Posterior cortical atrophy as an extreme phenotype of GRN mutations.

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: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:We encountered 2 patients with amyotrophic lateral sclerosis (ALS) with tongue enlargement and protrusion outside the oral cavity (macroglossia). To our knowledge, the relationship between macroglossia and ALS has not been reported in the literature. The objective of this article was to describe the clinical...

journal_title：JAMA neurology

authors： McKee HR,Escott E,Damm D,Kasarskis E

更新日期：2013-11-01 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: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:Progressive external ophthalmoplegia (PEO) is a common feature in adults with mitochondrial (mt) DNA maintenance disorders associated with somatic mtDNA deletions in muscle, yet the causal genetic defect in many patients remains undetermined. OBSERVATIONS:Whole-exome sequencing identified a novel, heterozyg...

journal_title：JAMA neurology

authors： Gorman GS,Pfeffer G,Griffin H,Blakely EL,Kurzawa-Akanbi M,Gabriel J,Sitarz K,Roberts M,Schoser B,Pyle A,Schaefer AM,McFarland R,Turnbull DM,Horvath R,Chinnery PF,Taylor RW

更新日期：2015-01-01 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: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: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: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：: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:The frequency and distribution of synucleinopathies and tauopathies manifesting with parkinsonism in the general population are poorly understood, thus affecting health care planning and research. OBJECTIVE:To investigate the incidence and distribution of specific types of parkinsonism and related proteinop...

journal_title：JAMA neurology

authors： Savica R,Grossardt BR,Bower JH,Ahlskog JE,Rocca WA

更新日期：2013-07-01 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:Magnetic resonance imaging markers of incipient cognitive decline among healthy elderly individuals have become important for both clarifying the biological underpinnings of dementia and clinically identifying healthy individuals at high risk of cognitive decline. Even though the role of hippocampal atrophy ...

journal_title：JAMA neurology

authors： Fletcher E,Raman M,Huebner P,Liu A,Mungas D,Carmichael O,DeCarli C

更新日期：2013-11-01 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: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:As the life expectancy of people with Down syndrome (DS) has markedly increased over the past decades, older adults with DS may be experiencing a higher incidence of aging conditions. In addition to longevity, the amyloid precursor protein gene located on chromosome 21 places individuals with DS at a high ri...

journal_title：JAMA neurology

authors： Bayen E,Possin KL,Chen Y,Cleret de Langavant L,Yaffe K

更新日期：2018-11-01 00:00:00

abstract：Importance:Advances in treatment of traumatic brain injury are hindered by the inability to monitor pathological mechanisms in individual patients for targeted neuroprotective treatment. Spreading depolarizations, a mechanism of lesion development in animal models, are a novel candidate for clinical monitoring in patie...

journal_title：JAMA neurology

authors： Hartings JA,Andaluz N,Bullock MR,Hinzman JM,Mathern B,Pahl C,Puccio A,Shutter LA,Strong AJ,Vagal A,Wilson JA,Dreier JP,Ngwenya LB,Foreman B,Pahren L,Lingsma H,Okonkwo DO

更新日期：2020-04-01 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:Epidemiological data on hereditary cerebellar ataxia (HCA) and hereditary spastic paraplegia (HSP) are scarce. OBJECTIVE:To present the prevalence and distribution of HCA and HSP in Portugal. DESIGN AND SETTING:Population-based, nationwide, systematic survey, from January 1, 1994, through April 15, 2004, i...

journal_title：JAMA neurology

authors： Coutinho P,Ruano L,Loureiro JL,Cruz VT,Barros J,Tuna A,Barbot C,Guimarães J,Alonso I,Silveira I,Sequeiros J,Marques Neves J,Serrano P,Silva MC

更新日期：2013-06-01 00:00:00

abstract：BACKGROUND:Approximately 10% to 14% of ischemic strokes occur in young adults. OBJECTIVE:To investigate the yield of diagnostic tests, neuroimaging findings, and treatment of ischemic strokes in young adults. DESIGN:We retrospectively reviewed data from our Get with the Guidelines-Stroke database from 2005 through 20...

journal_title：JAMA neurology

authors： Ji R,Schwamm LH,Pervez MA,Singhal AB

更新日期：2013-01-01 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：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:Fungal meningitis due to injections of contaminated methylprednisolone acetate can present with vascular sequelae in immunocompetent individuals. This is particularly germane to neurologists because better recognition of the clinical characteristics of patients with fungal meningitis and ischemic stroke will...

journal_title：JAMA neurology

authors： Kleinfeld K,Jones P,Riebau D,Beck A,Paueksakon P,Abel T,Claassen DO

更新日期：2013-09-01 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: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:Emerging yet contrasting evidence associates air pollution with incident dementia, and the potential role of cardiovascular disease (CVD) in this association is unclear. Objective:To investigate the association between long-term exposure to air pollution and dementia and to assess the role of CVD in that as...

journal_title：JAMA neurology

authors： Grande G,Ljungman PLS,Eneroth K,Bellander T,Rizzuto D

更新日期：2020-07-01 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: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：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