Efficacy and Safety of Deep Brain Stimulation in Tourette Syndrome: The International Tourette Syndrome Deep Brain Stimulation Public Database and Registry.

abstract：IMPORTANCE:To provide clinical and genetic diagnoses for patients' conditions, it is important to identify and characterize the different subtypes of spinocerebellar ataxia (SCA). OBJECTIVE:To clinically and genetically characterize a Spanish kindred with pure SCA presenting with altered vertical eye movements. DESIGN...

journal_title：JAMA neurology

authors： Serrano-Munuera C,Corral-Juan M,Stevanin G,San Nicolás H,Roig C,Corral J,Campos B,de Jorge L,Morcillo-Suárez C,Navarro A,Forlani S,Durr A,Kulisevsky J,Brice A,Sánchez I,Volpini V,Matilla-Dueñas A

更新日期：2013-06-01 00:00:00

abstract：OBJECTIVE:To assess the reliability of new magnetic resonance imaging (MRI) lesion counts by clinicians in a multiple sclerosis specialty clinic. DESIGN:An observational study. SETTING:A multiple sclerosis specialty clinic. PATIENTS:Eighty-five patients with multiple sclerosis participating in a National Institutes ...

journal_title：JAMA neurology

authors： Erbayat Altay E,Fisher E,Jones SE,Hara-Cleaver C,Lee JC,Rudick RA

更新日期：2013-03-01 00:00:00

abstract：Importance:Apolipoprotein E ε4 (APOEε4) is the single most important genetic risk factor for Alzheimer disease. While APOEε4 is associated with increased amyloid-β burden, its association with cerebral tau pathology has been controversial. Objective:To determine whether APOEε4 is associated with medial temporal tau pa...

journal_title：JAMA neurology

authors： Therriault J,Benedet AL,Pascoal TA,Mathotaarachchi S,Chamoun M,Savard M,Thomas E,Kang MS,Lussier F,Tissot C,Parsons M,Qureshi MNI,Vitali P,Massarweh G,Soucy JP,Rej S,Saha-Chaudhuri P,Gauthier S,Rosa-Neto P

更新日期：2020-04-01 00:00:00

abstract：Importance:Cardiovascular disease (CVD) risk has not been previously evaluated in a large matched cohort study in idiopathic intracranial hypertension (IIH). Objectives:To estimate the risk of composite cardiovascular events, heart failure, ischemic heart disease, stroke/transient ischemic attack (TIA), type 2 diabete...

journal_title：JAMA neurology

authors： Adderley NJ,Subramanian A,Nirantharakumar K,Yiangou A,Gokhale KM,Mollan SP,Sinclair AJ

更新日期：2019-07-08 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：BACKGROUND:Therapies designed to decrease the level of SOD1 are currently in a clinical trial for patients with superoxide dismutase (SOD1)-linked familial amyotrophic lateral sclerosis (ALS). OBJECTIVE:To determine whether the SOD1 protein in cerebral spinal fluid (CSF) may be a pharmacodynamic marker for antisense o...

journal_title：JAMA neurology

authors： Winer L,Srinivasan D,Chun S,Lacomis D,Jaffa M,Fagan A,Holtzman DM,Wancewicz E,Bennett CF,Bowser R,Cudkowicz M,Miller TM

更新日期：2013-02-01 00:00:00

abstract：IMPORTANCE:Voltage-gated potassium channel complex antibody (VGKCc-Ab) encephalitis is an immunotherapy-responsive syndrome usually associated with causative antibodies that target the leucine-rich, glioma inactivated 1 (LGI1) protein. Although it is expressed throughout the brain, LGI1 is not known to be expressed in ...

journal_title：JAMA neurology

authors： Naasan G,Irani SR,Bettcher BM,Geschwind MD,Gelfand JM

更新日期：2014-10-01 00:00:00

abstract：Importance:Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain. Objective:To provide an objective summary of the available evidence on the clinical presentation, investigations findings, and treatment ou...

journal_title：JAMA neurology

authors： D'Antona L,Jaime Merchan MA,Vassiliou A,Watkins LD,Davagnanam I,Toma AK,Matharu MS

更新日期：2021-01-04 00:00:00

abstract：IMPORTANCE:Given the high frequency of failure of first-line therapies, there is an urgent need for second-line treatment strategies for pediatric patients with multiple sclerosis (MS). OBJECTIVE:To report the use of natalizumab in pediatric MS. Natalizumab, a humanized monoclonal antibody targeting α4 integrin, is ef...

journal_title：JAMA neurology

authors： Kornek B,Aboul-Enein F,Rostasy K,Milos RI,Steiner I,Penzien J,Hellwig K,Pitarokoili K,Storm van's Gravesande K,Karenfort M,Blaschek A,Meyer A,Seidl R,Debelic D,Vass K,Prayer D,Kristoferitsch W,Bayas A

更新日期：2013-04-01 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:Estimated base rates of invalid performance on baseline testing (base rates of failure) for the management of sport-related concussion range from 6.1% to 40.0%, depending on the validity indicator used. The instability of this key measure represents a challenge in the clinical interpretation of test results ...

journal_title：JAMA neurology

authors： Abeare CA,Messa I,Zuccato BG,Merker B,Erdodi L

更新日期：2018-06-01 00:00:00

abstract：IMPORTANCE:Cerebrospinal fluid (CSF) is the compartment in closest proximity to the central nervous system (CNS) parenchyma and might reflect immune pathology in inflammatory CNS disorders like multiple sclerosis (MS). Multiparameter flow cytometry is used to characterize immunological alterations in the CSF of patient...

journal_title：JAMA neurology

authors： Alvermann S,Hennig C,Stüve O,Wiendl H,Stangel M

更新日期：2014-07-01 00:00:00

abstract：IMPORTANCE:Despite recessive inheritance, X-linked dystonia-parkinsonism (Lubag disease) has also been described in women presenting with a late-onset isolated parkinsonian syndrome. Interestingly, unlike in other populations, there is a slight female predominance in the prevalence of parkinsonism in the Philippines. ...

journal_title：JAMA neurology

authors： Domingo A,Lee LV,Brüggemann N,Freimann K,Kaiser FJ,Jamora RD,Rosales RL,Klein C,Westenberger A

更新日期：2014-09-01 00:00:00

abstract：Importance:Basket-design clinical trials that allow investigation of treatment effects on different clinical syndromes that share the same molecular pathophysiology have not previously been attempted in neurodegenerative disease. Objective:To assess the safety, tolerability, and pharmacodynamics of the microtubule sta...

journal_title：JAMA neurology

authors： Tsai RM,Miller Z,Koestler M,Rojas JC,Ljubenkov PA,Rosen HJ,Rabinovici GD,Fagan AM,Cobigo Y,Brown JA,Jung JI,Hare E,Geldmacher DS,Natelson-Love M,McKinley EC,Luong PN,Chuu EL,Powers R,Mumford P,Wolf A,Wang P,Sham

更新日期：2020-02-01 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：Importance:Early-onset Alzheimer disease (EOAD) is a rare form of Alzheimer disease (AD) with a large genetic basis that is only partially understood. In late-onset AD, elevated circulating cholesterol levels increase AD risk even after adjusting for the apolipoprotein E ε4 (APOE E4) allele, a major genetic factor for ...

journal_title：JAMA neurology

authors： Wingo TS,Cutler DJ,Wingo AP,Le NA,Rabinovici GD,Miller BL,Lah JJ,Levey AI

更新日期：2019-07-01 00:00:00

abstract：IMPORTANCE:While advances have been made in characterizing the C9ORF72 clinical phenotype, the hallmark features that discriminate between carriers and noncarriers remain unclear. OBJECTIVES:To determine the frequency of the C9ORF72 mutation in a frontotemporal dementia (FTD) cohort and to define the clinical, neurops...

journal_title：JAMA neurology

authors： Devenney E,Hornberger M,Irish M,Mioshi E,Burrell J,Tan R,Kiernan MC,Hodges JR

更新日期：2014-03-01 00:00:00

abstract：Importance:The safety and efficacy of intensive systolic blood pressure reduction in patients with intracerebral hemorrhage who present with systolic blood pressure greater than 220 mm Hg appears to be unknown. Objective:To evaluate the differential outcomes of intensive (goal, 110-139 mm Hg) vs standard (goal, 140-17...

journal_title：JAMA neurology

authors： Qureshi AI,Huang W,Lobanova I,Barsan WG,Hanley DF,Hsu CY,Lin CL,Silbergleit R,Steiner T,Suarez JI,Toyoda K,Yamamoto H,for ATACH-II trial investigators.

更新日期：2020-09-08 00:00:00

abstract：IMPORTANCE:Glioblastoma is the most common primary malignant brain tumor, but despite multimodal treatment with surgery, radiotherapy, and temozolomide chemotherapy, the prognosis is poor, with a median survival of 16 to 19 months and poor quality of life throughout the disease course. New treatments are needed. EVIDE...

journal_title：JAMA neurology

authors： Thomas AA,Brennan CW,DeAngelis LM,Omuro AM

更新日期：2014-11-01 00:00:00

abstract：Importance:Cerebrospinal fluid (CSF) core Alzheimer disease (AD) biomarkers have shown an excellent capacity for the in vivo detection of AD. Previous studies have shown that CSF levels of phosphorylated tau (p-tau) also correlate with tau pathology in frontotemporal lobar degeneration (FTLD) after accounting for AD co...

journal_title：JAMA neurology

authors： Lleó A,Irwin DJ,Illán-Gala I,McMillan CT,Wolk DA,Lee EB,Van Deerlin VM,Shaw LM,Trojanowski JQ,Grossman M

更新日期：2018-06-01 00:00:00

abstract：IMPORTANCE:Documentation of muscle pathology compatible with targeting of sarcolemmal aquaporin-4 (AQP4) by complement-activating IgG implies involvement of organs beyond the central nervous system in neuromyelitis optica spectrum disorders. OBSERVATIONS:We report on a 51-year-old woman who had relapsing optic neuriti...

journal_title：JAMA neurology

authors： Guo Y,Lennon VA,Popescu BF,Grouse CK,Topel J,Milone M,Lassmann H,Parisi JE,Pittock SJ,Stefoski D,Balabanov R,Lucchinetti CF

更新日期：2014-08-01 00:00:00

abstract：Importance:Race-specific and sex-specific stroke risk varies across the lifespan, yet few reports describe sex differences in stroke risk separately in black individuals and white individuals. Objective:To examine incidence and risk factors for ischemic stroke by sex for black and white individuals. Design, Setting, ...

journal_title：JAMA neurology

authors： Howard VJ,Madsen TE,Kleindorfer DO,Judd SE,Rhodes JD,Soliman EZ,Kissela BM,Safford MM,Moy CS,McClure LA,Howard G,Cushman M

更新日期：2019-02-01 00:00:00

abstract：Importance:The pathophysiologic mechanisms of Guillain-Barré syndrome (GBS) associated with Zika virus (ZIKV) infection may be indicated by differences in clinical features. Objective:To identify specific clinical features of GBS associated with ZIKV infection. Design, Setting, and Participants:During the ZIKV epidem...

journal_title：JAMA neurology

authors： Dirlikov E,Major CG,Medina NA,Lugo-Robles R,Matos D,Muñoz-Jordan JL,Colon-Sanchez C,Garcia M,Olivero-Segarra M,Malave G,Rodríguez-Vega GM,Thomas DL,Waterman SH,Sejvar JJ,Luciano CA,Sharp TM,Rivera-García B

更新日期：2018-09-01 00:00:00

abstract：Importance:Literature on ictal magnetoencephalography (MEG) in clinical practice and the relationship to other modalities is limited because of the brevity of routine studies. Objective:To investigate the utility and reliability of ictal MEG in the localization of the epileptogenic zone. Design, Setting, and Particip...

journal_title：JAMA neurology

authors： Alkawadri R,Burgess RC,Kakisaka Y,Mosher JC,Alexopoulos AV

更新日期：2018-10-01 00:00:00

abstract：Importance:It is unclear whether relapses and disease-modifying therapies are associated with the rate of disability accumulation in patients with secondary progressive multiple sclerosis (SPMS). Objective:To examine the association of relapses with the rate of disability accumulation in patients with SPMS and to asse...

journal_title：JAMA neurology

authors： Lizak N,Malpas CB,Sharmin S,Havrdova EK,Horakova D,Izquierdo G,Eichau S,Lugaresi A,Duquette P,Girard M,Prat A,Larochelle C,Trojano M,Grand'Maison F,Grammond P,Sola P,Ferraro D,Hupperts R,Bergamaschi R,Boz C,Van Pe

更新日期：2020-07-27 00:00:00

abstract：Importance:Identification of genetic factors that interact with the apolipoprotein e4 (APOE4) allele to reduce risk for Alzheimer disease (AD) would accelerate the search for new AD drug targets. Klotho-VS heterozygosity (KL-VSHET+ status) protects against aging-associated phenotypes and cognitive decline, but whether ...

journal_title：JAMA neurology

authors： Belloy ME,Napolioni V,Han SS,Le Guen Y,Greicius MD,Alzheimer’s Disease Neuroimaging Initiative.

更新日期：2020-07-01 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:Chronic subdural hematoma (CSDH) is a trauma-associated condition commonly found in elderly patients. Surgery is currently the treatment of choice, but it carries a significant risk of recurrence and death. Nonsurgical treatments remain limited and ineffective. Our recent studies suggest that atorvastatin re...

journal_title：JAMA neurology

authors： Jiang R,Zhao S,Wang R,Feng H,Zhang J,Li X,Mao Y,Yuan X,Fei Z,Zhao Y,Yu X,Poon WS,Zhu X,Liu N,Kang D,Sun T,Jiao B,Liu X,Yu R,Zhang J,Gao G,Hao J,Su N,Yin G,Zhu X,Lu Y,Wei J,Hu J,Hu R,Li J,Wang D

更新日期：2018-11-01 00:00:00

abstract：Importance:Atabecestat, a nonselective oral β-secretase inhibitor, was evaluated in the EARLY trial for slowing cognitive decline in participants with preclinical Alzheimer disease. Preliminary analyses suggested dose-related cognitive worsening and neuropsychiatric adverse events (AEs). Objective:To report efficacy, ...

journal_title：JAMA neurology

authors： Sperling R,Henley D,Aisen PS,Raman R,Donohue MC,Ernstrom K,Rafii MS,Streffer J,Shi Y,Karcher K,Raghavan N,Tymofyeyev Y,Bogert J,Brashear HR,Novak G,Thipphawong J,Saad ZS,Kolb H,Rofael H,Sanga P,Romano G

更新日期：2021-01-19 00:00:00

abstract：:We are at an inflection point in our study of the human genome as it relates to neurodegenerative disease. The sequencing of the human genome, and its associated cataloging of human genetic variation and technological as well as methodological development, introduced a period of rapid gene discovery over the past deca...

journal_title：JAMA neurology

authors： De Jager PL,Bennett DA

更新日期：2013-06-01 00:00:00