Association of Cerebrospinal Fluid Neurofilament Light Concentration With Alzheimer Disease Progression.

abstract：Importance:Neurofilament light protein (NfL) is elevated in cerebrospinal fluid (CSF) of a number of neurological conditions compared with healthy controls (HC) and is a candidate biomarker for neuroaxonal damage. The influence of age and sex is largely unknown, and levels across neurological disorders have not been co...

journal_title：JAMA neurology

authors： Bridel C,van Wieringen WN,Zetterberg H,Tijms BM,Teunissen CE,and the NFL Group.,Alvarez-Cermeño JC,Andreasson U,Axelsson M,Bäckström DC,Bartos A,Bjerke M,Blennow K,Boxer A,Brundin L,Burman J,Christensen T,Fialová L,Fo

更新日期：2019-06-17 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：: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: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:Mutations in the SQSTM1 gene, coding for p62, are a cause of Paget disease of bone and amyotrophic lateral sclerosis (ALS). Recently, SQSTM1 mutations were confirmed in ALS, and mutations were also identified in 3 patients with frontotemporal dementia (FTD), suggesting a role for SQSTM1 in FTD. OBJECTIVE:To...

journal_title：JAMA neurology

authors： Le Ber I,Camuzat A,Guerreiro R,Bouya-Ahmed K,Bras J,Nicolas G,Gabelle A,Didic M,De Septenville A,Millecamps S,Lenglet T,Latouche M,Kabashi E,Campion D,Hannequin D,Hardy J,Brice A,French Clinical and Genetic Research Net

更新日期：2013-11-01 00:00:00

abstract：Importance:The incidence of unprovoked seizures and epilepsy increases considerably in late life, with approximately one-third of seizures being of unknown etiology. While individuals with dementia have a high risk of developing unprovoked seizures, it is unknown whether older adults with late-onset unprovoked seizures...

journal_title：JAMA neurology

authors： Keret O,Hoang TD,Xia F,Rosen HJ,Yaffe K

更新日期：2020-06-01 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: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:Doses of fingolimod lower than 0.5 mg per day were not investigated during the fingolimod clinical development program. Whether lower doses of fingolimod might retain efficacy with fewer safety risks remains unknown. Objective:To evaluate the efficacy and safety of fingolimod, 0.5 mg, and fingolimod, 0.25 m...

journal_title：JAMA neurology

authors： Cree BAC,Goldman MD,Corboy JR,Singer BA,Fox EJ,Arnold DL,Ford C,Weinstock-Guttman B,Bar-Or A,Mientus S,Sienkiewicz D,Zhang Y,Karan R,Tenenbaum N,ASSESS Trial Investigators.

更新日期：2020-08-24 00:00:00

abstract：Importance:Randomized clinical trials have shown the efficacy of thrombectomy of large intracranial vessel occlusions in adults; however, any association of therapy with clinical outcomes in children is unknown. Objective:To evaluate the use of endovascular recanalization in pediatric patients with arterial ischemic s...

journal_title：JAMA neurology

authors： Sporns PB,Sträter R,Minnerup J,Wiendl H,Hanning U,Chapot R,Henkes H,Henkes E,Grams A,Dorn F,Nikoubashman O,Wiesmann M,Bier G,Weber A,Broocks G,Fiehler J,Brehm A,Psychogios M,Kaiser D,Yilmaz U,Morotti A,Marik W,

更新日期：2020-01-01 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: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: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

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: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: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: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：: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:Intracerebral hemorrhage (ICH) has the highest mortality of all stroke types and is the most serious complication of anticoagulation. Data regarding trends in ICH incidence and location-specific risk factors on the population level are conflicting. Objective:To assess long-term population-based trends in th...

journal_title：JAMA neurology

authors： Lioutas VA,Beiser AS,Aparicio HJ,Himali JJ,Selim MH,Romero JR,Seshadri S

更新日期：2020-06-08 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:Progressive multifocal leukoencephalopathy results from lytic infection of the glia by the JC polyomavirus (JCV); JCV granule cell neuronopathy is caused by infection with a mutated form of JCV, leading to a shift in viral tropism from the glia to cerebellar granule cells. This shift results in a clinical sy...

journal_title：JAMA neurology

authors： Dang L,Dang X,Koralnik IJ,Todd PK

更新日期：2014-04-01 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: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：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: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: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:Functional neurological disorders (FND) are common sources of disability in medicine. Patients have often been misdiagnosed, correctly diagnosed after lengthy delays, and/or subjected to poorly delivered diagnoses that prevent diagnostic understanding and lead to inappropriate treatments, iatrogenic harm, un...

journal_title：JAMA neurology

authors： Espay AJ,Aybek S,Carson A,Edwards MJ,Goldstein LH,Hallett M,LaFaver K,LaFrance WC Jr,Lang AE,Nicholson T,Nielsen G,Reuber M,Voon V,Stone J,Morgante F

更新日期：2018-09-01 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:In Huntington disease (HD), mutation severity is defined by the length of the CAG trinucleotide sequence, a well-known predictor of clinical onset age. The association with disease trajectory is less well characterized. Quantifiable summary measures of trajectory applicable over decades of early disease prog...

journal_title：JAMA neurology

authors： Langbehn DR,Stout JC,Gregory S,Mills JA,Durr A,Leavitt BR,Roos RAC,Long JD,Owen G,Johnson HJ,Borowsky B,Craufurd D,Reilmann R,Landwehrmeyer GB,Scahill RI,Tabrizi SJ,TRACK-HD and Track-On HD Groups.

更新日期：2019-08-12 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