abstract：Importance:Galcanezumab (LY2951742), a monoclonal antibody against calcitonin gene-related peptide (CGRP), is one of a novel class of new medicines for migraine prevention. Objective:To assess whether at least 1 dose of galcanezumab was superior to placebo for episodic migraine prevention. Design, Setting, and Partic...

journal_title：JAMA neurology

authors： Skljarevski V,Oakes TM,Zhang Q,Ferguson MB,Martinez J,Camporeale A,Johnson KW,Shan Q,Carter J,Schacht A,Goadsby PJ,Dodick DW

更新日期：2018-02-01 00:00:00

abstract：Importance:The goal of Alzheimer disease (AD) prevention together with advances in understanding the pathophysiology of AD have led to clinical trials testing drugs in cognitively unimpaired persons who show evidence of AD biomarkers. Data are needed to inform the processes of describing AD biomarkers to cognitively no...

journal_title：JAMA neurology

authors： Mozersky J,Sankar P,Harkins K,Hachey S,Karlawish J

更新日期：2018-01-01 00:00:00

abstract：Importance:Neurologic disorders with isolated symptoms or complex syndromes are relatively frequent among mitochondrial inherited diseases. Recessive RTN4IP1 gene mutations have been shown to cause isolated and syndromic optic neuropathies. Objective:To define the spectrum of clinical phenotypes associated with mutati...

journal_title：JAMA neurology

authors： Charif M,Nasca A,Thompson K,Gerber S,Makowski C,Mazaheri N,Bris C,Goudenège D,Legati A,Maroofian R,Shariati G,Lamantea E,Hopton S,Ardissone A,Moroni I,Giannotta M,Siegel C,Strom TM,Prokisch H,Vignal-Clermont C,Der

更新日期：2018-01-01 00:00:00

abstract：Importance:Biomarkers do not determine conversion to Alzheimer disease (AD) perfectly, and criteria do not specify how to take patient characteristics into account. Consequently, biomarker use may be challenging for clinicians, especially in patients with mild cognitive impairment (MCI). Objective:To construct biomark...

journal_title：JAMA neurology

authors： van Maurik IS,Zwan MD,Tijms BM,Bouwman FH,Teunissen CE,Scheltens P,Wattjes MP,Barkhof F,Berkhof J,van der Flier WM,Alzheimer’s Disease Neuroimaging Initiative.

更新日期：2017-12-01 00:00:00

abstract：Importance:Scientific and lay interest in negative outcomes associated with exposure to repetitive brain trauma (RBT) continues to strengthen. Concerns about the association between RBT and dementia began more than a century ago, but have resurfaced in the last decade with the more recently described chronic traumatic ...

journal_title：JAMA neurology

authors： Asken BM,Sullan MJ,DeKosky ST,Jaffee MS,Bauer RM

更新日期：2017-10-01 00:00:00

abstract：Importance:The utility of plasma total tau level as a prognostic marker for cognitive decline and dementia is not well understood. Objectives:To determine (1) the association between plasma total tau level, cognitive decline, and risk of mild cognitive impairment (MCI) and dementia; (2) whether this association differ...

journal_title：JAMA neurology

authors： Mielke MM,Hagen CE,Wennberg AMV,Airey DC,Savica R,Knopman DS,Machulda MM,Roberts RO,Jack CR Jr,Petersen RC,Dage JL

更新日期：2017-09-01 00:00:00

abstract：Importance:This study reports the long-term epidemiologic trends of amyotrophic lateral sclerosis (ALS) based on a prospective register. Objective:To examine the 20-year epidemiologic trends of ALS in the Piemonte and Valle d'Aosta regions of Italy. Design, Setting, and Participants:The Piemonte and Valle d'Aosta Reg...

journal_title：JAMA neurology

authors： Chiò A,Mora G,Moglia C,Manera U,Canosa A,Cammarosano S,Ilardi A,Bertuzzo D,Bersano E,Cugnasco P,Grassano M,Pisano F,Mazzini L,Calvo A,Piemonte and Valle d’Aosta Register for ALS (PARALS).

更新日期：2017-09-01 00:00:00

abstract：:A young man with normal neurodevelopment presented with 3 years of medically refractory, progressive epilepsy and myoclonus. Initial examination included neuroimaging, electroencephalography, and biochemical analyses, all of which were unremarkable except for mildly enlarged ventricles. Over the following year, the pa...

journal_title：JAMA neurology

authors： Cohen AL,Jones LK,Parisi JE,Klaas JP

更新日期：2017-06-01 00:00:00

abstract：Importance:Amyotrophic lateral sclerosis (ALS) is a common adult-onset neurodegenerative disease characterized by selective loss of upper and lower motor neurons. Patients with ALS have persistent peripheral and central inflammatory responses including abnormally functioning T cells and activated microglia. However, mu...

journal_title：JAMA neurology

authors： Zhao W,Beers DR,Hooten KG,Sieglaff DH,Zhang A,Kalyana-Sundaram S,Traini CM,Halsey WS,Hughes AM,Sathe GM,Livi GP,Fan GH,Appel SH

更新日期：2017-06-01 00:00:00

abstract：Importance:YARS2 mutations have been associated with a clinical triad of myopathy, lactic acidosis, and sideroblastic anemia in predominantly Middle Eastern populations. However, the identification of new patients expands the clinical and molecular spectrum of mitochondrial disorders. Objectives:To review the clinical...

journal_title：JAMA neurology

authors： Sommerville EW,Ng YS,Alston CL,Dallabona C,Gilberti M,He L,Knowles C,Chin SL,Schaefer AM,Falkous G,Murdoch D,Longman C,de Visser M,Bindoff LA,Rawles JM,Dean JCS,Petty RK,Farrugia ME,Haack TB,Prokisch H,McFarland R

更新日期：2017-06-01 00:00:00

abstract：Importance:A neurophysiologic signature of the melanopsin-mediated persistent constriction phase of the pupillary light reflex may represent a surrogate biomarker for the integrity of the retinohypothalamic tract, with potential utility for investigating alterations in homeostatic mechanisms associated with brain disor...

journal_title：JAMA neurology

authors： Meltzer E,Sguigna PV,Subei A,Beh S,Kildebeck E,Conger D,Conger A,Lucero M,Frohman BS,Frohman AN,Saidha S,Galetta S,Calabresi PA,Rennaker R,Frohman TC,Kardon RH,Balcer LJ,Frohman EM

更新日期：2017-05-01 00:00:00

abstract：Importance:Intravenous thrombolysis (IVT) followed by mechanical thrombectomy (MT) is recommended to treat acute ischemic stroke (AIS) with a large vessel occlusion (LVO). Most hospitals do not have on-site MT facilities, and most patients need to be transferred secondarily after IVT (drip and ship), which may have an ...

journal_title：JAMA neurology

authors： Gerschenfeld G,Muresan IP,Blanc R,Obadia M,Abrivard M,Piotin M,Alamowitch S

更新日期：2017-05-01 00:00:00

abstract：Importance:Mechanical thrombectomy (MT) improves clinical outcomes in patients with acute ischemic stroke (AIS) caused by a large vessel occlusion. However, it is not known whether intravenous thrombolysis (IVT) is of added benefit in patients undergoing MT. Objective:To examine whether treatment with IVT before MT wi...

journal_title：JAMA neurology

authors： Coutinho JM,Liebeskind DS,Slater LA,Nogueira RG,Clark W,Dávalos A,Bonafé A,Jahan R,Fischer U,Gralla J,Saver JL,Pereira VM

更新日期：2017-03-01 00:00:00

abstract：Importance:Periodic discharges (PDs) that do not meet seizure criteria, also termed the ictal interictal continuum, are pervasive on electroencephalographic (EEG) recordings after acute brain injury. However, their association with brain homeostasis and the need for clinical intervention remain unknown. Objective:To d...

journal_title：JAMA neurology

authors： Witsch J,Frey HP,Schmidt JM,Velazquez A,Falo CM,Reznik M,Roh D,Agarwal S,Park S,Connolly ES,Claassen J

更新日期：2017-03-01 00:00:00

abstract：Importance:Although levodopa remains the most effective oral pharmacotherapy for Parkinson disease (PD), its use is often limited by wearing off effect and dyskinesias. Management of such complications continues to be a significant challenge. Objective:To investigate the efficacy and safety of safinamide (an oral amin...

journal_title：JAMA neurology

authors： Schapira AH,Fox SH,Hauser RA,Jankovic J,Jost WH,Kenney C,Kulisevsky J,Pahwa R,Poewe W,Anand R

更新日期：2017-02-01 00:00:00

abstract：Importance:Endovascular therapy (ET) is typically not considered for patients with large baseline ischemic cores (irreversibly injured tissue). Computed tomographic perfusion (CTP) imaging may identify a subset of patients with large ischemic cores who remain at risk for significant infarct expansion and thus could sti...

journal_title：JAMA neurology

authors： Rebello LC,Bouslama M,Haussen DC,Dehkharghani S,Grossberg JA,Belagaje S,Frankel MR,Nogueira RG

更新日期：2017-01-01 00:00:00

abstract：:A 63-year-old man presented with a 4-year history of insidious onset and gradual progression of visual symptoms including right homonymous hemianopsia, alexia, and simultanagnosia with preserved memory. Magnetic resonance imaging, perfusion single-photon emission computed tomography, and fluorodeoxyglucose positron em...

journal_title：JAMA neurology

authors： Mitchell SB,Lucente D,Larvie M,Cobos MI,Frosch M,Dickerson BC

更新日期：2017-01-01 00:00:00

abstract：Importance:Prolonged seizures in super-refractory status epilepticus (SRSE) have been shown to cause neuronal death and reorganization, and visual inspection in individual case studies has demonstrated progressive cortical and subcortical atrophy. At present, magnetic resonance imaging (MRI) studies that evaluate brain...

journal_title：JAMA neurology

authors： Hocker S,Nagarajan E,Rabinstein AA,Hanson D,Britton JW

更新日期：2016-10-01 00:00:00

abstract：IMPORTANCE:Double-seronegative myasthenia gravis (dSNMG) includes patients with myasthenia gravis (MG) without detectable antibodies to the nicotinic acetylcholine receptor (AChR) or to muscle-specific tyrosine kinase (MuSK). The lack of a biomarker hinders the diagnosis and clinical management in these patients. Corta...

journal_title：JAMA neurology

authors： Cortés-Vicente E,Gallardo E,Martínez MÁ,Díaz-Manera J,Querol L,Rojas-García R,Illa I

更新日期：2016-09-01 00:00:00

abstract：IMPORTANCE:This dose-blinded extension of the phase 2 BOLD (BAF312 on MRI Lesion Given Once Daily) Study in relapsing-remitting multiple sclerosis provides evidence on disease activity and safety of a range of siponimod doses for up to 24 months. OBJECTIVE:To assess the safety and efficacy of siponimod for up to 24 mo...

journal_title：JAMA neurology

authors： Kappos L,Li DK,Stüve O,Hartung HP,Freedman MS,Hemmer B,Rieckmann P,Montalban X,Ziemssen T,Hunter B,Arnould S,Wallström E,Selmaj K

更新日期：2016-09-01 00:00:00

abstract：IMPORTANCE:Allogeneic hematopoietic stem cell transplantation (HSCT) has been the only treatment option clinically available during the last 20 years for juvenile metachromatic leukodystrophy (MLD), reported with variable outcome and without comparison with the natural course of the disease. OBJECTIVE:To compare the l...

journal_title：JAMA neurology

authors： Groeschel S,Kühl JS,Bley AE,Kehrer C,Weschke B,Döring M,Böhringer J,Schrum J,Santer R,Kohlschütter A,Krägeloh-Mann I,Müller I

更新日期：2016-09-01 00:00:00

abstract：OBJECTIVE:In this review article, we focus on the molecular pathogenic basis for genetic generalized epilepsies associated with mutations in the inhibitory γ-aminobutyric acid (GABAA) receptor γ2 subunit gene, GABRG2 (OMIM 137164), an established epilepsy gene. OBSERVATIONS:The γ-aminobutyric acid (GABAA) receptor γ2 ...

journal_title：JAMA neurology

authors： Kang JQ,Macdonald RL

更新日期：2016-08-01 00:00:00

abstract：IMPORTANCE:Focal cortical dysplasia (FCD), hemimegalencephaly, and megalencephaly constitute a spectrum of malformations of cortical development with shared neuropathologic features. These disorders are associated with significant childhood morbidity and mortality. OBJECTIVE:To identify the underlying molecular cause ...

journal_title：JAMA neurology

authors： Mirzaa GM,Campbell CD,Solovieff N,Goold C,Jansen LA,Menon S,Timms AE,Conti V,Biag JD,Adams C,Boyle EA,Collins S,Ishak G,Poliachik S,Girisha KM,Yeung KS,Chung BHY,Rahikkala E,Gunter SA,McDaniel SS,Macmurdo CF,Ber

更新日期：2016-07-01 00:00:00

abstract：IMPORTANCE:Cerebral microbleeds (CMBs) have been established as an independent predictor of cerebral bleeding. There are contradictory data regarding the potential association of CMB burden with the risk of symptomatic intracerebral hemorrhage (sICH) in patients with acute ischemic stroke (AIS) treated with intravenous...

journal_title：JAMA neurology

authors： Tsivgoulis G,Zand R,Katsanos AH,Turc G,Nolte CH,Jung S,Cordonnier C,Fiebach JB,Scheitz JF,Klinger-Gratz PP,Oppenheim C,Goyal N,Safouris A,Mattle HP,Alexandrov AW,Schellinger PD,Alexandrov AV

更新日期：2016-06-01 00:00:00

abstract：IMPORTANCE:Synaptic loss is an early pathologic substrate of Alzheimer disease (AD). Neurogranin is a postsynaptic neuronal protein that has demonstrated utility as a cerebrospinal fluid (CSF) marker of synaptic loss in AD. OBJECTIVE:To investigate the diagnostic and prognostic utility of CSF neurogranin levels in a l...

journal_title：JAMA neurology

authors： Tarawneh R,D'Angelo G,Crimmins D,Herries E,Griest T,Fagan AM,Zipfel GJ,Ladenson JH,Morris JC,Holtzman DM

更新日期：2016-05-01 00:00:00

abstract：IMPORTANCE:Metabolic syndrome (MetS) is thought to influence several autoimmune diseases, including multiple sclerosis (MS). Anti-inflammatory effects of treatments used for MetS, such as metformin hydrochloride and pioglitazone hydrochloride, have been demonstrated, although clinical evidence supporting use of these t...

journal_title：JAMA neurology

authors： Negrotto L,Farez MF,Correale J

更新日期：2016-05-01 00:00:00

abstract：IMPORTANCE:Gait and cognition have been related to mortality in population-based studies. This association is possibly mediated by cerebral small vessel disease (SVD), which has been associated with mortality as well. It is unknown which factors can predict mortality in individuals with SVD. Identification of high-risk...

journal_title：JAMA neurology

authors： van der Holst HM,van Uden IW,Tuladhar AM,de Laat KF,van Norden AG,Norris DG,van Dijk EJ,Rutten-Jacobs LC,de Leeuw FE

更新日期：2016-04-01 00:00:00

abstract：IMPORTANCE:The extent to which large-caliber axonal degeneration contributes to Alzheimer disease (AD) progression is unknown. Cerebrospinal fluid (CSF) neurofilament light (NFL) concentration is a general marker of damage to large-caliber myelinated axons. OBJECTIVE:To test whether CSF NFL concentration is associated...

journal_title：JAMA neurology

authors： Zetterberg H,Skillbäck T,Mattsson N,Trojanowski JQ,Portelius E,Shaw LM,Weiner MW,Blennow K,Alzheimer’s Disease Neuroimaging Initiative.

更新日期：2016-01-01 00:00:00

abstract：IMPORTANCE:Duchenne muscular dystrophy (DMD) is a candidate for the recommended universal screening panel based on evidence that early corticosteroid treatment improves outcomes and on new genetic therapies that require early diagnosis for effectiveness. Elevated creatine kinase levels in the neonatal period are the in...

journal_title：JAMA neurology

authors： Gatheridge MA,Kwon JM,Mendell JM,Scheuerbrandt G,Moat SJ,Eyskens F,Rockman-Greenberg C,Drousiotou A,Griggs RC

更新日期：2016-01-01 00:00:00

abstract：IMPORTANCE:Neurogranin (NGRN) seems to be a promising novel cerebrospinal fluid (CSF) biomarker for synaptic loss; however, clinical, and especially longitudinal, data are sparse. OBJECTIVE:To examine the utility of NGRN, with repeated CSF sampling, for diagnosis, prognosis, and monitoring of Alzheimer disease (AD). ...

journal_title：JAMA neurology

authors： Kester MI,Teunissen CE,Crimmins DL,Herries EM,Ladenson JH,Scheltens P,van der Flier WM,Morris JC,Holtzman DM,Fagan AM

更新日期：2015-11-01 00:00:00

abstract：IMPORTANCE:Smoking tobacco is a well-established risk factor for multiple sclerosis (MS), a chronic inflammatory disorder of the central nervous system usually characterized by bouts and remissions and typically followed by a secondary progressive (SP) course. However, it is not clear whether smoking after diagnosis is...

journal_title：JAMA neurology

authors： Ramanujam R,Hedström AK,Manouchehrinia A,Alfredsson L,Olsson T,Bottai M,Hillert J

更新日期：2015-10-01 00:00:00

abstract：IMPORTANCE:Converging evidence suggests that Alzheimer disease (AD) involves insulin signaling impairment. Patients with AD and individuals at risk for AD show reduced glucose metabolism, as indexed by fludeoxyglucose F 18-labeled positron emission tomography (FDG-PET). OBJECTIVES:To determine whether insulin resistan...

journal_title：JAMA neurology

authors： Willette AA,Bendlin BB,Starks EJ,Birdsill AC,Johnson SC,Christian BT,Okonkwo OC,La Rue A,Hermann BP,Koscik RL,Jonaitis EM,Sager MA,Asthana S

更新日期：2015-09-01 00:00:00

abstract：IMPORTANCE:Intravenous recombinant tissue plasminogen activator (alteplase) was approved by the US Food and Drug Administration in 1996 for the treatment of acute ischemic stroke. Nearly 20 years later, it remains the only approved treatment, despite limitations in both efficacy and safety. With a growing capacity for ...

journal_title：JAMA neurology

authors： Marshall RS

更新日期：2015-08-01 00:00:00

abstract：IMPORTANCE:The R47H variant in the triggering receptor expressed on myeloid cells 2 gene (TREM2), a modulator of the immune response of microglia, is a strong genetic risk factor for Alzheimer disease (AD) and possibly other neurodegenerative disorders. OBJECTIVE:To investigate a large family with late-onset AD (LOAD)...

journal_title：JAMA neurology

authors： Korvatska O,Leverenz JB,Jayadev S,McMillan P,Kurtz I,Guo X,Rumbaugh M,Matsushita M,Girirajan S,Dorschner MO,Kiianitsa K,Yu CE,Brkanac Z,Garden GA,Raskind WH,Bird TD

更新日期：2015-08-01 00:00:00

abstract：IMPORTANCE:Mild strokes have been poorly represented in thrombolytic trials and only a few series have reported outcomes after treatment with intravenous (IV) recombinant tissue plasminogen activator (rtPA) after mild stroke. OBJECTIVE:To report treatment complications and short-term outcomes in patients with mild str...

journal_title：JAMA neurology

authors： Romano JG,Smith EE,Liang L,Gardener H,Camp S,Shuey L,Cook A,Campo-Bustillo I,Khatri P,Bhatt DL,Fonarow GC,Sacco RL,Schwamm LH

更新日期：2015-04-01 00:00:00

abstract：IMPORTANCE:Whether cognition is influenced by arterial stiffness in the absence of vascular disease remains uncertain. OBJECTIVE:To test the hypotheses that indirect measures of arterial stiffness are important predictors of cognitive performance and that this relationship varies depending on the presence of vascular ...

journal_title：JAMA neurology

authors： Gutierrez J,Marshall RS,Lazar RM

更新日期：2015-03-01 00:00:00

abstract：IMPORTANCE:Apolipoprotein E (APOE) ε4 is an established risk factor for cognitive decline and the development of dementia, but other factors may help to minimize its effects. OBJECTIVE:Using APOE ε4 as an indicator of high risk, we investigated factors associated with cognitive resilience among black and white older a...

journal_title：JAMA neurology

authors： Kaup AR,Nettiksimmons J,Harris TB,Sink KM,Satterfield S,Metti AL,Ayonayon HN,Yaffe K,Health, Aging, and Body Composition (Health ABC) Study.

更新日期：2015-03-01 00:00:00

abstract：IMPORTANCE:There is a deficit of pituitary adenylate cyclase-activating polypeptide (PACAP) in patients with neuropathologically confirmed Alzheimer dementia. However, whether this deficit is associated with the earlier stages of Alzheimer disease (AD) is unknown. This study was conducted to clarify the association bet...

journal_title：JAMA neurology

authors： Han P,Caselli RJ,Baxter L,Serrano G,Yin J,Beach TG,Reiman EM,Shi J

更新日期：2015-03-01 00:00:00

abstract：IMPORTANCE:Understanding the relationships between age-related changes in brain structure and cognitive function has been limited by inconsistent methods for assessing brain imaging, small sample sizes, and racially/ethnically homogeneous cohorts with biased selection based on risk factors. These limitations have preve...

journal_title：JAMA neurology

authors： Gupta M,King KS,Srinivasa R,Weiner MF,Hulsey K,Ayers CR,Whittemore A,McColl RW,Rossetti HC,Peshock RM

更新日期：2015-02-01 00:00:00

abstract：IMPORTANCE:Posterior cortical atrophy (PCA) is characterized by progressive visuoperceptual and visuospatial deficits and commonly considered to be an atypical variant of Alzheimer disease. Mutations of the GRN gene are responsible for a large phenotypic spectrum, but, to our knowledge, the association of PCA with GRN ...

journal_title：JAMA neurology

authors： Caroppo P,Belin C,Grabli D,Maillet D,De Septenville A,Migliaccio R,Clot F,Lamari F,Camuzat A,Brice A,Dubois B,Le Ber I

更新日期：2015-02-01 00:00:00