.1111/ane.12794
  • Gender differences in Parkinson's disease: A clinical perspective
    • Authors: D. Georgiev; K. Hamberg, M. Hariz, L. Forsgren, G.-M. Hariz
      Abstract: Available data indicate that there are gender differences in many features of Parkinson's disease (PD). Precise identification of the gender differences is important to tailor treatment, predict outcomes, and meet other individual and social needs in women and men with PD. The aim of this study was to review the available clinical data on gender differences in PD. Original articles and meta-analyses published between 1990 and 2016 systematically exploring gender differences in PD were reviewed. There is slight male preponderance in incidence and prevalence of PD. PD starts earlier in men. Women tend to be more prone to develop tremor-dominant PD but are less rigid than men. Motor improvement after deep brain stimulation is equal in both sexes, but women tend to show better improvement in activities of daily living. Furthermore, women with PD show better results on tests for general cognitive abilities, outperform men in verbal cognitive tasks, show more pain symptoms, and score higher on depression scales. It seems, however, that the differences in cognition, mood, and pain perception are not disease specific as similar gender differences can be found in healthy subjects and in other neurological conditions. Despite PD being the most frequently studied movement disorder, studies investigating gender differences in PD are still scarce with most of the studies being cross-sectional. Good-quality, prospective, longitudinal studies analyzing gender differences in PD and comparing them to matched healthy controls are needed in order to properly address the issues of gender differences in PD.
      PubDate: 2017-07-02T22:26:02.673154-05:
      DOI: 10.1111/ane.12796
  • Prospective measurement of quality of life in myotonic dystrophy type 1
    • Authors: S. Peric; C. Heatwole, E. Durovic, A. Kacar, A. Nikolic, I. Basta, A. Marjanovic, Z. Stevic, D. Lavrnic, V. Rakocevic Stojanovic
      Abstract: IntroductionGeneric patient reported outcome measures have had varied success in tracking QoL in myotonic dystrophy type 1 (DM1).AimTo analyze changes of Individualized Neuromuscular Quality of Life questionnaire (INQoL) scores in clinic patients with DM1 over a 6-year period.MethodPatients completed the INQoL at baseline and after a 6-year period through their attendance in a neurology outpatient clinic. Severity of muscular involvement in DM1 was analyzed using the Muscular Impairment Rating Scale (MIRS).ResultsNinety-nine DM1 patients completed a baseline visit. Sixty-seven of these patients were retested at an interval time. The overall INQoL score improved in our sample of patients (P
      PubDate: 2017-06-28T23:10:40.810887-05:
      DOI: 10.1111/ane.12788
  • Parinaud syndrome: Any clinicoradiological correlation'
    • Authors: L. Pollak; T. Zehavi-Dorin, A. Eyal, R. Milo, R. Huna-Baron
      Abstract: IntroductionThe significance of MRI findings of patients with Parinaud syndrome (PS) with respect to clinical characteristics is poorly defined. Over the past decades, all patients with PS undergo magnetic resonance imaging which allows a better identification of the lesion localization. We compared the neuro-ophthalmological findings of patients with PS caused by intrinsic (intra-axial) vs extrinsic (pineal gland tumor) brainstem lesions.MethodsMedical records of patients with PS evaluated between 2000 and 2016 were retrospectively reviewed.ResultsTwenty-six patients with PS were included. Eight patients had pineal gland tumors and hydrocephalus. Two patients had hydrocephalus due to aqueduct stenosis and fourth ventricle tumor. Sixteen patients suffered from an intrinsic brainstem lesion and seven associated with hydrocephalus.The neuro-ophthalmological findings did not differ between patients with extrinsic and intrinsic brainstem lesions. No correlation was found between the grade of hydrocephalus and number of clinical findings except for more findings in low-grade hydrocephalus in intrinsic (40%) vs extrinsic (0%) lesions (P=.003). Patients with moderate brainstem lesions and hydrocephalus had more clinical findings (65%) than patients with the same grade of brainstem involvement without hydrocephalus (29%) (P=.03). The resolution rate of ophthalmological findings was comparable in all groups of patients.ConclusionsOur results did not show differences in neuro-ophthalmological findings between intra- and extra-axial lesions causing PS. However, the presence of hydrocephalus was an important factor influencing clinical findings. The prognosis of PS was less favorable than generally reported.
      PubDate: 2017-06-26T22:52:35.247136-05:
      DOI: 10.1111/ane.12795
  • Association between Parkinson's disease and diabetes: Data from NEDICES
    • Authors: E. De Pablo-Fernandez; F. Sierra-Hidalgo, J. Benito-León, F. Bermejo-Pareja
      Abstract: BackgroundDespite growing evidence showing an association between Parkinson's disease (PD) and diabetes, epidemiological studies have shown conflicting results.Aims of the studyTo evaluate the association between PD and diabetes and the impact of diabetes duration in this association in an elderly (≥65 years) Spanish population.MethodsData for this cross-sectional population-based analysis were obtained from NEDICES study. Subjects were identified from census list. Diagnosis of PD was confirmed by neurological examination. Diabetes was defined by self-report, being on antidiabetic medication or diagnosis on medical records. Logistic regression analysis adjusted by potential confounders was performed to estimate the association between both conditions and also after dividing patients into short-duration (
      PubDate: 2017-06-26T22:43:03.479658-05:
      DOI: 10.1111/ane.12793
  • Electro-clinical criteria and surgical outcome: Is there a difference
           between mesial and lesional temporal lobe epilepsy'
    • Authors: M. Wassenaar; F. S. S. Leijten, G.-J. Haan, S. G. Uijl, J. W. Sander
      Abstract: ObjectivesMesial temporal lobe epilepsy syndrome (MTLE) with specific electrophysiological and clinical characteristics and hippocampal sclerosis (HS) on MRI is considered the prototype of a syndrome with good surgical prognosis. Ictal onset zones in MTLE have been found to extend outside the hippocampus and neocortical seizures often involve mesial structures. It can, thus, be questioned whether MTLE with HS is different from lesional temporal epilepsies with respect to electro-clinical characteristics and surgical prognosis. We assessed whether MTLE with HS is distinguishable from lesional TLE and which criteria determine surgical outcome.MethodsPeople in a retrospective cohort of 389 individuals with MRI abnormalities who underwent temporal lobectomy, were divided into “HS only” or “lesional” TLEs. Twenty-six presented with dual pathology and were excluded from further analysis. We compared surgical outcome and electro-clinical characteristics.ResultsOver half (61%) had “HS only.” Four electro-clinical characteristics (age at epilepsy onset, febrile seizures, memory dysfunction and contralateral dystonic posturing) distinguished “HS only” from “lesional” TLE, but there was considerable overlap. Seizure freedom 2 years after surgery (Engel class 1) was similar: 67% (“HS only”) vs 69% (“lesional” TLE). Neither presence of HS nor electro-clinical criteria was associated with surgical outcome.ConclusionsDespite small differences in electrophysiological and clinical characteristics between MTLE with HS and lesional TLE, surgical outcomes are similar, indicating that aetiology seems irrelevant in the referral for temporal surgery.
      PubDate: 2017-06-18T23:05:23.595429-05:
      DOI: 10.1111/ane.12790
  • Controlled population-based comparative study of USA and international
           adult [55-74] neurological deaths 1989-2014
    • Authors: C. Pritchard; E. Rosenorn-Lanng, A. Silk, L. Hansen
      Abstract: ObjectivesA population-based controlled study to determine whether adult (55-74 years) neurological disease deaths are continuing to rise and are there significant differences between America and the twenty developed countries 1989-91 and 2012-14.MethodTotal Neurological Deaths (TND) rates contrasted against control Cancer and Circulatory Disease Deaths (CDD) extrapolated from WHO data. Confidence intervals compare USA and the other countries over the period. The Over-75's TND and population increases are examined as a context for the 55-74 outcomes.ResultsMale neurological deaths rose>10% in eleven countries, the other countries average rose 20% the USA 43% over the period. Female neurological deaths rose>10% in ten counties, averaging 14%, the USA up 68%. USA male and female neurological deaths increased significantly more than twelve and seventeen countries, respectively. USA over-75s population increased by 49%, other countries 56%. Other countries TND up 187% the USA rose fourfold. Male and female cancer and CDD fell in every country averaging 26% and 21%, respectively, and 64% and 67% for CDD. Male neurological rates rose significantly more than Cancer and CCD in every country; Female neurological deaths rose significantly more than cancer in 17 countries and every country for CDD. There was no significant correlation between increases in neurological deaths and decreases in control mortalities.ConclusionsThere are substantial increases in neurological deaths in most countries, significantly so in America. Rises in the 55-74 and over-75's rates are not primarily due to demographic changes and are a matter of concern warranting further investigation.
      PubDate: 2017-06-18T22:45:30.328765-05:
      DOI: 10.1111/ane.12789
  • Prognostic role of hypochloremia in acute ischemic stroke patients
    • Authors: H.-Z. Bei; S.-J. You, D. Zheng, C.-K. Zhong, H.-P. Du, Y. Zhang, T.-S. Lu, L.-D. Cao, X.-F. Dong, Y.-J. Cao, C.-F. Liu
      Abstract: ObjectivesThe impact of electrolyte imbalance on clinical outcomes after acute ischemic stroke (AIS) is still not understood. We investigated the association between hypochloremia and hyponatremia upon hospital admission and in-hospital mortality in AIS patients.Materials and methodsA total of 3314 AIS patients enrolled from December 2013 to May 2014 across 22 hospitals in Suzhou city were included in this study. Hypochloremia was defined as having a serum chloride concentration
      PubDate: 2017-06-14T04:07:41.838247-05:
      DOI: 10.1111/ane.12785
  • A rating scale for the severity of Guillain-Barré syndrome
    • Authors: Z. Guan; L. Shang, W. Zhang, Y. Guo, Y. Xue, X. Li, Y. Gong, X. Liu
      Abstract: IntroductionThe objective of this study was to develop a rating scale to assess the severity of Guillain-Barré syndrome (GBS).MethodsThe preliminary rating scale, which contained 11 items, was developed by the Delphi method, and data of 258 patients were collected to evaluate it. Item analysis was accomplished by 100 patients; the additional 158 patients were used to evaluate the reliability, validity, and discriminative ability of the rating scale. The structure of the rating scale was testified by the confirmatory factor analysis and also made a further evaluation by the correlation analysis.ResultsThe rating scale contained 10 items. The three factors mainly generalized the motor function, cranial nerve function and autonomic function. The results of reliability and validity showed that the structure of the rating scale was good (χ2=68.25, df=32, χ2/df=2.13, normed fit index (NFI)=0.919, non-normed fit index (NNFI)=0.936, comparative fit index (CFI)=0.96, a root mean square error of approximation (RMSEA)=0.085), and the Cronbach's α coefficient for the scale was .852, with the three dimensions ranging from .585 to .752.ConclusionReliability and validity of the rating scale are all satisfied. The scale contained the main clinical presentations of GBS, and it is suitable to evaluate the severity of GBS.
      PubDate: 2017-06-14T04:05:54.981828-05:
      DOI: 10.1111/ane.12786
  • Accelerated atherosclerosis in ANCA-associated vasculitis
    • Authors: I. González-Suárez; J. J. Ríos-Blanco, J. Arpa
      Abstract: ObjectivesCardiovascular disease, including myocardial infarction and stroke, is a major cause of mortality in ANCA-associated vasculitis (AAV). Although AAV affects small vessels, an accelerated atherosclerosis not explained by traditional cardiovascular risk factors (CVRF) has been demonstrated. We aimed to investigate the association of atherosclerosis measured by carotid intima-media thickness (CIMT) and cerebral small vessel disease in AAV-patients.Materials & MethodsTwenty-three AAV-patients in complete remission were recruited. Carotid ultrasonography (US), transcranial Doppler (TCD), brain magnetic resonance imaging (MRI), and SPECT after intravenous administration of tracer 99mTc-HMPAO (dose: 720MBq) were performed.ResultsAAV-patients presented higher CIMT compared to normative population. Multivariate linear regression analysis demonstrated an association of higher CIMT with increased pulsatility index in middle cerebral artery (PI-MCA) (P=.011), higher lesion load on ARWMC scale (P=.011) and abnormal SPECT (P=.008). No association between higher CIMT and CVRF (diabetes or hypertension) was demonstrated. Increasing internal carotid artery pulsatility index (PI-ICA) was associated with decreasing mean flow velocity (MFV)-MCA (P=.038), increasing PI-MCA (P=.008) and increasing white matter lesions on MRI (P=.011).ConclusionsOur study adds weight to the presence of increased atherosclerosis in AAV-patients. The association observed between CIMT and PI-ICA with small vessel cerebral disease, points the possible association of easy to use carotid US in predicting microvascular brain injury.
      PubDate: 2017-06-12T22:40:21.54541-05:0
      DOI: 10.1111/ane.12787
  • Pregnancy in CADASIL
    • Authors: I. Donnini; V. Rinnoci, S. Nannucci, R. Valenti, F. Pescini, G. Mariani, S. Bianchi, M. T. Dotti, A. Federico, D. Inzitari, L. Pantoni
      Abstract: ObjectivesCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral small vessel disease caused by NOTCH3 gene mutations. CADASIL women are frequently considered at high risk of systemic vascular events during pregnancy and often prescribed with antithrombotic drugs. This decision is not evidence-based considering the lack of data about pregnancy outcome in CADASIL. We describe our experience on pregnancy in CADASIL patients.Materials and methodsWe reviewed records of 50 CADASIL females followed in our center, and we collected prospective information in six patients for a total of 93 pregnancies.ResultsNo woman had the disease onset or suffered from cerebral vascular ischemic events during pregnancy. Sixteen miscarriages (17.2%) were recorded. There were 72 vaginal births, and five cesarean sections. Considering the six patients followed prospectively (for a total of eight pregnancies), data on fetal growth and newborns weight were in line with those from the general population. Considering gestational complications, we recorded mild proteinuria without hypertension in one patient and hyperinsulinemia and pre-eclampsia in another affected by a known nephropathy. Antithrombotic drugs were used in three patients, in one for an unrelated coexisting prothrombotic condition.ConclusionsCADASIL does not seem to be associated with an unfavorable outcome of pregnancy either for women and fetuses. Patients and treating physicians should be reassured that pregnancy can be safely initiated in CADASIL, as there is no evidence to support a specific preventive antithrombotic treatment during pregnancy in CADASIL. Larger studies are needed to definitively confirm these conclusions.
      PubDate: 2017-06-12T22:40:20.144848-05:
      DOI: 10.1111/ane.12784
  • Peripheral neuropathy in 30 duodopa patients with vitamins B
    • Authors: V. Rispoli; V. Simioni, J. G. Capone, N. Golfrè Andreasi, F. Preda, E. Sette, V. Tugnoli, M. Sensi
      Abstract: ObjectivesPeripheral neuropathy (PN) is a significant concern and potential cause of withdrawal in patients with Parkinson's disease (PD) treated with Levodopa/Carbidopa Intestinal Gel (LCIG) infusion. Vitamin B deficiency and/or hyperhomocysteinemia levodopa-related are considered possible causative factors. In this study, we evaluated PN incidence in LCIG-PD patients treated since the beginning of infusion with vitamins B supplementation.Materials & MethodsIn this prospective open-label pilot study, 30 consecutive patients with PD on LCIG infusion were evaluated with clinical, neurophysiological, and biochemical assessments for a mean follow-up of 42.4 months (range 24-72). All evaluations were repeated every 6 months.ResultsAt baseline, 21 of 30 presented no signs or symptoms of PN, and 9 of 30 had pre-existing chronic PN. In whole population, a progressive worsening in nerve conduction studies of sural sensory and peroneal motor nerves was observed during the long-term follow-up. 4 of 21 patients, with normal clinical, electrophysiological assessment at baseline, developed distal symmetrical axonal polyneuropathy that remained asymptomatic during the long-term follow-up. Patients with pre-existing PN (9 of 30) showed a mild worsening of electrophysiological features during the period of observation. In none PN was cause of discontinuation of LCIG therapy. No incident cases of acute-subacute PN were documented. No correlation was found with age, sex, Levodopa dosage, duration of levodopa exposure, and homocysteine plasma levels.ConclusionIn this consecutive series of 30 patients with PD on LCIG infusion, with early and continuous vitamins B integration, we observed a low rate (19%) of new onset peripheral polyneuropathy that remained stable after long-term follow-up. Larger studies, controlled, with blinded evaluation, are needed to confirm these findings.
      PubDate: 2017-06-12T22:20:21.611258-05:
      DOI: 10.1111/ane.12783
  • Transient ischemic attack and ischemic stroke patients with or without
           prior stroke
    • Authors: F. Buchwald; B. Norrving, J. Petersson
      Abstract: ObjectivesA history of stroke is common in patients with transient ischemic attack (TIA) and ischemic stroke (IS), but there are few reports characterizing this group of patients. We aimed to compare characteristics, risk factors, and secondary preventive treatment in patients with TIA or IS with vs without a history of stroke.MethodsHospital-based data on TIA and IS events, registered from July 2011 to June 2013, were obtained from the Swedish Stroke Register (Riksstroke). Previous stroke was not specified as hemorrhagic or ischemic.ResultsA history of stroke was present in 19.3% (2892/15012) of TIA and 24.6% (10853/44169) of IS patients. Patients with prior stroke were older, more often male, and more likely to have atrial fibrillation (AF) (TIA: 27.4% vs 16.4%; IS: 36.1% vs 28.0%), hypertension (TIA: 77.5% vs 56.4%; IS: 74.2% vs 59.0%), and diabetes mellitus (TIA: 22.2% vs 14.2%; IS: 26.3% vs 19.5%) compared with those without (all differences P
      PubDate: 2017-06-01T03:30:43.277139-05:
      DOI: 10.1111/ane.12782
  • Behavioural changes in patients with intellectual disability treated with
    • Authors: E. Andres; F. Kerling, H. Hamer, B. Kasper, M. Winterholler
      Abstract: ObjectivesThe aim of this cross-sectional retrospective study was to assess the tolerability and efficacy of perampanel in patients with drug-resistant epilepsy who also suffered from intellectual disability (ID).Patients and methodsWe used an industry-independent, non-interventional retrospective evaluation based on standardized, daily seizure records. Twenty-seven patients with ID and drug-resistant epilepsy were started on perampanel between September 2012 and November 2015 after a 3-month observation period without perampanel treatment. Perampanel was given at a maximum dosage of 4-12 mg daily. Evaluation was carried out after 6, 12 and 24 months, including calculation of the retention rate. Mean seizure frequency was compared between the 3-month baseline period and subsequent 3-month treatment periods. The Clinical Global Impression scale was applied to assess qualitative changes in seizure severity, and the Aggressive Behaviour Scale (ABS) gave further insights into challenging behaviour.ResultsPerampanel was efficacious and well tolerated in five of 25 patients. In 18 patients, perampanel treatment was stopped, mainly because of adverse events (n=6), lack of efficacy (n=3) or both (n=9). Behavioural changes were documented in 15 of 27 patients, with aggressive behaviour being the commonest effect; we observed ataxia (n=6) and sedation (n=8) in further patients. The ABS showed worsening of aggressive behaviour in six patients.ConclusionsPerampanel was well tolerated and efficacious in one-fifth of our patients. We observed challenging behaviour, ataxia and sedation in a relevant number of patients with ID under perampanel treatment. Further studies are warranted to explore the tolerability of perampanel in patients with ID.
      PubDate: 2017-06-01T03:30:33.332165-05:
      DOI: 10.1111/ane.12781
  • Role of P-glycoprotein inhibitors in children with drug-resistant epilepsy
    • Authors: H. A. Elkhayat; R. H. Aly, I. A. Elagouza, R. H. El-Kabarity, Y. I. Galal
      Abstract: ObjectiveThe role of P-glycoprotein (Pgp), one of the known multidrug transporters, has been suggested in drug-resistant epilepsy (DRE). The following study aimed to measure the serum level of Pgp as a possible indicator of tissue Pgp overexpression in patients with DRE and to assess the efficacy of verapamil (as a Pgp inhibitor agent) in these patients.Material and methodsA group of 24 patients with DRE were recruited and subdivided into two groups, one receiving verapamil and the other receiving a placebo in a double-blind randomized study. Pgp serum levels were measured at enrollment and 12 months later. Twenty medically controlled epileptic patients served as a control group.ResultsA significant statistical increase was found in the Pgp level of patients when compared the control group. Patients on both verapamil and the placebo showed improvement in seizure frequency and severity where statistical analysis showed no significant differences.ConclusionPgp serum levels in patients with DRE were significantly elevated compared to patients with medically controlled epilepsy. The effect of verapamil as Pgp inhibitor on DRE requires further evaluation and research.
      PubDate: 2017-05-31T01:45:27.673086-05:
      DOI: 10.1111/ane.12778
  • Viruses and endogenous retroviruses in multiple sclerosis: From
           correlation to causation
    • Authors: A.-F. A. Mentis; E. Dardiotis, N. Grigoriadis, E. Petinaki, G. M. Hadjigeorgiou
      Abstract: Multiple sclerosis is an immune-mediated disease with an environmental component. According to a long-standing but unproven hypothesis dating to initial descriptions of multiple sclerosis (MS) at the end of the 19th century, viruses are either directly or indirectly implicated in MS pathogenesis. Whether viruses in MS are principally causal or simply contributory remains to be proven, but many viruses or viral elements—predominantly Epstein-Barr virus, human endogenous retroviruses (HERVs) and human herpesvirus 6 (HHV-6) but also less common viruses such as Saffold and measles viruses—are associated with MS. Here, we present an up-to-date and comprehensive review of the main candidate viruses implicated in MS pathogenesis and summarize how these viruses might cause or lead to the hallmark demyelinating and inflammatory lesions of MS. We review data from epidemiological, animal and in vitro studies and in doing so offer a transdisciplinary approach to the topic. We argue that it is crucially important not to interpret “absence of evidence” as “evidence of absence” and that future studies need to focus on distinguishing correlative from causative associations. Progress in the MS-virus field is expected to arise from an increasing body of knowledge on the interplay between viruses and HERVs in MS. Such interactions suggest common HERV-mediated pathways downstream of viral infection that cause both neuroinflammation and neurodegeneration. We also comment on the limitations of existing studies and provide future research directions for the field.
      PubDate: 2017-05-23T22:55:25.833793-05:
      DOI: 10.1111/ane.12775
  • A prospective study of quality of life in amyotrophic lateral sclerosis
    • Authors: B. Jakobsson Larsson; A. G. Ozanne, K. Nordin, I. Nygren
      Abstract: ObjectsThe aim of this prospective and longitudinal study was to describe individual quality of life in patients with amyotrophic lateral sclerosis (ALS) and its correlations with physical function and emotional well-being from diagnosis and over time.Materials and methodsThirty-six patients were included in the study. Individual quality of life was measured with the Schedule of Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), illness severity was assessed using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALS FRS-R), and emotional distress was measured using the Hospital Anxiety and Depression Scale (HADS). Data were collected from diagnosis and thereafter, every six months for a period of two years. Twelve patients completed the 24-month follow-up.ResultsFamily, friends and own physical health were important for overall quality of life, from diagnosis and during the disease progression. Most patients had good quality of life, which remained stable, despite changed physical functions. Several patients scored above the cut-off score for doubtful and clinical anxiety and depression early on after diagnosis, and there was a significant decrease in anxiety over time. Soon after diagnosis, there was a correlation between depression and quality of life.ConclusionThe family, social relations and own physical health are important for overall quality of life in patients with ALS. Thus, supporting the family and facilitating so that patients can continue to stay in contact with friends are important aspects during the disease. Conducting an early screening for depression can be important for preventing decreased quality of life.
      PubDate: 2017-05-19T00:20:34.180499-05:
      DOI: 10.1111/ane.12774
  • Marital status is a prognostic factor in amyotrophic lateral sclerosis
    • Authors: R. Spataro; P. Volanti, D. Lo Coco, V. La Bella
      Abstract: Background and objectivesSeveral variables have been linked to a shorter survival in patients with amyotrophic lateral sclerosis (ALS), for example, female sex, older age, site of disease onset, rapid disease progression, and a relatively short diagnostic delay. With regard to marital status, previous studies suggested that living with a partner might be associated to a longer survival and a higher likelihood to proceed to tracheostomy. Therefore, to further strengthen this hypothesis, we investigated the role of marital status as a prognostic variable in a cohort of ALS patients.MethodsWe performed a retrospective analysis on 501 consecutive ALS patients for which a complete disease's natural history and clinical/demographic data were available. At diagnosis, 409 patients (81.6%) were married or lived with a stable partner, whereas 92 patients (18.4%) were single/widowed/divorced.ResultsIn our ALS cohort, being married was associated with a median longer survival (married, 35 months [24-50] vs unmarried, 27 months [18-42]; P
      PubDate: 2017-05-03T21:10:39.950762-05:
      DOI: 10.1111/ane.12771
  • Low-frequency rTMS of the unaffected hemisphere in stroke patients: A
           systematic review
    • Authors: L. Sebastianelli; V. Versace, S. Martignago, F. Brigo, E. Trinka, L. Saltuari, R. Nardone
      Abstract: The aim of this review was to summarize the evidence for the effectiveness of low-frequency (LF) repetitive transcranial magnetic stimulation (rTMS) over the unaffected hemisphere in promoting functional recovery after stroke. We performed a systematic search of the studies using LF-rTMS over the contralesional hemisphere in stroke patients and reviewed the 67 identified articles. The studies have been gathered together according to the time interval that had elapsed between the stroke onset and the beginning of the rTMS treatment. Inhibitory rTMS of the contralesional hemisphere can induce beneficial effects on stroke patients with motor impairment, spasticity, aphasia, hemispatial neglect and dysphagia, but the therapeutic clinical significance is unclear. We observed considerable heterogeneity across studies in the stimulation protocols. The use of different patient populations, regardless of lesion site and stroke aetiology, different stimulation parameters and outcome measures means that the studies are not readily comparable, and estimating real effectiveness or reproducibility is very difficult. It seems that careful experimental design is needed and it should consider patient selection aspects, rTMS parameters and clinical assessment tools. Consecutive sessions of rTMS, as well as the combination with conventional rehabilitation therapy, may increase the magnitude and duration of the beneficial effects. In an increasing number of studies, the patients have been enrolled early after stroke. The prolonged follow-up in these patients suggests that the effects of contralesional LF-rTMS can be long-lasting. However, physiological evidence indicating increased synaptic plasticity, and thus, a more favourable outcome, in the early enrolled patients, is still lacking. Carefully designed clinical trials designed are required to address this question. LF rTMS over unaffected hemisphere may have therapeutic utility, but the evidence is still preliminary and the findings need to be confirmed in further randomized controlled trials.
      PubDate: 2017-05-02T10:17:15.532886-05:
      DOI: 10.1111/ane.12773
  • First clinical experience with levodopa/carbidopa microtablets in
           Parkinson's disease
    • Authors: M. Senek; M. Hellström, J. Albo, P. Svenningsson, D. Nyholm
      Abstract: BackgroundLevodopa is the most effective symptomatic treatment throughout the course of Parkinson's disease, but as the disease progresses, there may be a need for individualized, fine-tuned treatments.AimTo evaluate individualized levodopa/carbidopa dosing using microtablets dispensed with a dose dispenser, with respect to efficacy and usability as perceived by patients.MethodsPatient records and dose dispenser reports from patients previously or currently treated with microtablets and a dose dispenser were reviewed, and a patient questionnaire concerning effect and usability was sent to patients.ResultsEleven patient records, four dose dispenser reports and nine survey responses were obtained. The treatment effect was considered to be improved by six of nine patients. One-third found their bradykinesia to be improved, and the non-troublesome dyskinesia was unchanged according to a majority of patients; however, some experienced the duration and magnitude of troublesome dyskinesia to be worse. The usability was generally rated as good. The four dose dispenser reports obtained showed 97(±5)% total adherence.ConclusionsThe experienced effect of treatment can, for some patients, be improved by the use of microtablets, and the dose dispenser was considered user-friendly. Further studies with a larger study population and prospective design are needed to confirm the results.
      PubDate: 2017-03-15T21:00:45.186294-05:
      DOI: 10.1111/ane.12756
  • Causally treatable, hereditary neuropathies in Fabry's disease,
           transthyretin-related familial amyloidosis, and Pompe's disease
    • Authors: J. Finsterer; J. Wanschitz, S. Quasthoff, S. Iglseder, W. Löscher, W. Grisold
      Abstract: ObjectivesMost acquired neuropathies are treatable, whereas genetic neuropathies respond to treatment in Fabry's disease (FD), transthyretin-related familial amyloidosis (TTR-FA), and Pompe's disease (PD). This review summarizes and discusses recent findings and future perspectives concerning etiology, pathophysiology, clinical presentation, diagnosis, treatment, and outcome of neuropathy in FD, TTR-FA, and PD.MethodsLiterature review.ResultsNeuropathy in FD concerns particularly small, unmyelinated, or myelinated sensory fibers (small fiber neuropathy [SFN]) and autonomic fibers, manifesting as acroparesthesias, Fabry's crises, or autonomous disturbances. FD neuropathy benefits from agalsidase alpha (0.2 mg/kg every second week intravenously) or from beta (1.0 mg/kg every second week intravenously). Neuropathy in TTR-FA is axonal and affects large and small sensory, motor, and autonomous fibers. Neuropathy in TTR-FA profits from liver transplantation and the TTR kinetic stabilizer tafamidis (20 mg/d). Neuropathy in PD particularly occurs in late-onset PD and manifests as mononeuropathy, polyneuropathy, or SFN. PD neuropathy presumably responds to alglucosidase-alpha (20 mg/kg every second week intravenously).ConclusionsNeuropathy in FD, TTR-FA, and PD is predominantly a SFN and can be the dominant feature in FD and TTR-FA. SFN in FD, TTR-FA, and PD needs to be recognized and benefits from enzyme replacement treatment or TT-kinetic stabilizers.
      PubDate: 2017-03-12T22:30:26.321625-05:
      DOI: 10.1111/ane.12758
  • Issue Information
    • Pages: 555 - 557
      PubDate: 2017-11-06T03:23:25.588805-05:
      DOI: 10.1111/ane.12685
  • Prevalence and predictors of anxiety in an African sample of recent stroke
    • Authors: A. Ojagbemi; M. Owolabi, R. Akinyemi, O. Arulogun, J. Akinyemi, O. Akpa, F. S. Sarfo, E. Uvere, R. Saulson, S. Hurst, B. Ovbiagele
      First page: 617
      Abstract: ObjectivesStudies considering emotional disturbances in the setting of stroke have primarily focused on depression and been conducted in high-income countries. Anxiety in stroke survivors, which may be associated with its own unique sets of risk factors and clinical parameters, has been rarely investigated in sub-Saharan Africa (SSA). We assess the characteristics of anxiety and anxiety-depression comorbidity in a SSA sample of recent stroke survivors.Materials and methodsWe assessed baseline data being collected as part of an intervention to improve one-year blood pressure control among recent (≤1 month) stroke survivors in SSA. Anxiety in this patient population was measured using the Hospital Anxiety and Depression Scale (HADS), while the community screening instrument for dementia was used to evaluate cognitive functioning. Independent associations were assessed using logistic regression analysis.ResultsAmong 391 participants, clinically significant anxiety (HADS anxiety score≥11) was found in 77 (19.7%). Anxiety was comorbid with depression in 55 (14.1%). Female stroke survivors were more likely than males to have anxiety (OR=2.4, 95% CI=1.5-4.0). Anxiety was significantly associated with the presence of cognitive impairment after adjusting for age, gender and education (OR=6.8, 95% CI=2.6-18.0).ConclusionsOne in five recent stroke survivors in SSA has clinically significant anxiety, and well over 70% of those with anxiety also have depression. Future studies will need to determine what specific impact post-stroke anxiety may have on post-stroke clinical processes and outcomes.
      PubDate: 2017-04-17T20:40:26.178893-05:
      DOI: 10.1111/ane.12766
  • Risk factors for mortality in patients with anti-NMDA receptor
           encephalitis; Comment on data sparsity
    • Authors: E. Ayubi; S. Safiri
      Pages: 737 - 737
      PubDate: 2017-11-06T03:23:24.763293-05:
      DOI: 10.1111/ane.12792
  • Risk factors for mortality in patients with anti-NMDA receptor
           encephalitis: Reply to comment on data sparsity
    • Authors: X. Chi; D. Zhou
      Pages: 738 - 738
      PubDate: 2017-11-06T03:23:23.745042-05:
      DOI: 10.1111/ane.12791
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