A 63-Year-Old Man With Progressive Visual Symptoms.

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 The search for novel Alzheimer disease (AD) genes or pathologic mutations within known AD loci is ongoing. The development of array technologies has helped to identify rare recessive mutations among long runs of homozygosity (ROHs), in which both parental alleles are identical. Caribbean Hispanics are known...

journal_title：JAMA neurology

authors： Ghani M,Sato C,Lee JH,Reitz C,Moreno D,Mayeux R,St George-Hyslop P,Rogaeva E

更新日期：2013-10-01 00:00:00

abstract：Importance:The goal of preclinical Alzheimer disease (AD) clinical trials is to move diagnosis and treatment to presymptomatic stages, which will require biomarker testing and disclosure. Objective:To assess the short-term psychological outcomes of disclosing amyloid positron emission tomography results to older adult...

journal_title：JAMA neurology

authors： Grill JD,Raman R,Ernstrom K,Sultzer DL,Burns JM,Donohue MC,Johnson KA,Aisen PS,Sperling RA,Karlawish J,A4 Study Team.

更新日期：2020-08-10 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: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: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: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: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: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: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:Large-scale genome-wide association studies in the European population have identified 90 risk variants associated with Parkinson disease (PD); however, there are limited studies in the largest population worldwide (ie, Asian). Objectives:To identify novel genome-wide significant loci for PD in Asian indivi...

journal_title：JAMA neurology

authors： Foo JN,Chew EGY,Chung SJ,Peng R,Blauwendraat C,Nalls MA,Mok KY,Satake W,Toda T,Chao Y,Tan LCS,Tandiono M,Lian MM,Ng EY,Prakash KM,Au WL,Meah WY,Mok SQ,Annuar AA,Chan AYY,Chen L,Chen Y,Jeon BS,Jiang L,Lim J

更新日期：2020-06-01 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: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: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: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: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:The development and expansion of intracranial hematoma are associated with adverse outcomes. Use of tranexamic acid might limit intracranial hematoma formation, but its association with outcomes of severe traumatic brain injury (TBI) is unclear. Objective:To assess whether prehospital administration of tran...

journal_title：JAMA neurology

authors： Bossers SM,Loer SA,Bloemers FW,Den Hartog D,Van Lieshout EMM,Hoogerwerf N,van der Naalt J,Absalom AR,Peerdeman SM,Schwarte LA,Boer C,Schober P,BRAIN-PROTECT collaborators.

更新日期：2020-12-07 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: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:Mild cognitive impairment (MCI) is a transition state between normal cognitive aging and dementia that increases the risk for progressive cognitive decline. Preventing cognitive decline is a public health priority. Objective:To determine whether behavioral activation prevents cognitive and functional declin...

journal_title：JAMA neurology

authors： Rovner BW,Casten RJ,Hegel MT,Leiby B

更新日期：2018-12-01 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: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：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: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: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:Interictal epileptiform discharges (IEDs) in electroencephalograms (EEGs) are a biomarker of epilepsy, seizure risk, and clinical decline. However, there is a scarcity of experts qualified to interpret EEG results. Prior attempts to automate IED detection have been limited by small samples and have not demon...

journal_title：JAMA neurology

authors： Jing J,Sun H,Kim JA,Herlopian A,Karakis I,Ng M,Halford JJ,Maus D,Chan F,Dolatshahi M,Muniz C,Chu C,Sacca V,Pathmanathan J,Ge W,Dauwels J,Lam A,Cole AJ,Cash SS,Westover MB

更新日期：2020-01-01 00:00:00

abstract：IMPORTANCE:The literature on cingulate gyrus epilepsy in the magnetic resonance imaging era is limited to case reports and small case series. To our knowledge, this is the largest study of surgically confirmed epilepsy arising from the anterior or posterior cingulate region. OBJECTIVE:To characterize the clinical and ...

journal_title：JAMA neurology

authors： Alkawadri R,So NK,Van Ness PC,Alexopoulos AV

更新日期：2013-08-01 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: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