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Observation | May 21, 2012 
Two in OneReport of a Patient With Spinocerebellar Ataxia Types 2 and 10
Sachin S. Kapur, MD; Jennifer G. Goldman, MD, MS
ObjectiveObjective To report a rare case of the coexistence of 2 spinocerebellar ataxia (SCA) mutations in a single patient.DesignDesign Case report.SettingSetting University ...
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Arch Neurol. Published online May 21, 2012.   doi: 10.1001/archneurol.2011.3044
Images in Neurology | May 21, 2012 
Multiple Fusiform Intracranial Aneurysms 14 Years After Atrial Myxoma ResectionMultiple Fusiform Intracranial Aneurysms
Alejandro Santillan, MD; Dimitri Sigounas, MD; Matthew E. Fink, MD; Y. Pierre Gobin, MD
A 68-year-old patient with past medical history significant for an atrial myxoma and multiple transitory ischemic attacks presented with an incidental finding ...
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Arch Neurol. Published online May 21, 2012.   doi: 10.1001/archneurol.2011.3503
Original Contribution | May 21, 2012 
C9orf72 Hexanucleotide Repeat Expansions as the Causative Mutation for Chromosome 9p21–Linked Amyotrophic Lateral Sclerosis and Frontotemporal DementiaC9orf72 Hexanucleotide Repeat Expansions
Hussein Daoud, PhD; Hamid Suhail, PhD; Mike Sabbagh, MD; Veronique Belzil, MSc; Anna Szuto, MSc; Alexandre Dionne-Laporte, MSc; Jawad Khoris, MD; William Camu, MD; Francois Salachas, MD; Vincent Meininger, MD; Jean Mathieu, MD, MSc, FRCPC; Michael Strong, MD, FRCPC; Patrick A. Dion, PhD; Guy A. Rouleau, MD, PhD, FRCPC
Includes: Supplemental Content
ObjectiveObjective To further assess the presence of a large hexanucleotide repeat expansion in the first intron of the C9orf72 gene identified as ...
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Arch Neurol. Published online May 21, 2012.   doi: 10.1001/archneurol.2012.377
Observation | May 14, 2012 
A Novel Mutation in PNPLA2 Leading to Neutral Lipid Storage Disease With MyopathyMutation in PNPLA2 Leading to NLSD With Myopathy
Daniel B. Ash, BS; Dimitra Papadimitriou, MD; Arthur P. Hays, MD; Salvatore DiMauro, MD; Michio Hirano, MD
BackgroundBackground Mutations in PNPLA2, a gene encoding adipose triglyceride lipase, lead to neutral lipid storage disease with myopathy.ObjectiveObjective To report the clinical ...
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Arch Neurol. Published online May 14, 2012.   doi: 10.1001/archneurol.2011.2600
Images in Neurology | May 14, 2012 
Multiple Septic Brain Emboli in Infectious EndocarditisBrain Emboli in Infectious Endocarditis
Jonathan Tisdell, MD; Thomas W. Smith, MD; Susanne Muehlschlegel, MD, MPH
A 34-year-old woman with known intravenous drug use presented with fever, somnolence, dysarthria, ataxia, and a generalized papulopustular rash. Cerebrospinal fluid and ...
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Arch Neurol. Published online May 14, 2012.   doi: 10.1001/archneurol.2011.3563
Original Contribution | May 14, 2012 
Frontotemporal Dementia in a Brazilian Kindred With the C9orf72 MutationFrontotemporal Dementia and C9orf72 Mutation
Leonel T. Takada, MD; Maria Lucia V. Pimentel, MD, PhD; Mariely DeJesus-Hernandez, BS; Jamie C. Fong, MS, CGC; Jennifer S. Yokoyama, PhD; Anna Karydas, BA; Marie-Pierre Thibodeau, MD; Nicola J. Rutherford, BSc; Matthew C. Baker, BSc; Catherine Lomen-Hoerth, MD, PhD; Rosa Rademakers, PhD; Bruce L. Miller, MD
Includes: Supplemental Content
ObjectivesObjectives To describe the clinical features of a Brazilian kindred with C9orf72 frontotemporal dementia–amyotrophic lateral sclerosis and compare them with other described ...
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Arch Neurol. Published online May 14, 2012.   doi: 10.1001/archneurol.2012.650
Original Contributions | May 7, 2012 
Increased Cerebral Metabolism After 1 Year of Deep Brain Stimulation in Alzheimer DiseaseIncreased Cerebral Metabolism After 1 Year of DBS
Gwenn S. Smith, PhD; Adrian W. Laxton, MD; David F. Tang-Wai, MDCM, FRCPC; Mary Pat McAndrews, PhD; Andreea Oliviana Diaconescu, PhD; Clifford I. Workman, BS; Andres M. Lozano, MD, PhD, FRCSC
BackgroundBackground The importance of developing unique, neural circuitry–based treatments for the cognitive and neuropsychiatric symptoms of Alzheimer disease (AD) was the impetus ...
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Arch Neurol. Published online May 7, 2012.   doi: 10.1001/archneurol.2012.590
Original Contribution | May 7, 2012 
Cellular Immune Suppression in Paraneoplastic Neurologic Syndromes Targeting Intracellular AntigensCellular Immune Suppression in PNDs
Dana Orange, MD; Mayu Frank, NP; Suyan Tian, MSc; Athanasios Dousmanis, MD, PhD; Ronen Marmur, MD, PhD; Noreen Buckley, NP; Salina Parveen, MS; Jerome J. Graber, MD, MPH; Nathalie Blachère, PhD; Robert B. Darnell, MD, PhD
Includes: Supplemental Content
BackgroundBackground Tumor treatment is the mainstay of therapy for paraneoplastic neurologic disorders (PNDs), but it is only effective in some cases and ...
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Arch Neurol. Published online May 7, 2012.   doi: 10.1001/archneurol.2012.595
Original Contribution | April 23, 2012 
Frontotemporal Dementia in Elderly Individuals
Atik Baborie, MD; Tim D. Griffiths, DM; Evelyn Jaros, PhD; Parastoo Momeni, PhD; Ian G. McKeith, MD; David J. Burn, MD; G. Keir, PhD; Andrew J. Larner, MD; David M. Mann, PhD; Robert Perry, MD
Includes: Supplemental Content
ObjectiveObjective To determine whether cases of frontotemporal lobar degeneration (FTLD) do exist in elderly individuals and have clinical and neuropathological features distinct ...
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Arch Neurol. Published online April 23, 2012.   doi: 10.1001/archneurol.2011.3323
Original Contribution | April 23, 2012 
Amyloid-β–Associated Clinical Decline Occurs Only in the Presence of Elevated P-tauAβ-Associated Clinical Decline and Elevated P-tau
Rahul S. Desikan, MD, PhD; Linda K. McEvoy, PhD; Wesley K. Thompson, PhD; Dominic Holland, PhD; James B. Brewer, MD, PhD; Paul S. Aisen, MD; Reisa A. Sperling, MD; Anders M. Dale, PhD; for the Alzheimer's Disease Neuroimaging Initiative
ObjectiveObjective To elucidate the relationship between the 2 hallmark proteins of Alzheimer disease (AD), amyloid-β (Aβ) and tau, and clinical decline over ...
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Arch Neurol. Published online April 23, 2012.   doi: 10.1001/archneurol.2011.3354
Images in Neurology | April 23, 2012 
Thoracal Radiculopathy Owing to Disc HerniationThoracal Radiculopathy Owing to Disc Herniation
Brynhildur Hafsteinsdottir, MD; Elias Olafsson, MD, PhD
A sharp nonradiating pain suddenly developed in the right flank of a 59-year-old man. The pain lasted 3 hours, then disappeared completely. ...
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Arch Neurol. Published online April 23, 2012.   doi: 10.1001/archneurol.2012.15
Editorial | April 23, 2012 
CSF Biomarkers for Secondary Prevention TrialsWhy Markers of Amyloid Deposition and Neurodegeneration Are Both ImportantCSF Biomarkers for Secondary Prevention Trials
David M. Holtzman, MD
A major challenge in developing therapies for Alzheimer disease (AD) as well as most neurodegenerative diseases is that by the time even ...
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Arch Neurol. Published online April 23, 2012.   doi: 10.1001/archneurol.2012.587
Images in Neurology | April 23, 2012 
Spinal Cord Glioma Metastasizing to the BrainSpinal Cord Glioma Metastasizing to the Brain
Shiv Saidha, MRCPI; Carlos A. Pardo, MD
A 50-year-old woman presented with a 2-month history of Lhermitte sign, progressive gait and limb ataxia, weakness, and urinary hesitancy. Seven years ...
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Arch Neurol. Published online April 23, 2012.   doi: 10.1001/archneurol.2012.8
Images in Neurology | April 16, 2012 
The Diagnostic Pitfall of Infratentorial Subdural EmpyemaInfratentorial Subdural Empyema
Kirsten S. Adriani, MD; Diederik van de Beek, MD, PhD; Dirk Troost, MD, PhD; Matthijs C. Brouwer, MD, PhD
A 58-year-old woman presented at the emergency department at midnight with a minor head trauma. On presentation, she had a normal temperature ...
Arch Neurol. Published online April 16, 2012.   doi: 10.1001/archneurol.2012.151
Observation | April 16, 2012 
Treatment of γ-Aminobutyric AcidBReceptor–Antibody Autoimmune Encephalitis With Oral CorticosteroidsCorticosteroid Treatment of GABAB Encephalitis
Daniel M. Goldenholz, MD, PhD; Victoria S. S. Wong, MD; Lisa M. Bateman, MD, FRCPC; Michelle Apperson, MD, PhD; Bjorn Oskarsson, MD; Shahrzad Akhtar, MD; Vicki Wheelock, MD
BackgroundBackground Autoimmune encephalitis is increasingly identified as a cause of nonviral, idiopathic encephalitis. Present treatment algorithms recommend costly immune-modulating treatments and do ...
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Arch Neurol. Published online April 16, 2012.   doi: 10.1001/archneurol.2012.197
Observation | April 16, 2012 
Anti–Glutamic Acid Decarboxylase Limbic Encephalitis Without Epilepsy Evolving Into Dementia With Cerebellar AtaxiaAnti-GAD Limbic Encephalitis Without Epilepsy
Marisol Mirabelli-Badenier, MD; Giovanni Morana, MD; Francesca Pinto, MD; Antonio Uccelli, MD; Edvige Veneselli, MD, PhD; Francesca Maria Battaglia, PhD; Roberta Biancheri, MD, PhD; Maria Giuseppina Baglietto, MD, PhD; Angela Vincent, FRCP; Maria Margherita Mancardi, MD, PhD
ObjectivesObjectives To expand the spectrum of the clinical presentation of anti–glutamic acid decarboxylase antibodies–related limbic encephalitis and to improve the recognition of ...
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Arch Neurol. Published online April 16, 2012.   doi: 10.1001/archneurol.2012.234
Original Contribution | April 16, 2012 
Effects of Age and Sex on Aquaporin-4 AutoimmunityAge and Sex Effects on Aquaporin-4 Autoimmunity
Amy M. L. Quek, MBBS; Andrew McKeon, MD; Vanda A. Lennon, MD, PhD; Jayawant N. Mandrekar, PhD; Raffaele Iorio, MD; Yujuan Jiao, MD, PhD; Chiara Costanzi, MD; Brian G. Weinshenker, MD; Dean M. Wingerchuk, MD; Claudia F. Lucchinetti, MD; Elizabeth A. Shuster, MD; Sean J. Pittock, MD
ObjectiveObjective To determine the sex and age distribution of aquaporin-4 (AQP4) autoimmunity using data derived from clinical service laboratory testing of 56 ...
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Arch Neurol. Published online April 16, 2012.   doi: 10.1001/archneurol.2012.249
Original Contribution | April 16, 2012 
ω-3 Fatty Acid Treatment in Multiple Sclerosis (OFAMS Study)A Randomized, Double-Blind, Placebo-Controlled Trialω-3 Fatty Acid Treatment in Multiple Sclerosis
Øivind Torkildsen, MD, PhD; Stig Wergeland, MD; Søren Bakke, MD; Antonie G. Beiske, MD, PhD; Kristian S. Bjerve, MD, PhD; Harald Hovdal, MD; Rune Midgard, MD, PhD; Finn Lilleås, MD; Tom Pedersen, MD; Bård Bjørnarå, MD; Frøydis Dalene, MD; Grethe Kleveland, MD; Jan Schepel, MD; Inge Christoffer Olsen, PhD; Kjell-Morten Myhr, MD, PhD
Includes: Supplemental Content
ObjectiveObjective To investigate whether ω-3 fatty acids reduce magnetic resonance imaging (MRI) and clinical disease activity in patients with multiple sclerosis, both ...
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Arch Neurol. Published online April 16, 2012.   doi: 10.1001/archneurol.2012.283
Observation | April 9, 2012 
Minocycline-Induced Fulminant Intracranial HypertensionMinocycline-Induced Intracranial Hypertension
Clare L. Fraser, MBBS, MMed; Valerie Biousse, MD; Nancy J. Newman, MD
ObjectiveObjective To describe the clinical course of an unusually severe case of minocycline-induced intracranial hypertension.DesignDesign Case study.SettingSetting Academic medical center.PatientPatient Twelve-year-old girl ...
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Arch Neurol. Published online April 9, 2012.   doi: 10.1001/archneurol.2012.144
Original Contribution | April 9, 2012 
Painful Legs and Moving Toes SyndromeA 76-Patient Case SeriesPainful Legs and Moving Toes Syndrome
Anhar Hassan, MBBCh, FRACP; Farrah J. Mateen, MD; Elizabeth A. Coon, MD; J. Eric Ahlskog, PhD, MD
ObjectiveObjective To better characterize the clinical features, electrophysiologic features, and treatment outcomes of painful legs and moving toes (PLMT) syndrome.DesignDesign Large case ...
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Arch Neurol. Published online April 9, 2012.   doi: 10.1001/archneurol.2012.161

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