49,957 results match your criteria Neurology[Journal]


Lamotrigine and hemophagocytic lymphohistiocytosis.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Department of Neurology and Neurological Sciences (K.J.M.), Stanford University School of Medicine, Palo Alto, CA; and the School of Pharmacy and Department of Neurology (B.E.G.), University of Wisconsin, Madison.

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http://dx.doi.org/10.1212/WNL.0000000000007518DOI Listing

Prognostic value of "tissue-based" definitions of TIA and minor stroke: Population-based study.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Centre for the Prevention of Stroke and Dementia, Nuffield Department of Clinical Neurosciences, University of Oxford, UK.

Objective: Since use of diffusion-weighted imaging (DWI) positivity in the "tissue-based" definition of stroke in patients with a clinical TIA is supported by the high associated 90-day risk of recurrent stroke, we aimed to determine long-term prognostic significance, stratified by etiologic subtype, and whether the same tissue-based distinction is predictive in minor strokes.

Methods: Consecutive eligible patients with TIA or minor stroke (NIH Stroke Scale [NIHSS] ≤3) in the population-based Oxford Vascular Study underwent brain MRI at baseline. Stroke risk on 10-year follow-up was stratified by NIHSS (0/1 vs 2/3) and Trial of Org 10172 in Acute Stroke Treatment classification of the initial event. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007531DOI Listing

Erenumab in chronic migraine: Patient-reported outcomes in a randomized double-blind study.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Department of Neurology (R.B.L.), Albert Einstein College of Medicine, Bronx, New York; Geisel School of Medicine at Dartmouth (S.J.T.), Hanover, NH; Department of Neurology (U.R.), Charité Universitätsmedizin Berlin, Germany; Jefferson Headache Center (S.S.), Thomas Jefferson University, Philadelphia, PA; Sutter Health (W.F.S.), Walnut Creek, CA; and Amgen Inc. (J.N., D.K.L., P.D., S.C., D.D.M., R.L.), Thousand Oaks, CA.

Objective: To determine the effect of erenumab, a human monoclonal antibody targeting the calcitonin gene-related peptide receptor, on health-related quality of life (HRQoL), headache impact, and disability in patients with chronic migraine (CM).

Methods: In this double-blind, placebo-controlled study, 667 adults with CM were randomized (3:2:2) to placebo or erenumab (70 or 140 mg monthly). Exploratory endpoints included migraine-specific HRQoL (Migraine-Specific Quality-of-Life Questionnaire [MSQ]), headache impact (Headache Impact Test-6 [HIT-6]), migraine-related disability (Migraine Disability Assessment [MIDAS] test), and pain interference (Patient-Reported Outcomes Measurement Information System [PROMIS] Pain Interference Scale short form 6b). Read More

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http://dx.doi.org/10.1212/WNL.0000000000007452DOI Listing

Hemophagocytic lymphohistiocytosis associated with the use of lamotrigine.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Division of Pharmacovigilance, Office of Surveillance and Epidemiology, US Food and Drug Administration, Silver Spring, MD.

Objective: To describe adverse event reports of hemophagocytic lymphohistiocytosis (HLH) reported in association with lamotrigine.

Methods: The Food and Drug Administration Adverse Event Reporting System database of spontaneous adverse event reports and medical literature databases were searched for cases of HLH reported in association with lamotrigine. Cases were included if they met the case definition of suspected or confirmed HLH and if causal association was assessed as robust or supportive. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007517DOI Listing

Clinical complications and outcomes of angiographically negative subarachnoid hemorrhage.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Departments of Neurological Surgery (C.L.N., S.O., L.R., G.L.) and Neurology (E.F.W., A.A.R.), Mayo Clinic, Rochester, MN.

Objective: To define the in-hospital course, complications, short- and long-term functional outcomes of patients with angiographically negative subarachnoid hemorrhage (anSAH), particularly those with aneurysmal-pattern anSAH (aanSAH).

Methods: Retrospective cohort study of patients with aneurysmal subarachnoid hemorrhage (aSAH), aanSAH, and perimesencephalic-pattern anSAH (panSAH) treated at a single tertiary referral center between January 2006 and April 2018. Ninety-nine patients with anSAH (33 aanSAH and 66 panSAH) and 464 patients with aSAH were included in this study. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007501DOI Listing
April 2019
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Network-targeted stimulation engages neurobehavioral hallmarks of age-related memory decline.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Departments of Medical Social Sciences (A.S.N., E.L.K., J.L.V.), Ken and Ruth Davee Department of Neurology (M.-M.M., S.V., J.L.V.), and Psychiatry and Behavioral Sciences (S.W., J.L.V.), Cognitive Neurology and Alzheimer's Disease Center (A.S.N., M.-M.M., S.W.), Feinberg School of Medicine, Northwestern University, Chicago, IL.

Objective: To test whether targeting hippocampal-cortical brain networks with high-frequency transcranial magnetic stimulation in older adults influences behavioral and neural measures characteristic of age-related memory impairment.

Methods: Fifteen adults aged 64 to 80 years (mean = 72 years) completed a single-blind, sham-controlled experiment. Stimulation targets in parietal cortex were determined based on fMRI connectivity with the hippocampus. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007502DOI Listing
April 2019
1 Read

Erenumab in chronic migraine with medication overuse: Subgroup analysis of a randomized trial.

Neurology 2019 Apr 17. Epub 2019 Apr 17.

From the Geisel School of Medicine at Dartmouth (S.J.T.), Hanover, NH; Department of Neurology (H.-C.D.), University of Duisburg-Essen, Germany; Department of Neurology (M.A.), Danish Headache Center, Rigshospitalet Glostrup, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark; Department of Neurology (J.L.B.), Vanderbilt University School of Medicine; Nashville Neuroscience Group (J.L.B.), TN; Neurology and Neurotherapeutics and Ophthalmology (D.I.F.), University of Texas Southwestern Medical Center, Dallas; Department of Neurology (U.R.), Charité Universitätsmedizin Berlin, Germany; and Amgen Inc (S.C., J.N., D.K.L., R.A.L., D.D.M.), Thousand Oaks, CA.

Objective: To determine the effect of erenumab, a human anti-calcitonin gene-related peptide receptor monoclonal antibody, in patients with chronic migraine and medication overuse.

Methods: In this double-blind, placebo-controlled study, 667 adults with chronic migraine were randomized (3:2:2) to placebo or erenumab (70 or 140 mg), stratified by region and medication overuse status. Data from patients with baseline medication overuse at baseline were used to assess changes in monthly migraine days, acute migraine-specific medication treatment days, and proportion of patients achieving ≥50% reduction from baseline in monthly migraine days. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007497DOI Listing
April 2019
1 Read

Author Correction: Biomarkers for closed-loop deep brain stimulation in Parkinson disease and beyond.

Nat Rev Neurol 2019 Apr 16. Epub 2019 Apr 16.

Division of Neurology, Department of Clinical Neuroscience, Geneva University Hospital, Geneva, Switzerland.

In the originally published article, one of the affiliations for Paul Krack was omitted - these should have included 'Movement Disorders Center, Department of Neurology, University Hospital (Inselspital) and University of Bern, Bern, Switzerland.' This error has been corrected in the HTML and PDF versions of the manuscript. Read More

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http://dx.doi.org/10.1038/s41582-019-0189-xDOI Listing

Association of the MMP-9 polymorphism and ischemic stroke risk in southern Chinese Han population.

BMC Neurol 2019 Apr 16;19(1):67. Epub 2019 Apr 16.

Department of Neurosurgery, Affiliated Haikou Hospital of Xiangya Medical College in Central South University, Haikou, Hainan, 570208, China.

Background: Stroke is a serious cardiovascular disease and is also the leading cause of long-term disability in developing and developed countries. Because matrix metalloproteinase-9 (MMP-9) is associated with the risk of many cardiovascular diseases, we investigated the relationship between single nucleotide polymorphisms (SNPs) in MMP-9 and the risk of Ischemic stroke (IS) in a southern Chinese Han population.

Methods: This study included 250 stroke patients and 250 healthy controls. Read More

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https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-
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http://dx.doi.org/10.1186/s12883-019-1285-7DOI Listing
April 2019
1 Read

Distinct tau filaments in CTE.

Authors:
Rebecca Kelsey

Nat Rev Neurol 2019 Apr 15. Epub 2019 Apr 15.

Nature Reviews Neurology, .

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http://www.nature.com/articles/s41582-019-0185-1
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http://dx.doi.org/10.1038/s41582-019-0185-1DOI Listing
April 2019
1 Read

Immunoneuropsychiatry - novel perspectives on brain disorders.

Nat Rev Neurol 2019 Apr 15. Epub 2019 Apr 15.

Department of Neurology, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany.

Immune processes have a vital role in CNS homeostasis, resilience and brain reserve. Our cognitive and social abilities rely on a highly sensitive and fine-tuned equilibrium of immune responses that involve both innate and adaptive immunity. Autoimmunity, chronic inflammation, infection and psychosocial stress can tip the scales towards disruption of higher-order networks. Read More

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http://www.nature.com/articles/s41582-019-0174-4
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http://dx.doi.org/10.1038/s41582-019-0174-4DOI Listing
April 2019
3 Reads

Teaching NeuroImages: A disintegrating rock.

Neurology 2019 Apr;92(16):e1937-e1938

From the Departments of Neurology and Internal Medicine (M.A.H.), FLENI, Buenos Aires, Argentina; and Department of Neurology (A.A.R.), Mayo Clinic, Rochester, MN.

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http://dx.doi.org/10.1212/WNL.0000000000007299DOI Listing

Teaching NeuroImages: Waardenburg syndrome type 2.

Neurology 2019 Apr;92(16):e1935-e1936

From the Departments of Neuroradiology (M.R.J.), Radiology (L.M.S.), and Otorhinolaryngology (B.F.R., H.F.R.), Hospital Universitário Cassiano Antônio de Moraes da Universidade Federal do Espírito Santo-HUCAM/UFES/EBSERH, Vitória ES, Brazil.

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http://dx.doi.org/10.1212/WNL.0000000000007318DOI Listing

Teaching NeuroImages: The curious case of the brainstem kink.

Neurology 2019 Apr;92(16):e1933-e1934

From the Department of Pediatrics (R.S.), Division of Neurology, Faculty of Medicine & Dentistry, Edmonton Clinic Health Academy, University of Alberta (F.-D.M.-J.), Edmonton, Canada.

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http://dx.doi.org/10.1212/WNL.0000000000007320DOI Listing

Right Brain: Silence.

Authors:
Ricardo Varela

Neurology 2019 Apr;92(16):772-773

From the Neurology Department, Centro Hospitalar e Universitário de Coimbra, Portugal.

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http://dx.doi.org/10.1212/WNL.0000000000007297DOI Listing

Exceptional involvement of medulla oblongata in Wilson disease.

Neurology 2019 Apr;92(16):770-771

From the Neurology Department (A.P., F.W.), National Reference Centre for Wilson's Disease (A.P., F.W.), and Neuroradiology Department (A.G.), AP-HP, Lariboisière University Hospital, Paris, France.

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http://dx.doi.org/10.1212/WNL.0000000000007321DOI Listing

Persistent connection.

Authors:
Andrew Silverman

Neurology 2019 Apr;92(16):768-769

From the Department of Neurology, Yale School of Medicine, New Haven, CT.

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http://dx.doi.org/10.1212/WNL.0000000000007304DOI Listing

What's Happening in Neurology® Genetics.

Authors:

Neurology 2019 Apr;92(16):756

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http://dx.doi.org/10.1212/WNL.0000000000007303DOI Listing

Correction to Lancet Neurol 2019; 18: 504-12.

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Lancet Neurol 2019 Apr 12. Epub 2019 Apr 12.

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http://dx.doi.org/10.1016/S1474-4422(19)30156-5DOI Listing

Effects of the blood urea nitrogen to creatinine ratio on haemorrhagic transformation in AIS patients with diabetes mellitus.

BMC Neurol 2019 Apr 13;19(1):63. Epub 2019 Apr 13.

Center of Cerebrovascular Diseases, Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Background: The effect of the blood urea nitrogen (BUN) to creatinine (Cr) ratio (henceforth BUN/Cr) on haemorrhagic transformation (HT) of acute ischaemic stroke (AIS) patients is unclear.

Methods: AIS patients in the West China Hospital, Sichuan University, Chengdu, China, admitted within seven days from stroke onset (2012-2016) were included in the study. Baseline data, including BUN and Cr levels, were collected. Read More

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http://dx.doi.org/10.1186/s12883-019-1290-xDOI Listing

Optimal management after paediatric lumbar puncture: a randomized controlled trial.

BMC Neurol 2019 Apr 13;19(1):64. Epub 2019 Apr 13.

Department of Infectious Diseases, Beijing Children's Hospital, Capital Medical University, Nalishi Road 56#, Xicheng District, Beijing, 100045, China.

Background: To evaluate whether a shorter time of lying supine without a pillow and fasting for solids and liquids (LSFSL) after a lumbar puncture (LP) is associated with a higher risk of post-lumbar puncture headache (PLPH) and post-lumbar puncture lower back pain (PLPBP) in a randomized, assessor-blinded, controlled trial.

Methods: Paediatric patients who underwent their first LP after hospital admission were randomly allocated to either the group with half an hour of LSFSL (0.5 h LSFSL) or 4 h of LSFSL (4 h LSFSL) immediately after LP. Read More

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http://dx.doi.org/10.1186/s12883-019-1275-9DOI Listing
April 2019
2.040 Impact Factor

Stent-retriever assisted vacuum-locked extraction (SAVE) versus a direct aspiration first pass technique (ADAPT) for acute stroke: data from the real-world.

BMC Neurol 2019 Apr 15;19(1):65. Epub 2019 Apr 15.

Department of Neuroradiology, University Medical Center Goettingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany.

Background: Embolectomy is the standard of care in acute ischemic stroke (AIS) caused by large vessel occlusion (LVO). Aim of this study was to compare two techniques: A Direct Aspiration First Pass Technique (ADAPT) and Stent-retriever Assisted Vacuum-locked Extraction (SAVE) stratified by the occluded vessel.

Methods: One hundred seventy-one patients (71 male) treated between January 2014 and September 2017 with AIS due to LVO of the anterior circulation (55 carotid T, 94 M1, 22 M2) were included. Read More

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https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-
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http://dx.doi.org/10.1186/s12883-019-1291-9DOI Listing
April 2019
3 Reads

A biomechanical study of gait initiation in Down syndrome.

BMC Neurol 2019 Apr 15;19(1):66. Epub 2019 Apr 15.

Department of Electronics, Information and Bioengineering, Politecnico di Milano, Piazza Leonardo da Vinci 32, 20133, Milan, Italy.

Background: Gait Initiation (GI) is a functional task that challenges the balance control requiring weight shift and a transition from standing to walking. Individuals with Down Syndrome (DS) walk with low velocity, prolonged stance and shorter steps beside an increased support base. However, no studies performed GI analysis on this population. Read More

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http://dx.doi.org/10.1186/s12883-019-1288-4DOI Listing

CSF and blood biomarkers for Parkinson's disease.

Lancet Neurol 2019 Apr 10. Epub 2019 Apr 10.

Section of Neurology, Department of Medicine, University of Perugia, Perugia, Italy; IRCCS Fondazione Santa Lucia, Rome, Italy.

In the management of Parkinson's disease, reliable diagnostic and prognostic biomarkers are urgently needed. The diagnosis of Parkinson's disease mostly relies on clinical symptoms, which hampers the detection of the earliest phases of the disease-the time at which treatment with forthcoming disease-modifying drugs could have the greatest therapeutic effect. Reliable prognostic markers could help in predicting the response to treatments. Read More

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http://dx.doi.org/10.1016/S1474-4422(19)30024-9DOI Listing

Daniel Friedman.

Authors:

Lancet Neurol 2019 Apr 10. Epub 2019 Apr 10.

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http://dx.doi.org/10.1016/S1474-4422(19)30113-9DOI Listing

Correction to Lancet Neurol 2019; 18: 357-75.

Authors:

Lancet Neurol 2019 May;18(5):e4

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http://dx.doi.org/10.1016/S1474-4422(19)30120-6DOI Listing

Correction to Lancet Neurol 2018; 17: 394-95.

Authors:

Lancet Neurol 2019 May;18(5):e4

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http://dx.doi.org/10.1016/S1474-4422(19)30109-7DOI Listing

Clinical application of next-generation sequencing to the practice of neurology.

Lancet Neurol 2019 May;18(5):492-503

Program in Neurogenetics, Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles, CA, USA; Department of Human Genetics, David Geffen School of Medicine, University of California, Los Angeles, CA, USA; Clinical Neurogenomics Research Center, Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles, CA, USA. Electronic address:

Next-generation sequencing technologies allow for rapid and inexpensive large-scale genomic analysis, creating unprecedented opportunities to integrate genomic data into the clinical diagnosis and management of neurological disorders. However, the scale and complexity of these data make them difficult to interpret and require the use of sophisticated bioinformatics applied to extensive datasets, including whole exome and genome sequences. Detailed analysis of genetic data has shown that accurate phenotype information is essential for correct interpretation of genetic variants and might necessitate re-evaluation of the patient in some cases. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S14744422193003
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http://dx.doi.org/10.1016/S1474-4422(19)30033-XDOI Listing
May 2019
1 Read

Rufus Olusola Akinyemi.

Authors:

Lancet Neurol 2019 May;18(5):426

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http://dx.doi.org/10.1016/S1474-4422(19)30119-XDOI Listing

Pitfalls in using neonatal brain NAA to predict infant development.

Authors:
Lars Walløe

Lancet Neurol 2019 May;18(5):423

Division of Physiology, Institute of Basic Medical Sciences, University of Oslo, Oslo NO-0317, Norway. Electronic address:

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http://dx.doi.org/10.1016/S1474-4422(19)30111-5DOI Listing

Pitfalls in using neonatal brain NAA to predict infant development - Authors' reply.

Lancet Neurol 2019 May;18(5):423-424

Centre for Perinatal Neuroscience, Imperial College London, London W12 0HS, UK. Electronic address:

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http://dx.doi.org/10.1016/S1474-4422(19)30116-4DOI Listing

Recovery from brain injury: a surprising new drug target.

Lancet Neurol 2019 May;18(5):421-422

Department of Neurology, Washington University School of Medicine, St Louis, MO, USA.

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https://linkinghub.elsevier.com/retrieve/pii/S14744422193011
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http://dx.doi.org/10.1016/S1474-4422(19)30117-6DOI Listing
May 2019
2 Reads

The risks of ignoring scientific evidence.

Lancet Neurol 2019 May;18(5):415

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http://dx.doi.org/10.1016/S1474-4422(19)30089-4DOI Listing

FSHD1 and FSHD2 form a disease continuum.

Neurology 2019 Apr 12. Epub 2019 Apr 12.

From the Peripheral Nervous System (S.S., M.G., C.C., A.P.), Muscle & ALS Department, Pasteur 2 Hospital, Centre Hospitalier Universitaire de Nice, and Institute for Research on Cancer and Aging of Nice (S.S., C.B., N.L., P.N., G.C.), CNRS, INSERM, Université Côte d'Azur; Department of Genetics and Molecular Biology (A.B.-S., S.R., M.J.), Cochin Hospital, Paris, France; Department of Human Genetics (R.J.L.F.L., S.M.v.d.M.), Leiden University Medical Center, the Netherlands; Rare Neuromuscular Diseases Centre (C.C.), Department of Human Neuroscience, Sapienza University of Rome, Italy; Pathology Department (F.C.), CHRU of Caen, INSERM U1075, University of Caen, Normandy; Myology Institute (T.S., A.B., B.E.), Center of Research in Myology, APHP, Sorbonne Université, UPMC Univ Paris 06, INSERM UMRS974, CNRS FRE3617, Paris; Electromyography and Neuromuscular Department (C.V., F.B., P.P.), Neurologic Hospital, Lyon East Hospital Group, Lyon-Bron, France; Neuromuscular Center, Department of Neuroscience (M.C., E.P.), and Clinical Genetics Unit, Department of Women's and Children's Health (L.S.), University of Padova, Italy; Institut Imagine, Imagine Bioinfomatics Platform (M.B.), Paris Descartes University; Département de Neurologie (A.E.-L.), Hôpitaux Universitaires, Strasbourg; Nord/Est/Ile de France Neuromuscular Center (P.L.), Neurology Department, Raymond Poincaré Teaching Hospital, Garches; INSERM U1179 (P.L.), END-ICAP, Versailles Saint-Quentin-en-Yvelines University, Montigny-le-Bretonneux, France; and IRP Città della Speranza (L.S.), Padova, Italy.

Objective: To compare the clinical features of patients showing a classical phenotype of facioscapulohumeral muscular dystrophy (FSHD) with genetic and epigenetic characteristics of the FSHD1 and FSHD2 loci D4Z4 and .

Methods: This is a national multicenter cohort study. We measured motor strength, motor function, and disease severity by manual muscle testing sumscore, Brooke and Vignos scores, clinical severity score (CSS), and age-corrected CSS, respectively. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007456DOI Listing
April 2019
3 Reads

Incidence of frontotemporal lobar degeneration in Italy: The Salento-Brescia Registry study.

Neurology 2019 Apr 12. Epub 2019 Apr 12.

From the Center for Neurodegenerative Diseases and the Aging Brain (G.L., M.P., C.Z., R.C., R. Tortelli, P.B., R.B.), Department of Clinical Research in Neurology, and Department of Basic Medical Sciences, Neuroscience and Sense Organs (G.L., M.P., P.B.), University of Bari "Aldo Moro"; "Pia Fondazione Cardinale G. Panico" (G.L., M.P., C.Z., R.C., R. Tortelli, P.B., R.B.), Tricase, Lecce; IRCCS Centro San Giovanni di Dio Fatebenefratelli (G.B., S.F., L.B., R.G., S.F.C.); Department of Clinical and Experimental Sciences (R. Turrone, A.P., A.A., B.B.), Neurology Unit, University of Brescia; Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA) (P.B.), University of Firenze; and Department of Neurology (E.B.), Ospedali Riuniti, Foggia, Italy.

Objective: The goal of the present work, based on a collaborative research registry in Italy (the Salento-Brescia Registry), was to assess the incidence of frontotemporal lobar degeneration (FTLD) and to define the frequencies of different FTLD phenotypes in the general population.

Methods: The study was conducted from January 1, 2017, to December 31, 2017, in 2 Italian provinces: Lecce (in Puglia) in the south (area 2,799.07 km, inhabitants 802,082) and Brescia (in Lombardy) in the north (area 4,785. Read More

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http://www.neurology.org/lookup/doi/10.1212/WNL.000000000000
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http://dx.doi.org/10.1212/WNL.0000000000007498DOI Listing
April 2019
1 Read

Recognizing autoimmune encephalitis as a cause of seizures: Treating cause and not effect.

Neurology 2019 Apr 12. Epub 2019 Apr 12.

From the Division of Epilepsy (J.W.B.) and ICREA and Institut d'Investigacions Biomèdiques August Pi i Sunyer Hospital Clínic (J.D.), Universitat de Barcelona, Spain; and Department of Neurology(J.D.), University of Pennsylvania, Philadelphia.

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http://dx.doi.org/10.1212/WNL.0000000000007444DOI Listing
April 2019
1 Read

Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABAR encephalitis.

Neurology 2019 Apr 12. Epub 2019 Apr 12.

From the Departments of Neurology (M.A.A.M.d.B., A.v.S., M.H.v.C.-H., A.E.M.B., P.A.E.S.S., M.J.T.), Immunology (M.W.J.S.), and Pediatric Neurology (R.F.N.), Erasmus MC University Medical Center, Rotterdam; Haga Hospital (A.v.S.), the Hague; Department of Neurology (R.P.W.R., M.H.J.M.M.), Maastricht UMC+, Maastricht; Department of Neurology (C.A.v.D.), Maasstad Hospital, Rotterdam; Department of Neurology (M.J.M.M.), Academic Center of Epileptology Kempenhaeghe; Sophia Children's Hospital (R.F.N.), Rotterdam; Department of Neurology (R.D.T.), Stichting Epilepsie Instellingen Nedederland (SEIN), Heemstede; and Department of Neurology (R.D.T.), Leiden University Medical Center (LUMC), the Netherlands.

Objective: This nationwide cohort study evaluates seizure responses to immunotherapy and antiepileptic drugs (AEDs) in patients with anti-leucine-rich glioma-inactivated 1 (LGI1), anti-NMDA receptor (NMDAR), and anti-gamma-aminobutyric-acid B receptor (GABAR) encephalitis.

Methods: Anti-LGI1, anti-NMDAR, and anti-GABAR encephalitis patients with new-onset seizures were included. Medical information about disease course, AEDs and immunotherapies used, effects, and side effects were collected. Read More

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http://www.neurology.org/lookup/doi/10.1212/WNL.000000000000
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http://dx.doi.org/10.1212/WNL.0000000000007475DOI Listing
April 2019
2 Reads

Attention and corpus callosum volumes in individuals with mucopolysaccharidosis type I.

Neurology 2019 Apr 12. Epub 2019 Apr 12.

From the Departments of Pediatrics (K.K., I.N., V.K., K.A.D., J.B.E., E.G.S.), Psychiatry (J.R.W., B.A.M., K.O.L.), and Genetics and Metabolism (C.B.W.), University of Minnesota Medical Center; Division of Biostatistics (K.D.R.), University of Minnesota School of Public Health, Minneapolis; Department of Psychology (E.G.M., J.R.), Hospital for Sick Children-Toronto, Ontario, Canada; Department of Human Genetics (N.A., S.C.), Emory University, Atlanta, GA; and Division of Gastroenterology and Nutrition (P.H.), UCSF Benioff Children's Hospital Oakland, San Francisco, CA. Dr. Kovac is now at the School of Medicine, Washington University in St. Louis, MO. Dr. Raiman is now at the Department of Inherited Metabolic Diseases, Birmingham Children's Hospital, UK. K.A. Delaney is now at Biomarin Pharmaceuticals, San Rafael, CA.

Objective: Previous research suggests attention and white matter (WM) abnormalities in individuals with mucopolysaccharidosis type I (MPS I); this cross-sectional comparison is one of the first to examine the relationship of WM structural abnormalities as measured by corpus callosum (CC) volumes with attention scores to evaluate this relationship in a larger sample of patients with MPS I.

Methods: Volumetric MRI data and performance on a computerized measure of sustained attention were compared for 18 participants with the severe form of MPS I (MPS IH), 18 participants with the attenuated form of MPS I (MPS I), and 60 typically developing age-matched controls.

Results: The MPS I groups showed below-average mean attention scores ( < 0. Read More

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http://dx.doi.org/10.1212/WNL.0000000000007496DOI Listing

FSHD1 or FSHD2: that is the question: The answer: It's all just FSHD.

Neurology 2019 Apr 12. Epub 2019 Apr 12.

From the Department of Neurology (N.E.J.), Virginia Commonwealth University, Richmond; and the Department of Neurology (J.M.S.), Kansas University Medical Center, Kansas City.

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http://dx.doi.org/10.1212/WNL.0000000000007446DOI Listing

Diffusion tensor imaging for assessment of microstructural changes associate with treatment outcome at one-year after radiofrequency Rhizotomy in trigeminal neuralgia.

BMC Neurol 2019 Apr 12;19(1):62. Epub 2019 Apr 12.

Department of Diagnostic Radiology, Chang Gung Memorial Hospital Chiayi Branch, No.6 Chia-Pu Rd. West Sec., Chiayi County, Taiwan.

Background: Trigeminal neuralgia (TN) is characterized by facial pain that may be sudden, intense, and recurrent. Neurosurgical interventions, such as radiofrequency rhizotomy, can relieve TN pain, but their mechanisms and effects are unknown. The aim of the present study was to investigate the microstructural tissue changes of the trigeminal nerve (TGN) in patients with TN after they underwent radiofrequency rhizotomy. Read More

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https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-
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http://dx.doi.org/10.1186/s12883-019-1295-5DOI Listing
April 2019
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A novel PDGFRB sequence variant in a family with a mild form of primary familial brain calcification: a case report and a review of the literature.

BMC Neurol 2019 Apr 12;19(1):60. Epub 2019 Apr 12.

Department of Clinical Genetics, Odense University Hospital, J. B. Winsløvs Vej 4, DK-5000, Odense, Denmark.

Background: Primary familial brain calcification is a rare autosomal dominant or recessive neurodegenerative disease, characterized by bilateral brain calcifications in different areas of the brain. It is a clinically heterogeneous disease and patients are reported to exhibit a wide spectrum of neurological and psychiatric symptoms. Mutations in five genes have been identified so far including SLC20A2, PDGFRB, PDGFB, XPR1, and MYORG. Read More

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http://dx.doi.org/10.1186/s12883-019-1292-8DOI Listing

Michigan Neural Distinctiveness (MiND) study protocol: investigating the scope, causes, and consequences of age-related neural dedifferentiation.

BMC Neurol 2019 Apr 12;19(1):61. Epub 2019 Apr 12.

Department of Psychology, University of Michigan, 530 Church Street, Ann Arbor, MI, 48109, USA.

Background: Aging is often associated with behavioral impairments, but some people age more gracefully than others. Why? One factor that may play a role is individual differences in the distinctiveness of neural representations. Previous research has found that neural activation patterns in visual cortex in response to different visual stimuli are often more similar (i. Read More

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http://dx.doi.org/10.1186/s12883-019-1294-6DOI Listing

Stroke genetics: discovery, biology, and clinical applications.

Lancet Neurol 2019 Apr 8. Epub 2019 Apr 8.

Program in Medical Population and Genetics, Broad Institute, Cambridge, MA, USA; Henry and Allison McCance Center for Brain Health, and Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA.

Stroke, a leading cause of long-term disability and death worldwide, has a heritable component. Recent gene discovery efforts have expanded the number of known single-gene disorders associated with stroke and have linked common variants at approximately 35 genetic loci to stroke risk. These discoveries have highlighted novel mechanisms and pathways implicated in stroke related to large artery atherosclerosis, cardioembolism, and small vessel disease, and defined shared genetic influences with related vascular traits. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S14744422193004
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http://dx.doi.org/10.1016/S1474-4422(19)30043-2DOI Listing
April 2019
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